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pmc-6169535-1
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A 50-year-old woman presented, in 2010, with galactorrhoea and oligomenorrhea of 4 years. Her past medical history included a right nephrectomy following a road traffic accident as a child. She had five children. The galactorrhea persisted since the birth of her last son when she was 31 years old. Endocrinology assessment revealed raised serum prolactin at 1444 mu/L with suppressed gonadotrophins and oestradiol. The patient was not taking any medication at the time. Renal function and thyroid function were both normal. A pituitary MR scan showed a 6 mm diameter microadenoma with inferior extension towards the sphenoid sinus on the left hand side (). The patient also suffered from headaches and visual disturbance, although the tumour was confined within the sella turcica and was not thought to be causal. Taken together, these findings were interpreted as diagnostic of a microprolactinoma.
After the patient’s eldest daughter died in her early 20s in 2013 from ovarian cancer, genetic testing was carried out within the family. The patient, as well as the patient’s mother and half-sister were found to carry a DICER1 gene mutation. There is no other significant family history of pituitary disorders. As part of DICER1 follow-up, she had regular scans of her thyroid, which revealed the expected multinodular goitre, and in 2018, she was diagnosed with a differentiated papillary carcinoma thyroid.
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pmc-6169932-1
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A 36-year-old man experienced acute pain above the right heel accompanied by an audible snap while sprinting. He immediately had difficulty walking and 3 hours later consulted an on-call GP. Posterior ankle swelling with a tender depression 3 cm proximal to the calcaneum was found. Active plantar flexion against resistance was weak and Simmonds–Thompson test was ‘partially positive’ on applying a strong calf-squeeze. Based on these findings, calf muscle rupture was diagnosed as the Achilles tendon was thought to be intact. The patient was advised to elevate the foot and wait 2 weeks for improvement. Two days later a second GP, who was aware of a history of an audible snap, considered complete tendon rupture and reexamined the patient. Findings included an absent right heel raise due to weakness, minimal active plantar flexion against gravity and lying prone, significant right ankle swelling without bruising, and an altered angle of declination. Palpation elicited no ankle bony tenderness, yet a painful gap was identified 6 cm proximal from the calcaneal attachment, along the line of the Achilles tendon. Simmonds–Thompson's test was clearly positive. The positive Simmond’s triad indicated a clinical diagnosis of complete rupture of the Achilles tendon.
A 3.4–8 MHz linear array probe PSUD (VScan™ dual probe, GE Healthcare), set at a depth of 3.5 cm, was used under the supervision of a rheumatologist experienced in ultrasound. The tendon was enlarged from 1 cm to 6 cm above the calcaneal insertion, where a clear gap was seen (). Two hours later a radiologist-performed ultrasound (LOGIQ E9™, GE Healthcare) and reported an enlarged distal tendon and a complete rupture at 5–6 cm from the calcaneal attachment, creating a 2.7 cm blood-filled gap (). Surgical exploration 8 days post-injury found a complete Achilles tendon rupture ‘5–10 cm above the ankle joint’.
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pmc-6169946-1
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A 57-year-old female presented to her GP with a 3-month history of left-sided catarrh and epistaxis from her left nostril. Clinical examination was unremarkable and the patient was initially diagnosed with sinusitis. However, the symptoms did not resolve following treatment for sinusitis. On further examination, her dentist noted left palatal swelling and referred her to the maxillofacial clinic by which time she had been suffering from these symptoms for 18 months. In hindsight, her epistaxis might have been a warning sign, and on reflection, the GP highlighted the need to take new epistaxis seriously. Clinical examination by the maxillofacial team revealed diffuse palatal swelling of the hard palate. Subsequent magnetic resonance imaging (MRI) showed a palatal tumour extending into the floor of the left nasal cavity and projecting into the left maxillary antrum through the medial wall. Biopsy of the palate showed an invasive tumour indicative of an ACC of minor salivary glands in the palate. Staging was T4N0M0.
She underwent a left hemimaxillectomy where the palate and floor of the nasal cavity were excised. The defect was covered with a removable obturator. She also had postoperative radiotherapy.
The patient remained in remission for 8 years. She underwent several surgeries during this time such as alar repositioning surgery to help reduce facial asymmetry and augmentation rhinoplasty to help support the nasal collapse that was secondary to the hemimaxillectomy and radiotherapy. She also had fat grafting to her upper lip to improve the lip seal. As a result of her disease process and treatment, she had Eustachian tube dysfunction and had a number of grommets inserted. She experienced problems in accessing an adequate palatal obturator requiring referral to Birmingham Dental School. Counselling from local hospice charity LOROS was also sought to help the patient come to terms with the psychological and physical impact of major and disfiguring surgery.
After 8 years of being in remission, she presented to the GP with a tingling and burning sensation of her left mandible and tip of tongue. Clinical examination did not show any lesion in the oral cavity and oropharynx with no cervical lymphadenopathy. The patient was subsequently referred to the consultant maxillofacial surgeon who had a low index of suspicion for recurrence at this late stage so investigations were not urgently undertaken. The chest X-ray was organised by the GP at the request of the maxillofacial team and an MRI scan was organised by the maxillofacial team. Fortunately, the personal list system at the practice ensured that the GP was well informed and able to act promptly. However, even after MRI scanning, the diagnosis of recurrent disease was still unclear so a multidisciplinary team discussion regarding the need for biopsy took place. Ultimately, at biopsy she was found to have recurrent disease in the left masticator space extending up to the base of the skull (). In addition, there was involvement of the left trigeminal nerve, explaining the patient’s unusual sensations. Computed tomography (CT) of the thorax additionally showed possible solitary metastasis, with a diameter of 1.5 cm in the left upper lobe, which was subpleural in location, though chest X-ray had been unremarkable.
Following a further multidisciplinary meeting, the patient underwent a left selective neck dissection, craniofacial resection including a lip split mandibulotomy, and reconstruction using a left radial forearm free-flap. shows the CT scan following the surgery.
Ten months after surgery, the patient underwent left video-assisted thoracoscopic surgery involving wedge resection of the subpleural, left upper lobe nodule. Histological examination confirmed this to be ACC. Since then, the patient has been in remission for 18 months. While medically she is in remission, she suffers from the psychosocial implications of the facial deformity following the surgery and the discomfort with the prosthesis. This continues to affect her quality of life, her confidence to be in public places, and ability to eat.
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pmc-6170324-1
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A 42-year-old female patient (BMI – 23, non - smoker) was presented to our clinic with no severe complaints, for extirpation of retrorectal mass. 3 months ago, during the preventive gynecological examination, ultrasound analysis showed a mass incidentally found in the left side of the pelvis. Pelvic computed tomography with contrast was decided to perform. It revealed a well circumscribed adipose tissue mass with septums, which was located behind the uterus, at the left ovary projection and covers the entire pelvic cavity. The size of a mass was 11.5 cm × 6.5 cm in diameter. It presupposed diagnosis of dermoid cyst or lipoma. What is more, a congenital renal tract abnormality-duplicated collecting system, was detected. To clarify diagnosis and localization of lipoma the magnetic resonance imaging (MRI) was performed. It showed a giant retrorectal homogeneous adipose tissue opacity mass, surrounded by thin fibrous capsule, 12 cm × 6.7 cm × 8.6 cm in diameter, spreading toward the left obturator foramen (Photos 1). Surgery was indicated due to exclude malignant process certainly, because it is difficult to differentiate lipoma from lowgrade liposarcoma on MRI []. A laparoscopic extirpation of the retrorectal tumour was planned. We decided to perform laparoscopic approach instead of laparotomy to reduce postoperative complications, length of hospital stay and pain, furthermore, minimally invasive surgery brings better cosmetic results.
Informed patient consent had been obtained before the procedure. The patient was brought under general anesthesia with endotracheal intubation; surgery was performed by the laparoscopic approach. The patient was placed in the Trendelenburg position. After preparation of the surgical field and pneumoperitoneum formation, the camera port for video laparoscope (10 mm) was placed. Organs of the abdominal cavity were explored, some adhesions in the true pelvis and right iliac region were found. Under the control of the laparoscope, three trocars were introduced: one 12 mm trocar in the right iliac region and two 10 mm trocars on right and left sides of the paraumbilical region, 5 cm below the umbilicus. The harmonic scalpel and two bowel forceps were inserted. Rectum dislocated to the right side of pelvis and two ureters were found. The soft tumor arranged between rectum and the left wall of the bony pelvis was palpated. After the pelvic peritoneum was revealed and the anterior surface of the lipoma was exposed, the tumor was dissected from the mesorectum, presacral fascia and the lateral side of the bony pelvis (Photo 2, 3). The resected tumor was removed in a retrieval bag through the 12 mm in the right iliac region, which was enlarged for its delivery. One drain in the tumor bed was inserted. The trocar wounds were sutured. The tumor of the adipose tissue structure was approximately 15 cm × 10 cm × 8 cm (Photos 4). The overall operative time was 80 min. Histological examination of the removed tissue was performed and the final diagnosis of the pathology was lipoma. The operation and early postoperative period showed no major complications. The drain was removed on the 1st postoperative day. On the 2nd postoperative day, in a satisfactory condition, the patient was discharged from the hospital. Currently, two weeks after the surgery, there is no complications related to the surgery and the patient is feeling well.
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pmc-6170515-1
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A 42-year-old man presented to our outpatient clinic because of the presence of a pararectal tumor that was found incidentally during a routine medical examination. The patient had no urinary or gastrointestinal symptoms and no previous medical history. In the physical examination, a smooth-margined, hard elastic mass was felt, and in a digital rectal examination, the rectal mucosa appeared to be normal.
The computed tomography (CT) scan showed a 5-cm, well-defined, solid mass in the left ischiorectal fossa abutting the left anal wall and extending into the inferior perineum (Fig. ). Contrast-enhanced CT showed intense heterogeneous enhancement that persisted during the delayed phase (Fig. , ), and a feeding vessel was visible around the mass (Fig. ). Intense arterial enhancement suggested a hypervascular nature and persistent delayed enhancement suggested a fibrous nature of the mass. The differential diagnosis was an SFT, gastrointestinal stromal tumor (GIST), aggressive angiomyxoma, leiomyoma, neurogenic tumor, or soft tissue sarcoma. There was no evidence of distant metastasis in the chest or abdomen.
Magnetic resonance imaging (MRI) revealed several useful findings for differential diagnosis (Fig. ). T1-weighted images of the mass showed homogenous intermediate signal intensity (Fig. ). T2-weighted images yielded heterogeneous intermediate and low signal intensity (Fig. , ). Images of the mass contained areas of low signal bands (Fig. ) and heterogeneous high signal intensity (Fig. , ). The gadolinium contrast-enhanced fat-suppressed T1-weighted images showed homogenous enhancement in the delayed phase (Fig. ). Persistent delayed enhancement and low signal bands on T2-weighted images suggested a fibrous component of the mass. High signal intensity on T2-weighted images was suggestive of various components such as myxoid or necrotic cysts.
Although contrast-enhanced CT showed nonspecific findings that were not inconsistent with a benign tumor, an SFT was suspected particularly from the MRI findings. Our patient did not hope for surgery if the tumor was benign; however, most SFTs are benign but have malignant potential; therefore, an ultrasound-guided, transperineal core needle biopsy was performed for a more precise diagnosis to decide on a treatment strategy.
Microscopic examination showed tumor cells appearing as spindle and fibroblast-like cells within a collagenous stroma (Fig. ). Immunohistochemistry identified cluster of differentiation (CD) 34 and vimentin, supporting the diagnosis of an SFT (Fig. ).
The patient consented to excision of the mass. He was placed in a prone jackknife position, and the tumor was removed transperineally using a posterior approach (modified Kraske procedure). A 5-cm vertical incision was used to approach the mass (Fig. ). Following a short split of the gluteus maximus muscle at the cranial part of the incision, a careful blunt dissection was performed while compressing the tumor to the anal side from inside the rectum with the index finger (Fig. ). The tumor was separated from the rectal wall, the levator ani muscle, and the external sphincter muscles and avoided cutting into the mass itself. The levator ani muscle, the external sphincter muscles, and rectum were not involved and easily separated from the tumor. Considering the potential for tumor seeding along the needle tract, en bloc excision of the needle tract with the tumor was successfully performed with no complication, and the integrity of the peripheral structures was preserved. The posterior approach provided good exposure without excision of the coccyx. The patient was discharged 5 days after the surgery without complications.
Gross examination of the tumor demonstrated a 53 × 35 × 25-mm, well-defined, completely encapsulated mass (Fig. ). The sectioned gross specimen demonstrated a solid, yellowish-white cut surface with hemorrhagic areas (Fig. ). Microscopically, the tumor cells appeared as spindle and fibroblast-like cells within a collagenous stroma, and it was consistent with an SFT. Necrosis and cytological atypia were not present. Mitotic activity was one mitosis per ten high power fields (HPF). Immunohistochemical analysis of the tumor cells showed positive results for CD34 and vimentin but negative for CD31, epithelial membrane antigen (EMA), α-smooth muscle antibody (SMA), desmin, and S-100.
There was no local recurrence of the tumor during the year following surgery.
An SFT is a rare neoplasm that occurs in approximately 2.8 of every 100,000 people. Recent advances in electron microscopy and immunohistochemistry have demonstrated a mesenchymal rather than a mesothelial origin of SFTs. While the majority of SFTs occur within the pleura, approximately 30% of SFTs arise in the peritoneal cavity, retroperitoneal soft tissue, and pelvis [, ]. However, SFTs occurring in the ischiorectal fossa are extremely rare.
Pelvic SFTs usually occur during the fifth decade of life (age range, 20–70 years) and do not exhibit any gender predilection []. Most patients are asymptomatic, but large tumors may result in the compression of adjacent structures. Refractory hypoglycemia (Doege-Potter syndrome) has been reported in 5% of patients [].
Tumors involving the ischiorectal fossa are most often secondary to direct extensions of primary anorectal, prostate, and sacral tumors []. Primary tumors are rare and diverse, benign (e.g., aggressive angiomyxomas [–], plexiform neurofibromas [, ], GIST [, ], leiomyomas []) to malignant (e.g., metastatic disease [], lymphomas [], liposarcomas [], epithelioid sarcomas []). In these primary tumors, solid well-circumscribed and slow-growing tumors are often benign and tend to be followed up depending on their size. Most SFTs are also benign, but approximately 10–15% of SFTs are malignant, recurring locally or as metastases [].
Typical SFTs show an architecture characterized by a combination of alternating hypocellular and hypercellular areas separated by thick bands of hyalinized, somewhat keloidal collagen and branching hemangiopericytoma-like vessels. Myxoid changes, areas of fibrosis, and interstitial mast cells are commonly observed. Some SFTs may contain mature adipocytes and/or giant multinucleated stromal cells, thus overlapping morphologically with the so-called lipomatous hemangiopericytomas and giant cell angiofibromas. Malignant SFTs are usually hypercellular lesions showing at least focally moderate to marked cytological atypia, tumor necrosis, numerous mitoses (≥ 4 mitoses per ten HPF) and/or infiltrative margins []. Tumor cells in SFTs are characteristically immunoreactive for CD34 (90 to 95% of cases) and CD99 (70%). Twenty to 35% of SFTs are positive for EMA, BCL2, and SMA []. Because of their rarity, reports detailing radiological findings of SFTs of the ischiorectal fossa are few [, , ]. CT scans usually show a smooth-margined, soft-tissue mass with uniform soft-tissue attenuation and heterogeneous enhancement of secondary to myxoid degeneration, hemorrhage, or necrosis []. Calcification arising within an SFT is rare and is usually seen only in large tumors [].
MRI shows intermediate signal intensity on T1-weighted images and heterogeneous high and low signals of mixed intensity with multiple flow voids representing prominent vascular channels on T2-weighted images [, ]. On T2-weighted images, areas of low signal intensity suggest mature fibrous tissue containing few cells and abundant collagen stroma. In contrast, areas of high signal intensity suggest edema, cellularity, myxoid degeneration, hemorrhage, or necrosis on T2-weighted images []. Intense heterogeneous enhancement that persists in the delayed phase images may be obtained following contrast material administration. Intense arterial enhancement indicates the hypervascular nature of the mass []. Large collateral feeding vessels are present in 35% of SFTs; however, the presence of the collateral vessels is not specific, and other hypervascular tumors also show these findings []. Persistent delayed enhancement suggests the fibrous nature of the tumor []. Hemorrhage [], tumor necrosis, and infiltrative margins (in approximately 10% of cases) [] are mostly observed in locally aggressive or malignant tumors.
The differential diagnosis of primary tumors in the ischiorectal fossa with hypervascular and fibrous nature are GIST, leiomyoma, and soft tissue sarcoma. MRI findings based on tissue characterization of tumors can narrow the differential diagnosis; however, they often overlap. Therefore, further accurate and less invasive investigations for diagnosis are necessary to determine an appropriate treatment strategy.
Given the perceived potential for tumor seeding along the needle tract even for SFTs [, ], percutaneous biopsy of a potentially malignant lesion is controversial. According to Berger-Richardson’s [] report that examined seeding after percutaneous needle biopsy for sarcoma, in the setting of a retroperitoneal sarcoma, a review of pooled data from four large tertiary care referral centers demonstrated a 0.37% risk of needle tract seeding. They concluded that the benefits of pretreatment biopsy in patients with mesenchymal tumors outweigh the potential risks of needle tract seeding. They also concluded that although en bloc excision of the needle tract with the primary tumor is often performed, this practice is not associated with improved oncologic outcomes. Buchs et al. [] described that the biopsy can alter the management of the patient. Certain cases of aggressive angiomyxoma can be treated initially with goserelin acetate (GnRH agonist, Zoladex), some GIST with imatinib, and some desmoids fibromatosis with tamoxifen and sulindac; a reduction in the size of the lesion or a reduction of the local invasion has been observed.
Complete surgical excision is the only established curative treatment for an SFT. However, surgery may become difficult due to extensive adhesions to adjacent vital structures. If complete surgical excision is not possible with an acceptable safety margin, local recurrence is a possibility. Generally, the posterior approaches in the prone jackknife position (modified Kraske procedure) are commonly used for incision of the ischiorectal fossa and presacral tumors [, –]. The details of approach are dictated by the location and the size of the mass and its relationship with adjacent structures. In the posterior approach, an incision starting from the lower mid-sacrum or the tip of the coccyx to the side of the anus, parasacral incision, or vertebral para-anal incision is used [, –]. The ipsilateral gluteus maximus and sacrotuberous ligament are divided over the ischial tuberosity and edge of the sacrum, and if not given sufficient exposure for the removal of the tumor, the division of the lower sacrum below the border of the S3 vertebra may follow [, ]. This approach can allow entry into the presacral space, at the point proximally above the location of the tumor. Low-lying tumors (the upper limit of the lesion is below the level of S4) can be removed by the posterior approach [, , ]. For small lesions (less than 5 cm), the lower sacrum can be spared [, , ]. If the superior border of the tumor can be palpated during the digital examination, the posterior approach should be successful []. The posterior approach provides good access to the caudal component, but the major drawbacks are the absence of control over pelvic vessels and the potential for injury to the lateral pelvic nerves []. An anterior transabdominal approach is recommended for large and high-lying tumors which extend superiorly through the levator muscles) []. In our case, bleeding during surgery was quite limited, but preoperative embolization of highly vascular SFTs may be used to reduce intraoperative blood loss [, ]. In unresectable cases, surgical cytoreduction with chemotherapy and radiation therapy might be useful [, ]. There is no consensus concerning follow-up for SFTs, but continued long-term surveillance is advised due to the indolent nature of the tumor and the possibility of late recurrence up to 20 years after initial treatment [, ].
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pmc-6170624-1
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A 72-years old female was referred to the IRCCS SDN with medical prescription to undergo a Magnetic Resonance Imaging with simultaneous Positron Emission Tomography (MRI-PET) exam in order to investigate whether the MH she referred had an epileptic nature. She has 5 years education and she is housewife; she does not display neurological illnesses, neurodegenerative disorders, or psychopathological conditions. The onset of MH was sudden, six months since neurological evaluation, coinciding with a period of stressful events affecting the health of her husband. From the onset, her MH present daily, mainly during the afternoon.
From the clinical interview made by the neuropsychologist, emerged that familiar Neapolitan, English, and French songs (3–4 current songs with no special preference, accompanied with music, without amplitude alterations neither memories/emotions associated) characterize patients’ MH that do not interfere with the sleep and with daily activities and are slightly weakened by listening radio or watching television. The patient always hears the same songs, in particular singers’ original voice without instrumental contribution. The patient did not report co-existing auditory hallucinations.
She also experienced hearing impairment assessed by an otolaryngologist who diagnosed presbyacusys and recommended the use of bilateral mobile acoustic prostheses.
The present study was carried out in accordance with the Declaration of Helsinki and the local ethics committee approved it. Written informed consent was obtained by the subject, also specifically for the publication of collected data, in anonymous form, in the present case report.
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pmc-6170626-1
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A 30-year-old female presented with a 10-year history of pain in the lumbosacral spine; she had had casual radiation to both lower limbs. After her first delivery, she developed back pain. The MRI demonstrated a tethered cord at the L4 level and a filum terminale lipoma. The MRI also showed tortuous veins on the spinal cord surface (Figure ). Nine years later, during the second pregnancy, she noted weakness and sensory loss, imbalance with urinary and fecal incontinence. On admission, she had bilateral plantar flexion weakness (grade 4 according to modified Medical Research Council system) and reduced sensation in the gluteal regions and legs, and plantar response. The Babinski and Rossolimo signs were present bilaterally. A repeat MRI revealed intraspinal T2 hyperintensive changes in the thoracic spine and conus (Figures ). Those changes were consistent with neurologic deficit and, after exclusion of inflammatory demyelinating diseases, based on a brain MRI and an aquaporin-4 antibody test, diagnosed as myelopathy. Due to described torturous veins on the spinal cord surface (Figure ), the patient underwent spinal digital subtraction angiography (DSA). The range of DSA was from the Th6 level to the coccygeal artery. The results did not reveal any vascular malformation. Therefore, the preliminary diagnosis was symptomatic TCS and filum terminale lipoma. In view of weakness and neurological deficit surgical spinal cord de-tethering, without lipoma resection was planned.
The sacral canal was opened with a median incision. The dura was thin and transparent. After a midline dura and arachnoid incision, a tumor was visualized that appeared to be a lipoma. It engulfed the filum terminale and spinal nerve roots caudally, to the S2 level. The S1 and S2 nerve roots were positioned lateral to the tumor. A thickened, tortuous, bright-red vessel was also noted on the filum terminale, running in the cranial direction. The external surface of the dura was explored bilaterally at the sacral level in search for a SDAVF. A clearly visible nidus was identified at the S3 level on the left side. An empirical test was performed to support this finding. A clip was placed on the arterialized vein, intradurally, for a few minutes. Then, the vessel on the filum terminale clearly changed color, from bright red into bluish gray, and its tension decreased. The SDAVF was then intradurally disconnected, and the nidus was extradurally coagulated. In addition, the filum terminale was cut, and samples of lipoma were taken for histology. The dura was sutured in a watertight fashion, and the wound was closed in layers.
The postoperative course was uneventful. A few days later, the patient was discharged. A follow-up MRI showed resolution of the myelopathic changes (Figures –). At the 1-year follow-up, the patient had considerably improved, with no incontinence or motor deficit.
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pmc-6170626-2
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A 33-year-old male presented with a 2-year history of pain in the lumbosacral spine with progressive lower-limb weakness, urinary frequency, and incontinence. Symptoms intensified after exercise and prolonged standing. Lumbar spine MRI demonstrated a tethered cord at the L3 level, a filum terminale lipoma, and edema in the spinal cord. It also showed tortuous veins on the posterior surface of the spinal cord (Figures ).
The neurological examination revealed bilateral hip-joint flexion weakness (grade 4 according to modified Medical Research Council system), plantar flexion weakness (grade 3 according to modified Medical Research Council system), and reduced sensation in the S2–S3 dermatomes. The Babinski and Rossolimo signs were present bilaterally. Based on the clinical and radiological signs, a SDAVF was suspected. A DSA confirmed the SDAVF at the S2-S3 level, in the dura supplied by lateral sacral arteries and branches from the internal iliac arteries. Tortuous draining veins were present on the posterior surface of the entire spinal cord and terminated intracranially (Figures ).
The patient underwent three endovascular embolizations, each with a different agent. First, embolization was performed with Onyx™ 18, administered in two microcatheters. This treatment resulted in an occlusion of the fistula. The patient significantly improved, but due to poor penetration of the draining vein, after a few weeks, the fistula recanalized and symptoms returned.
A second embolization was performed 4 months later. The vascular access to the fistula was more complicated. A 25% Phil™ injection was feasible only through the small sacral branches of the left internal iliac artery. This procedure achieved a significant reduction of flow through the fistula. Eighteen months later embolization of the fistula was attempted with diluted glue. Despite good occlusion of the fistula observed in a DSA, the tortuous vessels remained visible in the follow-up MRI (Figures ). The symptoms persisted, and the patient was qualified for surgical treatment.
The goal of surgery was to occlude the draining vein and the arterial feeder and to reduce the lipoma. With a median incision, the sacral canal was opened on the right side. The dura was thick, and there were multiple vessels filled with embolic agents. There was one large, bright-red vessel extradurally and one intradurally at the S2-S3 level. A clip was placed upon the arterialized vein intradurally for a couple of minutes. After that, the vessel changed color and the tension decreased. Both intra- and extradural vessels were coagulated. Afterwards, the lipoma was visualized and partially removed. The dura was sutured in a watertight fashion, and the wound was closed in layers.
The postoperative course was uneventful, and a few days later, the patient was discharged. After 6 months, a follow up MRI showed the absence of tortuous vessels on the posterior surface of the spinal cord and a significant reduction in the myelopathic changes (Figure ). On follow-up, the patient considerably improved, with no incontinence, some urinary frequency and mild weakness (grade 4 according to modified Medical Research Council system).
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pmc-6170650-1
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In 2014, an 83-year-old woman with no history of known autoimmune disease was diagnosed as having a right leg superficial spreading melanoma, initially T2b N0 M0. Eight months later, she developed iterative local and in transit cutaneous metastases on the same leg and she underwent four times surgical excision.
In 2016, at the fourth recurrence, surgery was not chosen. Baseline full-body computed tomography revealed no other metastasis (T2 N0 M1a). Mutation tested on a tumor sample excluded the presence of any BRAF mutation. Administration of pembrolizumab therapy was started at 2 mg/kg every 3 weeks, resulting in complete remission (CR) within 3 months (cycle 4). In March 2017, after 14 cycles, she remained in CR, and the pembrolizumab therapy was stopped at her request.
In October 2017, 6 months after pembrolizumab discontinuation, she complained of oral pain and was referred to our hospital. Clinical examination revealed gingivitis with one tense blister, a large pseudomembrane-covered erosion with a tweezers sign, an atrophy and pseudo lichenoid lesions. Other MM and skin were not involved. Gingival biopsy showed a subepithelial cleavage with the overlying intact epithelium (Figure ). A moderate perivascular infiltration consisting of lymphocytes and histiocytes was observed, with no lichenoid infiltrates. Direct immunofluorescence (DIF) microscopy revealed linear IgG (++) and C3 (++) immune deposits along the basement membrane zone (BMZ) (Figure ). Standard indirect immunofluorescence (IIF) microscopy on rat esophagus failed to detect circulating anti-BMZ antibodies. A diagnosis of mild MMP was made. Further immunological investigations demonstrated that the immune deposits identified using direct immunoelectron microscopy (IEM) were strictly localized in the lamina densa (Figure ), a site consistent with autoantibodies against the laminin 332 or the C-terminal extremity of BP180 antigen (BP180). IIF on salt-split skin, immunoblot using amniotic extracts, and enzyme-linked immunosorbent assays (ELISAs) with BP180-NC16A epitope and BP230 antigen (BP230) had negative results.
Doxycycline therapy (100 mg/day) and mouth washes with corticosteroid (betamethasone 2 mg) three times daily were initiated, which led to the control of the MMP within 2 weeks and CR under this minimal therapy in 6 weeks. After 3 months of treatment with doxycycline therapy, the patient decided on her own to discontinue it, and no MMP relapse had occurred 3 months later. No clinical or radiological evidence of relapse of the melanoma was observed on computed tomography imaging after a 14-month follow-up since discontinuation of the pembrolizumab therapy.
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pmc-6170999-1
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A 56-year-old HIV-infected female presented to Kiruddu General Hospital, Kampala, Uganda accompanied by her 17-year-old daughter. Her daughter reported a three-week history of gradual onset confusion, neck pain, and generalised body weakness. In addition, she had urinary incontinence for several months. She reported no systemic symptoms of TB.
She had been on antiretroviral therapy (ART) and prophylactic cotrimoxazole for 12 years. She attended her HIV clinic every 1–2 months, with good compliance. She had undergone two previous changes to her antiretroviral regimen: the first three years prior due to virologic failure, from zidovudine, lamivudine and nevirapine to second line tenofovir, lamivudine and atazanavir/ritonavir. Her second change three months prior to admission was after having discontinued ART for two weeks when developing urinary incontinence. Thereafter, her tenofovir was switched to abacavir. Her most recent HIV viral load was 251 copies/ml with a CD4 of 384 cells/μL (9 months prior to admission). Repeat plasma HIV viral load during the current admission was 1840 copies/mL, and CSF viral load was 32,000 copies/mL.
She was treated for cryptococcal meningitis 12 years prior and had been taking fluconazole secondary prophylaxis since. In addition, she completed treatment for pulmonary TB (confirmed by sputum Xpert) three years prior. She was known to have type II diabetes mellitus, and took regular metformin and glibenclamide.
On admission, she was afebrile with other vital signs within normal range, and a random blood glucose of 7.4 mM (133 mg/dL). On examination, she had a Glasgow coma scale score of 14 (of 15 possible) due to confusion but no focal neurological signs. She had no evidence of wasting and no palpable lymphadenopathy. She had mild generalised abdominal tenderness with no palpable masses or organomegaly, and no abnormalities on thoracic examination.
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pmc-6171147-1
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Patient 1 (Fig. ): A 42-year-old woman with no medical history of note presented with right hemiparesis and aphasia, and was admitted to our hospital. The actual onset time was unknown. On arrival, her National Institutes of Health Score Scale (NIHSS) was 20. Diffusion-weighted brain magnetic resonance imaging (MRI) showed a hyperintense signal in the left middle cerebral artery (MCA) territory, and MR angiography (MRA) indicated occlusion of the left superior M2 (Fig. , ). Because the infarct area seemed to match with the occluded artery territory, reperfusion therapy was not performed. After admission, we performed examinations to investigate the cause of cerebral infarction. Transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) showed no remarkable findings. A 24-h Holter electrocardiogram (ECG) did not show atrial fibrillation or other arrhythmia. Carotid echography and carotid MRA did not show atherosclerotic changes at proximal arteries. Blood tests were conducted to investigate the possibility of coagulation disorders, such as antiphospholipid antibody syndrome, collagen disease, protein S and C deficiency, antithrombin III deficiency, and tumor markers. However, the results were unremarkable, except for elevation of D-dimer (1.4 μg/mL) and CA 125 (395 U/mL; normal, < 35 U/mL). Whole body enhanced computed tomography (CT) revealed no malignancy. Pelvic MRI showed uterine adenomyosis (Fig. ).
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pmc-6171147-2
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Patient 2 (Fig. ): A 50-year-old woman with no medical history of note presented with right hemiparesis and mixed aphasia, and was admitted to a local hospital. The onset time was unknown. Diffusion-weighted imaging (DWI) in brain MRI revealed a hyperintense area in the left MCA territory. MRA showed occlusion at M1 (Fig. , ). The patient was referred to our hospital for further examination and treatment. On arrival, her NIHSS was 23. Emergent digital subtraction angiography (DSA) was performed and partial reperfusion of the left MCA was found (Fig. ). We hesitated to perform endovascular treatment because of the large infarction. After admission, we performed examinations to investigate the cause of cerebral infarction. TTE and TEE showed no remarkable findings, and a 24-h Holter ECG did not show atrial fibrillation or other arrhythmia. DSA and carotid echography did not show atherosclerotic changes at proximal arteries. Blood tests performed to investigate the presence of coagulation disorders (as listed above for case 1) were unremarkable, except for elevation of D-dimer (3.7 μg/mL) and CA125 (143 U/mL; normal, < 35 U/mL). Whole body enhanced CT revealed no malignancy. Pelvic MRI showed uterine adenomyosis (Fig. ). Her aphasia gradually improved, but motor aphasia remained.
Based on the above findings, both cases were finally diagnosed with cerebral infarction due to Trousseau syndrome-like hypercoagulability associated with adenomyosis. For secondary prevention, the first patient was treated with warfarin and the second patient was treated with rivaroxaban, and there has been no recurrence for 68 and 19 months and modified rankin scale is 1 and 4, respectively.
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pmc-6171188-1
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The 70-year-old male patient was admitted to the Department of Neurology with isolated left upper extremity weakness and clumsiness. He had wrist drop and was unable to grip with the fingers. He complained of no sensory loss or numbness. His past medical history included long-term oral anticoagulation due to recurrent lower extremity deep vein thrombosis, glaucoma, and a non-significant aortic valve stenosis, with vascular risk factors including a 5-year history of treated hypertension, hypercholesterolemia, and a history of non-significant stenosis of the left anterior descendent coronary artery. On admission, no abnormality could be detected by physical examination, apart from the neurological signs, which included a wrist drop on the left side with decreased grip strength (Fig. ). Pronation and wrist dorsiflection were lost, as well as the ability to form a ring with the thumb and the index. The reflexes of the left upper extremity were slightly brisk, with not pathological reflexes present and no sensory deficits. Laboratory parameters were without abnormal findings except for a slightly elevated fasting blood glucose level. Cranial CT revealed contrast-enhancing, irregularly shaped lesions with diameters of 7, 10, and 9 mm (in the temporal, parietal, and frontal lobes, respectively), surrounded by perifocal edema. Carotid duplex ultrasonography did not demonstrate signs of focal atherosclerotic plaques, circulatory disturbance or stenosis on either side. In accordance with the findings of the CT scan, the cranial MRI confirmed the tumor in the right precentral gyrus, corresponding to the ‘hand knob’, accompanied by further similar lesions posteriorly in the parietal and the temporal lobes, and in the left frontal lobe (Fig. ). Electromyography and nerve conduction studies did not support a peripheral origin underlying the paresis. The neuropsychological examination revealed a deficit in the Luria three-step test as a single alteration, which performance could, however, be remarkably improved by verbal clues. Chest X-ray performed as part of the search for primary tumor revealed no abnormality. Non-contrast and contrast-enhanced chest CT detected an irregularly shaped mass in segment 10 of the left lung, with inhomogeneous enhancement of the contrast agent and multiple associated lymph node conglomerates, suggesting lung cancer as the primary tumor. Abdominal ultrasonography did not detect malignancy. The patient received palliative steroid therapy, with no improvement in the paresis during the observation period. Based on the recommendation of the tumor board, the patient was transferred to the Department of Pulmonology for bronchoscopic tissue sampling. The verification of the diagnosis by this means, however, was unsuccessful. The diagnostic process had to be suspended due to an acute bleeding duodenal ulcer, and the patient received blood transfusion and local hemostatic treatment in the Department of Surgery. In association with the antibiotic treatment, the patient developed pseudomembranous colitis caused by Clostridium difficile. In addition to the persisting colitis, fever occurred accompanied by hemoptysis and coughing. The subsequent chest X-ray did not confirm pneumonia. Despite the applied antibiotic and oxygen therapy, respiratory failure developed, and the patient passed away 2 months after the onset of the neurological symptom. The autopsy and the histopathological analysis identified an adenocarcinoma (mucinous tubular adenocarcinoma with solid anaplastic parts) both in the brain and the lung; (Fig. ).
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pmc-6171195-1
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A previously healthy 58-year-old female presented to our clinic with a sudden painful visual loss in her right eye for 2 days. Ocular movement significantly aggravated her pain. Four weeks before the presentation, she developed a group of vesicles on the erythematous base over the right ophthalmic branch of the trigeminal nerve including the tip of her nose, which was diagnosed as HZO. At that time, she was treated with intravenous acyclovir (30 mg/kg/day) for 10 days. The group of vesicles soon disappeared and turned to hyperpigmented macules and patches (Fig. ).
At our clinic, an ophthalmic examination revealed best-corrected visual acuity of light perception in the right eye, compared with 20/20 in the left eye. A relative afferent pupillary defect (RAPD) was present in the right eye. Intraocular pressures were 12 mmHg in both eyes. Ocular motility, anterior segment, and a fundus examination were unremarkable bilaterally. Neither proptosis nor ptosis was observed. The neurological examination was significant for hypoesthesia in the area supplied by the right ophthalmic branch of the trigeminal nerve. A clinical diagnosis of HZO-related right retrobulbar ON was made. To exclude other possible causes of atypical ON, a blood test including a complete blood count (CBC), erythrocyte sedimentation rate (ESR), c-reactive protein (CRP), Venereal Disease Research Laboratory (VDRL), Treponema pallidum hemagglutination (TPHA), antinuclear antibody (ANA), and aquaporin 4-antibody were performed, which all showed normal results. MRI of the brain and orbit showed enhancement and restricted diffusion of a right-sided intraorbital, intracanalicular, and prechiasmatic optic nerve (Fig. ). Notably, linear hyperintense T2 lesions in vertical orientation extending from the right dorsolateral pons down to the medulla without any enhancement or restricted diffusion were also found (Fig. ). These vertical lesions represented the anatomical location of the spinal trigeminal nucleus and tract (STNT) along the brainstem. Lumbar puncture showed mild lymphocytic pleocytosis (22 cells, 98% lymphocytes) with normal protein and a negative polymerase chain reaction (PCR) for VZV.
Treatment was started with intravenous acyclovir (30 mg/kg/day) along with 1 g/day of intravenous methylprednisolone. Intravenous acyclovir was continued for 14 days, then reduced to 800 mg oral acyclovir daily. Acyclovir was discontinued in the third month. Oral prednisolone (1 mg/kg/day) was started after 5 days of intravenous methylprednisolone, then gradually tapered and discontinued in the third month. After the completion of the 2 month treatment, the best-corrected visual acuity was counting fingers and 20/20 in the right and left eyes, respectively. An ophthalmic examination detected a right optic disc atrophy with normal physiological cupping. MRI of the brain and orbit showed stable brainstem STNT abnormalities and resolution of the ON.
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pmc-6171202-1
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The patient is a 75-year-old man referred to our facility for the further management of ST-elevation myocardial infarction (STEMI). He had presented 3 days earlier to an outside hospital with shortness of breath and productive cough of one-day duration. The evaluation was positive for right-sided pneumonia and was started on intravenous ceftriaxone and oral azithromycin. He was gradually improving but on day 3 of admission developed worsening shortness of breath with tachypnea and oxygen desaturation. He denied any precordial pain. His electrocardiogram (EKG) showed ST-segment elevation in anterolateral precordial leads with QT/QTc interval prolongation (). Troponin T then was 0.01 ng/mL (reference range: <0.01 ng/mL). Chest X-ray (CXR) showed diffuse pulmonary edema. He was started on intravenous (IV) furosemide and intubated for hypoxic respiratory failure. He was then transferred to our facility for expectant cardiac catheterization.
On presentation, the patient was intubated, and history was obtained from the patient's family. According to them, the patient was functionally active at baseline prior to presentation. He ambulated with a cane but was able to go up a flight of stairs without anginal symptoms. His past medical history was significant for heart failure with reduced ejection fraction, polycythemia vera, obstructive sleep apnea, hypertension, hyperlipidemia, aortic stenosis, anemia, chronic kidney disease stage IV, and stroke. He had undergone cardiac catheterization 2 years ago which had shown 40–50% stenosis of ostial left anterior descending (LAD) artery and 50% stenosis of the first diagonal branch. The patient was diagnosed with JAK2 V617F mutation-positive polycythemia vera 22 years ago and was maintained on hydroxyurea and darbepoetin alfa. Hydroxyurea was discontinued 2 weeks prior to presentation for anemia (hemoglobin 7 g/dL; reference range: 14–17 g/dL) requiring a blood transfusion and was started on ruxolitinib. Hydroxyurea was restarted 2 days prior at the referring hospital for worsening thrombocytosis.
On examination, the patient was afebrile with a pulse of 80 beats per minute (bpm), blood pressure of 98/56 mmHg, respiratory rate of 28/min, and 99% saturation on FiO2 of 70% intubated on assist control mode of ventilation. Chest examination revealed diffuse crackles and aortic stenosis murmur. He did not have pedal edema. His platelet count was 3,321,000/μL (reference range: 140,000–400,000/μL) and was described as giant platelets on peripheral smear. During the stay, his platelet count peaked at 4,145,000/μL. His white cell count was 13,100/μL (reference range: 4000–11,000/μL), and hemoglobin was 7.5 g/dL. Creatinine was elevated at 3.1 mg/dL (reference range: 0.7–1.4 mg/dL; baseline: 2.3–2.5 mg/dL) His probrain natriuretic peptide (pro-BNP) was 12,885 pg/mL (reference range: <450 pg/mL). Troponin T was 0.99 ng/mL. As EKG in the outside hospital showed ST elevation, he received 2 units packed red cells to reach goal hemoglobin between 9–10 g/dl. Repeat hemoglobin was 9.3 g/dL. He was continued on dual antiplatelet therapy and metoprolol tartrate 50 mg twice daily. He was started on heparin infusion. Transthoracic echocardiography showed an ejection fraction of 35% and moderately decreased left ventricular systolic function with dyskinetic to akinetic distal half of both ventricles consistent with takotsubo cardiomyopathy (). Cardiac catheterization revealed nonobstructive coronary artery disease (). Heparin infusion was discontinued. Post catheterization, his hospital stay was uneventful. He was successfully extubated and discharged a week later (hydroxyurea 1000 mg and ruxolitinib 5 mg both twice daily).
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pmc-6171208-1
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A 48-year-old woman with breast cancer underwent mastectomy (histology: invasive ductal carcinoma, histology grade 2; estrogen receptor: positive; progesterone receptor: positive; HER2: positive; Ki67: 10%, n+[27/28]) at the department of surgery in a previous hospital. Subsequently, she underwent chemotherapy with paclitaxel and doxifluridine and hormonal therapy with tamoxifen. Six years after surgery, bone metastasis was noted in the vertebra, and she was treated with a combination of radiotherapy and chemotherapy with trastuzumab. However, the metastatic disease progressed. Liver metastasis was also noted at 57 years of age, and the treatment was switched to capecitabine plus lapatinib, which was shortly discontinued because of adverse effects. Disease progression continued, although fulvestrant was also added. Eventually, she underwent chemotherapy with trastuzumab emtansine (T-DM1).
For the inhibition of bone metastasis, zoledronate was initiated at 54 years of age and was continued for 5 years until renal failure. After discontinuation of zoledronate, denosumab was used for 3 years until the detection of AFFs in both proximal femurs on the bone scintigraphy at 62 years of age (). Eventually, BMAs had been administered for 8 years. Right hip pain occurred temporarily, whereas left hip pain persisted for a long time. She experienced a left displaced femoral subtrochanteric fracture after falling at the age of 63 years (). At that point, the doctors in the previous hospital made a diagnosis of a pathological fracture caused by bone metastasis and consulted with our department for specialized treatment. After the patient was transferred to our hospital, we examined whether that fracture was due to bone metastasis, but no metastatic lesion was noted at the fracture site. In addition, radiography of the fracture area exhibited a beak on the lateral side of the fracture site associated with cortical bone sclerosis, which was characteristic of an AFF () []. Considering the long-term administration of BMAs, the fractures were diagnosed as AFFs.
For treating the displaced AFF, we could select either IMN or prosthetic reconstruction. For appropriate selection, evaluation of the patient's prognosis is required because recovery of ADL/QOL is the priority in cancer patients with limited life expectancy. The Katagiri score as a predictor of prognosis in patients with skeletal metastasis was high () [], and similarly, the score of another scoring system for metastatic breast cancer was also high () []. Thus, both predicted a poor prognosis. IMN is a less invasive approach, and it might successfully induce bone healing. However, delayed union/nonunion and implant failure are possible issues. To achieve early weight bearing and avoid these issues, we performed endoprosthetic reconstruction (Zimmer Biomet, OSS Proximal Femur) on the seventh day after the injury (). Two weeks after surgery, she achieved walker-assisted gait. After subsequent rehabilitation, prophylactic IMN was performed for the right incomplete AFF (). Six months after surgery, the Musculoskeletal Tumor Society (MSTS) score recovered (47%) to almost the preoperative level (50%) before the injury (). However, she died of breast cancer 1 year and 2 months after the endoprosthetic reconstruction.
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pmc-6171214-1
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A 23-year-old man (weight 65 kg, height 175 cm, and BSA 1.8 m2) with a diagnosis of primitive right atrial enlargement from foetal age was referred to our Centre for cardiological evaluation. Cardiac examination showed increased heart size on percussion and a grade II/VI Levine systolic murmur. No significant pathological findings were found on pulmonary examination. Electrocardiography showed a regular sinus rhythm with a rate of approximately 60 beats/min associated with an abnormal morphology and duration of P wave (enlargement of P wave with duration of 130 msec), together with a low amplitude of QRS complexes in the limb leads. All routine laboratory studies were within normal limits. Chest radiography showed an abnormal cardiac silhouette with increased convexity in the lower half of the right cardiac border and cardiomegaly ().
Transthoracic two-dimensional echocardiography demonstrated a huge right atrium of about 6.2 cm and a volume of 230 ml/m2, with a thick smoke pattern and mild tricuspid regurgitation. The pulmonary arterial pressure was normal (). The tricuspid valve was normal without significant annular dilation. No stenosis or abnormal displacement of the tricuspid valve leaflets was detected. No significant regurgitation of the tricuspid valve was found despite a partial distortion of the anterior leaflet and compression of the right ventricle inflow. The right ventricle appeared small and compressed anteriorly by the right atrium (area of RV: 11 cm2).
Cardiac magnetic resonance imaging showed a marked right atriomegaly (right atrium area: 66.50 cm2, volume: 220 ml/m2) and normal size of the left atrium (left atrium area: 7.02 cm2). The right ventricle was regular in size and global contractility but was partially compressed and dislocated posteriorly, due to the massive enlargement of the right atrium. The left ventricle was regular in dimension, thickness of the wall, and global/segmental contractility (FE VS = 61%). No evident transvalvular jets or areas of late gadolinium enhancement were found. The pericardium was visualized without focal abnormalities or pericardial effusion ().
Due to the high risk of arrhythmias and thrombus formation in the right atrium, which is a potential risk for pulmonary embolism, the patient underwent cardiac surgery. Through a median sternotomy, cardiopulmonary bypass was established with standard aorta and bicaval cannulation. After the pericardium was opened, the entire anterior surface of the heart was found to be covered with a thin wall in continuity with the right atrium. No atrial appendage as such was apparent. The right atrium was fully opened. The inferior border of the atriotomy was sewn around the anterior part of the tricuspid annulus, and the superior border was brought over the lateral wall of the right atrium as a flap and sewn near the interatrial groove. This provided adequate reduction of the atrial size and reinforcement of the atrial wall ().
The histology of the resected atrial wall showed focal hyperplasic areas of smooth muscle cells with polymorphic nuclei surrounded by a few scattered areas of hypertrophic fibrous tissue.
Postoperative transesophageal echocardiogram showed a significant reduction of the right atrium area (23 cm2, volume: 93 ml).
The patient was extubated 11 hours after surgery. Complications arose postoperatively with the early appearance of pericardial effusion with leukocytosis and elevated inflammatory markers. This was resistant to conventional medical therapy, which in the end required surgical drainage. Medical therapy of the postpericardiotomy syndrome (ibuprofen 600 mg/TID and colchicine 1 mg/OD) was continued over the subsequent 6 follow-up months without further recurrence of pericardial effusion.
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pmc-6171215-1
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A 54-year-old Caucasian male with past medical history positive only for diverticulitis with a resultant sigmoidectomy presented to a pain management specialist with progressively worsening bilateral low back, buttock, and radicular leg pain, weakness, and numbness for five weeks after falling from an 8 ft. ladder onto concrete. At the time of the fall, no notable injury was sustained and the patient reported only minimal low back pain without radiculopathy. Over the next several days, the patient began to experience numbness and weakness of the bilateral lower extremities. Evaluation in the ER, which included a hip X-ray and MRI of the lumbar spine (seen in ), revealed only mild lumbar facet arthropathy and moderate left neural foraminal stenosis at L3-L4 without any concomitant hip or spine fracture.
Following discharge, the patient reported worsening low back pain and bilateral lower extremity weakness in both L5 and S1 distributions. Several weeks after the initial fall, the patient was referred to pain management where he was evaluated and eventually underwent a radionuclide bone scan. This revealed mildly increased uptake at L5/S1 but was suspected to be a result of facet osteoarthritis. The patient denied any fever at this time, constitutional symptoms, or recent illness. Review of symptoms and physical exam were unremarkable aside from mild paraspinal tenderness and lumbosacral radiculopathy. The rest of the patient's neurologic exam was normal. The patient was instructed to follow up in one week's time. At the return visit, he reported continued worsening of symptoms with no relief. At this point in time, the patient mentioned that he had been experiencing shaking chills at night. Other review of symptoms and physical exam remained unchanged, and the patient was noted to be afebrile. A CT scan () and subsequent MRI of the lumbar spine () were conducted which revealed extensive findings of discitis/osteomyelitis at L5–S1 as well as an epidural abscess resulting in severe narrowing of the central spinal canal. An emergent decompression laminectomy and discectomy was performed at L5–S1 with evacuation of the epidural abscess. Intraoperative tissue and wound cultures revealed Salmonella enterica serovar Agbeni. The patient recovered well and was discharged on eight weeks of IV ceftriaxone as dictated by culture sensitivities. Patient has recovered well with no neurologic deficits.
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pmc-6171225-1
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A 19-year-old, unmarried woman presented with complaint of chronic pelvic pain and a palpable mass at the lower pelvic midline region. On physical examination, a mobile and painless mass was palpated at the lower pelvic midline region. The external genitalia were normal in appearance. Transabdominal ultrasonography reported a cystic structure in the lower pelvic region communicating with the uterus (likely dilated vagina) with endometrial cavities and absent right kidney. A cystic structure with internal septae was also reported in the left adnexal region.
MR imaging with contrast was performed and showed duplication of the uterine bodies, endometrial canals, uterine cervices and vaginal canals. The right vaginal canal was significantly dilated. There was communication between the two cervices well seen in the axial T2W sequence. A small tubular structure with internal fluid signal along the anterolateral aspect of the dilated right hemivagina represented blind ectopic ureter (the mesonephric remnants) (Figs. and ). Right kidney was not visualized in included sections of the upper abdomen (Fig. ).
A heterogeneous cystic structure was seen in the left ovary with hemorrhagic components.
The patient underwent surgery under general anesthesia. The septum was excised and hematocolpos was drained. The hemorrhagic, infected left adnexal cyst was also excised.
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pmc-6171255-1
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The patient was a healthy 28-year-old woman who works as a book binder with no history of interest to this report. The fingers of her dominant hand were caught in a binding machine during work. Immediately after the injury, sensory disturbance and numbness occurred and persisted. Furthermore, she experienced severe neuropathic pain and anesthesia of her index finger. The patient came to our clinic 2 weeks after the injury.
A wound scar was observed near the distal interphalangeal (DIP) joint of the ulnar side of the index finger (). Sensory examination revealed hypoesthesia in the thumb, index, middle, and little fingers. In the index finger, in particular, the area of the ulnar side beyond the wound was red (indicating loss of protective sensation) based on Semmes-Weinstein monofilament score used by tactile and contact force tester (2SA01, Kono Seisakusho Co. Ltd., Japan) [] (). Tinel's sign was observed, consistent with the wound. Severe neuropathic pain was observed both at rest and during movement. The visual analog scale (VAS) for pain was 10/10 mm. There were no radiological findings suggestive of fracture (). The range of motion of the index finger (injured/healthy side) was restricted due to neuropathic pain: metacarpophalangeal (MP) joint: 0°/70°, proximal interphalangeal (PIP) joint: 0°/40°, and DIP joint: 0°/60°. The total active range of motion (TAM) was 63%.
In a sensory nerve conduction study (NCS), we attempted to derive sensory nerve action potentials (SNAPs) of the thumb, index, and middle fingers. Using the antegrade recording method, SNAPs were collected by stimulating the digital nerve; they were derived from the forearm just above the median nerve using a surface electrode. No derivation of SNAP was observed in the distal area of the index finger (). We also attempted to use ultrasound for diagnosis, but definitive diagnosis proved difficult due to the small size of the digital nerve.
Neurotmesis was suspected based on clinical findings and NCS. Although we attempted to control her neuropathic pain, her symptoms were uncontrollable by conservative therapy. If neurotmesis was not denied by NCS, we recommended the patient to conduct intraoperative diagnosis in our facility. Surgery under general anesthesia was performed 5 weeks after injury. Intraoperative findings revealed severe adhesion around the digital nerve, but the nerve preserved its continuity. The intraoperative diagnosis was nerve crush injury. After undergoing neurolysis, 0.5 ml of PRP was intraneurally injected at two locations of the injured area that was identified based on intraoperative findings (total administration volume = 1 ml) (). A 25-gauge needle was used for infiltration, and PRP was injected from the proximal side of the injured site under direct vision.
After 1 week of immobilization, rehabilitation of active finger motion was initiated. A decrease in neuropathic pain was observed immediately after surgery (). Improvement of restricted finger range of motion was recognized from 2 weeks postoperatively and became normal 4 weeks after surgery. In the Semmes-Weinstein monofilament test, the sensory disorder recovered gradually and completely recovered 6 months postoperatively (–(d)). The derivation of SNAP was confirmed 3 months postoperatively, and it became normal 9 months postoperatively (Figures and ). Neuropathic pain disappeared 9 months after surgery.
PRP was elaborated according to PRGF-Endoret technology (BTI Biotechnology Institute, Vitoria-Gasteiz, Spain) in the cell-processing factory unit of our hospital which fulfills the criteria for good manufacturing product (GMP). Briefly, a total of 36 ml of peripheral venous blood was withdrawn into four tubes of 9 ml containing 3.8% (w/v) sodium citrate. Blood was centrifuged at 580 g for 8 minutes at room temperature (24–26°C). The upper volume of plasma (platelet-poor plasma; PPP), which contains a similar platelet count to that of peripheral blood, was drawn off and discarded in a collection tube. The 2 ml plasma fraction (PRP) located immediately above the sediment of red blood cells, but not including the buffy coat, was collected from each tube (total 8 ml PRP) and transferred to another tube. This tube was taken to the operation room ready for use. This plasma contains a moderate enrichment of platelets (two- to threefold the platelet count of peripheral blood) with scarce leukocytes, being a P2-x-Bβ PRP according to the classification system proposed by DeLong et al. []. The concentration rate of administered PRP in this case was 2.23 (). To initiate clotting, calcium chloride (10% w/v) was added to the liquid PRP aliquots just before injection. All procedures were performed under sterile conditions.
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pmc-6171295-1
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A 47-year-old white female was referred at 1 AM to our Urology department from the Emergency Room for admitted migration inside the bladder of a metallic urethral dilator used for sexual stimulation. The patient stated that she had bought the object through a dedicated internet site. An ultrasound study revealed a partially full bladder with an echogenic internal structure (Fig. ). An X-ray plate of the pelvis clearly visualized the presence of a high-density object shaped like a rifle bullet about 6 cm long, placed obliquely above the pubic symphysis. It was referred by the Radiologist as “likely intrauterine device” (Fig. ).
Since the patient had no symptoms, she opted to return home under oral antibacterial treatment with Ciprofloxacin, and endoscopic extraction was planned in 2 days time, with the program of introducing through the urethra under sedation a 24 F nephroscope and to extract the dilator placing it in line with the instrument axis and retrieving it with a 3-pronged rigid grasper.
The following day, when contacted by telephone again, the patient refused hospitalization, stating that she had be able to self-manipulate retrogradely the dilator through the urethra outside the bladder (Table ). An US and X-ray study of the pelvis confirmed the absence of the foreign body (Fig. ).
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pmc-6171728-1
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A 44-year-old Hispanic female presented with a three-week history of recurrent sharp interscapular pain radiating to the mid-sternal and epigastric region associated with refractory nausea and vomiting. She underwent cholecystectomy for intermittent epigastric pain two years ago. CT abdomen at that time showed a subcarinal mass measuring 5.4 X 5.0 cm (
). Subsequent EUS diagnosed it as a bronchogenic cyst. EBUS guided aspiration resulted in an incomplete drainage and she was discharged after partial improvement.
Current physical examination showed a heart rate of 126/min (normal range: 60–100/min) and respiratory rate of 20/min (normal range: 12–20/min). Initial labs showed white cell count of 10.58X10
3/uL (normal range: 4000–11X10
3uL), elevated inflammatory markers [ESR of 63mm/hr (normal range: 0–20 mm/hr); CRP of 116 mg/L (normal range: <3.0 mg/L)], and hypokalemic metabolic alkalosis. Electrocardiogram showed non-specific T wave changes. Chest X-ray showed right posterior mediastinal mass (
).
CT chest showed an increase in the size of the bronchogenic cyst (9.64 X 7.7 cm) with small right pleural effusion (
).
The X-ray and CT findings were consistent with partial cyst rupture or an infected cyst. X-ray esophagogram ruled out esophageal compression or contrast extravasation. The patient’s symptoms were refractory to conservative analgesic and antiemetic measure like Dilaudid (hydromorphone) 1 mg IV every 3 hourly and Zofran (Ondansetron) 4 mg IV every 4 hourly for pain and nausea/vomiting respectively. Cardiothoracic surgery was consulted and the patient underwent right thoracotomy and surgical cyst excision. Cyst pathology was consistent with severe inflammatory changes. Within 24–48 hours after the surgery, the resolution in the patient’s symptoms were noted in terms of decrease in need of pain and nausea medications. Repeated labs showed resolution of leukocytosis.
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pmc-6171780-1
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A 66-year-old male with a past medical history of hypertension and pancreatic adenocarcinoma presented to our hospital with complaints of nausea, vomiting, and generalized weakness in the arms and legs. The patient was diagnosed with locally advanced, pancreatic cancer, T1 N0 M0 a year prior to presentation. Magnetic resonance cholangiopancreatography (MRCP) revealed a 1.7 cm mass at the head of his pancreas, locally invasive but without the involvement of lymph nodes, superior mesenteric artery, superior mesenteric vein or portal vein. Endoscopic biopsy revealed adenocarcinoma. The patient was a poor surgical candidate due to social issues, alcoholism, residence at a nursing home and was at a high-risk for post-surgical complications. The patient was treated palliatively with nine cycles of gemcitabine and paclitaxel. The initial dose of gemcitabine was 2000 mg. The tumor decreased in size and CA 19-9 level declined from an initial level of 2000 to 26 units/mL. Later the dose of gemcitabine was reduced to 1400 mg (20% reduction) after the sixth cycle due to pancytopenia.
On admission to our hospital, the patient reported abdominal pain that was sharp and located in the right lower quadrant (RLQ). He denied fevers or chills. The patients' vital signs were: temperature 99.3 °F, heart rate of 73 beats per minute, blood pressure 129/60 mmHg, respiratory rate of 17 breaths per minute and oxygen saturation 100% on room air. The physical examination was remarkable for RLQ tenderness. The laboratory data revealed hemoglobin (Hb) 6.5 g/dL, hematocrit (Hct) 19.8, mean corpuscular volume (MCV) 83.2fL /red cell, red cell distribution width (RDW) 19.1 %, white cell count of 9.44 x 109/L, platelets of 54 x 109/L, alanine transaminase (ALT) 133 IU/L, aspartate transaminase (AST) 222 IU/L, alkaline phosphatase (ALP) of 147 IU/L and a total bilirubin of 5 umol/L. BUN was 42 mg/dl, creatinine 2.12 mg/dl (baseline creatinine of 0.8), LDH was 1700 u/l, reticulocyte count was 7.8%. Peripheral smear showed microcytic anemia with frequent schistocytes consistent with a microangiopathic hemolytic process (Figures -). Urinalysis was positive for 1+ blood and 1+ albumin. Computed tomography (CT) scan of the abdomen without contrast showed a stable pancreatic mass and no signs of hydronephrosis (Figure ). ERCP revealed choledocholithiasis. Choledocholithotomy was performed and subsequently the bilirubin improved. Blood cultures grew Klebsiella. He was treated with piperacillin-tazobactam. The patient received intravenous (IV) fluids, blood and platelets when the Hb and platelets declined to Hb of 6.4 g/dl and platelet counts of 8 x 109. He was treated with methylprednisone 30 mg IV q24 and the platelet count increased to 20 x 109. His creatinine increased to 12 mg/dl and BUN to 112 mg/dl. ADAMTS13 activity was 34%. A diagnosis of GiHUS was made. The patient was offered plasmapheresis, but he opted for hospice.
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pmc-6172139-1
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A 74-year-old male presented in February 2016 with increasing dysphagia. There was no history of prior abdominal infection or surgery. On esophagogastroscopy, a necrotic and circumferential friable tumor was seen at 33 to 40 cm from the incisors, with an endoscopic appearance of involvement of gastroesophageal (GE) junction and the proximal 2 cm of the stomach. Biopsies of the distal esophageal tumor confirmed poorly differentiated adenocarcinoma. The patient was anemic with a hemoglobin of 89 g/L. Staging endoscopic ultrasound suggested a breach of muscularis propria and four enlarged paraesophageal nodes. Neoadjuvant chemoradiotherapy followed by esophagectomy was initially considered; however, a staging positron emission tomography (PET) scan demonstrated 18-fluorodeoxyglocose (FDG) uptake not only in the primary tumor, but also in the paraesophageal region near the GE junction and upper abdominal lymph nodes extending as far inferiorly as the right renal vessels, in a retrocaval location (Figure ).
A radiation oncology consultation was sought regarding treatment options of such extensive lymphadenopathy. Palliative radiation therapy (RT) was recommended. The patient was also evaluated by a medical oncologist who advised that chemotherapy may be considered after assessing the response to palliative radiotherapy.
From March 21, 2016 to April 5, 2016, the patient received palliative RT to the symptomatic primary tumor and closest adjacent nodes using a pair of anterior and posterior fields. A total dose of 30 Gray (Gy) was prescribed over 10 daily fractions. As the lymphadenopathy in the lower abdomen was not symptomatic, and would have contributed to increased toxicity, this region was deliberately excluded from the high dose RT volume (Figure ). Other than very mild odynophagia, the patient had no other RT-related side effects. On the first follow-up visit, one month following treatment completion, he had improved swallowing function and a weight gain of six pounds.
Follow-up computed tomography (CT) scan was obtained on May 24, 2016 to evaluate for the suitability of chemotherapy and to serve as a baseline during systemic therapy. This demonstrated persistent thickening of the lower esophagus, with lymphadenopathy reported to have decreased in size and no significant retroperitoneal adenopathy. When given the option of receiving palliative chemotherapy, the patient declined and chose to continue on observation only. Further CT scans in August and October 2016 showed a complete response in the irradiated primary tumor and nodes, with a stable 10 mm lymph node at the right renal vein.
In January 2017, due to symptoms of increasing dysphagia, the patient was assessed by a thoracic surgeon for consideration of esophageal stent placement. Endoscopy on January 12, 2017 noted that there was a possible small amount of residual tumor at the GE junction, but there was no significant narrowing or stricture, and no biopsies were taken. A further CT scan on April 10, 2017 showed minor circumferential thickening of the distal esophagus, but unchanged from previous. Paraesophageal lymphadenopathy was reported to be unchanged. The PET-positive lymph node at the renal vein decreased from 10 mm to 5 mm.
The patient’s symptom of dysphagia resolved spontaneously, and an evaluation was made with a further PET scan on May 19, 2017 (Figure ). This demonstrated mild residual FDG activity within the distal esophagus, more likely inflammatory change rather than malignancy. The FDG activity within all the lymph nodes, both treated and untreated, had unexpectedly resolved.
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pmc-6172140-1
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A 64-year-old male with a history of urothelial bladder cancer, who was a former smoker of a packet of cigarettes a day for 15 years, presented at his private medical doctor’s (PMD) practice with complaints of exertional chest pain. This chest pain was located in the middle of his chest, was pressure-like in nature, and was exacerbated with exertion and relieved with rest. The patient denied any associated diaphoresis or palpitations. An exercise stress test was performed, which showed inferior wall ischemic changes, and the patient was subsequently presented at our hospital for an elective angiogram. Upon admission, he was afebrile with a blood pressure of 147/85 mm/Hg and a regular heart rate of 79 beats/minute. The electrocardiogram (ECG) showed a sinus rhythm with a right bundle branch block and no ST segment or T wave changes indicative of ischemia (Figure ). The lab analyses, such as troponin, lipid profiles, and fasting blood glucose, were within normal limits. The patient was referred to the catheterization lab for a coronary angiogram (CAG) through the right femoral artery. Cannulation of the right coronary ostia revealed the anomalous origin of the left circumflex artery (LCx) and the right coronary artery (RCA) from a shared ostium. The RCA was found to have 80% stenosis in the middle and distal segments with the right posterior descending artery (RPDA) having a 70% stenosis in the proximal segment (Figure ). The LCx showed no significant stenosis. In the pursuit to locate the left coronary ostia, the discovery of the anomalous origin of the left anterior descending artery (LAD) from the right coronary cusp was made. It arose just anterior to the shared ostium of the RCA and LCx. The LAD had no stenosis. The procedure was thus aborted to perform a cardiac multidetector computed tomography (MDCT) to further study the anatomy of the patient in order to decide whether he would benefit from a surgical intervention or a percutaneous coronary intervention (PCI) of his obstructive coronary artery disease (CAD) (Figure ). The MDCT confirmed the findings of the CAG. Considering the favorable anatomy of the LAD with no intramural course, a separate ostium, and no significant stenosis, the patient was referred back to the catheterization lab for a PCI. He received two drug-eluting stents in his RCA and one stent in his RPDA with excellent angiographic results. During the patient’s four-week follow-up appointment, he admitted to having better exercise tolerance and was free of exertional chest pain.
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pmc-6172416-1
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In March 2016, a 47-year-old male patient was admitted to the Oral and Maxillofacial Surgery and Traumatology service of a public hospital, referred by the medical team with the chief complaint of respiratory difficulty after the appearance of a soft palate lesion. On anamnesis, the patient reported rapid weight loss, drug use for more than 10 years, and the development of a hard palate lesion three years earlier, which was left untreated. In addition, the patient reported the development of a soft palate lesion one month earlier. Intraorally, an extensive mass of mucosa-like color was present in the palate region, which obstructed the oropharynx and consequently caused dyspnea.
The sagittal computed tomography (CT) image reveals a soft tissue mass in the nasal cavity extending to the posterior portion of the nasopharynx and the lower part of the sphenoid sinus, with lowering of the entire musculature of the soft palate (A). The coronal CT image shows density loss of the septum in the nasal cavity and nasal conchae, maxillary sinus opacification, and lesion extending to the sphenoid cavity floor (B). The axial CT image reveals the soft tissue mass observed in the nasal cavity with opacification of the maxillary sinuses (C).
After the initial examinations, the patient was submitted to tracheostomy (A) and intraoral incisional biopsy under general anesthesia (B). Intraoperatively, the tumor had a fibrous consistency and showed normal bleeding. The specimen was sent for histopathological analysis (A). Microscopically, the presence of a densely collagenated connective tissue was observed, with numerous blood vessels that were usually of small caliber and sometimes congestive; also, foci of hemosiderosis were visible (B). Based on the microscopic reports, clinical-radiological characteristics and physical examination, the diagnosis of NA was established.
The patient was referred for excision of the lesion. However, due to the extension of the lesion and the patient's systemic conditions, the teams of different specialties decided not to perform surgical resection or radiotherapy, opting for embolization. However, as a technology procedure was not available at the time, the patient remained hospitalized awaiting treatment availability. Approximately one month after the incisional biopsy, bilateral swelling in the submandibular region and parapharyngeal regions was noted (A and B). In addition, the patient presented severe headache, difficulty in closing the eyes, dysphagia and dyspnea, trismus, left facial paralysis and episodes of fainting. The patient was under palliative care and died after a few weeks.
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pmc-6172567-1
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A 41-year-old female presented with a 7 months history of a palpable breast lump in right upper outer quadrant. No abnormalities were discovered on physical examination. Results of laboratory tests were all within the reference range. Sonography and mammographic findings showed no evidence of abnormality in either breast or axillae. The Fine Needle Aspiration (FNA) was atypical cytology, and showed scattered or rarely clustered, uniformly round cells with small nuclei and a moderate amount of cytoplasm, suggesting a benign or low grade malignant tumor. Core needle biopsy (CNB) of the mass was diagnosed as Infiltrating Ductal carcinoma-NOS. Modified radical mastectomy was done and sent for histopathological examination.
On gross examination, the tumor was a 2.5 × 2.0 × 1.0 cm, gray white with a rubbery consistency, with a well-defined border and a slightly lobulated appearance (). Microscopically, the tumor showed a diffuse infiltrative growth patterns with small acinar or glandular structures mixed with solid nests (, ). Most of the tumor was comprised of monotonous round cells with a finely granular, weakly eosinophilic, or clear vacuolated cytoplasm resembling acinar cells of the salivary glands. Some neoplastic cells had a clear cytoplasm. The nuclear grade of the tumor cells was determined to be grade 2. Foci of vascular invasion were also present. Lymphatic permeation was occasionally seen, but the sentinel lymph node was free of metastasis. The mitotic count was up to 0–3/high power field. The Final surgical margins were negative. Cells with eosinophilic granules and globules were strongly periodic acid–Schiff (PAS) (diastase resistant) positive.
Immunohistochemically, tumor cell populations were strongly positive for lysozyme (A and D) and were negative for estrogen receptor, progesterone receptor and human epidermal growth factor receptor-2 (HER2/neu). Because the patient was diagnosed as having invasive breast cancer with a triple-negative phenotype, postoperative radiotherapy (50Gy-/25 fractions) followed by adjuvant chemotherapy (TC: docetaxel 75mg/m2 and cyclophosphamide 600 mg/m2 administered intravenously every 3 weeks for 4 cycles) was administered. Although the followup period to date has been short (1 year), there have thus far been no signs of recurrence.
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pmc-6172666-1
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A 2-month-old male infant was admitted under the paediatric team with a 1-day history of non-bilious vomiting, pyrexia, and irritability a day after receiving his first-dose rotavirus vaccination. On examination he was haemodynamically stable and had no focal signs of sepsis. His abdominal examination revealed a soft, non-distended abdomen with no palpable masses. He had passed normal stool within the preceding 24 hours. Following initial assessment, he underwent a full septic screen including lumbar puncture, the results of which were all within normal range.
After developing bilious vomiting overnight a paediatric surgical review was obtained and an upper gastrointestinal contrast study was performed. This revealed no evidence of malrotation. An abdominal X-ray was subsequently performed which revealed a soft tissue mass in the right hypochondrium, dilated proximal small bowel loops, and a paucity of distal bowel gas, in keeping with small bowel obstruction (). An urgent ultrasound scan was obtained which showed dilated proximal small bowel loops and the characteristic target sign typically seen in intussusception (). The child received full resuscitation before an air enema reduction was performed under fluoroscopic guidance. This was successful at first attempt.
The following day the patient was well, tolerating feeds, and passing normal stools. He was subsequently discharged home. Following discussion with Public Health England the child’s parents were advised to decline the second-dose rotavirus vaccination.
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pmc-6172667-1
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A 5-year-old girl presented to her GP with a history of an erythematous rash that appeared on her left cheek associated with eating certain foods including strawberries, apples, and sweets. The rash would appear immediately on mastication and would entirely disappear within 30 minutes of ingestion.
Her medical history was unremarkable apart from a road traffic accident at 3 years of age when she suffered facial and chest trauma leading to a mandibular fracture and right lower lobe collapse. The patient required intubation and ventilation for 9 days on the paediatric intensive care unit and underwent maxillofacial surgery for the mandibular fracture.
Physical examination revealed a well-grown child with no systemic abnormalities or eczema. Within a few seconds of eating candy a facial flushing appeared on her left cheek, stretching from her the temporal region to the corner of her mouth. This faded within a few minutes as demonstrated in. There was no associated lip or tongue swelling or difficulty in breathing.
The patient was referred for skin prick testing which was performed on an extended panel and was negative. She was reviewed by a consultant paediatrician who made a diagnosis of Frey’s syndrome and counselled the family with regard to the non-allergic pathogenesis of this condition.
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pmc-6172805-1
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A 51-year-old man presented to the emergency department with fever, abdominal pain and jaundice. Past and recent medical history were unremarkable and the patient did not report any recent trip, risky sexual behaviour, parenteral drug intake or ingestion of potentially contaminated food. The physical examination revealed right hypochondrium and epigastric tenderness and no signs of peritonitis. Lab tests showed Aspartate AminoTransferase (AST) 3560 UI/L, Alanine AminoTransferase (ALT) 4513 UI/L, hyperbilirubinemia (total 16 mg/dL), alkaline phosphatase, GGT 90/418 UI/L, PT 50%, normal pancreatic enzymes and normal renal function. Abdominal ultrasound showed no alterations.
Twenty-four hours after the admission, liver function rapidly declined, with PT 40% and maximum total bilirubin of 47 mg/dL. In parallel, blood test showed an elevation of LDH, haptoglobin consumption, reticulocytosis and AKI stage 3 with creatinine of 4 mg/dL and a peripheral blood smear suggestive of hemolysis. Urinalysis was positive for bilirubin and hemoglobin, while the urinary sediment discarded the presence of red blood cells. Serological tests were positive for IgM Hepatitis A Virus (HAV). In addition, a previously unknown complete glucose-6-phosphate dehydrogenase deficiency was detected.
On the basis of these findings a diagnosis of acute hepatitis A infection complicated with massive hemolysis due to glucose-6-phosfate dehydrogenase deficiency was done. Hemolysis was probably triggered by fitomenadione administration and its diagnosis was partially masked by high bilirubin levels due to the severe hepatitis. AKI was interpreted as the result of pigmented-cast nephropathy. The haemolytic crisis was initially managed with 2 sessions of plasma exchange. However, considering the need of dialysis and the presence of CPH, continuous renal replacement treatment with a HCO filter (Septex™, 1.1m2, Gambro-Baxter, Hechingen, Germany; Fig. ) in CVVHD modality was started. In order to measure the CPH clearance, plasma was collected in EDTA tubes from arterial, venous and dialysate ports of the CRRT circuit and CPH was measured with Drabkin-based spectrophotometric analysis at 540 nm. The Sieving coefficient (SC) was calculated as CD/[CIn + COut)/2] where CD, CIn and COut represent CPH concentration at the dialysate, blood inlet and blood outlet side. CPH clearance was calculated as {CD/[CIn + COut)/(2)]}Qe, where Qe is the effluent flow. This filter was used for 48 h, observing a CPH value of 4,24 g/L at the beginning and of 3,72 g/L at the end of the treatment. The calculated SC for CPH was 0.08 at treatment start, later decreasing to 0.02 after 24 h. The calculated clearance of CPH declined as well, from 2,87 ml/min on the first day to 0,76 ml/min after 24 h (Table for treatment data and CPH clearance profile, Table for common laboratory data before and after CVVHD treatment).
After this treatment, as the patient was still oliguric, seven sessions more of intermittent hemodialysis were performed. Eventually, 4 weeks after admission urine output ensued and the patient’s renal function started to recover. At the time of discharge, total bilirubin was 3.4 mg/dL and creatinine 2.8 mg/dL. Finally, 3 months after discharge the patient presented normal renal and hepatic function.
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pmc-6172811-1
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We present the case of a 21-year-old Greek woman who presented to the Emergencies Department of our hospital with breast pain, abdominal distension, and weakness of approximately 1 week’s duration. Her individual, gynecological, and family history were unremarkable.
She had a high breathing rate (~ 22 breaths/minute), tachycardia (~ 110 beats/minute), hypotension with mean arterial pressure (MAP) of 55 mmHg, lethargy, swollen and painful breasts, abdominal dilatation with diffuse sensitivity to palpation and dullness on percussion, and low grade fever (~ 37.5 °C).
She was directly subjected to ultrasound (U/S) of her upper and lower abdomen that showed enlarged ovaries as well as a large amount of free ascitic fluid. Complete laboratory testing and blood gases were obtained and an urgent computed tomography (CT) scan of her upper and lower abdomen was performed, confirming the findings of the U/S: enlarged and inflammatory ovaries, pleural effusions, and large amount of free ascitic fluid (Fig. ). Laboratory tests showed neutrophilic leukocytosis with white blood cells (WBC) 30,000/μL, polymorphonucleocytes (PMN) 95%, and thrombocytopenia with platelets (PLT) 90,000/μL with signs of disseminated intravascular coagulation (DIC), increased urea (U) and creatinine (Cr) levels, increased bilirubin (Bil), increased serum glutamic oxaloacetic transaminase (SGOT) and serum glutamic pyruvic transaminase (SGPT), and severe lactic and metabolic acidosis. Control for viral and human immunodeficincy virus (HIV) infection was negative. With these data and due to further deterioration of our patient’s clinical condition, it was decided to conduct a research laparotomy.
Intraoperatively there were found enlarged and inflamed ovaries, a large amount of ascitic fluid, and an inflamed appendix, which was removed and sent for biopsy. The peritoneum was clear and free of any visible implants. A biopsy was also obtained from both her ovaries and cytology and ascitic fluid cultures were sent, a suprapubic fluid drainage tube was placed and due to the reproductive age of our patient it was decided not to remove her ovaries. After surgery, she was transported to the Intensive Care Unit (ICU), intubated, and mechanically ventilated; she was hemodynamically unstable, presenting hypoxemia with partial pressure of oxygen in arterial blood/fraction of inspired oxygen (PaO2/FiO2) of 150 and severe metabolic and lactic acidosis.
The differential diagnosis included ovarian hyperstimulation syndrome (OHSS) and other ovarian tumors. OHSS is a systemic disorder attributed to the release of vasoactive agents released from the ovaries after overstimulation []. The pathophysiology of OHSS is characterized by increased capillary permeability leading to large fluid extravasation, accumulation in the third space, and intravascular dehydration []. The serious manifestations of the syndrome include thrombosis, renal and hepatic insufficiency, and acute respiratory distress syndrome (ARDS), which cause severe morbidity []. Mortality from the syndrome is fortunately rare, with only sporadic references in the literature []. Women should be aware that mild forms of OHSS are common and complicate 33% of in vitro fertilization (IVF) cycles, while moderate and severe forms occur in 3–8% of cases of OHSS []. The majority of serious OHSS cases occur after IVF cycles, but the syndrome may also occur after any form of ovulation induction, such as clomiphene and gonadotropins []. The incidence of the syndrome, particularly the complex form, is higher in young women, women with polycystic ovaries, and in gestational cycles. Laboratory tests may show high hematocrit levels (> 55%), hypoproteinemia, and leukocytosis []. The treatment of OHSS is initially supportive until the situation resolves [].
She was initially treated as severe septic shock; blood cultures were obtained and broad-spectrum antibiotic treatment was administered. Due to acute renal failure, she was placed in continuous venous-venous hemofiltration (CVVHDF).
While she was in our ICU she showed progressive clinical, gasometric, and hemodynamic improvement, draining ~ 2000 ml of ascitic fluid/day; on the third day of admission an attempt was made to wean her from the ventilator, pending the results of the cultures and ovarian and appendix biopsies. She was febrile (~ 38.4 °C), hemodynamically stable with normal hourly diuresis, and improved laboratory testing, therefore CVVHDF was removed. Severe leukopenia (WBC 2000/μL) was evident, for which she received subcutaneous granulocyte growth factor. On the fourth day of admission, the results of blood and ascites fluid cultures were negative and biopsy results showed high-grade Burkitt lymphoma of the ovaries and the appendix. With these data our patient was transported to a specialized oncology center for immediate onset of chemotherapy and further treatment.
She was gradually weaned from mechanical ventilation and was successfully extubated on the 12th day of her hospitalization. On the sixth day she received a combined chemotherapy regimen intravenously. On the 15th day she left the ICU and on the 28th day she was discharged from hospital, presenting improved clinical and laboratory condition, waiting for further cycles of chemotherapy.
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pmc-6172832-1
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A round tumor with a diameter of approximately 10 mm that involved the posterior end of the nasal septal mucosa at the midline of the epipharynx was discovered in a 58-year-old man while screening for laryngeal cancer (Fig. ). The patient was referred to our department for further evaluation, whereupon imaging analyses and a regional biopsy were performed under local anesthesia using a biopsy fiberscope. Pathological findings resembled an inverted ductal papilloma of the salivary glands, but did not produce a definitive diagnosis. The patient had a history of renal cancer for which he had undergone surgery 5 years prior.
Plain computed tomography (CT) revealed a soft tissue shadow tumor approximately 10 mm in size in the vault of the nasopharynx at the junction of the nasal septum and roof (Fig. ). Magnetic resonance imaging (MRI) showed a 10 mm mass at the same location as that observed on CT. T1- and T2-weighted images showed the same intensities as that of the nasal concha, and a regular contrast effect was observed (Fig. ). These MRIs suggested a benign tumor. Furthermore, positron emission tomography did not show any abnormal uptake of 18F-fludeoxyglucose in the nasopharynx, thyroid gland, or elsewhere in the body.
To excise the tumor and obtain a definitive pathological diagnosis, surgery was performed under general anesthesia using an endoscopic endonasal approach. First, an electrocautery needle was used for electrocoagulation and excision. Next, a suction curette was used for exfoliation of the tumor. Finally, the suction probe of the electrocautery device was used for electrocoagulation to stanch the bleeding.
Hematoxylin-eosin staining showed that the tumor had a papillary structure lined by a columnar epithelium with a hyalinized fibrous core, and was additionally composed of sheets of spindle cells (Fig. ); these two types of structures merged imperceptibly. A negative tumor margin was confirmed after surgery, and immunohistochemical studies showed that both columnar and spindle cells were diffusely positive for CK7 (Fig. ), TTF-1 (Fig. ), CK19, and vimentin (data not shown); however, they were negative for CK20, p63, smooth muscle actin (SMA), S-100, Epstein-Barr-encoded RNA (EBER), p16, human papillomavirus (HPV), and thyroglobulin (data not shown). The Ki-67 index was approximately 2–3%. P53 was irregularly positive in a small number of cells, suggesting wild-type status.
We diagnosed the tumor as a biphasic LGNPPA with a prominent spindle cell component []. No postoperative adjuvant treatment was administered. The patient is well and free of disease 34 months after the surgery.
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pmc-6172840-1
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A 76-year-old Japanese woman presented with neutrophilia, mild renal dysfunction, and proteinuria and was referred to our Department in year X (Table ). Her neutrophilia was first discovered during a medical check-up when she was 74 years old (year X-2), and thereafter, her neutrophil count progressively increased. Her serum G-CSF was 161 pg/mL (Table ), which was far beyond the normal range (< 39.0 pg/mL).
Her 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT) scan showed intense uptake in the bone marrow but did not show any evidence of a malignant solid tumor or an occult abscess related to bacterial infection. Fluorescence in situ hybridization was performed on peripheral blood smears, and there was no clonality in the patient’s neutrophils. Her urine had tested positive for urinary occult blood since she was 50 years old, and she developed proteinuria after her neutrophil count increased above 20.0 × 103/μL in year X-1.
Her blood pressure was 118/59 mmHg; other vital signs were also normal. Physical examinations were unremarkable. When she was admitted in year X, the results of laboratory tests were as follows: white blood cell (WBC) count, 35.9 × 103/μL (neutrophil count, 31.3 × 103/μL; 87.2% of the WBC count); hemoglobin level, 11.5 g/dL; platelet count, 27.3 × 104/μL; serum creatinine, 0.85 mg/dL; estimated glomerular filtration rate, 49.4 mL/min/1.73 m2; blood urea nitrogen, 16 mg/dL; lactate dehydrogenase, 170 U/L; total protein/albumin, 8.0/3.6 g/dL; and immunoglobulin G (IgG), 2901 mg/dL. Serum immunoelectrophoresis revealed a monoclonal IgG λ peak; however, the levels of other immunoglobulins were normal, and bone marrow aspiration showed less than 10% clonal plasma cells, thereby yielding the diagnosis of monoclonal gammopathy of undetermined significance (MGUS). Tumor markers including carcinoembryonic antigen, squamous cell carcinoma antigen, and cytokeratin fragment were all within normal range. Both the levels of C-reactive protein and complements were normal, and both antinuclear antibody and rheumatoid factor were negative. Tests for hepatitis B virus and hepatitis C virus were negative. Her urinary protein excretion was 2.55 g per gram urinary creatinine, and the proteinuria selectivity index was 0.17. We found hematuria (20–29 red blood cells/high-power field) and granular casts in her urinary sediment.
A renal biopsy was performed, and sections evaluated by light microscopy showed many lobulated glomeruli. Mesangial proliferation and focal double contouring of the glomerular capillary walls were also present (Fig. ). Additionally, endocapillary proliferation was observed in the glomeruli (Figs. ). Immunoperoxidase staining for neutrophil elastase revealed some neutrophils infiltrating the glomeruli (Fig. ). Double immunofluorescence staining for neutrophils and macrophages showed that the infiltration of macrophages was mainly around the glomeruli, and the glomeruli contained more neutrophils than macrophages (data not shown). We identified intratubular cellular casts and interstitial expansion in the tubulointerstitial area (Fig. ). Immunoperoxidase staining for G-CSF did not show a specific staining pattern (data not shown). Immunofluorescent staining showed no deposition of immunoglobulins or complements (data not shown). Expansion of the subendothelial space suggesting injury of glomerular endothelial cells and effacement of the podocyte foot processes were seen in sections evaluated by electron microscopy (Fig. ). There were no electron dense deposits. Based on these biopsy findings, we diagnosed the patient with MPGN-like glomerulopathy.
The patient was conservatively treated with an angiotensin II type 1 receptor blocker, but her proteinuria did not resolve. On the contrary, her proteinuria fluctuated in accord with the changes in her blood neutrophil count. Finally, the neutrophil count decreased without any medication, and the proteinuria diminished considerably (Table ).
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pmc-6172932-1
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An 84-year-old male with multiple comorbidities including prior ischemic stroke without any residual deficits was admitted to the hospital for worsening lethargy and weakness due to progressive dysphagia to solids and liquids and weight loss of 15 pounds in the past 6 to 8 months. According to the family, he had a long-standing history of coughing and choking while eating and complained about food getting stuck in his chest. His vitals were stable in the emergency department and was breathing on ambient air. Esophagogram was ordered for the workup of his chronic dysphagia. During esophagogram, he started coughing and choking after which the study was terminated. After 5 hours of study, he started becoming hypoxic with oxygen saturation of 86% on room air requiring a non-rebreather mask, fever of 101°F, tachycardia 112/min, and hypotension to 90s mm Hg systolic blood pressure. Aggressive intravenous (IV) resuscitation was done, IV antibiotics including vancomycin and piperacillin-tazobactam were started, and he was upgraded to intensive care unit for a higher level of care.
Esophagogram showed early laryngeal penetration of contrast and subsequent presence of contrast in the trachea and bronchial tree with minimal contrast in the esophagus ().
Chest X-ray showed contrast highlighting the tracheobronchial tree and bilateral upper lungs (). He was managed conservatively with IV fluids and IV antibiotics. He started to improve within 6 hours of aspiration event with stabilization of vital signs including resolution of hypotension and tachycardia. Oxygen requirement also improved to 2 L through nasal cannula within 24 hours. Repeat chest X-ray showed the advancement of contrast into bilateral bronchioles and alveoli with left-sided predominance (). His respiratory status remained stable; however, later on, due to his other comorbidities, family opted for hospice care.
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pmc-6173321-1
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A 71 year-old-male with past medical history of hypertension, hyperlipidemia, diabetes, chronic obstructive pulmonary disease and marijuana abuse presented with intermittent retrosternal pleuritic chest pain for two weeks, sharp in nature, 7/10 in intensity, non-radiating, and aggravated by sitting up from a lying position, deep breathing, and exertion. The patient reported intermittent nonproductive cough, fatigue, decreased appetite and a 10-pound weight loss in the past eight months. Chest radiography and physical exam findings were all noted as normal one year prior to presentation. He had a 40 pack year’s history of smoking; he quit 25 years ago.
On examination, he was afebrile, pulse rate 88 beats per minute, blood pressure of 133/70 mmHg, with an oxygen saturation of 98% on room air. An electrocardiogram revealed diffuse ST-segment elevation. A chest radiograph revealed cardiomegaly, congestion, and a pleural-based opacity in the left upper lung field. Transthoracic echocardiography showed a left ventricular ejection fraction of 30%, grade 1 diastolic dysfunction, inferior vena cava dilation with blunting of respirophasic changes (less than 50% variation), and a large circumferential pericardial effusion with “swinging” of the heart, suggestive of cardiac tamponade physiology. Laboratory investigations revealed a hemoglobin of 9.3 g/dL, glycosylated hemoglobin at 9.4 g%, troponin I obtained 8 hours apart were in normal range (0.014 ng/mL and 0.024 ng/mL). Computed tomography of the chest with contrast revealed a 4.5 cm left upper lobe mass, left hilar and mediastinal lymphadenopathy, and a large pericardial effusion. A pericardial window was placed and 750 ml of serosanguinous pericardial fluid was drained. Pericardial fluid cytology was negative for malignant cells and bacteria. Elevated lactate dehydrogenase (7157 units per liter) and glucose level (266 milligrams per deciliter), reactive mesothelial cells and numerous white blood cells were noted. A computed tomography-guided biopsy of the left lung mass was performed; hematoxylin and eosin stains showed poorly differentiated malignant neoplasm. Immunohistochemical analysis revealed malignant pleural mesothelioma. The pericardial biopsy did not show any mesothelioma cells or fibrous plaques. The patient has been lost to follow-up since the diagnosis.
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pmc-6173349-1
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We report a case of a 70 year-old male, with a history of dyslipidaemia and
smoking habits, who suffered an inferior ST elevation myocardial infarction
(STEMI). Given the impossibility to achieve a timely percutaneous coronary
artery intervention, thrombolysis was performed within 4 hours of symptoms
onset. Advanced atrioventricular block requiring a transcutaneous pacemaker
occurred soon after, followed by cardiorespiratory arrest in ventricular
fibrillation, which was reversed after one cycle of advanced life support. The
patient was transported by airplane to a percutaneous coronary intervention
(PCI)-capable centre. Coronary angiography showed a 50-60% stenosis in the
proximal segment of the right coronary artery, which was treated with a bare
metal stent. Echocardiography showed a moderate left ventricular systolic
dysfunction (estimated ejection fraction of 35%), with inferior, inferolateral
and inferoseptal akinesia and moderate mitral regurgitation. On the fifth day,
the patient was transferred to our centre, after a 10-hour flight. On admission
to intensive care unit, the patient was in cardiogenic shock with inotropes and
non-invasive ventilation. A bedside transthoracic echocardiography revealed a
severe mitral valve regurgitation of uncertain mechanism, along with moderate
left ventricle systolic dysfunction and right ventricle systolic compromise.
Additional characterisation by transoesophageal echocardiography revealed a 9 mm
disruption of the posteromedial papillary muscle consistent with a contained,
albeit morphologically imminent, rupture. The instability of the sub-valvular
apparatus, leading to a broad posterior leaflet prolapse, caused severe mitral
regurgitation with an eccentric jet with Coanda effect, reaching the left atria
roof (). The patient underwent
urgent mitral valve replacement with a biological prosthetic valve (St. Jude
#29), with preservation of anterior and posterior leaflets. The patient
experienced a favourable post-operative recovery and was discharged 12 days
after surgery with anticoagulant therapy for three months, in addition to dual
antiplatelet therapy. On the fourth month after surgery, the patient initiated
progressive heart failure symptoms (NYHA class III) without any further
complaints. Additional transthoracic and transoesophageal evaluations were
performed, revealing a significant restriction of the prosthetic mitral valve
leaflets mobility due to thrombotic material deposition, leading to severe
obstruction, with a mean gradient of 19 mmHg and an effective orifice area
estimated by PISA method of 0.4 cm. Additionally, in continuity with the prosthesis, a large
mural thrombus was present covering the left atrial posteroseptal wall (). Urgent surgery, within twenty-four
hours after diagnosis, was performed involving mitral bioprosthesis replacement
with another biologic prosthesis with significant improvement in clinical
status. After an extensive study, no evidence was found of atrial fibrillation
or thrombotic disorders. Pathology examination of the excised prosthetic
material confirmed prosthetic thrombosis, with no signs of endocarditis.
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pmc-6173734-1
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The patient is a 3-year-old girl who was diagnosed with a brain tumour at 5 months of age. She presented initially with vomiting and seizures and an MRI showed a heterogeneous mass measuring 6 × 3 × 2 cm in the right lateral ventricle. Following gross total resection pathology showed predominance of large epithelioid and spindle-shaped cells with mild pleomorphism, mitotic index of 14 per 10 high power fields and a Ki67 proliferative index of 40%. The tumour showed patchy positivity for GFAP, strong nuclear staining for p53, and was negative for synaptophysin, chromogranin, NeuN, BRAF V600E, H3K27M and ATRX. She was diagnosed with a HGG and was treated with an infant brain tumour protocol with 13 cycles of chemotherapy.
Four months after completing treatment, she had disease progression in the tumour bed with multiple nodules in the lateral and third ventricles. Further tumour debulking confirmed recurrent HGG. After 6 months, a new mass in the tumour bed was subtotally resected and she received focal radiotherapy of 54 Gy to the tumour bed. The resected tumour was profiled on a pilot personalised medicine study. Three months following completion of radiation therapy, she represented with difficulty walking, drowsiness, vomiting and irritability. MRI showed widespread progressive disease with increased enhancement at the resection site, and enlarging suprasellar and subependymal nodules in the lateral and third ventricles. Dexamethasone was continued at 1.5 mg daily. The parents were told that she was incurable, and she was referred to palliative care for symptom management.
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pmc-6173833-1
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A 43-year-old Sudanese male was admitted to Acibadem University Hospital in Istanbul, Turkey with hyperpigmented painful skin rashes on his whole body. He was experiencing these symptoms intermittently for a year and self-medicated himself with non-steroid anti-inflammatory drugs with no fever or other health problems. He had recently experienced joint pains. A complete blood count during admission showed normal erythrocyte counts (5.1 × 106/µL) and Hb levels (13.9 g/dL) with a high white blood cell levels (23.710/µL, of which 85% were lymphocytes) and low neutrophil (10.500/µL) and platelet (128.000/µL) levels. Investigation of a peripheral blood smear revealed 29% large granular lymphocytes (LGLs). Flow cytometric analysis of peripheral blood confirmed that 95% of lymphocytes (CD3+/TCRαβ+ population) were positive for pan-T antigens (CD2, CD5, and CD7) and CD8, but negative for CD4 and CD56. Ultrasonography and FDG-PET-CT evaluation of the abdominal area found hepatomegaly, splenomegaly, and hypermetabolic supra-infradiaphragmatic lymph nodes as well as a hypermetabolic spleen. He had a history of malaria, but HCV and HIV tests were negative. These results were compatible with CD8+ T cell lymphoproliferative disorder with skin involvement. Therefore, a 0.5-cm-deep skin punch biopsy was performed in an inner part of the leg showing lesions.
LGL leukaemia is a rare lymphoproliferative disease and presents with anaemia, neutropenia, and an increase in the number of LGLs []. About 85% of LGL leukaemias are derived from a T cell lineage (T-LGL leukaemic cells express CD3, CD8, CD16, and CD57), while the rest are derived from the natural killer (NK) cell lineage (NK-LGL leukaemic cells express CD2, CD16, CD56, and CD57) [, ]. Furthermore, CD8+ T cell lymphoproliferative disorder is a very rare form of T-LGL with poorly defined clinical, aetiological, immunophenotypic, molecular and pathological features []. Although T-LGL is an indolent disease, it may chronically affect the immune system and cause recurrent infections, symptomatic anaemia, and autoimmune conditions such as rheumatoid arthritis. Prednisone, methotrexate, and cyclosporine have been used for T-LGL treatment. Therefore, the patient with this pre-diagnosis was prescribed methotrexate (20 mg/week) and Prednol® (80 mg) for 6 weeks, and further immunopathological parameters were evaluated in skin lesions.
Microscopic evaluation of skin sections by haematoxylin–eosin (HE) staining showed that the epidermis was minimally spongy and the upper dermis was oedematous with mild perivascular lymphocyte infiltration. However, the deep dermis was infiltrated by intra- and peri-vascular small lymphocytes (Fig. a). Standard analysis of paraffin-embedded sections by Benchmark-XT (Ventana Medical Systems) with its inner controls showed that 99% of the total lymphoid population was CD3+, CD2+, and CD5+, among which 90–95% were positive for CD8 (Fig. b, red arrow shows a histiocyte with no CD8 staining), while only 5–10% were positive for CD4. Notably, Granzyme B showed a similar staining pattern as CD8 (data not shown). In contrast, CD20 (B cells) and CD56 (NK cells) were negative. At the bottom of the tissue, a few giant multinuclear histiocytic cells were noticed (Fig. a, red arrow). Notably, these cells contained small intra-cytoplasmic microorganism-like structures that were not discernibly stained with any dye specific for fungi or bacteria (PAS, Alcian Blue, Grocott’s methenamine silver stain, and Ziehl–Neelsen stain) (Fig. a, white arrows). Overall, the patient was finally diagnosed with CD8+ T cell lymphoproliferative disorder involving both the periphery and skin.
After this final diagnosis, methotrexate and Prednol were administered for 6 weeks. Six weeks later, blood values had normalized, skin and arthritis symptoms subsided, and the patient was discharged. No other follow-up could be performed because the patient returned to his country. However, because the patient was from Sudan, a malaria endemic region, and the unusual presence of small intra-cytoplasmic microorganism-like structures in histiocytic cells, the pathologist, who had no experience with malaria cases, consulted a malaria specialist at Osaka University, Japan.
Careful evaluation of skin biopsy samples by polarized microscopy revealed birefringent crystalloid structures resembling malarial haemozoin (Fig. a, arrows show representative shiny crystalloid structures) []. Haemozoin is a by-product of haemoglobin metabolism in Plasmodium parasites and readily captured by macrophages and the reticuloendothelial system of the host, which can be easily recognized as birefringent crystals under polarized light [, ]. Haemozoin-like structures were mainly loaded in macrophages and giant histiocytes. To further investigate the possibility of asymptomatic submicroscopic chronic malaria infection, we performed nested PCR to detect Plasmodium parasites []. After purification of DNA from the skin biopsy samples by a tissue DNA extraction kit (NucleoSpin, Macherey–Nagel), P. falciparum DNA was amplified (Fig. b).
The co-presence of EBV infection with malaria is a well-known aetiology of lymphoma. Hence, EBV-early RNA (EBER) transcripts were investigated in paraffin-embedded tissue samples and found to be positive in macrophage-like histiocytes (Fig. ).
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pmc-6173835-1
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A 60-year-old man was referred to our hospital for kidney transplantation. His wife, a 59-year-old woman, volunteered to donate her kidney to him when he started hemodialysis at age 59. The proposed transplant was ABO incompatible, from a donor with blood-type A to a recipient with blood-type O, and the recipient’s anti-A blood-type IgG antibody titer was measured at 4096-fold dilution.
Preoperative testing included HLA-DNA typing, which revealed a mismatch in 6 antigens. Initial flow cytometric crossmatch testing (FCXM) was negative. Moreover, the flow cytometric panel reactive antibody (Flow PRA) screening test was negative for human leukocyte antigen (HLA) class I and class II. Single antigen testing was also negative.
Three months prior to surgery, mycophenolate mofetil (MMF) 750 mg/day was initiated and the anti-CD20 monoclonal antibody Rituximab (200 mg) was administered according to our pre-transplantation regimen (Fig. ). Following 3 months of desensitization therapy, the patient underwent two sessions of double filtration plasmapheresis (DFPP).
Anti-blood type antibody titers (IgG/IgM) were then assayed using the column agglutination technology (gel microcolumn) method (Bio-Rad®, Japan). Our target antibody titer level was < 128-fold dilution; however, the anti-A blood-type IgG antibody titer decreased to only 512-fold dilution (Table ). In addition, serum IgG before the induction of DFPP was 1428 mg/dl. The high titer state following plasmapheresis was considered “refractory rebound”, and the planned transplant was postponed in order to resume desensitization therapy (MMF 750 mg/day). Four months following the initial start of desensitization therapy (MMF), the anti-A blood-type antibody level rebounded to 1024-fold dilution.
Shortly after the re-initiation of desensitization therapy (150 days from the initial start of therapy), the patient developed herpes zoster infection. He was treated with anti-viral medication and the MMF dose was reduced from 750 mg/day to 500 mg/day. After 1 month, the MMF dose was increased back to 750 mg/day.
Transplantation was rescheduled to occur 210 days from the initiation of MMF. The pre-transplantation regimen was as follows. Rituximab was administered at 200 mg and 100 mg at 21 days and 1 day before transplant, respectively. Twelve days prior to surgery, the dose of MMF was increased to 1000 mg/day (At 11 days prior to surgery, serum MMF Area Under the Curve (AUC)0–12 was 35.6 ng/ml.). The initial dose of extended-release tacrolimus (TacER) (0.15 mg/kg/day) was administered 13 days prior to transplantation and the dose was adjusted based on serum concentration. Because the initial DFPP sessions did not decrease the anti-A blood-type antibody titers, 4 sessions of selective plasma exchange (PE) were used to remove the anti-blood type antibody. With these interventions, the anti-A blood-type IgG antibody decreased to 128-fold dilution and the serum IgG level decreased to 357 mg/dl on the day of transplant (Table ).
The renal graft was transplanted into the right iliac fossa without incident. Subsequently, the graft became pink and urine was produced immediately. The post-transplant induction immunosuppression protocol consisted of TacER, MMF 2000 mg/day, basiliximab 20 mg administered on postoperative day (POD) 0 and 4, and systemic steroids starting on POD 0. A graft biopsy performed 1 h after reperfusion demonstrated no evidence of hyperacute rejection (Fig. ). The serum creatinine (s-Cr) level began to decrease immediately. On POD 6, the s-Cr level was 1.5 mg/dl, and anti-A blood-type IgG and IgM antibodies were measured at 16-fold and 4-fold dilutions, respectively. The antibody titer levels remained at these levels throughout the post-operative course. However, serum IgG increased to 957 mg/dl. On POD 12, cytomegalovirus (CMV) antigenemia was diagnosed. The antiviral medication baragancyclovir was initiated and the dose of MMF was decreased to 1000 mg/day. No further post-operative complications were observed.
The sCr fluctuated between 1.29 and 1.42 mg/dl during the 1.5 months after ABO-iLKT. A protocol biopsy was performed on POD 50. The histopathological examination revealed i) acute T cell-mediated rejection IB, and ii) no evidence of acute antibody-mediated rejection (Fig. ). Steroid pulse therapy (500 mg for 3 consecutive days) was administered.
At the time of 18 months after ABO-iLKT, the sCr level was between 1.30 and 1.49 mg/dl (estimated glomerular filtration rate (eGFR)-calculation of 38.0–40.8 ml/min/1.73m2). Urinalysis showed urine albumin of 30–80 mg/L, urine red blood cells of 0–1 /high power field, urine white blood cells 0–1 /high power field and no granular casts. His blood pressure was maintained as 132/80–138/88 mmHg on Amlodipine 5 mg once daily. Anti-A blood-type IgG and IgM antibody titers were stable at 16-fold and 8-fold dilutions, respectively. Serum IgG was preserved within the normal range. The patient was maintained on triple immunosuppression therapy consisting of TacER, MMF 1000 mg/day, and steroid 5 mg/day.
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pmc-6173840-1
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A 72-year-old Japanese woman with a 10-year history of T2DM had symptoms of diarrhea and persistent pain in left lower abdomen for 2 days and visited the emergency room in Kawasaki Medical School. She had an approximately 10-year history of hypertension and dyslipidemia. At that time, she was taking 4 mg/day of benidipine hydrochloride and 20 mg/day of azilsartan for the treatment of hypertension, and 25 mg/day of alogliptin and 500 mg/day of metformin for T2DM, and 2.5 mg/day of rosuvastatin for dyslipidemia. She had no remarkable family history. She was a housewife and she did not smoke tobacco or drink alcohol. She had no past history of digestive disease or obstetrics and gynecology disease. She had mild tenderness to palpation in her abdomen. Her height and body weight were 150.0 cm and 69.5 kg, respectively. Her vital signs were as follows: blood pressure 150/87 mmHg, heart rate 110 beats/minute, and temperature 36.4 °C. Inflammation markers were markedly elevated: white blood cell (WBC), 20,110/μL (neutrophil, 89.0%); C-reactive protein (CRP), 16.12 mg/dL. Anemia and mild hypoalbuminemia were observed although their causes remained unknown: red blood cell, 304 × 104/μL; hemoglobin (Hb), 9.3 g/dL; total protein (TP), 6.8 g/dL; and albumin (Alb), 3.2 g/dL. Her liver and renal function were within normal range as follows: aspartate aminotransferase (AST), 14 U/L; alanine aminotransferase (ALT), 9 U/L; gamma-glutamyl transpeptidase (γ-GTP), 8 U/L; lactate dehydrogenase (LDH), 202 U/L; creatinine (Cre), 0.81 mg/dL; blood urea nitrogen (BUN), 7 mg/dL; Na, 134 mEq/L; K, 3.8 mEq/L; Cl, 99 mEq/L; Cre clearance, 66.9 mL/minute; and urinary Alb, 15.1 mg/g·Cr. As shown in Fig. , her abdominal computed tomography (CT) on admission revealed a large tumor with calcification in left side of intrapelvis (upper middle panel) which was not observed in abdominal CT 1 year before (upper left panel). She had abdominal CT 1 year before by a urologist because bladder diverticulum was suspected at an annual medical checkup with abdominal ultrasonography. The tumor size was as large as 65 mm in diameter. In addition, as shown in Fig. , MRI showed a large tumor in left side of intrapelvis at the same lesion site observed in CT. An axial T1-weighted (T1W) image through the pelvis showed a markedly dilated fallopian tube posterior to the left ovary (upper left panel). Axial T2-weighted (T2W) image showed a slightly higher intensity (upper right panel). Axial diffusion-weighted (DW) image and contrast-enhanced T1W image showed a high intensity lesion at the same place (lower left and right panels). Based on these findings, we finally diagnosed her as having pyosalpinx.
On admission, she had symptoms of diarrhea and persistent pain in left lower abdomen, but there were no findings in physical and neurological examinations. Her glycemic control was relatively good: HbA1c, 6.6%; glycoalbumin, 23.6%. In addition, her HbA1c levels were around 6% for over 1 year with the medication (metformin 500 mg, alogliptin 25 mg). However, during the acute phase of infection, we treated her with intensive insulin therapy using insulin aspart and insulin glargine. Tumor makers were within normal range: carcinoembryonic antigen (CEA), < 1.0 ng/mL; cancer antigen (CA) 19-9, 6.0 U/mL; and CA-125, 11.0 U/mL. Pathogenic bacteria were not detected. Her Treponema pallidum hemagglutination (TPHA) was positive but rapid plasma reagin (RPR) for Treponema pallidum was negative. Candida antigen and β-D-glucan were negative. Neisseria gonorrhoeae deoxyribonucleic acid (DNA) and Chlamydia trachomatis DNA in the urine were negative. Although pathogenic bacteria were not detected, we started antibiotics therapy for pyosalpinx (13.5 g/day of tazobactam/piperacillin and 500 mg/day of levofloxacin) (Fig. ). We discussed the necessity of surgery such as laparoscopy with gynecologists, but finally we selected antibiotics therapy without laparoscopy because her symptoms and laboratory data were very much improved. Her laboratory data 14 days after starting the antibiotics were improved and became within normal range (WBC, 5500/μL (neutrophil 47.4%); CRP, 0.04 mg/dl); we stopped antibiotics (Fig. ). Laboratory data were within normal range even after stopping antibiotics. T2DM was well controlled with orally administered anti-diabetes drugs (metformin 500 mg, alogliptin 25 mg, and gliclazide 10 mg): HbA1c, 6.0%; glycoalbumin, 16.5%. The tumor gradually reduced in size: 25 days later (upper right panel, Fig. ), 3 months later (lower left panel, Fig. ), and 6 months later (lower middle panel, Fig. ) compared to that on admission (upper middle panel, Fig. ); however, the tumor did not disappear completely even 6 months later. Finally she was discharged without any symptoms and/or problems. After discharge, she had no symptoms and/or problems, and her inflammation markers remained within normal levels for at least 6 months.
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pmc-6173903-1
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A 75-year-old man on hemodialysis for end-stage renal disease was hospitalized for cough, fever, and altered mental status. His temperature was 103 °F, pulse 68, blood pressure 124/69, respiratory rate 22, and O2 saturation 96% on 2 L of O2 by nasal cannula. He was lethargic, with bibasilar crackles and a normal cardiac examination. His white blood cell (WBC) count was 20,100 cells/mm3 and his plasma procalcitonin was 1.1 ng/mL. Chest X-ray showed bibasilar infiltrates. Microscopic examination of Gram-stained sputum showed profuse polymorphonuclear leukocytes (PMNs) and Gram-positive rods, many of which appeared to be intracellular (Fig. ). Sputum culture yielded overwhelmingly predominant Corynebacteria, with rare P. aeruginosa. The Corynebacteria was identified as C. propinquum by MALDI-TOF. Blood cultures and viral PCR were negative. The patient was initially treated with vancomycin, cefepime, and metronidazole. Based on the predominance of Corynebacteria with the absence of other bacteria on Gram stain and a negative PCR for respiratory viruses, his pneumonia was attributed to C. propinquum, and only vancomycin was continued. He responded and was discharged to complete a 10-day course of linezolid.
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pmc-6173903-2
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A 61-year-old man was found unresponsive post laryngectomy and tracheostomy for laryngeal squamous cell carcinoma. His temperature was 97.8 °F, pulse 99, blood pressure 114/86, and respiratory rate 22. Breath sounds were decreased bilaterally. WBC count was 7200 cells/mm3, lactate 1.2 mg/dL. Chest X-ray showed a new left-sided infiltrate. Sputum Gram stain revealed many Gram-positive rods, including those found within PMNs. Sputum yielded many Corynebacteria, identified by MALDI-TOF as C. propinquum, and few S. aureus. Sputum was liquefied with 2% N-acetyl cysteine and diluted serially; aliquots were cultured on blood agar and the number of colony forming units (CFU) was calculated []. The specimen contained 2 × 109 CFU of Corynebacteria and < 105 CFU of S. aureus per mL. Blood cultures and viral PCR were negative. The patient was initially given vancomycin, cefepime, and ampicillin; treatment was switched to ampicillin/sulbactam after the Corynebacterium was reported susceptible. His mental status rapidly improved, and he was subsequently discharged to complete a 10-day course of amoxicillin/clavulanate.
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pmc-6173903-3
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A 59-year-old male with widely metastatic squamous cell carcinoma of the tongue was admitted for bleeding from his tracheostomy site. His temperature was 99 °F, blood pressure 108/84, pulse 119, respiratory rate 24, and oxygen saturation 82% on room air. He had blood at the tracheostomy site and bibasilar rhonchi. WBC count was 22,000 cells/mm3, hemoglobin 11.1 g/dL, and lactate 1.8 mg/dL. Chest X-ray revealed a left upper-lobe infiltrate. Sputum sample showed profuse PMNs and Gram-positive rods. Culture yielded C. striatum (confirmed by MALDI-TOF) and few Escherichia coli. The patient was placed on comfort care and died 8 days later.
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pmc-6173919-1
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A 13-year-old male adolescent, with no history of any medical illnesses, presented to the emergency room complaining of severe continuous backache and fatigability for 3 days. He had recently traveled to the southern region of Saudi Arabia. No bowel or bladder symptoms were present. Written informed consent was obtained from the patient by King Fahd Hospital of the University, Imam Abdulrahman Bin Faisal University.
Clinical examination revealed tenderness of the lower back region on palpation and a reduction in the strength of both knees and hip during extension and flexion (grade 3/5), with sensory loss in both lower limbs on pinprick examination. Other parameters on neurological examination were intact. Laboratory results revealed anemia with mild leukocytosis and peripheral eosinophilia. All other results of routine laboratory tests were within the reference range.
An X-ray examination of the lumbar spine showed no gross abnormality. Emergent magnetic resonance imaging (MRI) revealed cord edema with an abnormal signal intensity in the thoracic and lumbar regions (Figs. , ). The clinical and laboratory findings of the radiological features indicated acute transverse myelitis secondary to infectious or inflammatory changes. However, the possibility of other differentials remained. A lumbar puncture was performed using standard procedures. Gram staining and culture of the cerebrospinal fluid yielded negative results. No isolated parasitic eggs were present in the urine or stool specimens. Brain MRI findings were unremarkable. However, the Schistosoma serology titer showed a marked elevation.
Therefore, a presumptive diagnosis of neuroschistosomiasis was made, and an experimental oral treatment for schistosomiasis was prescribed without any spinal intervention. The patient’s symptoms and signs rapidly subsided, with a regression of the spinal myelitis pattern on follow-up MRI examination (Figs. , ). Subsequently, he was discharged from the hospital within 1 week in dependable health and continued his anti-microbial regimen for 1 month. Follow-up examinations at the neurology clinic revealed a gradual improvement in the patient’s clinical condition. The patient was referred for further follow-up in the infection clinic. The patient was followed for 1 year and showed a complete long-term resolution of symptoms.
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pmc-6174051-1
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The patient was a 35-yr-old man with gastric cancer history for one year referred to Golestan Hospital, Ahvaz southwest of Iran in 2015 due to clinical signs including vomiting, loss of consciousness, food intolerances, impotence, icterus and paleness for 1 year. He had undergone chemotherapy bytaxotel (50mg), cisplatin (35mg) and flucytosine (500mg) according to his practitioner recommendation for four days. The chemotherapy schedule was repeated 4 times. The vital sign and biochemical parameters were: BP: 100/60 mm/Hg, PR: 110/min, RR= 28 /min, OT: 38.5C. The other biochemical parameters were:
ALT = 166, AST= 250, WBC: 23.80x 10 3, HB: 7.4, PLT: 56x10 3, FBS: 93, Na: 130, K: 5.9, Ca: 8.1, P: 3.9, bilirubin (T): 33.6, bilirubin (D): 30.1BUN= 149, Cr= 5.3 and in urinalysis: PRO 2+, Hb: 3+.
The patient was transferred to ICU due to respiratory distress. After few days, several larvae and pupa stage were seen in nasal and oral cavity (). The larvae and pupa were removed by forceps and transferred to parasitology department for precise diagnosis (, ). Nasal myiasis was recognized by infectious department consulting.
The patient was treated with turpentine, meropenem, vancomycin, and fluconazole for myiasis, pneumonia and candidiasis infection. The patient died after 5 d. The identification of third stages of larvae was performed according to internal and external morphological characteristics. Anterior and posterior air spiracles were removed from the body of maggots and examined microscopically (–). The pupa was cultured in room temperature for recognizing adult fly (). According to larvae, pupa and adult characters, Lucilia spp. was identified ().
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pmc-6174053-1
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We present a male case of a 14 yr old 2016 met at Legal Medicine Bihor County Service, Romania who bought an energy drink and while he was consuming it, he was feeling sick and felt down on the ground. He was resuscitated for about 1 hour, but unfortunately, he was declared dead. After we discussed with his family, he felt ill for two weeks, accusing chest pain, drowsiness, fatigue, productive cough and low-grade fever treated withtylenol and aspirin. He was at attending physician the day before his death and he was given tylenol and aspirin because he was diagnosed as having flu. No complementary examination like complete blood count or imaging exams (computed tomography) was made.
We followed the human subjects’ procedure, established by our institution. The research was conducted with the rules of good conduct in scientific research. The identity of the participant in the research is confidential when the results of this study are published. Informed consent was taken from the relatives of the patient.
External exam of the corpse did not reveal any violent lesions neither pathological.
Examination of the oral cavity showed normal dentition, but the gums corresponding to the teeth 1, 1, 1, 2.1, 3, 2.1, 2.2 were red and swollen, specific for gingivitis. There was no visible sign of illness at the level of head and neck so we could exclude the orocervicofacial form of this disease.
Macroscopic the lungs were described as pneumonia, emphysema and pulmonary edema; the entire myocardium looked like myocarditis () and on the posterior wall of the left ventricle was an area with cardiosclerosis () which made us think that it could be a scar from a myocardial infarction.
Inside the bronchi from the lungs inflammatory granulomas with A. israelii (not stained periodic acid Schiff-PAS negatively) were visible (). In myocardium bacterial colonies, microabscesses, diffuse cardiosclerosis, myocytes with necrosis and polymorphonuclear cells were visible (). Therefore microscopic exam revealed acute myocardial infarction, heart failure, myocardosclerosis, chronic myocarditis and into the lungs infection with A. israelii.
Corroborating the clinical features, autopsy findings and complementary examinations it was established that this sudden death was caused by an acute myocardial infarction as a result of chronic myocarditis and pulmonary pneumonia with A. israelii.
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pmc-6174063-1
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An 83-year-old Japanese man awoke from sleeping and fell out of a bed that was approximately 40 cm high. He called an ambulance 3 hours later because of persistent left-sided chest and back pain and was transferred to our hospital. He had also fallen 3 days before presenting at our hospital and had hit his left arm and the occipital region of his head, for which he had received treatment elsewhere. He had a medical history of cerebral infarction, atrial fibrillation, and prostate cancer, and he had been prescribed apixaban 2.5 mg twice daily and bicalutamide 80 mg/day. His habitual history and familial history were unremarkable. He was a retired medical doctor and lived with his wife’s sister. On arrival at the emergency room, his vital signs were as follows: temperature, 36.2 °C; pulse, 68 beats per minute with an irregular rhythm; respiratory rate, 24 breaths per minute; blood pressure, 143/64 mmHg; and oxygen saturation, 100% on 6 L/minute with a simple oxygen mask. His status on the Glasgow Coma Scale was 13 (E3V4M6), indicating slightly affected consciousness due to mild brain injury. On examination, he was found to be drowsy, pale, and restless. His heart sounds were unremarkable. Cardiac apex was not palpable. His trachea was central and left-sided chest expansion was reduced. There was significant left-sided chest tenderness. Coarse crackles were heard with decreased breath sounds over the left side of his chest. His abdomen was not distended. There was no hepatosplenomegaly. His cranial examination was normal. His limbs examination was normal except for his left arm which had a bruise. Arterial blood gas (ABG) analysis revealed the following: pH, 7.38; partial pressure of carbon dioxide (PCO2), 30 mmHg; partial pressure of oxygen (PO2), 211 mmHg; bicarbonate (HCO3−), 17.5 mmol/L; base excess, − 6.5 mmol/L; hemoglobin, 12.2 g/dL; and lactate, 6.0 mmol/L. Chest radiography and computed tomography (CT) revealed left hemothorax with fractures of the 9th to 12th ribs, which we suspected was associated with the injury sustained during his first fall 3 days before admission (Fig. –). His blood pressure gradually decreased to 93/45 mmHg after CT assessment, and intensive fluid resuscitation was then initiated. Contrast-enhanced CT (CECT) 4 hours later showed worsening hemothorax with contrast extravasation in the region supplied by the tenth intercostal artery (Fig. –). A tube thoracostomy at the fifth intercostal space initially drained 950 mL of hemothorax. TAE resulted in hemodynamic stabilization (Fig. ). He was admitted to the intensive care unit (ICU) after TAE, but experienced persistent chest tube drainage at a rate of > 200 mL/hour, eventually reaching a total volume of 1820 mL. Measurements of ABG revealed that his hemoglobin had fallen to 7.6 g/dL despite a transfusion with 4 units of red blood cells (RBC).
A thoracotomy at 4 hours after TAE revealed active bleeding from a partial-thickness wound at a peripheral site of his left diaphragm, corresponding to the edge of the broken tenth rib (Fig. ). A crushed bleeding lesion was removed from his diaphragm and the site was directly sutured. His fractured ribs were fixed, hemodynamic stabilization was confirmed, and further hemothorax did not arise (Fig. ). He required 6 units each of RBC and fresh frozen plasma during surgery. He was discharged from the ICU on hospital day 4 and the chest tube was removed on the following day. His postoperative course was uneventful and he was transferred to another hospital for rehabilitation without complications on day 29. He stayed at a nursing home and received no follow-up visit after discharge. He was managed at the hospital where he used to work and no adverse events were observed at 6 months after discharge.
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pmc-6174068-1
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A 76-year-old man presented initially for evaluation of refractory anemia. His history included a diagnosis of Felty syndrome at the age of 64 years, after he was found to have rheumatoid arthritis, neutropenia, and an enlarged spleen. A diagnosis of anemia of chronic disease was made after complete workup including a BM examination was negative for malignancy. He received multiple therapeutic agents over time to control his rheumatoid arthritis, including adalimumab, methotrexate, abatacept, infliximab, azathioprine, rituximab, and prednisone. He underwent splenectomy at 67 years.
His anemia progressed and he became transfusion dependent at the age of 68 years. He was diagnosed with myelodysplastic syndrome (MDS) after a repeat BM examination revealed minimal morphologic dysplasia with a diploid karyotype. He received erythropoietin support for 1 year with worsening of his anemia. He was next treated with adjusted-dose lenalidomide (5 mg every other day) and rapidly achieved transfusion independence and a hemoglobin level of 13 g/dL. He progressively lost his response and became transfusion dependent. After 5 years, lenalidomide was discontinued and he received one cycle of azacitidine. Azacitidine was discontinued after BM examination at that time showed no morphologic support for residual MDS, and he continued to receive transfusions for nearly one year before presenting to our institution.
A BM core biopsy and aspiration on initial evaluation demonstrated hypercellular marrow with trilineage dysplasia, moderate reticulin fibrosis, and 2% blasts (Fig. a, b). Flow cytometry demonstrated changes consistent with MDS, including markedly decreased side scatter in granulocytes (cytoplasmic hypogranularity), absence of hematogones, and a small number of aberrant myeloid blasts that had expression pattern of increased CD13 and CD34 and decreased CD38. Dendritic cells with a CD123bright, CD4+, HLA-DR+, CD56− immunophenotype were 0.2% of all analyzed cells (Fig. , top panel). Cytogenetic studies showed a normal diploid karyotype, and no mutations were identified on next-generation sequencing of 28 leukemia-associated genes. He was treated on clinical protocol with oral decitabine and had decreased transfusion requirements. Repeat BM aspirations showed persistence of dysplastic features and no significant increase in blasts.
Flow cytometry of a surveillance BM aspirate sample approximately 8 months after decitabine initiation showed the emergence of a minimal population of aberrant plasmacytoid dendritic cells (CD123bright, CD4+, HLA-DR+, CD56+), comprising 0.79% of cells (Fig. , middle panel). This represented an immunophenotypic shift from CD56− plasmacytoid dendritic cells cells at presentation. After 1 year of decitabine treatment, he experienced increasing fatigue and developed scattered violaceous plaques on his abdomen, upper chest, and forearms (Fig. c) in addition to bilateral discoloration of the inferior conjunctiva. BM aspiration and biopsy revealed 70% immature appearing cells with finely dispersed nuclear chromatin, conspicuous nucleoli, and scant, eccentrically placed cytoplasm imparting a “hand-mirror” appearance. Immunohistochemical studies showed the immature cells were positive for CD4, CD56, CD123, and TCL1, and negative for CD34, MPO, and lysozyme. Flow cytometry showed a distinct population of CD45dim cells with CD123bright expression. These cells were CD4+, HLA-DR+, CD56+, CD34−, CD117−, CD64−, CD14−, and made up 35% of analyzed events (Fig. , bottom panel). This constellation of findings was diagnostic for BPDCN with extensive BM involvement. Cytogenetic studies showed a normal diploid karyotype and next-generation sequencing studies identified mutations in KRAS, NOTCH1, and RUNX1. Biopsy of an abdominal wall lesion showed BPDCN skin involvement (Fig. d, e). He was enrolled in a clinical trial (NCT02113982) of single-agent SL-401, a recombinant human IL-3 conjugated to truncated diphtheria alpha-toxin, which targets CD123. After 1 cycle, he experienced a decrease in BM blasts to 22%, resolution of skin lesions (Fig. f) and ophthalmic lesions, and marked improvement in performance status. After the second cycle, his BM aspirate showed 2.5% residual aberrant blastic plasmacytoid dendritic cells.
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pmc-6174197-1
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A 63-year-old man came to the clinical observation because of a rapid onset of dyspnea and dysphonia along with the development of a bulky node in the left side of the neck. He had been working as an interventional cardiologist in an angiographic room for 15 years at the local Hospital. Family history was negative with regard to malignancies and thyroid disease.
The relevant medical history included hypertension treated with valsartan and hydrochlorothiazide and non-insulin-dependent diabetes mellitus treated with metformin. There was no previous history of thyroid disease. Two years before the admission, he was treated with warfarin because of a deep venous thrombosis of the left leg occurred after a short bed rest for prostatitis. He was a heavy smoker.
The iodine status of the patient was not known; however, he was from a non-Alpine region and he was still living in the same area which is considered as a mildly iodine insufficient [].
On physical examination, the patient had a 8 × 10-cm firm left-sided neck mass with a right-sided shift of the larynx. On ultrasound examination, a nodule of the left thyroid lobe was found measuring 5 and 6-cm in its antero-posterior (AP) and transverse (T) diameters, respectively. The nodule was hypoechoic but inhomogeneous, with no vascularization; at the strain elastography, the nodule ranged from a medium elasticity to a hard pattern. The volume of the right thyroid lobe was reduced with a small hypoechoic nodule. No enlarged lymphnodes were found at the neck ultrasound. Computed tomography (CT) of the neck confirmed a 7 × 5 × 13-cm (T × AP × Long diameters) large, inhomogeneous neck mass originating from the left lobe that caused displacement of the trachea, the left common carotid artery and the left internal jugular vein. No evidence of primary malignancies or suspicious for secondary lesions was found at the CT of the head, abdomen, and pelvis. The chest CT showed a 6-mm round-shaped nodule not suspicious for malignancy close to the parietal pleura at the lower lobe of the right lung.
A fine needle aspiration cytology (FNAC) of the mass was performed which yielded hemorrhagic smears with few groups of large, epithelioid cells, with vesicular, severely atypical nuclei and eosinophilic dense cytoplasms. A diagnosis of malignancy was given (Category 6 according to Bethesda 2010) with a suggestion for an anaplastic carcinoma (Figure ).
The patient underwent a total thyroidectomy and lymphadenectomy of central and left lateral cervical nodes. At the gross pathology examination, the tumor measured 6 × 6 × 12 cm (T × AP × Long diameters) and was partially circumscribed by a fibrous pseudocapsule. The mass had a gray, tan and red cut surface, with areas of hemorrhagic necrosis. Histology showed a vasoformative high grade neoplasia characterized by large epithelioid cells growing in sheets and lining abnormal vascular spaces; some cells showed intracytoplasmic lumina. There were areas of spontaneous necrosis and hemorrhage and a brisk mitotic activity; angioinvasion was noted. The tumor immunostained for vascular markers (CD31, ERG, CD34, factor VIII and vimentin), whereas epithelial differentiation markers were negative (cytokeratins, thyroid transcription factor 1, thyroglobulin, and EMA). The final histologic diagnosis was primary epithelioid angiosarcoma of the thyroid, grade 3 according to FFCCS (Figures ). This diagnosis was confirmed at a second opinion from a different institution. The tumor was restricted to the thyroid with free surgical margins. The mass had substernal extension and displaced the surrounding structures but it did not infiltrate the thyroid capsule, the strap muscles, or other neck tissues. The remaining thyroid tissue had nodular colloid goiter. No lymphnode metastases were detected.
Fifteen days after the thyroidectomy, the patient was operated to prevent rupture of an aneurysm of the abdominal aorta. One month after thyroidectomy, the chest CT showed multiple pulmonary nodular lesions some of them with a solid pattern surrounded by a ground-glass halo, 12 mm in maximum diameter. There was no consensus as to the oncologic relevance of these lesions, therefore, no biopsy was performed. A bone scintigraphy yielded negative results.
Chemotherapy with Epirubicin, Ifosfamide, and Mesna was administered but it was discontinued after 4 cycles because of pancytopenia and infection by Klebsiella Pneumoniae, treated with piperacillin/tazobactam, and by Clostridium difficile, treated with vancomycin. The patient recovered from the infections and, at a 6-month follow-up, the chest CT showed a reduction of the number and volume of the lung lesions with only three of them remaining in the medial lobe of the right lung.
At a further 18-month control, the chest CT was unchanged. The 6 mm round-shaped nodule close to the parietal pleura at the lower lobe of the right lung was also found to be stable. One year later, the patient developed pneumonitis and recovered after antibiotic therapy. At that time, he was investigated by neck, chest, abdomen and pelvis CT as well as with FDG-PET without any evidence of disease recurrence.
Afterward, a 6-month CT follow-up program was started which is still ongoing. At present, the patient is alive with no evidence of disease after 62 months from initial diagnosis.
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pmc-6174357-1
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A 14-years-old girl came to our attention because of severe and persistent lymphopenia during an episode of autoimmune hemolytic anemia. Her familiar history was negative for invasive infections and autoimmune diseases. Patient medical history was unremarkable for infections. In addition, previous blood counts were normal. The study conformed to all the protocols of Asst Spedali Civili of Brescia. Informed consent for blood tests and genetic studies was obtained from her parents.
She presented with acute onset anemia (hemoglobin 5.5 g/dl) with positive direct antiglobulin test (Coombs test, IgG 2+), normal platelets (299.000/μL), and white cell count (total leucocytes 5.760/μL, neutrophils 4.160/μL, lymphocytes 1.330/μL). At the beginning she was treated with oral prednisone (2 mg/kg/day), but poor response to the treatment was observed. Therefore, she was switched to four intravenous pulses of methylprednisolone each one at 2 mg/kg within 72 h, followed by intravenous immunoglobulins (1 g/kg).
The laboratory tests showed normal white cell counts, except for marked lymphopenia (Figure ), reduction of CD4+ cells (ranging from 50 to 300 cells/μL), increase of fetal hemoglobin, (6.4–13.9% during follow-up), supposedly related to reticulocytosis. While autoantibodies, including Anti-Nuclear Antibodies, Extractable Nuclear Antigen, Anti-DNA antibodies, Anti-Smooth Muscle Antibodies, Anti-phospholipid Antibodies, complement factors, and serum immunoglobulins were within the normal ranges. Immunological screening for celiac and thyroid disease were also negative. Serologic tests for Parvovirus B19, EBV, CMV, and Waaler-Rose test, were consistent with prior infection or non-immunized state. The fecal occult blood test was negative; chest radiograph, echocardiography and abdomen ultrasound were all unremarkable.
She underwent also bone marrow aspirate, that showed normal proportion of myeloid cells, presence of megakaryocytes, and mild dyserythropoiesis, without evidence of cytogenetic abnormalities.
During corticosteroid therapy, hemoglobin levels slowly increased and Coombs test became negative within 3 months. Prednisone (1 mg/kg/bid) was continued for 10 months with progressive tapering. However, this therapy was associated with multiple side effects, such as alopecia, weight gain, moon face, stretch marks, including lymphopenia. This was also associated with reduction of IgG concentrations (IgG 520, n.v. 640–1909 mg/dl; IgM 115, n.v. 59–297 mg/dl, IgA 121, n.v. 61–301 mg/dl) which prompted us to start IVIG every 4 weeks for 3 months. In addition, because of the risks related to lymphopenia and to prolonged immunosuppressive therapy with corticosteroids, prophylaxis with cotrimoxazole and acyclovir was prescribed.
In order to exclude an underling immunodeficiency, we examined lymphocyte subsets. Flow cytometric immunophenotyping showed marked alterations in the distributions of the T-lymphocytes (Table ): reduction of the CD3+CD4+ T cells (19.7%), especially the Recent Thymus Emigrants (RTE) CD4+ T Cells (2.9%), and increase of the TCR γ/δ T cells (24.7%). Transitional B cells (0.4%), and terminal differentiated B cell subsets (0.06%) were reduced, while Switched Memory B cells (34.2%) were increased. T-cell Receptor Excision Circles (TRECs) and Kappa-deleting Recombination Excision Circles (KRECs) were below normal range, while analysis of the T-cell receptor repertoire showed a marked monoclonal expansion of TCRBV6 expressing cells. Increase of TCR γ/δ T cells could be related to many conditions, including infections. CD3+/CD4−/CD8− (double negative T cells) subset were in the normal range, thus ruling out the hypothesis of Autoimmune lymphoproliferative syndrome.
Next, we analyzed by next generation sequencing (Ion Torrent pgm, Thermo Fischer) a panel of genes that are associated with SCID or CID/lymphopenia. Genetic analysis revealed an heterozygous mutation in exon 10 of the gene encoding for adenosine deaminase (ADA, p.S291L c.561C>T), while other genes that are associated with autoimmune diseases, including LRBA and CTLA4 were negative for mutations. Sanger sequencing of ADA gene coding and non-coding flanking regions revealed the same mutation. The assessment of adenosine and deoxyadenosine nucleotides levels in the patient at the time of lymphopenia revealed a moderate increase of both metabolites: adenosine was 3.6 μMol/ml RBC (n.v. 0.8–1.6) while deoxyadenosine was 0.006 uMol/ml RBC (n.v. < 0.005), respectively. Total AXP and dAXP in RBC were quantified by high performance liquid chromatography measuring the levels of Ado and dAdo formed by treating neutralized acid extracts with alkaline phosphatase and venom phosphodiesterase ().
Analysis of ADA activity in red blood cells lysates, as measured by spectrophotometric analysis of the absorbance change at 265 nm (), was reduced but within the normal range (0.8 U/g Hb; n.v. 0.8–2.5) while PNP activity in red blood cells lysates was normal (200 μ/g Hb; n.v >330). Analysis of ADA activity, of total AXP and dAXP in RBC in her parents were normal; unfortunately, their genetic status has not been investigated for parental decision.
After corticosteroid tapering the patient's total numbers of lymphocytes gradually increased and reached normal levels (Figure ). One year after stopping corticosteroid therapy lymphocyte count was 1.410 cells/μL, with 550 CD4+cells/uL. Thus, she interrupted the prophylactic therapy with acyclovir and cotrimoxazole.
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pmc-6174561-1
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A 46-year-old Asian man without any significant past medical history presented to an out-patient clinic complaining of fever, epigastric pain, and back pain. He was diagnosed as having gastric ulcer by upper gastrointestinal endoscopy and prescribed a proton pomp inhibitor; however, his fever of approximately 38 °C and his back pain remained. Two weeks later, his back pain had worsened, and the laboratory data of the out-patient clinic showed an elevated C-reactive protein level (17.2 mg/dL); thus, he came to our hospital for further evaluation. His medication included only orally administered azelnidipine for hypertension. There was no significant family medical history. He denied smoking tobacco, alcohol consumption, and exposure to toxins. He worked at a ceremonial hall without any ill contacts. He had a fever of 37.9 °C, heart rate of 90 beats per minute (bpm), respiratory rate of 20 breaths/minute, blood pressure of 126/78 mmHg, and oxygen saturation of 97% on room air. A physical examination including a neurological examination showed a well man without any specific abnormal findings. Blood tests at the first encounter revealed a white blood cell count of 10,300/μL with 70% neutrophils, 14% lymphocytes, and 16% monocytes, and the platelet count was 275,000/μL. His lactate dehydrogenase level was 299 IU/L (normal range, 119–229 IU/L), his alkaline phosphatase level was 983 U/L (normal range, 103–335 U/L), and his gamma-glutamyl transpeptidase level was 256 IU/L (normal range, 0–73 IU/L). His C-reactive protein level was 23.47 mg/dL (normal range, 0–0.29 mg/dL). Other results are shown in Table . A contrasted computed tomography (CT) scan showed edema around his gallbladder without gallstones or bile duct dilation, along with left adrenal enlargement without contrast, suggesting necrosis and slight pleural effusion (Fig. ). His right adrenal gland was contrasted normally. Contrasted magnetic resonance imaging (MRI) of his adrenal glands was also performed, and the results showed necrosis of his left adrenal gland with a slight possibility of infarction and no specific evidence of hemorrhage. He was hospitalized for further investigation into the cause of the unilateral adrenal necrosis.
Lupus anticoagulant and don't break the value complex antibody were measured, and both were negative, which suggested a low possibility of antiphospholipid syndrome. He did not meet the criteria for diagnosis of systemic lupus erythematosus. We considered the possibility of adrenal insufficiency or pheochromocytoma and measured several types of adrenal hormones, such as serum cortisol, adrenocorticotropic hormone, plasma renin activity, plasma aldosterone activity, and urinary metanephrine and normetanephrine, but none of them explained our patient’s condition. The culture results from blood drawn at the first encounter were all negative. We performed CT-guided needle biopsy of his left adrenal gland, which revealed necrosis and the formation of fibrotic granulomatous tissue (Fig. ). There was no epithelioid granuloma, malignant lymphoma cells, or hemosiderin deposition, suggesting a low possibility of the involvement of a hemorrhagic etiology. The bacterial culture of this biopsy tissue was also negative. After the biopsy was finished, he was discharged. However, 1 week later, severe thrombocytopenia (5000/μL) appeared, and he was rehospitalized. His creatinine level had increased to 1.03 mg/dL from the initial value of 0.85 mg/dL. Bone marrow aspiration first resulted in a dry tap, but subsequent results showed increased megakaryocytes and hypercellular marrow with fibrosis classified as MF-1 according to the European consensus on bone marrow fibrosis staging (Fig. ). A contrasted CT scan showed new left axillary lymphadenopathy with a size of 15 mm, right pleural effusion, and increased ascites (Fig. ). Because our patient’s condition was worsening, we needed to start immediate treatment for any possible underlying causes, including bacterial infection and autoimmune disease, before obtaining the exact diagnosis. The laboratory data from the second hospitalization are shown in Table . The clinical course of this case is shown in Fig. . The initial treatment included ampicillin/sulbactam and a methylprednisolone pulse followed by orally administered prednisolone and intravenous immunoglobulin therapy (400 mg/kg for 5 days), considering the underlying causes mentioned above, such as severe bacterial infection or autoimmune diseases including antiphospholipid syndrome and immune thrombocytopenia; however, all of these treatments seemed to be ineffective. We also used recombinant thrombomodulin (380 U/kg) for 7 days to cope with the possibility of a thrombotic event or disseminated intravascular coagulation. Because the blood and adrenal gland biopsy culture results were both negative, we stopped the antibiotic treatment. On hospital day 9, we performed a left axillary lymph node needle biopsy, which showed no evidence of malignant lymphoma. With the edema, severe thrombocytopenia, fever above 37.5 °C, reticulin myelofibrosis (MF), mild lymphadenopathy, and progressive renal insufficiency and with other diseases excluded, we diagnosed this patient as having TAFRO syndrome according to the diagnostic criteria []. The administration of intravenously administered tocilizumab (8 mg/kg) was begun on the same day with tapering prednisolone dose; his C-reactive protein and alkaline phosphatase levels gradually improved, along with his renal function and fever (Fig. ). For the anasarca, furosemide and potassium canrenoate were used and were highly effective.
Because the thrombocytopenia remained, we added eltrombopag, a thrombopoietin receptor agonist, on hospital day 14, followed by tocilizumab administered on hospital day 16. Then, his platelet count began to increase. Under the strong immunosuppressive treatment, he contracted methicillin-resistant Staphylococcus epidermidis bacteremia on hospital day 20 and cytomegalovirus viremia on hospital day 31, which were successfully treated with vancomycin and ganciclovir, respectively. Tocilizumab was administered a third time on hospital day 47, and our patient was discharged on hospital day 48. After discharge, he remained afebrile and with an alkaline phosphate level within normal limits, and tocilizumab administration was no longer necessary. Eltrombopag administration was stopped because his platelet count increased and remained stable within normal limits. Nine months after the first treatment, this patient continues to do well. He is only being treated with low-dose prednisolone at approximately 5 mg per day and is still tapering carefully because of the presence of unilateral adrenal necrosis, considering the possibility of adrenal insufficiency.
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pmc-6174575-1
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A 22-year-old, right-hand-dominant woman was referred to our Plastic Surgery Department from her sexual health clinic 1 day postinsertion of a contraceptive implant (Implanon) in her left arm. It was explained that the implant was inserted by a nurse at the clinic who felt that the implant went in at “a slight angle, rather than superficial,” after the patient had flinched on insertion of the trochar. The subject experienced pain and paresthesia along her arm that had then subsided; however, she returned later that same day with worsening symptoms. On examination in the clinic, the implant was not palpable. The patient described paresthesia along the ulnar distribution of her hand and the forearm, as well as shooting pain on palpating the course of the ulnar nerve. Ultrasonography found the implant to be lying in the subfascial plane. On exploration in the operation theater, the implant was found lying in the perineurium, with the nerve itself intact (). The medial intermuscular septum was released and the implant was removed in one piece without the need to repair any structures. She recovered well postoperatively. On review in the clinic 4 weeks later, she had persistent hypersensitivity of the dorsoulnar aspect of the distal forearm and reduced sensation in the ulnar digital and radial digital nerves of the little finger. The power of the intrinsic muscles in the hand was normal. Three months after removal of the implant, all her ulnar nerve functions apart from a slight residual sensory alteration had returned to normal.
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pmc-6174731-1
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A 76-year-old male linguistics professor was referred to the general internal medicine service by emergency medicine for a 2-week history of worsening confusion. He was independent for his activities of daily living (ADLs) and his instrumental activities of daily living (IADLs) at his baseline 6 months ago. The family endorsed a cognitive decline that started with memory issues, word-finding difficulty, and unsteady gait. They also endorsed a history of agitation and hallucinations at night. In the 2 weeks prior to his emergency room visit, his symptoms progressed at an even more rapid pace, with him being too weak to ambulate, and experiencing new incontinence of urine and stool. Until the worsening of his cognitive deficits, he was still working as a linguistics professor at the postsecondary level.
His past medical history was significant for coronary artery disease, hypertension, type 2 diabetes, asthma, and benign prostatic hyperplasia. There was no personal or family history of malignancy or dementia. He had never been screened for malignancy. There was no history of hunting or consuming game meat. His medications included ASA, candesartan, hydrochlorothiazide, metformin, glimepiride, iron supplements, multivitamins, and timolol eye drops. There were no over-the-counter medications, illicit drugs, or alcohol. On exam, his vitals were stable. His mucous membranes were dry, and his JVP was flat. His cardiac, respiratory, and abdominal exams were unremarkable. His neurological exam revealed a slight upward gaze palsy and velocity-dependent hypertonia in the upper extremities. There were no fasciculations or myoclonus. Reflexes and sensation were intact.
His white blood cell count was 2.7 × 109 (normal 3.5–10.5), his hemoglobin was 134 g/L, and his platelets were 196 × 109. The electrolytes and extended electrolytes were within normal limits aside from sodium of 125 mmol/L (normal 136–145). LTFs and bilirubin were within normal limits, and TSH was 2.35 (normal), and B12 level was 278 pmol/L (normal). Syphilis and HIV serologies were both negative, as was the antinuclear antibody (ANA). A diffusion-weighted MRI demonstrated diffuse parenchymal volume loss that was prominent for age and mild microangiopathic changes. His EEG was abnormal but nonspecific with irregular periods of 6–7 Hz theta activity, intermingled with short 2–4 Hz delta rhythms most prominent in the frontal regions. There was no alpha activity or obvious epileptiform, focal, or lateralizing features. CSF showed a nucleated cell count of 6 (normal 0–5), normal glucose, and slightly elevated protein at 0.55 g/L (normal 0.15–0.45 g/L). Oligoclonal bands were not detected in the CSF. CSF was negative for tau and 14-3-3 protein but positive for end-point quaking-induced conversion (EP-QuIC) at the National Microbiology Laboratory in Winnipeg. The paraneoplastic (anti-hu, ri, yo, ma2, cv2, and amphiphysin) antibody panel (mitogen) was negative.
He developed myoclonus and mutism, and he was discharged to a palliative care facility. He passed away 8 weeks after his initial emergency room presentation. The postmortem autopsy of the brain demonstrated microspongiosis, neuronal loss, and gliosis in the cortex, hippocampus, basal ganglia, and cerebellum, consistent with sCJD.
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pmc-6174732-1
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A 20-year-old male patient with no past medical history presented to the emergency room (at Jahra Hospital, Kuwait) with history of fever associated with malaise and arthralgia for 7 days. On arrival in the causality, he was febrile (39°C). Rest of the physical examination was normal. A history of ingesting unpasteurized camel's milk was reported, and a serological test for brucellosis was positive. A complete blood count (CBC) showed hemoglobin 145 g/L, WBCs 7.6 × 109/L with 45.6% lymphocytes and 49.2% neutrophils, and platelets 4 × 10/L. Other laboratory tests showed urea 3.6 mmol/L, creatinine 75.25 µmol/L, total bilirubin 16.1 µmol/L, ALT 48 IU/L, and AST 52 IU/L. There was severe isolated thrombocytopenia (4000), but no evidence of skin or mucous membranes bleeding. He denied use of any over-the-counter drugs, painkillers, or NSAIDs. The ER physician advised for hospital admission but he refused. Therapy with doxycycline and rifampicin was prescribed, but then he left against medical advice. Two days later, he returned to the emergency room with complaints of epistaxis, gingival bleeding, and hematuria. No hematemesis or melena was reported. He was febrile with a body temperature of 38.7°C, an arterial blood pressure of 110/75 mmHg, and a heart rate of 72 beats/min. There was nonitchy flat purpuric rash, particularly on his lower extremities. His physical examinations of the cardiovascular, respiratory, abdominal, and central nervous systems were normal. Repeat CBC showed hemoglobin 128 g/L, WBCs 4.9 × 109/L with 51.8% lymphocytes and 41.1% neutrophils, and platelets 2 × 109/L. A peripheral blood smear examination showed normal RBCs, WBCs morphology, and marked thrombocytopenia with occasional giant platelets and no platelet clumps. When true thrombocytopenia was confirmed and to exclude other causes of thrombocytopenia, malaria blood film, coagulation profile, fibrinogen level, antinuclear antibody (ANA), double-stranded DNA antibody (anti-ds DNA), and serological tests for HbsAg, anti-HCV, HIV, cytomegalovirus, and Ebstein–Barr virus all were unremarkable. Brucella agglutination test was positive (titer 1 : 1280). We considered a diagnosis of brucellosis with immune thrombocytopenic purpura. Due to the very low platelet count and the mucous membrane hemorrhage, 12 units of platelets were infused. Treatment with intravenous immunoglobulin (IVIg) was initiated at a dose of 500 mg/kg/day, combined with prednisolone (1 mg/kg/day), doxycycline (100 mg/12 hours), and rifampicin (600 mg/day). Fever resolved on the second day of treatment, platelet count started to rise, and bleeding manifestations improved. On the 6th day, his platelet count was 66000, and he was discharged from hospital on tablet prednisolone of 25 mg daily with gradual tapering, tablet rifampin of 600 mg daily, and capsule doxycycline of 100 mg twice a day for total six weeks. Blood cultures performed on specimens obtained at the time of admission showed no growth. He was planned for follow-up one month after discharge from the hospital, but he did not show up. A follow-up phone call confirmed that all his symptoms resolved and he finished his treatment course.
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pmc-6174736-1
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A previously healthy 41-year-old woman was diagnosed with CML, after presentation of symptoms caused by hypersplenism with gravity sensation under the right costal margin. Initial blood tests demonstrated severe leukocytosis 227 × 109/L (normal range 4–11 × 109/L) together with increased serum concentration of lactate dehydrogenase (LDH) 969 U/L (200–450 U/l) and cobalamin 2834 pmol/L (150–840 pmol/L). A blood smear demonstrated dominance of myeloid precursor with increased metamyelocytes and rods. CML was confirmed by identification of the Philadelphia chromosome t(9; 22) by using conventional G-banding analyses.
Treatment was initiated with hydroxyurea combined with interferon, and the patient reached morphological remission, then proceeded to allo-SCT for consolidation treatment. The transplantation was performed by a myeloablative condition (MAC) regime with busulphan 1 mg/kg for four days followed by cyclophosphamide 60 mg/kg for two days, followed by bone marrow-derived stem cell from her human leukocyte antigen- (HLA-) matched sister. Standard graft versus host disease (GVHD) prophylaxis by using cyclosporine A and methotrexate on day 1, 3, 6, and 11 after transplant was given []. No severe complications were observed after transplant, and especially, she did not develop any signs of acute or chronic GVHD.
After the development of polymerase chain reaction (PCR) analysis for BCR-ABL1 transcripts [], this test has been regarded as mandatory in the follow-up of CML patients []. Six years after the allo-SCT, an e13a2 transcript of BCR-ABL1 was detected by nested PCR. She was therefore controlled twice yearly, without signs of progression judged from karyotyping and interphase fluorescence in situ hybridization (FISH) of 200 interphases with probes against BCR and ABL1 in the bone marrow. By standardization of quantitative real-time (RT) PCR, yearly analyses were performed [], and low but detectable transcript levels were still observed, although molecular remission (MR) levels were below MR3.
Her transcript levels then suddenly increased rapidly, and she lost her MR (). This was confirmed by analysis at two different laboratories. The patient proceeded to bone marrow examination showing normal metaphases by G-banding and only one cell with BCR-ABL1 of 245 interphases by FISH using dual fusion probes, and this was regarded as insignificant. The bone marrow smear was hypercellular with increased myeloid precursors and megakaryocytes, although without evidence of increased myeloblasts. Hence, we maintained the diagnosis of CML with molecular relapse appearing 25 years after initial allo-SCT. The patient was screened for other mutations commonly occurring in myeloid malignancies, including mutations in BCR-ABL1, but no additional mutations were detected. Donor chimerism status revealed that the majority of myeloid cells (99%) were of donor origin. We thought that her relapsed disease was likely to be sensitive to treatment with first-generation TKI, and she was given imatinib 400 mg daily. She tolerated the treatment well without major side effects; after three months, she obtained MR4 status (), and imatinib treatment was continued with regular monitoring of BCR-ABL1 quantitative RT-PCR.
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pmc-6174737-1
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A 39-year-old woman, who had been taking medication of quetiapine as an antipsychotics for depression, experienced mild dysarthria and visited the department of neurology in our hospital. Her symptom was diagnosed as drug-induced lip dyskinesia, which disappeared in a week. Screening head magnetic resonance imaging (MRI) at this time revealed stenosis of the left MCA with no brain parenchymal lesions (), why she was consulted to our department. We performed angiography, confirming moderate M1 portion stenosis (Figures and ). SPECT showed no apparent laterality in CBF, thereby we decided to observe her with no treatment.
Seven months later, the patient experienced mild weakness and numbness in her right hand and visited our department. Although MRI showed no apparent ischemic change in her brain, arterial spin labelling (ASL) of MRI detected the decrease of CBF in the left cerebrum (), which was thought to well correspond for her symptoms. She was admitted and treated with an antiplatelet agent. Two weeks later, she still complained of numbness in her right hand; thereby, we decided to perform left STA-MCA anastomosis to prevent deterioration of her symptoms. Preoperative SPECT showed no apparent laterality in CBF (). On operation, left temporal craniotomy was performed, and the parietal branch of the STA was anastomosed with the M4 portion on the temporal lobe (Figures and ). The intraoperative course was uneventful, and the patient recovered from anesthesia without any new neurological symptoms
Postoperatively, her speech was normal until postoperative day 1 (POD1). On POD2, she exhibited mild speech disturbance, which worsened day by day finally resulting in complete motor aphasia on POD6. Her comprehension was kept normal. On POD3, generalized convulsion occurred, which ceased quickly by diazepam, and levetiracetam was initiated. On the same day, she presented with mild weakness of right upper extremity, which improved gradually and disappeared on POD7. MRI and CT showed no ischemic or hemorrhagic changes, but ASL and SPECT revealed remarkable increase of CBF in the left cerebrum (Figures and ), by which the symptoms were diagnosed as CHPS. Despite the treatment with strict blood pressure and the administration of edaravone and minocycline, complete motor aphasia remained unchanged on POD21. MRI showed no abnormality except slightly hypointense changes on T2 weighted images and FLAIR (). At this point, the patient was discharged partly because of the request from the patient, and we continued to follow her in outpatient visit. One month after the surgery, the patient started to utter words that were not fluent, when SPECT and MRI showed normalization (). Thereafter, the improvement of her speech was slow, and totally more than three months was taken for full recovery after the surgery.
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pmc-6174740-1
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A 59-year-old woman with epigastralgia was evaluated in gastroenterology and an upper digestive endoscopy was indicated. During the endoscopic examination a gastric polyp was found and the patient was referred to our hospital for treatment.
Endoscopic examination of the upper digestive tract revealed extensive atrophic gastritis and a sessile lesion of 15 mm of reddish coloration in the distal gastric body (). Magnifying endoscopy with Fuji Intelligent Color Enhancement (FICE) of the polyp showed an irregular microsurface pattern at the apex, noticing a demarcating line, highly suggestive changes of malignancy (). Endoscopic mucosal resection (EMR) was performed with lateral safety margins (Figures and ).
The histopathological evaluation of the resected polyp revealed that the base corresponded to a hyperplastic polyp, in which a tubular adenoma with high-grade dysplasia was established with focal well-differentiated intramucosal tubular adenocarcinoma (). The lesion was resected completely with a lateral margin greater than 2 mm. No evidence of lymphovascular invasion was noticed. For this reason, it was concluded that the EMR was successfully performed fulfilling the criteria of histological cure []. The polyp with focal adenocarcinoma was classified as early gastric cancer, type 0-Is according to the Paris classification, and T1a according to the TNM classification []. Histopathological evaluation of the surrounding mucosa revealed atrophic gastritis in the body and antrum, with no evidence of Helicobacter pylori infection.
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pmc-6174746-1
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Case 1. Dustin, a five-year-old White male, was brought to the clinic by his mother for her chief concern of defiance and emotional dysregulation. During the first diagnostic interview, Dustin's worries included any social situations that included scrutiny from others (e.g., he refused to stand in front of the church for his baptism), death, feeling anxious in places where he does not have control, and intense fear of bugs. Worries occurred almost daily and appeared somewhat uncontrollable. At the second interview, two weeks later, his worries remained the same but now included getting lost. At school, his negative emotionality could escalate into tantrums of screaming and trying to bang his head on a wall, which could last two hours. At home, getting him to take a bath could involve an hour of crying and protesting. Symptoms first appeared at one-and-a-half years but because he was preverbal at that age his mother could not give examples that clearly met GAD criteria.
Physical symptoms present during Dustin's periods of worry included feeling restless, on edge, difficulty concentrating, and irritability. Functional impairment included a slight impact on parental relationships, a moderate impact on the relationship with his daycare provider, and a severe impact on the child's ability to go out in public. His mother almost always accommodated him by rarely taking him outside of the home.
Dustin met all of the criteria for GAD, ODD, and SAD. His treatment involved helping learn ways to calm himself and help his parents manage his behavior. He improved markedly by the end of the school year and treatment terminated over the summer. But his behavior flared up when school resumed and treatment had to be restarted.
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pmc-6174746-2
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Case 2. Ivan, a five-year-old white male, was brought to the clinic by his mother for her chief concern of his worries about death. During the first diagnostic interview, Ivan was described as having excessive worries related to something happening to his family, death, Earth being sucked into a black hole, criminals harming someone in the family, and separating from parents when going to school. He worried that other children did not like him and that he would not do a good job on tasks. Symptoms first appeared at age four years.
His worries appeared clearly uncontrollable to his mother. Physical symptoms present during Ivan's periods of worry included difficulty concentrating. Functional impairment was endorsed in the parental relationship.
No excessive worries were endorsed during the second diagnostic interview even though those interviews were only two weeks apart. However, when his medical record was reviewed for this paper, it was clear that he had the same worries consistently for two years following those interviews up to the present time.
Ivan met all of the criteria for GAD plus SAD. He had not yet improved markedly after two years of treatment.
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pmc-6174746-3
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Case 3. Alani was a six-year-old Pacific Islander female who was brought to the clinic by her mother for her chief concern of fear of bad weather. No other excessive worries were endorsed during the first interview, so her fear of weather was conceptualized as PTSD initially. During the second interview however Alani was described as having excessive worries related to peers making fun of her, grades, sickness, and worries about the safety of other people. Her treating clinician had been unaware of these. Her worries appeared clearly uncontrollable to her mother. Her symptoms first appeared at age four years.
Physical symptoms present during Alani's worry episodes included restlessness, feeling on edge, irritability, and problems sleeping. No functional impairments or accommodations were endorsed by her mother. Alani's clinician reported however that Alani experienced marked impairment due to her worries and severe restrictions on her activities, as her impairment was a central focus of the therapy.
Alani met all of the criteria for GAD, PTSD, and ODD. She improved markedly over thirteen sessions of psychotherapy.
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pmc-6174749-1
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A 58-year-old male presented to the emergency department via private vehicle with multiple complaints. Complaints included “chest discomfort”, low back pain, shortness of breath, generalized weakness throughout all extremities, and “numbness” of bilateral lower extremities. These symptoms began acutely at around 10 hours prior to arrival when he awoke from a dream. The patient stated during the dream that he was in an earthquake and his legs were trapped and crushed in the earthquake; when he awoke both of his lower extremities were numb and weak. He states that all of his symptoms are progressively getting worse and now he “can't move my legs.”
The patient's past medical history is significant for chronic back pain, anxiety, bipolar disorder, schizophrenia, major depressive disorder, and an episode of previous “paralysis.” The patient states in 1997 that he had a lumbar fusion and while in rehab he “became paralyzed and couldn't move my legs or walk” and that episode of weakness gradually improved and paralysis resolved without any medical intervention. The patient takes a total of 23 for his medical conditions that include zolpidem, methocarbamol, hydrocodone, carisoprodol, alprazolam, and gabapentin.
Vital signs at time of presentation are benign and reveal a temperature of 99.20 F, HR 68, BP 156/84, and Sating 95% on RA. General exam reveals a nontoxic patient in no acute distress, with a disheveled appearance. Neurological exam reveals that patient is alert and orientated X 3, with a GCS of 15, CN 2-12 intact, and 5+ bilateral upper extremity strength, normal finger to nose movement. Decreased bilateral patellar DTRs and decreased bilateral lower extremity strength 4/5. Otherwise physical exam was within normal limits.
A CBC, CMP, CK, sed rate, UA, UDS, Troponin-I, D-dimer, EKG, noncontrast CT's of head and C-spine, and CT of chest/abdomen/pelvis with IV contrast to evaluate aorta with spinal reconstruction were ordered. Pertinent labs results include normal Troponin and D-dimer, calcium 10, sed rate of 9, CK of 437, UDS positive for benzodiazepines and opiates (both of which are chronic medications prescribed to patient), and otherwise unremarkable labs and all imaging within normal limits.
The patient was now reevaluated. We discussed how his evaluation is unremarkable and cannot explain his symptoms. He responds with “Doc, it's getting worse and now I can't move my legs at all.” A repeat neurological exam revealed decreased sensation and bilateral lower extremity strength now 0/5. Pinprick sensation not detected and elicited no movement, despite pinprick drawing blood.
At this point, the evaluation had ruled out electrolyte abnormalities, acute myocardial infarction, pulmonary embolism, aortic dissection, acute CVA, spinal pathology, or tick paralysis as potential causes for the patient's acute weakness. The differential diagnosis continued to include Multiple Sclerosis, Myasthenia Gravis, medication reaction, conversion disorder, and Guillain-Barré syndrome. In light of the patient's psychiatric history, atypical complaints, disheveled appearance, medication list, and history of onset related to a dream in which his lower extremities were crushed, the overall clinical picture suggests a psychosomatic conversion disorder. However, this is a diagnosis of exclusion after CNS, peripheral nervous syndrome, infections, vascular, cardiopulmonary, and other potentially life threatening etiologies are excluded and therefore, the medical decision was made to proceed with lumbar puncture and CSF analysis, as well as MRI to fully rule out neurological pathology.
The procedure was complicated secondary to patient's history of lumbar fusion surgery from L4-S1 twenty years previously. Scar tissue at the L3-L4 spinal space made the procedure difficult and failed three times. Pt was informed of the difficulty and he states “please Doc, try one more time.” At the insistence of the patient, a fourth attempt at lumbar puncture was made and successfully returned CSF that revealed RBC 0, WBC 2, glucose 66, and protein 73.
Furthermore, an MRI was performed that revealed enhancement of bilateral nerve roots throughout entire thoracic and lumbar spine consistent with Acute Inflammatory Demyelinating Polyneuropathy (AIDP), a form of Guillain-Barré syndrome.
The patient was admitted to ICU, underwent 5 rounds of plasmapheresis, physical, and occupational therapy, improved, and was able to ambulate out of the hospital less than 2 weeks later. He never required intubation.
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pmc-6174750-1
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A 70-year-old woman underwent a right-sided mastectomy and axillary lymph node excision because of invasive adenocarcinoma in 2007. She received additional radiotherapy on the mastectomy site and the right axilla. Extensive lymphedema of the right arm developed and formed ecchymosis, which persisted despite a microsurgical lymphovenous derivation in 2009. On clinical examination in 2017, a painful nodular and purpuric transformation of the skin was noted. Because of increasing pain in the upper arm, a biopsy of the skin was performed, revealing a cutaneous epitheloid angiosarcoma. The patient was referred for magnetic resonance imaging (MRI) of the right upper arm to evaluate local extent.
Axial Tau Inversion Recovery (TIR) and T1-weighted images of the right upper arm (Figure and ) show a diffusely thickened cutis and subcutis with extensive lymphedema (arrowheads), as well as muscle edema (asterisk). There is an amorphous mass extending from the ventral cutis to the biceps muscle (arrows in Figure and ) with spicular infiltration reaching the neurovascular bundle. This mass has an intermediate signal on T1-weighted and a heterogeneous high signal on TIR images, and shows heterogeneous contrast enhancement (Figure ).
The patient was treated with an amputation of the affected arm. Follow-up CT examination revealed progressive disease with new subcutaneous and intramuscular metastases in the right hemithorax, and the additional follow up MRI revealed diffuse skeletal metastasis.
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pmc-6174754-1
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A 67-year-old female, with a typical presentation of carpal tunnel syndrome in the right hand, presented initially with numbness in digit IV and V a year later. A few months later, she developed numbness in all five fingers on the left side. Furthermore, the patient mentioned that she had had a burning sensation in both feet for a few years. Another few months later she complained of weakness in all four limbs and was sent for a neurological consultation with electromyography. Clinical examination revealed a Hoffmann-Trömner reflex on the left side, mild loss of strength in both hands, loss of vibratory sense and hypoesthesia in the distal end of all four limbs and loss of proprioception in both legs. The electromyography showed disturbed sensorimotor signals in the left hand and mildly disturbed sensorimotor signals in the right hand. The disturbed electromyography was attributed to carpal tunnel syndrome, which was presumably less severe on the right side due to treatment with long acting corticosteroid injections. The paresthesia in digit IV, digit V and both feet, however, could not be explained with the diagnosis of carpal tunnel syndrome. Therefore, magnetic resonance imaging (MRI) of the cervical spine was performed. Imaging showed an extra-dural soft tissue mass posterior to the odontoid process of the axis. The mass extruded through the transverse ligament of the atlas with severe compression of the myelum and myelomalacia at the level of C1 (Figures , and ).
A plain radiograph of the cervical spine was performed to check for atlanto-axial instability. The radiograph during flexion shows a slightly widened atlantodental interval, measuring 4 mm (normal value: <3 mm) (Figure and ).
Pre-operative imaging of the cervical spine also revealed an anatomical variant of the craniocervical junction, atlanto-occipital assimilation of the massa lateralis bilaterally (Figure ).
The patient was treated with laminectomy of C1 to decompress the spinal canal followed by posterior fixation of C1-C2 for stability.
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pmc-6174758-1
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An 85-year-old female with past medical history of recurrent deep venous thrombosis, pulmonary embolism on anticoagulation with a vena cava filter in place, rheumatoid arthritis, hypertension, hyperlipidemia, hypothyroidism, and type 2 diabetes mellitus presented to the emergency department with complaint of exertional dyspnea and chest pain. She denied fever, chills, or lower extremity edema and had no history of malignancy, weight loss, or night sweats. Initial vitals revealed BP of 86/62 mmHg that decreased to 79/60 mmHg with inspiration. Initial pulse was 95 bpm, and respiratory rate was 20. White blood cell count was mildly elevated at 11.5, troponins were normal, and electrocardiogram was unremarkable. D-dimer was elevated at 1290. Patient was sent for CT scan to evaluate for pulmonary embolism, and a moderate pericardial effusion was found (). Subsequently, an echocardiogram was done to further delineate the effusion which revealed a large circumferential effusion with mild respiratory variation concerning for impending cardiac tamponade (). She was admitted to the intensive care unit and underwent emergent pericardiocentesis. Flow cytometry of the pericardial fluid revealed a population of monoclonal B-cells with significant large cell component (). The overall morphologic and immunophenotypic features were consistent with high-grade B-cell lymphoma with t(8; 14) (). Bone marrow biopsy demonstrated monotypic B-cells compatible with the diagnosis of large B-cell lymphoma. The patient was started on rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) with an excellent initial response. She was transferred out of the ICU within days and discharged home for outpatient follow-up.
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pmc-6174764-1
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A 62 year-old male was diagnosed with yellow nail syndrome in 2000. He had a long history of recurrent sinusitis from 1983 and had developed numerous respiratory tract infections since 1996. Primary lower limb lymphoedema was diagnosed in 2000. Shortly thereafter he developed recurrent, initially right pleural effusions. Repeat thoracocenteses had revealed cloudy, thick fluid, exudative in nature. A right video-assisted thoracoscopic pleural biopsy was performed which showed chronic inflammation and reactive mesothelial changes but no malignancy. The diagnosis of yellow nail syndrome was made when xanthonychia developed in 2000. The clinical course continued with the development of bronchiectasis in 2003, complicated by recurrent chest infections and bilateral effusions.
The subject had a history of heavy prior tobacco smoking and had worked as a general manager, policeman, clothing design director, and dark room technician.
He died in 2010 following an infective exacerbation of his bronchiectasis.
A CT scan performed shortly before his death showed extensive right pleuroparenchymal disease, including right diffuse pleural thickening, bronchiectasis and right airspace shadowing ().
A postmortem examination was performed. External examination revealed the presence of yellow discolouration affecting the finger- and toenails (), along with bilateral lower leg oedema. Examination of the respiratory system showed extensive bilateral pleural adhesions, diffuse visceral pleural thickening and parietal pleural plaques (). Microscopical examination confirmed the presence of paucicellular hyaline collagenous plaques with ‘basket-weave' pattern, bilateral diffuse pleural fibrosis composed of similarly paucicellular collagen, and occasional lymphoid aggregates (). Septal lymphatics were noted to be markedly dilated (). A right-sided lobar pneumonia with organisation was present. Careful inspection of multiple lung sections by light microscopy failed to detect any asbestos body formation. There was a talc pleurodesis reaction in the right pleural space. Within the lung tissue bilaterally, remote from pleura, platy form polarisable material was seen consistent with talc particulates (). Other organ systems showed no significant abnormality.
Mineral analysis performed on tissue from the left lung by transmission electron microscopy and energy dispersive X-ray spectrometry detected: no commercial or noncommercial amphibole asbestos fibres or serpentine chrysotile asbestos. Fibrous mullite, a nonasbestos aluminium silicate, fibrous silica and muscovite were detected, confirming a technically successful analysis. Non-fibrous mineral analysis detected silicon, talc, aluminium, titanium, and iron.
Cause of death was recorded as yellow nail syndrome complicated by infection (lobar pneumonia) and diffuse pleural fibrosis (immediate cause).
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pmc-6174765-1
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This is a retrospective case study of a slim 37-year-old man exhibiting associated lifestyle risk factors (chronic marijuana and tobacco abuse, but neither alcohol excess, nor other illicit drugs) who suffered an acute thalamomesencephalic stroke, rapidly worsening to a comatose state.
Familial and personal medical history was negative for associated cardiocerebrovascular pathology or other specific risk factors.
In the evening that preceded the cerebral infarction, he submitted a large and elaborate tattoo over the left hypochondrium and abdominal (lumbar) flank and smoked a few cigarettes with cannabis.
The following morning, he experienced acute onset of dizziness, visual, speech, and gait disturbances.
He was admitted to the emergency room with walking difficulties, disturbed balance and coordination of movements, slurred speech, diplopia, confusion, and left palpebral ptosis. Neurological examination revealed right-sided severe ataxic hemiparesis, dysarthria, left palpebral ptosis and mydriasis, divergent strabismus, and fluctuating consciousness (Glasgow coma scale, GCS 10/15).
Blood tests (white blood cells count, hemoglobin, electrolytes, liver, and renal function) revealed normal results. Urine toxicology at admission was positive only for tetrahydrocannabinol; no other illicit drugs were present on tox screen. Electrocardiogram (EKG) and chest X-ray findings were normal. Clotting tests were normal [antithrombin III was 108% (>80%), homocysteine was 7.5 μmol (≤ 12), lupus anticoagulant was negative, antinuclear antibodies were 0.3 UM (<0.7), C protein was 117% (70-130)].
Emergent computed tomography (CT) scan on the day of admission showed no gross abnormality and no evidence of cerebral hemorrhage or encephalitis.
In a few hours he become comatose (GCS 7/15) and was transferred to the intensive therapy unit. Intubation and ventilation support were not necessary. EKG monitoring during admission in the intensive care unit did not revealed pathological aspects.
At about 20 hours after the onset of stroke, magnetic resonance imaging (MRI) of the brain and angiography (MRA) were also performed (). These revealed acute paramedian thalamic ischemic lesions extending to the rostral midbrain (asymmetrically, mainly on the left side). The imaging showed no evidence of cerebral venous occlusion, infiltrative neoplasm, severe infectious and inflammatory lesion, or a large embolus at the basilar tip, with stroke in the posterior circulation. MRA showed patency of the basilar and vertebral arteries, a normal appearance of the left P1 arterial segment and left PCA, and a right-sided full FPCA. Our 1.5-Tesla MRI device failed to visualize the TPAs; the left AOP was just presumed.
He recovered from a coma after 4 days and exhibited a slow, progressive evolution. Initially, he presented with severe alternating (superior) oculomotor hemiplegia (Weber syndrome), with left-sided oculomotor nerve palsy, a drooping eyelid and fixed-width pupil pointed down and out, diplopia, and dysarthria associated with contralateral severe ataxic hemiparesis.
Based on clinical and neuroimaging findings, the positive diagnosis was acute ischemic stroke in the territory of the left AOP. The clinical spectrum of the AOP infarct was outlined in the frame of a “thalamopeduncular" syndrome, associated with the typical symptoms of bilateral paramedian thalamic infarcts (confusion and coma), accompanied by with oculomotor disturbances, contralateral hemiplegia, and cerebellar ataxia.
After the acute episode, he was admitted on the neurorehabilitation department. He clinically manifested a paramedian midbrain syndrome, combining the previously described left-sided oculomotor impairment with moderate right-sided ataxic hemiparesis without hemianesthesia, tremor, dysmetria, dysarthria, and depression. Repeated EKG and blood tests and a transthoracic echocardiogram, respectively, did not reveal pathological aspects.
The diagnosis was established retrospectively, after a delay of 20 hours, too late for thrombolytic management. He initially received anticoagulant therapy (heparin for 3 weeks in the acute stroke department), followed by a novel oral anticoagulant for another 5 weeks during rehabilitation. He was discharged with small doses of aspirin, up to six months, as secondary prophylaxis. Statins were not administered, either in the acute or during the subacute stage.
He had a good evolutive trend and was discharged with a modified Rankin score (mRS) 3. Psychological evaluation emphasized a marked improvement of his masked depression and augmentation of the Mini-Mental State Examination (MMSE) score (from 23 to 29/30).
He completely changed his lifestyle, with abstinence from both tobacco and cannabis, and continued the rehabilitation program as an outpatient. He exhibited favorable outcomes, with no vascular recurrence.
Four months after the acute stroke he achieved a mRS 2 and was slightly disabled and still unable to carry out all previous activities, especially professional ones (driver). Most symptoms abated, except for slight visual blurring, diplopia, and residual left third cranial nerve palsy.
Favorable neurological results were consistent with repeated neuroimaging tests for control. Contrast-enhanced MR angiography remained unchanged. MRI showed no acute recurrences, but only small residual lacunae.
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pmc-6174767-1
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An 11-year-old girl presenting with involuntary movements in the face and extremities, clumsiness, and slurred speech was admitted to our hospital. She had no family history of neuropsychiatric disorders. Early psychomotor development was normal, although mild mental retardation was suspected at school. At age 10 years, she developed transient vocal tics. About 3 months before admission, she had episodes of choreiform movements. There were no events preceding these symptoms. The symptoms gradually worsened to include dropping eating utensils, and her body weight decreased by 4 kg in 3 months because of difficulty in eating. About a week before admission, she could not walk without assistance and did not attend school, because of gait difficulties. These symptoms were not observed during sleep. There was no indication of recent infection, and she had no history of fever during the 3 months before admission.
On examination, she exhibited notable choreoathetoid movements of the face and extremities. She was unable to walk without assistance. Muscle cramping in the cheeks and palpebrae-like tics were observed. She was alert and cooperative with the examiners, and her orientation was maintained. However, she exhibited emotional lability, sudden loud vocalizations, and resistance to restraint by caregivers. She showed severe irritability, and rage attacks were circumscribed. Muscle tonus and deep tendon reflexes were normal.
Blood testing showed no abnormalities. Antistreptolysin O titer (ASOT) (301.3 IU/ml; normal, 0–330 IU/ml) and thyroid studies on admission were normal, but ASOT was mildly elevated (414.6 IU/ml) at 8 days after admission (). GGS was isolated in a throat culture. Tests for rheumatoid factor, antinuclear antibody, and anticardiolipin antibody yielded negative results. Examination of cerebrospinal fluid (CSF) showed no pleocytosis or increase in protein level. Homovanillic acid (HVA) level in CSF was mildly elevated, at 80.9 ng/ml (normal, <50 ng/ml). Brain magnetic resonance imaging and electroencephalography findings were unremarkable. Serum IgM and IgG antibodies to lysoganglioside were positive. In SC and PANDAS, antilysoganglioside antibodies react with the neuronal cell surface because of a cross-reactive immune response with streptococcal antigen N-acetyl-beta-D-glucosamine (GlcNAc) [, ]. She was treated with oral ampicillin and intravenous immunoglobulin (IVIG) (400 mg/kg/day) for 5 days, and her symptoms eventually resolved. Her overall intelligence quotient on the Wechsler Intelligence Scale for Children, Third Edition was 50, which indicates mild intellectual disability.
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pmc-6174774-1
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A 4-year-old boy was treated for fever, mild cough, and nasal discharge at another hospital. Thereafter, he was diagnosed with influenza A infection and was treated with oseltamivir (4 mg/kg/day, 5 days). However, his condition deteriorated and fatigue, low activity, and breathing difficulty progressed; he was admitted to our hospital 7 days after the diagnosis of influenza A infection. He had no medical history of recurrent bacterial infections or growth failure. Physical examination at admission revealed remarkable respiratory distress and consciousness disturbance (Glasgow Coma Scale, E4V3M4). His body temperature was 38.0°C, blood pressure was 126/77 mmHg, heart rate was 155 beats/min, respiratory rate was 60 breaths/min, and oxygen saturation was 90% at room air. Right breath sounds were reduced, and an end-inspiratory crackle was detected in the right upper lung. In addition, red, cracked lips, strawberry tongue, and trunk and bilateral feet erythema were observed. Neither bilateral conjunctival injection, cervical lymphadenopathy nor edema was detected. Laboratory examination revealed a white blood cell count (WBC) of 20,000/μL with 95.1% neutrophils, hemoglobin level of 14.0 g/dL, and platelet count of 22.7 × 104/μL. Inflammatory biomarkers were elevated; C-reactive protein level was 20.54 mg/dl, procalcitonin level was 45.23 ng/mL, lactate dehydrogenase (LDH) level was 512 U/L, ferritin level was 261 ng/mL, and soluble interleukin-2 receptor (sIL-2R) level was 6,176 U/mL. The levels of several cytokines were also increased: IL-1β was 1.3 pg/mL; IL-6 was 233 pg/mL; IL-10 was 67 mg/mL; and TNF-α was 2.6 pg/mL, whereas IL-2, IL-3, IL-4, IL-5, and IL-12 were all normal. A chest X-ray and chest computed tomography revealed consolidation and a large-right pleural effusion (Figures and ). Based on these findings, the patient was diagnosed with sepsis and pleural empyema due to GAS infection.
The clinical course of the patient is shown in . Initially, continuous chest-tube drainage, intravenous administration of antibiotics (CTRX) and immunoglobulin (150 mg/kg/day, 3 days), and prednisolone (1 mg/kg/day, 3 days) were prescribed for sepsis and pleural empyema. The aspirated pleural fluid was serological with a yellowish brown color and pH of 7.002, WBC of 33,900/µL, protein of 4.7 g/dL, LDH of 9,121 U/L, and adenosine deaminase of 173.7 U/L (). No bacteria were detected by blood culture, whereas GAS tests conducted on both pleural fluids and throat swab on day 1 after culture were positive. The genotype of GAS was found to be emm1/speA/speB/speF, which is known as a virulence factor gene. Based on this data, the antibiotics were changed from CTRX to ampicillin (ABPC) and clindamycin (CLDM). However, fever and tachypnea persisted. Alternatively, the chest tube was considered as being obstructed or failing to drain, and hence, the chest tube was replaced, and an additional fibrinolytic therapy (urokinase 40,000 units in 40 mL 0.9 percent saline, intrapleural) was included; however, the patient's clinical condition still did not improve. On day 9, video-assisted thoracoscopic surgery (VATS) was performed to remove the thick fibrous septations. Thoracoscopy of the thoracic cavity revealed an adhered pulmonary and parietal pleura with fibrin; the cavity was then peeled off and washed using saline (). The patient's clinical condition improved following VATS, and he was discharged on day 33. All inflammatory biomarkers and cytokines had reduced to normal levels. At the time of the final follow-up, the patient had been healthy without any symptoms.
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pmc-6174775-1
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A 67-year-old female with a history of ovarian carcinosarcoma presented to the hospital with one week of headache and neck pain.
Her malignancy had been diagnosed one year prior to presentation after she had presented to her primary care physician with abdominal pain. Radiographic imaging at that time showed a large pelvic mass, and the patient subsequently underwent radical cytoreductive surgery which included total abdominal hysterectomy, bilateral salpingo-oophorectomy, and omentectomy. Pathology showed a focal left ovarian carcinosarcoma with metastases to the right ovary, omentum, and posterior cul-de-sac. The patient underwent six cycles of carboplatin and paclitaxel.
Eight months after completion of chemotherapy, the patient presented to her oncologist with new right pelvic pain. Pelvic imaging showed a new, deep right pelvic mass, and the patient underwent surgical resection which confirmed disease recurrence. The patient was set to begin localized radiation therapy and further chemotherapy when she developed headache and neck pain and presented to the hospital.
Upon current presentation, she noted an intractable bandlike headache and neck pain. Physical examination revealed normal vital signs, a normal mental status assessment, and a nonfocal neurological examination. She had restricted range of motion at the neck and midline point tenderness in the upper thoracic spine.
Laboratory testing demonstrated a normal complete blood count, normal renal function, and normal serum electrolyte levels. Magnetic resonance imaging (MRI) of the head and spine were obtained and showed a lytic mass centered in the left clivus and occipital condyle, as well as an expansile soft tissue lesion in the T4 spinous process (). A positron emission tomography-computed tomography (PET-CT) was also obtained (). In the setting of known ovarian recurrence, these findings were assumed to be metastases.
However, a 1.83 g/dL M-spike (reference range: 0.80–1.70 g/dL) was detected on serum protein electrophoresis, and a monoclonal gammopathy with immunoglobulin G (IgG) lambda monoclonal immunoglobulin was seen on immunofixation. Lambda free light chains were elevated at 49.1 mg/L (reference range: 5.7–26.3 mg/L), and kappa free light chains were borderline decreased at 5.3 mg/L (reference range: 3.3–19.4 mg/L). The free kappa to free lambda ratio was abnormal at 0.12 (reference range: 0.26–1.65). No M-spike was detected on urine protein electrophoresis. A biopsy of the T4 lesion showed a plasma cell neoplasm, and a bone marrow biopsy showed a clonal population of >10%, confirming the diagnosis of multiple myeloma ().
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pmc-6174776-1
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A 78-year old Caucasian male patient, without relevant medical history and in good health, presented to the emergency department with severe pain in the right hip after a high energy trauma due to a fall. On clinical examination, the right leg was shortened and externally rotated. There was no neurovascular deficit in the ipsilateral limb. A comminuted intertrochanteric hip fracture was diagnosed on a plain X-ray of the painful hip and pelvis (grade 31-A2 according to the AO classification) (). Intravenous pain medication was administered at the emergency department, and the patient was transferred to the operating room five hours after admission.
Closed reduction and internal fixation with a 170 mm 125° intramedullary nail (proximal femoral nail antirotation (PFNA; Synthes®), 11 mm diameter) were performed. The patient was placed supine on a fracture table with traction and the hip in adduction and internal rotation. The nail could be introduced without any particular difficulty. A 115 mm blade and a 38 mm distal locking screw were inserted with the use of the aiming arm. For distal locking, a drill sleeve, a protection sleeve, and a 4.2 mm calibrated drill bit (340 mm) were used. Drilling was guided, however not guarded. There were no intraoperative or immediate postoperative complications. Postoperative radiographs were satisfactory. Postoperatively, three weeks nonweight bearing were instructed because of the high energy impact of the trauma. Also, low molecular weight heparin (Enoxaparin 40, 1 subcutaneous injection per day) was administered for six weeks.
On clinical and radiographic checkup six weeks after surgery, no particular difficulties were noticed. The patient was able to walk with one crutch, there was no obvious swelling of the limb, and radiographs showed a good position of the intramedullary nail ().
Eight months postoperatively, the patient presented to polyclinical consultation because of a progressive swelling of the right thigh. There was no recent trauma, episode of fever, or illness. Clinical examination revealed a nontender diffuse swelling over the proximal part of the right thigh without well-defined borders, redness, or fluctuation. Peripheral pulses of the lower limbs were palpable, although stronger in the left limb, and capillary refill was normal. Mobilisation of the hip was painless. Ultrasound revealed a calcified old muscular hematoma localised medial to the femoral diaphysis. Additional X-rays confirmed the presence of a medial mass centred over the distal locking screw with calcification of the peripheral borders, suggestive for an (infected) hematoma. Compression and severe osteolysis of the medial border of the femur were seen (). Contrast-enhanced computed tomography (CT) was performed, showing an active extravasation in the hematoma which led to the diagnosis of pseudoaneurysm (). Laboratory tests showed a haemoglobin value of 11 g/dL and an elevated CRP (42 mg/L). Other laboratory parameters were within normal range. On retrospective analysis of the X-rays six weeks postoperatively, the additional mass could have been already noticed. However, because of the very subtle signs, it was misdiagnosed.
The patient was referred to the department of vascular surgery for open drainage. Preoperative findings showed a PFA lesion facing the distal locking screw which was managed by direct arterial suture. The screw was not visible and therefore left in place. No weight-bearing limits were made within the context of the scalloping of the medial femoral cortex because of patient's compliance and a good consolidation of the fracture. The patient was followed up regularly, and further evolution was uneventful.
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pmc-6174779-1
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Patient A was a 65-year-old female with history of dyslipidemia, anemia, and postural hypotension with syncope. She experienced palpitations once every three months, each lasting about six hours which spontaneously resolved. She was referred from her General Practitioner's clinic for fever with upper respiratory tract symptoms for two days and a few hours of palpitations. There was no chest pain or shortness of breath. Her physical examination was largely unremarkable apart from regular tachycardia and blood pressure 166/61 mmHg. Her arrival electrocardiograph (ECG) revealed regular narrow complex tachycardia (see ). She was placed in Trendelenburg position and instructed to take in deep breaths and subsequently hold her breath for five seconds before exhalation. She did this and converted to sinus rhythm (see ) within five breaths. She tolerated the HDDB maneuver well. The immediate postmaneuver BP was 171/64 and 30 minutes later it was 121/65. She did not have any complications and was later discharged from the emergency department (ED).
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pmc-6174779-2
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Patient B was a 68-year-old female who had a history of hypertension and dyslipidemia. She felt sudden onset of palpitations associated with chest pain about 45 minutes before ED arrival. She had chest pain which radiated to her right shoulder and neck. She experienced some sweatiness but had no dyspnea or fever. At the ED, she was alert and not in pain or distress. On examination, she was tachycardic with normal blood pressure. Her lungs had clear air entry. Her ECG revealed SVT (see ). She was subjected to the similar head down deep breathing (HDDB) maneuver as described above and converted to sinus rhythm without any complications. Her postmaneuver BP was 131/73. Her initial serum Troponin T was 10 ng/L (normal lab range 0-29 ng/L). However, in view of the earlier presence of chest pain with cardiovascular risk factors, she was admitted to cardiology for observation. Her subsequent Troponin T levels remained normal, and an echocardiogram the next day showed normal left ventricular (LV) ejection fraction and diastolic function, with no structural heart disease. She was discharged thereafter.
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pmc-6174791-1
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A ninety-year-old man with no significant medical history presented to the emergency department with abrupt onset of hemoptysis. The patient was hemodynamically stable. Lung exam revealed bilateral lung crackles. His chest X-ray showed left upper lobe opacity with widened mediastinum due to aortic dilatation (). CT of the chest revealed the aneurysmatic dilatation of the ascending arch and the descending aorta and pulmonary infiltrates in left upper lobe suspicious of aorto-bronchial fistula with bleeding in the lung (Figures , , and ). The patient was started on supportive care, including intubation and mechanical ventilation. Cardiology and Cardiothoracic Surgery were consulted. Cardiothoracic surgery agreed with the diagnosis of ascending aortic aneurysm and bronchopulmonary fistula; however due to his poor functional status and overall frailty the risk of surgery outweighed the benefits; hence surgical intervention was not pursued. During his second day of hospitalization the patient developed another episode of massive hemoptysis resulting in hypovolemic shock, and despite the best efforts of the medical personnel, the patient expired.
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pmc-6174797-1
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A 33-year-old Caucasian female with significant family history of inflammatory bowel disease (IBD) presents with profuse, bloody diarrhea for 5 days and associated tenesmus and urgency. One day prior to admission, she completed a one-week course of ampicillin for a urinary tract infection and noted that her symptoms began three days after she had initiated treatment. On presentation, patient was hemodynamically stable, afebrile, with mild lower abdominal pain, and a positive guaiac exam. Laboratory findings showed WBC 12.4 bil/L (normal values 3.3–10.7 bil/L), neutrophils 11.0 bil/L (normal values 1.6–7.2 bil/L), Hgb 13.1 g/dL (normal values 12.1–15.0 g/dL), platelets 275 bil/L (normal values 150–400 bil/L), lactic acid 1.4 mmol/L (normal values 0.5–2.2 mmol/L), and liver function tests within normal limits. Initial stool studies that included stool culture, ova and parasite, and Clostridium difficile toxin PCR were negative. A colonoscopy was planned as the patient had an extensive family history of IBD and presented with bloody diarrhea. Klebsiella oxytoca testing was requested on the stool culture after Clostridium difficile PCR came back negative, given her previous use of penicillins. Colonoscopy was notable for ulcerated mucosa with erythema and easy friability, suggestive of moderate colitis throughout the colon with rectosigmoid sparing (). Colonic biopsy was remarkable for mucosal congestion and ischemia suggestive of ischemic colitis (). Subsequently, requested stool culture was positive for many Klebsiella oxytoca. The patient's hematochezia resolved prior to discharge on day 3 of hospitalization, four days after cessation of ampicillin. She was advised to avoid future use of penicillins and minimize nonsteroidal anti-inflammatory drug (NSAID) use.
The patient has continued to follow with her gastroenterologist 10 months after her colonoscopy. She has had epigastric abdominal pain relieved by daily omeprazole. She no longer has documented hematochezia and there has been no repeat colonoscopy.
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pmc-6174802-1
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A 58-year-old female presented to the emergency department with intermittent, crampy right-sided abdominal pain, nausea, and vomiting, which began approximately 18 hours previously. Her past medical history was significant for hypertension and her surgical history included a thyroidectomy for treatment of thyroid cancer and a Caesarean section. A contrast enhanced CT abdomen and pelvis was obtained, demonstrating multiple fluid-filled, dilated small bowel loops in the right abdomen, which were predominantly anterolateral to the ascending colon and cecum (). In addition, two transition points were identified in the right lower quadrant, with one transition point at the distal ileum just proximal to the cecum and a second transition point in the proximal ileum. The two transition points were in close proximity to each other, indicative of closed loop obstruction. Decreased wall enhancement of the dilated small bowel loops was concerning for ischemia. Mucosal hyperenhancement of the ileum at the proximal transition point was felt to relate to ischemia or decompressed state (). Given the patient's symptoms and findings of closed loop obstruction on CT, the patient was taken to operating room. In the operating room, an internal hernia with closed loop obstruction was confirmed and resulted from herniation of small bowel through an adhesion of a transverse colon epiploic appendage to the ascending colon mesentery. The herniated small bowel was nonviable and a total of 60 cm of small bowel was resected (). Retrospectively, kinking of the ascending and transverse colon could be seen on the initial abdominal CT and was felt to correspond with the site of adhesion ().
After resection, the patient's small bowel was left in discontinuity and an abdominal wound-vac was placed. The following day, the patient returned to the operating room, at which time the terminal ileum was also found to be nonviable. An ileocecectomy with enterocolonic anastomosis was performed.
The patient had a complicated postoperative course, but was ultimately discharged approximately two weeks after the initial surgery.
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pmc-6174808-1
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A morbidly obese 43-year-old female with a history of hypertension, dyslipidemia, and diabetes mellitus presented with severe abdominal pain. She had an episode of acute pancreatitis one year ago. She complained of right upper quadrant pain radiating to the back over 6-hour duration alongside six episodes of vomitus. A review of systems was only notable for a headache and dizziness. She reported no family history of dyslipidemia or acute pancreatitis. She denied tobacco, alcohol, or illicit substance use. There was no history of gallstones, appendectomy, new medications, procedures (including ERCP), or any complications related to her diabetes.
Admission vitals revealed afebrile patient with a heart rate of 103/min, respiratory rate of 20/min, BP 116/62 mmHg, and oxygen saturation of 96% on a nasal cannula at 5 liter/min. The patient was alert and orientated but was in moderate distress. The abdomen was obese and soft and with tenderness in the epigastric region. There was no guarding, rigidity, or Murphy's sign. Her body mass index (BMI) was 47.1. Other systemic signs of elevated triglycerides including xanthelasma, corneal arcus, and tendon xanthoma were absent.
Initial laboratory investigations showed an elevated white cell count of 16.9 μ/L (4.5 -11 μ/L), haemoglobin 12.2 g/dL (12-16 g/dL), platelet count 368000 mm3 (130,000-400,000mm3), sodium 129 mEq/L (136-144 mEq/L), potassium 3.8 mEq/L (3.5-5mEq/L), anion gap of 2 (8-16), BUN 45 mg/dL ( 7-20 mg/dL), creatinine 0.6 (0.4-1.3), glucose 206 mg/dL (74-117 mg/dL), and serum calcium 7.9 mg/dL (8.5-10.2 mg/dL). Liver function tests showed total bilirubin 0.5 mg/dL (0.1-1.2mg/dL), aspartate aminotransferase 16 IU/L (8-46 IU/L), alanine aminotransferase 11 U/L (7-55 IU/L), total protein 8.5 g/dL (6.1-7.9 g/dL), albumin 4 g/dL (3.5-5.5 g/dL), and PT 11.6 seconds (9.8-13.4 seconds). Initial arterial blood gas (ABG) analysis showed pH 7.5, pCO2 37.5, and pO2 67 at FiO2 of 40%.
The patient had an elevated lipase of 4143 U/L (reference 22-51 U/L), total cholesterol of 694 mg/dL (100-199 mg/dL), and a triglyceride level of 600 mg/dL (reference 0-149 mg/dL). Computed tomography (CT) of the abdomen showed diffuse enlargement of the pancreas consistent with pancreatitis (). There was no evidence of gallstones or biliary sludge which was also confirmed by an ultrasound abdomen. Other competing etiologies including alcohol, autoimmune pancreatitis, abdominal trauma, pancreatic divisum, sphincter of Oddi dysfunction (SOD), viral infection, drugs, and toxins were all ruled out, making the most likely diagnosis hypertriglyceridemia-induced AP (HTG AP).
The patient was treated with boluses of intravenous (IV) normal saline and supportive care. On the second day of admission, the patient developed hypotension and had persistent tachycardia. She likely developed acute respiratory distress syndrome (ARDS) from complications of acute pancreatitis. Fluid overload was ruled out with a sonogram of the inferior vena cava (diameter of 1.5 cm), a central venous pressure of 6 cm of H2O, and echocardiography indicating left ventricular ejection fraction of 55% with no left ventricular diastolic dysfunction. The patient was subsequently transferred to the intensive care unit for hypovolemic shock and respiratory distress requiring intubation and mechanical ventilation. As per revised Atlanta classification, she was categorized as severe acute pancreatitis based on Marshall scoring system for organ failure with a score of 3, with a score of 1 from respiratory failure (PaO2/FiO2 of 240) and two from cardiovascular (systolic BP <90, not fluid responsive). Patient's fasting blood glucose was 288 mg/dL (74-117 mg/dl), and diabetic ketoacidosis (DKA) was ruled out with the absence of serum or urine ketones, no elevated anion gap, and no acidosis. ABG analysis showed pH of 7.5, bicarbonate of 23, and an anion gap of 2.
Following the patients decline into shock, an ABG indicated an acidic pH of 6.99, PCO2 9.4, PO2 134, and HCO3 of 5.4. The serum bicarbonate level was low at 15mmol/L and the calculated anion gap was elevated at 22 (normal 8–16). Lactic acid level was 3.8 mmol/L (0.5-1.9 mmol/L).
Repeat abdominal contrast-enhanced CT on the third day showed marked pancreatic and peripancreatic infiltration consistent with AP and no signs of necrosis. Continuous insulin infusion was started for HTG from day 1, lowering her triglyceride levels down to 247 mg/dL over five days as shown in . She was also started on a liquid diet via nasogastric tube from day 5. After nine days in the ICU, the patient was successfully extubated and was switched to oral diet on day 11, which was gradually advanced as tolerated. She was downgraded to the floor on the same day and discharged on day 17. The patient required nasal oxygen to maintain saturation as she was recovering from ARDS. She was discharged on gemfibrozil 600mg twice daily to prevent further episodes of HTG AP. Patient has been followed upon discharge and remains compliant with gemfibrozil, leading to a controlled triglyceride level of 123 mg/dL and no further episodes of AP.
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pmc-6175182-1
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A 50-year-old male presented with a rapidly enlarging lesion on his back. He had multiple skin lesions affecting most of his upper body, arms, and face (Figure A). The patient had first noticed skin tumors around the age of 18 years, and had multiple lesions removed, which were confirmed as cylindromas and spiradenomas. He was severely affected with multiple scalp tumors, such that he underwent total scalp excision with skin grafting. He had affected relatives, and sequencing of the CYLD gene in peripheral lymphocyte DNA revealed a novel pathogenic heterozygous truncating mutation (c.2476C>T; p.Gln823*) (Figure B), consistent with a diagnosis of CCS. The patient underwent surgical excision of the lesion on the back, which showed low-grade spiradenocarcinoma. Two years after this a further enlarging tumor was excised from his suprapubic skin and confirmed to be a primary spiradenocarcinoma. Five years since his first spiradenocarcinoma, neither lesion has recurred.
Histopathology of the tumor from his back showed an ulcerated spiradenocarcinoma arising in a spiradenoma. The spiradenocarcinoma was characterized by increased cellularity and absence of the dual cell population seen in spiradenoma. The neoplastic cells were arranged in nodules and had minimal cytoplasm, and some showed a slightly spindled morphology (Figure A,B). The ductal structures (highlighted by carcinoma embryonic artigen staining; data not shown) appeared compressed and pushed to the periphery. There was loss of the diffuse infiltrate of small lymphocytes. Within the neoplastic nodules, the cells showed increased mitotic activity (15/10 hpf). The spiradenoma in comparison was characterized by a dual population of cells arranged in trabeculae. The cells were a mixture of small basaloid cells with small dark nuclei, and a second cell type with a larger more irregular vesicular nucleus and more cytoplasm. Mitotic activity was very low in the benign component (1/10 hpf).
Immunohistochemistry for p63 and SMA was performed on spiradenoma and spiradenocarcinoma. p63 showed strongly positive cells closely associated with occasional weak p63 staining cells in spiradenoma and showed uniform but weak p63 staining in spiradenocarcinoma (Figure A,B). SMA showed a mixture of SMA positive cells and SMA negative cells diffusely throughout the lesion in spiradenoma, and nodules of SMA negative cells with SMA positive cells compressed at the periphery in spiradenocarcinoma (Figure A-C). These nodules were most easily recognized on low power magnification (Figure A). Similar features were seen in the spiradenocarcinoma arising in the suprapubic skin.
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pmc-6175201-1
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A 43-year-old female patient known to have a past medical history of depression, anxiety, and who used an etonogestrel/ethinyl estradiol vaginal ring for contraception, presented to the emergency department (ED) with new onset witnessed grand mal tonic-clonic seizure lasting at least two minutes with post-ictal confusion. The patient denied any previous seizure history. She did report drinking alcohol occasionally and had ingested two alcoholic drinks the previous evening. There was no associated trauma. Her vital signs on admission were: temperature 36.2ºC, pulse 119 beats per minute, respiratory rate 25 breaths per minute, blood pressure 140/105 mmHg, SpO2 99% on room air, and body mass index of 33.7 kg/m2. A thorough review of systems was negative other than nausea, diarrhea, and seizure. Physical examination, including a full neurological examination, was unremarkable. Laboratory data included: potassium 3.2 meq/L, bicarbonate 15 mmol/L, glucose 171 mg/dL, hemoglobin 11.5 g/dL, hematocrit 33.7%, platelet 134,000/mm3.
Computed tomography (CT) head without intravenous contrast showed trace right parieto-occipital extra-axial collection and parenchymal hemorrhage of the right parieto-occipital and temporal regions, with an adjacent subarachnoid hemorrhage (SAH) (Figure ). The acute right parieto-occipital intraparenchymal hemorrhage with scattered adjacent SAH was secondary to an extensive acute thrombosis of the right venous sinuses (transverse, sigmoid, and jugular). The patient was admitted to the intensive care unit (ICU) for further evaluation of her brain hemorrhage. She was started on nicardipine drip for a target systolic blood pressure of <140 mmHg and kept on hemorrhagic stroke protocol.
Neurology was consulted, and further evaluation included magnetic resonance angiogram (MRA) of the head without contrast. Findings were consistent with right transverse dural venous sinus thrombosis (Figure ). Brain magnetic resonance imaging (MRI) with and without contrast showed intraparenchymal hemorrhage of the right parieto-occipital and temporal regions secondary to venous thrombosis. Patchy meningeal enhancement is seen at the right parieto-occipital and temporal regions, likely related to venous stasis versus reactive changes secondary to thrombosis. Also, a small focus of hyperintense signal is seen on isotropic diffusion-weighted and fluid-attenuated inversion recovery (FLAIR) sequences in the left splenium of the corpus callosum without significant associated decreased signal on apparent diffusion coefficient (ADC) maps, this is likely due to subacute ischemia (Figure ).
Additionally, MRI venogram of the head without contrast showed a complete absence of flow-related enhancement in the right transverse and sigmoid sinuses and right internal jugular vein consistent with high-grade thrombosis of the right transverse sinus (Figure ). Neurosurgery service was also consulted and recommended no surgical intervention.
On the third day of admission, after starting heparin drip, the patient complained of right ear fullness and increasing headache. A repeat head CT scan without IV contrast showed enlargement of the intracranial hemorrhage (Figure ). Heparin infusion was held, with neurological assessments completed every hour. The patient was reassessed by neurosurgery and neurology teams, both of which recommended resumption of anticoagulation and starting warfarin for bridging with target international normalized ratio (INR) of 2-3. Once stable, the patient was transferred to a neurological center for further close follow-up and evaluation. The patient improved clinically over the following days and the cerebral hemorrhage improved on repeat CT imaging (Figure ).
The patient was discharged home on warfarin with a plan to continue for a minimum of six months. On follow-up, six months later, the patient reported complete resolution of symptoms and CT head without contrast revealed significant improvement in the size of intracranial hemorrhage (Figure ).
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pmc-6175202-1
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A 68-year-old female presented with gradual onset of negative sensory symptoms like numbness, and weakness particularly on extremities bilaterally. She also had some balance problems for the same time duration. On neurological examination, there was diffusely reduced muscle strength of 4/5 on Medical Research Council (MRC) muscle power grading scale, along with the reduced perception to all modalities conducting either by dorsal column lemniscus or spinothalamic pathway. She also had some loss of sense of vibration and sense of proprioception peripherally. Moreover, there was a generalized hyporeflexia and gait examination showed a positive Romberg sign. On further inquiry, there was no previous history of similar symptoms or recent history of having any upper respiratory tract infection or diarrhea. There was no history of recent travel. Her current medications included losartan (50 mg) for her blood pressure control and the multi-vitamins. Her blood pressure was under control and lab results from the medical record of last year were normal. Initial workup for her unexplained neuropathy included serum TSH, vitamin B12, HbA1c along with routine baseline laboratory investigations, to rule out the more prevalent causes of these symptoms. These laboratory tests turned out to be normal. The patient then developed acute urinary incontinence and severe orthostatic hypotension. She also developed symptomatic bradycardia, severe enough to place a temporary pacemaker to relieve her symptoms.
Meanwhile, further workup was ordered which showed M spike on serum electrophoresis with IgM kappa on immunofixation. IgM titers were surprisingly high; 568 mg/dl (normal 40–230 mg/dl). Initially, the probable diagnosis was monoclonal gammopathy of undetermined significance (MGUS) related neuropathy. Hematological workup was then extending, which revealed anti-MAG antibody titers >1:102400 (normal < 1:1600). Bone marrow biopsy showed small atypical lymphoid cells which stained positive for CD20, PAX-5, with rare CD138 positive plasma cells. These findings were consistent with a small B-cell lymphoproliferative disorder. She is currently being treated with rituximab with significant improvement in her neuropathic symptoms. Acute autonomic symptoms can be a rare [] and a confusing clinical manifestation of anti-MAG neuropathy.
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pmc-6175203-1
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A 70-year-old Caucasian female presented to an outpatient Partial Psychiatric Hospital Program (PPHP) after an episode where the patient was reported missing, and subsequently found by the police hiding under a tree with minimal clothing in freezing cold temperatures. The patient’s brother and sister-in-law, whom she resides with, were unable to locate her, and reported her missing to the police. Upon discovery by the police, the patient agreed to attend the outpatient PPHP. Upon admission, the patient explained that she ran away from her home due to feelings of guilt and “feeling like a burden to her family.” She then revealed that her feelings of guilt are primarily regarding her perceived parasite infestation, which she states has plagued her for decades. The patient states there are “little white bugs crawling in and out of my skin.” She further explained that they are difficult for her to capture because they dive deep into her skin. The patient expressed fear that her family members will also become infected, prompting her recent episode of escaping her home. She also mentioned feeling embarrassed about the issue, particularly because she is from a rural town and feels everyone will know.
The patient admits she has seen several healthcare providers including her primary care provider, parasitologists, and dermatologists, all reporting negative findings. When explaining this, the patient became very agitated and repeated, “I’m not crazy, but no one believes me.” Additional past medical history was benign other than the patient revealing she underwent an abortion at the age of 35. She does not link the parasite infestation with this event, but does express guilt over this decision.
The patient denied a history of physical or sexual abuse. She denied alcohol or drug abuse. She has never been married and has no children. Upon exam, the patient looked appropriate and stated her age. She maintained eye contact and spoke with coherence. The patient was anxious. Immediate retention and recall, recent memory, remote memory and fund of knowledge appeared to be fair. Insight and judgment were poor. The patient was not impulsive. The patient's current medications include Zyprexa 2.5 mg per os (PO) at bedtime and Zoloft 75 mg PO daily. A toxicology screening of the patient was negative.
From reviewing the medical records, it was elucidated that the patient was admitted to the Behavioral Health Unit two times previously with the same complaint of parasite infestation. The patient could not pinpoint when the supposed infestation began, but does state that it had been many years, possibly 15 years.
When questioned why the patient was at the PPHP, she states that after she was found by the police, she was taken to the Emergency Department (ED) and was medically cleared, but her family demanded she attend the outpatient PPHP because of her delusions. We were also able to ascertain that a computed tomography (CT) scan was taken in the ED, which demonstrated microvascular ischemic changes and cortical atrophy.
On completion of the interview, the patient was observed in group therapy, and a decision was made that the patient was not a good candidate for the PPHP, as her delusions were too severe and becoming disruptive to the group. At the request of her family, she was then transferred to a free-standing private psychiatric hospital for further evaluation and treatment. She currently remains there and is undergoing treatment.
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pmc-6175253-1
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A 32-year-old female with history of diabetes mellitus type one and a successful RYGB for morbid obesity three years ago presented to our clinic with the complaints of cough, greenish yellow sputum production, chills and night sweats for the last three months. She was feeling more fatigued, generalized weakness and unintentionally lost 33 pounds during that time. She denied recent history of travel out of state. The patient was in the United States and had never been to a country with endemic tuberculosis. She denied recent remote history of incarceration. She also denied hemoptysis, shortness of breath, headache or fever. She had no history of being diagnosed with TB or history of recent sick contacts. On physical examination, her temperature was 36.9°C, blood pressure was 116/60 mm Hg, pulse was 84 per minute and respiratory rate was 16 per minute. On chest auscultation, few rhonchi were present in the right upper lung and the rest of the physical examination was unremarkable. The Initial blood work showed sodium of 134 mmol/L (136–145 mmol/L), potassium of 4.2 mmol/L (3.5–5.1 mmol/L), bicarbonate of 28 mEq/L (23–31 mEq/L), blood urea nitrogen (BUN) of 6 mg/dL (9–21 mg/dL), creatinine of 0.33 mg/dL (0.6–1.1 mg/dL), glucose of 150 mg/dL (80–115 mg/dL) and liver function tests were within normal range. Her white blood cell count was 14,500/µL (4500–11000/µL) with 81% neutrophils. Her chest X-ray showed multiple small nodular opacities throughout the right lung with a probable cavity in the right lung apex (Figure ). Suspicion was raised for possible active TB and she was admitted in airborne isolation. Two peripheral blood cultures, sputum culture along with acid-fast bacilli (AFB) were ordered. Computed tomography (CT) chest without contrast showed multiple thick-walled cavities in the right upper lobe. The largest cavity was 7 cm. There were innumerable centrilobular nodules and tree in bud opacities throughout the right lung (Figures , ). Mycobacterium tuberculosis bacillus was detected by sputum AFB staining and later confirmed by culture and polymerase chain reaction (PCR) assay. She also had a positive QuantiFERON test. She was started on anti-tuberculosis treatment (ATT) medications including Isoniazid 300 mg daily, Rifampin 300 mg twice daily, Ethambutol 800 mg daily, Pyrazinamide 1000 mg daily and Pyridoxine 50 mg daily for the first two months and then two drugs Isoniazid and Rifampin for the next four months. After three days of hospital stay, she started showing some improvement in her symptoms such as cough and night sweats. She understood the isolation precautions, especially respiratory precautions and use of mask in the presence of other individuals. She was later discharged in stable condition with the above-mentioned medications. She had complete resolution of her symptoms in two months and completed six months course of ATT.
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pmc-6175255-1
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A 49-year-old female with a history of acromegaly, status post-transsphenoidal pituitary resection three weeks prior, presented to the emergency room with a headache and clear nasal discharge present since the removal of a nasal splint. The patient described the cephalgia as severe, intermittent and throbbing, exacerbated by standing up and coughing and relieved by lying down and acetaminophen. On physical examination, her vital signs were normal and the results of neurological examination were normal, but a minimal clear nasal discharge was noted. A computed tomography (CT) scan of the head showed multiple air loculi in the basal cisterns, lateral, third and fourth ventricles and numerous air-filled spaces also scattered in the brain. No mass effect or midline shift was seen (Figure ).
Initial management consisted of bed rest in the Fowler position at 30° and instructions to avoid Valsalva maneuver such as analgesia, coughing, and sneezing. Besides the supportive treatment, the headache worsened, and a repeat CT scan showed mild increased diffuse PNC with intracranial air loculi in the parafalcine region, anterior horn of the left ventricle, posterior fossa, and left middle fossa. There was also an increased amount of air in the posterior fossa causing a mass effect on the pons (Figure ).
She underwent a transsphenoidal endoscopic exploration of the sphenoid and sellar floor, with septoplasty and packing of the sphenoid sinus with abdominal fat graft, and with the insertion of a lumbar drain. After these procedures, she showed a significant improvement of her symptoms, the lumbar drain was removed after five days, and her headache and nasal leakage resolved. She was discharged on day 10 of hospitalization. At the time of follow up, the patient was free of symptoms, and repeat CT-scan revealed resolved PNC (Figure ).
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pmc-6175263-1
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A 60-year-old male presented with growth over the glans penis for two months associated with pain. There was no history of contact bleeding. The patient was not circumcised. He had noticed a swelling on the scrotum for the past six months. On examination, there was a 2 x 3 cm ulceroproliferative growth over the glans extending over the corona onto the shaft of penis. The ulcer was fixed to the ventral aspect of glans penis with induration proximally extending up to mid shaft of penis (Figure ).
Bilateral inguinal lymph nodes were palpable, 3-4 on each side, 0.5 cm in size, firm and mobile in nature. The patient also had a right-sided primary vaginal hydrocele. A clinical diagnosis of SCC of penis was made and a wedge biopsy done. Histopathological analysis revealed an ulcerated stratified squamous epithelial lining with underlying lesion composed of fascicles of spindle cells with moderate pleomorphism. These were very few in number to proceed with IHC stains. A possibility of a primary sarcoma of penis was suggested.
Fine needle aspiration cytology (FNAC) was done from bilateral inguinal nodes which revealed reactive hyperplasia. All blood parameters, liver and renal function tests were normal. The chest X-ray and ultrasound abdomen were unremarkable.
Total penectomy with perineal urethrostomy was performed. The hydrocele was aspirated under aseptic precautions, three days before surgery. Post-operative course was uneventful. The patient was discharged on the eighth post-operative day and was advised to follow up with the regional cancer center for further management.
The final histopathological examination revealed moderate pleomorphic spindle cells arranged in sheets of fascicles, the overlying squamous epithelium was dysplastic. Focal areas of necrosis were seen. No definite feature of keratinization was seen (Figure ).
The tumor cells were strongly positive for pancytokeratin and vimentin, and negative for epithelial membrane antigen (EMA), neuron-specific enolase, CD 34, S100, Melan A and HMB 45 (Figure ).
The final report was sarcomatoid carcinoma (spindle cell carcinoma) of penis involving the glans. The urethra, proximal resected skin and shaft were free of tumor.
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pmc-6175264-1
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A 55-year-old male with a history of multiple cerebral cavernomas presented to the emergency department of an outside hospital for back pain radiating to the right chest region. He attributed his pain to muscle strain associated with fixing a popup camper. An extensive workup was done and was negative except for magnetic resonance imaging (MRI) of the spine. This showed evidence of an intradural extramedullary lesion at the T3-T4 level, located dorsally and directed rightward. The lesion appeared hyperintense on T1 and T2 with compression of the cord (Figure and Figure ).
The patient was referred to the neurosurgery clinic, where he had been seen one month prior for decreasing dexterity of the left hand of one year’s duration. The past medical history was significant for seizures beginning at age 15, for which the patient underwent separate partial resections of the right frontal and temporal lobes. Multiple new cavernomas had been found following a breakthrough seizure at age 50. When the patient first presented to the neurosurgery clinic at age 54, he reported a decrease in left-hand dexterity. The only interval change in the MRI at the time was an enlargement of a right porencephalic cyst in the context of the patient’s previous surgeries.
Neurological exam revealed right-sided hyperreflexia but no weakness of the upper or lower extremities. He was diagnosed with thoracic myelopathy. Given the symptomatic presentation with severe radiculopathy and cord compression, the patient was offered surgery.
Under general anesthesia with neuromonitoring, a T3-T4 hinge laminotomy was performed. Ultrasound was used to confirm the location of the lesion within the dura. Under the magnification of the operating microscope, a curvilinear durotomy was performed. The lesion was hemorrhagic and highly friable. It appeared to be attached to the T3 dorsal nerve rootlets. Gross total resection was achieved in a piecemeal fashion using tumor forceps. After the tumor was mobilized off the spinal cord and the inner surface of the dura, it was seen to have left an impression on the thoracic spinal cord in the T3-T4 area. The durotomy was then closed in a water-tight fashion and the wound was closed in multiple layers. No complications were noted during the procedure and no changes were seen on neuromonitoring. Postoperative scans demonstrated no residual lesion (Figure ). The patient was relieved of his mid-back or thoracic radiculopathy and remained neurologically at baseline immediately post-operatively and at six months.
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pmc-6175265-1
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We report a 24-year-old Caucasian woman who presented to the hematology clinic with a history of easy nontraumatic bruising on both thighs and legs. She had a recent history of fall, with bruises on the left hip, bilateral arms, and the lower left quadrant of the abdomen. Her family history was negative for easy bruisability, bleeding, or clotting disorder. She denied any bleeding gums, heavy menstrual blood flow, nasal bleeds, blood in stool, or blood in urine. She also had an unexplained loss of appetite and loss of weight over the last six months. Her medication history included inhaled albuterol as needed. She has not had any surgeries in the past. She smokes a pack of cigarettes per day and drinks a pint of vodka every night as well as three cans of beer per week. She is unmarried and has no children. She is sexually active with male partners. Her maternal grandmother had lung cancer.
The physical examination was unremarkable except for few bruises on bilateral thighs. The computed tomography (CT) scans of the chest, abdomen, and pelvis were ordered to screen for her unexplained weight loss which was unremarkable. The complete blood count, comprehensive metabolic panel, factor VIII, prothrombin time and partial thromboplastin time, D-Dimer, and antithrombin activity were normal. Her von Willebrand factor activity was much lower compared to the antigen (Table ). From the reports, it was established that her von Willebrand factor activity was 20% (reference range is 40%-163%) whereas the antigen was low normal at 45% (reference range is 45%-150%) and the ratio is less than 0.5 (normal ratio is more than 0.7). Repeat testing in a week consolidated the previous findings with the factor activity and the antigen as less than 19% and 37%, respectively. The reports of her von Willebrand factor multimer analysis were normal. The platelet count was normal.
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pmc-6175266-1
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In December 2012, a 70-year-old woman presented with the chief complaint of “shaking” head movements. The movements began one month previously, shortly after she began taking hydroxychloroquine for seropositive rheumatoid arthritis (RA).
In addition to RA, the patient had a past medical history of atrial fibrillation, cardioembolic cerebrovascular accident, two transient ischemic attacks, Sjögren’s syndrome, pseudogout, calcium pyrophosphate disease, osteoarthritis, pacemaker placement, left-sided multinodular goiter, osteoporosis, chronic hypertension, heart failure with preserved ejection function, recurrent bilateral lower extremity deep vein thromboses, iron deficiency anemia, anxiety disorder, major depressive disorder, and dyslipidemia. She did not smoke tobacco, drink alcohol, or use illicit drugs. She danced once or twice a week to maintain physical fitness. Her family history was not contributory. She did not have allergies. Her medications included atorvastatin, carvedilol, cyclosporine, docusate, ergocalciferol, ferrous sulfate, folic acid, furosemide, losartan, melatonin, methotrexate, omeprazole, polyethylene glycol, prednisone, hydroxychloroquine, and warfarin.
Blood pressure was 135/72 mmHg, temperature was 97.7 Fahrenheit (38.6 Celsius), and body mass index was 41.05 kg/m2. She was alert and oriented to person place and time, and not in acute distress. Cranial nerves 2–12, and sensation to pinprick, vibration, and joint position were intact. Reflexes were 2+ at all the tendons, and strength was 5/5 in all the extremities. Gait was ataxic and she had choreiform movements affecting her head, upper extremities, and lower extremities. There was some dysmetria with finger-to-nose testing. The heart had normal rate and rhythm with a holosystolic murmur in the aortic region. The pulmonary, abdominal, and integumentary exams were unremarkable.
Arrangements were made to contact the patient's rheumatologist to determine if the head movements were related to hydroxychloroquine and obtain medical records from outside facilities. Results from a computed tomography (CT) scan of the head performed elsewhere four months previously were unremarkable except for some abnormalities around the pituitary region.
By February 2013, the truncal ataxia and abnormal head movements were still present. Hydroxychloroquine was halted, and rituximab was started, in case the former medication was the cause of her abnormal movements; however, the movements continued. Complete blood count (CBC), complete metabolic panel (CMP), thyroid function tests (TFTs), parathyroid hormone, lipid panel, vitamin B12 level, human immunodeficiency virus (HIV) screen, rapid plasma reagin, iron panel with ferritin, and creatine kinase (CK), were all within the normal limits. Antinuclear antibody, anti-Ro antibodies, rheumatoid factor, and anti-cyclic citrullinated peptide were positive; anti-La antibody was negative. A paraneoplastic antibody panel demonstrated P/Q and N-type V-G calcium channel antibodies. Table shows the full results of the paraneoplastic antibody panel. By November 2013, the cerebellar ataxia had resolved; however, the patient reported occasional sensations of feeling like she was “on a ship” and had some difficulties maintaining her balance.
In January 2014, the cerebellar ataxia returned prompting a workup for malignancy. Based on the US Preventative Task Force's recommendations about age-appropriate cancer screening for a 70-year-old-female a colonoscopy and screening mammograms were ordered. The colonoscopy was only remarkable for diverticulosis. Bilateral screening mammograms (Figures -) and ultrasounds done in April 2014 identified a 2.1 × 2.0 × 1.7 centimeter (cm) mass and a 0.7 × 0.5 cm hypoechoic area at the upper outer quadrants of the left and right breasts, respectively. Two months later, bilateral core needle biopsies confirmed bilateral breast cancer.
In September 2014, she presented again with complaints of intermittent abnormal movements of the head, with choreiform and athetoid characteristics; the movements were absent at rest and present with movement. In the same month, she underwent total bilateral mastectomies. The left breast specimen was 2.2 × 1.6 × 1.4 cm with a negative sentinel lymph node, and that from the right breast was 1.1 × 1.0 × 0.6 cm, with three negative lymph nodes. Histopathology revealed mixed invasive ductal and lobular carcinoma. Immunohistochemistry was weakly estrogen receptor (ER) positive, weakly progesterone receptor (PR) positive, and human epidermal growth factor 2 (HER2) negative. Antigen Ki-67 results predicted a low risk of recurrence. Tumor protein P53 was positive in the left breast but negative in the right breast.
In April 2015, she continued to have intermittent episodes of abnormal movements that also were unresponsive to trails of gabapentin and clonazepam.
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pmc-6175268-1
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A 66-year-old male presented to the emergency room due to worsening leg pain. His past medical history was significant for peripheral artery disease and iron deficiency anemia. Initial laboratory tests revealed an unexpectedly low hemoglobin level of 5.4 g/dl. He received three units of packed red blood cells and subsequently the hemoglobin level increased to 6.9 g/dl. Our gastroenterology department was consulted for evaluation of occult gastrointestinal bleeding. There was no hematochezia, melena, hematemesis, fatigue, or abdominal pain. The patient had been taking oral iron supplementation for the last five years for iron deficiency anemia. Previous upper and lower endoscopies were negative. On physical examination, the patient had pale conjunctivae. The abdomen was noted to be soft and non-tender. No masses, organomegaly or vascular bruits were detectable. The vital signs were stable, and the laboratory investigations were as follows: a hemoglobin (hb) level of 6.9 g/dL, a mean corpuscular volume of 73.5, a hematocrit level of 22.7% with normal white blood cell and platelet counts. The analyses for iron-deficiency anemia showed ferritin levels of 6 ng/mL, serum iron levels of 25 μg/dL, total iron-binding capacity of 535 μg/dL, and transferrin saturation of 5%. Upper and lower endoscopy showed no active bleeding or suspicious lesions. A small bowel capsule endoscopy was performed, which revealed a suspicious lesion over the jejunum with evidence of fresh blood (Figure ).
For further investigation, double balloon enteroscopy was performed, which revealed a proximal jejunal soft submucosal mass (25 mm) with mild superficial ulceration/erosions of the surface mucosa. Biopsy samples were taken from the mass and the patient was referred for further management and surgical evaluation. The final pathology results revealed a cavernous hemangioma without evidence of malignancy.
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pmc-6175539-1
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A 63-year-old right-handed Danish Caucasian lady, with no previous medical illnesses or family history of neurodegenerative diseases, was admitted urgently to her local stroke unit with a 5-day history of abrupt onset fluent dysphasia in October 2009. Detailed speech examination revealed preserved fluency and comprehension but markedly impaired repetition, reminiscent of conduction aphasia. Computed tomography of her brain was unremarkable, and she was subsequently discharged with secondary prevention measures for stroke, after 3 days. Nine days following hospital discharge, she returned with sudden onset right-sided paresthesia, and thereafter, her clinical complex evolved rapidly through a sequence of dysarthria, nonfluent speech, dyslexia, dysgraphia, motor and verbal perseveration, startle, myoclonus, akinetic mutism, and finally death over the period of 10 weeks.
The patient has 2 older sisters, both of whom are alive and well in their 70s at the present time. Her father died of cancer at the age of 80 years, while her mother lived until the age of 90 years; neither parent had neurological or cognitive symptoms in life. The patient's father had a sister who died in “old age” of an unknown cause; her mother had 2 other siblings who died of cancer at 63 and 73 years of age, respectively.
Magnetic resonance imaging (MRI) of her brain revealed restricted diffusion in her caudate heads, anterior putamina, and predominantly left-sided cortical ribboning. Her electroencephalogram (EEG) showed left frontotemporal slowing of 1–2 Hz, with occasional sharp waves over the left hemisphere. Her cerebrospinal fluid had 3 white cells and 308 red cells but normal protein and glucose levels; protein 14.3.3 was positive, and neuron-specific enolase (NSE) was raised 101 ng/ml (<35 ng/mL); real-time quaking-induced conversion assay was not performed.
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pmc-6175539-2
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A 76-year-old right-handed British Caucasian woman, with no family history of neurodegenerative diseases, developed abrupt onset bilateral upper limb postural and action myoclonus. In the following week, she exhibited unusual sitting postures (axial apraxia), and her gait assumed a narrow-based shuffling character (gait apraxia). She then developed a rapidly progressive nonfluent dysphasia that rendered her effectively mute within 3 weeks. In tandem with that, she became socially withdrawn, abulic, and completely indifferent to her surroundings. Subsequently, she developed visual hallucinations, exaggerated startle, severe myoclonus, incontinence, and akinetic mutism. She died 8 weeks after symptom onset; a postmortem examination was not carried out.
The patient was the only child. Her father died of bone cancer at the age of 73 years, while her mother died of lung cancer at the age of 57 years.
Her MRI brain showed asymmetrical cortical ribboning with a left-sided emphasis and bilateral anterior basal ganglia diffusion restriction, while her EEG showed generalized periodic complexes. cerebrospinal fluid cell count and routine biochemistry were normal, but no sample was analyzed for protein 14.3.3, S100B, or real-time quaking-induced conversion assay.
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pmc-6176285-1
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A 47-year-old Norwegian male presented at the general causality clinic with right eye irritation. Previously, the same day he had been working using a hammer and chisel to repair his car without any eye protection, and he thought a foreign body had struck his right eye. The examination of his right eye revealed congestion and laceration of the conjunctiva, and the general practitioner started treatment with a broad-spectrum topical antibiotic (chloramphenicol). Seven days following his first presentation to the general causality clinic, he woke up with decreased vision, redness, and minimal pain in his right eye, and he presented to the nearby ophthalmology department on the same day. The best-corrected visual acuity (BCVA) decimal had decreased from 1.0 to 0.7. The orbit computed tomography scans detected a metallic intraocular foreign body (), and he was referred urgently to the Department of Ophthalmology at Oslo University Hospital for surgical removal of the foreign body. On arrival, the BCVA decimal had decreased from 0.7 to hand motion. The biomicroscopic examination revealed intense conjunctival and ciliary injection, most likely self-sealed conjunctival laceration, corneal oedema, 3+ anterior chamber cells with fibrin, and a thin layer of hypopyon in the anterior chamber and posterior synechiae. A layer of fibrin mesh covered the anterior surface of the lens. The changes in ocular media obscured the fundus view. The B-scan ultrasonography revealed an echogenic foreign body in the posterior vitreous cavity with dense vitreous opacities and attached retina and posterior vitreous. His left eye was unremarkable, and he was otherwise in good health. A clinical diagnosis of exogenous endophthalmitis secondary to penetrating eye injury with retained intraocular metallic foreign body was made.
He underwent an emergency 23-gauge pars plana vitrectomy with both undiluted and diluted vitreous biopsy and anterior chamber tap. The attempt to remove the intraocular foreign body was unsuccessful even after the removal of fibrin mesh layer covering the anterior surface of the lens due to poor surgical visualization of the posterior segment. At the end of the surgery, vancomycin (1 mg/0.1 ml), ceftazidime (2 mg/0.1 ml), and fungizone (0.00549 mg/0.1 ml) were administered intravitreally and gentamicin (20 mg/0.5 ml) subconjunctivally. He received treatment with topical steroids and antibiotics (Maxitrol eye drops consisting of dexamethasone, neomycin, and polymyxin B eye drops) hourly during daytime and intravenous cefuroxime (750 mg 3 times a day) postoperatively. In addition, he received oral steroids (prednisolone 60 mg daily) only for 2 days prior to repeated pars plana vitrectomy. Due to poor visualization of the posterior segment initially, he underwent surgical removal of the metallic intraocular foreign body (2 × 1.5 × 1 millimetres) 7 days after the first operation. The day after surgical removal of the foreign body, the intravenous cefuroxime was discontinued, and he was prescribed a 10-day course of ciprofloxacin (750 mg three times a day) peroral treatment. He continued with topical steroids and antibiotics (dexamethasone, neomycin, and polymyxin B eye drops) three times a day. The routine postoperative eye examination on the 9th day following removal of the foreign body revealed asymptomatic rhegmatogenous retinal detachment from 10 to 2 o'clock with fovea on and with a BCVA decimal of 0.4. He underwent his third pars plana vitrectomy with gas tamponade. Three weeks following the retinal detachment repair, he reached a BCVA decimal of 0.8, and the retina was attached. Six months following the retinal detachment repair, he reached a BCVA decimal of 1.0.
Direct microscopy of the undiluted vitreous sample showed pleomorphic rods with coccoid or club-shaped appearance suggesting coryneform bacteria.
A broad PCR and DNA sequencing targeting 16S rRNA-encoding gene was performed on the undiluted vitreous body sample using EZI DNA tissue kit (Qiagen, Thermo Fisher Scientific, Hilden, Germany). The 5' half of the 16S rRNA gene was amplified by PCR. The PCR product was sequenced using BigDye Terminator Cycle Sequencing Kit (Thermo Fisher Scientific, Hilden, Germany). The bacterium was identified by searching the GenBank (the NIH genetic sequence database) with the obtained DNA sequence. The obtained 740-base pair DNA sequence revealed 100% identity with GenBank sequences of the Dietzia species including D. natronolimnaea, D. dagingensis, and D. cercidiphylli. Although, additional DNA sequencing on the rest of the 16S rRNA gene (total 1462 base pairs) was performed, and this did not give an unambiguous identification.
The undiluted and diluted vitreous samples were cultured on sheep blood agar and chocolate agar plates (Oslo University Hospital, Oslo, Norway). The plates were incubated at 35°C in 5% carbon dioxide (CO2) for 7 days. The growth of bacteria was detected after two days under aerobic incubation at 35°C in 5% CO2. Anaerobic culture using horse blood agar (Oslo University Hospital, Oslo, Norway) did not detect any bacterial growth, and yeast culture on Sabouraud agar (Oslo University Hospital, Oslo, Norway) did not reveal any growth. No bacterial or yeast growth was detected from the anterior chamber tap aspirate.
The Gram stain of the bacterial colonies demonstrated Gram-positive cocci and polymorphic rods. The isolate from the culture was identified as D. natronolimnaea using MALDI-TOF MS (MALDI Biotyper, Bruker Daltonics GmbH, Bremen, Germany). MALDI-TOF was unable to distinguish D. natronolimnaea from D. dagingensis and D. cercidiphylli because the MALDI-TOF database does not contain the sequences for the latter two species.
Antimicrobial susceptibility tests were performed on Mueller-Hinton agar (Oslo University Hospital, Oslo, Norway) using MIC test strips (Liofilchem, Teramo, Italy). There are no determined breakpoints for Dietzia species, and therefore, the results were reported with a minimum inhibitory concentration (MIC) value. The MIC values of ciprofloxacin, gentamycin, tetracycline, and vancomycin against Dietzia species were low, indicating that these antibiotics have clinical effect.
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pmc-6176286-1
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Case 1. A 68-year-old postmenopausal woman (gravida 2; body mass index [BMI], 32.4 kg/m2) presented at a local gynecology clinic 20 months ago with a chief complaint of vaginal spotting. Transvaginal ultrasonography showed no thickness of the endometrium, and endometrial cytology was negative. At the three-month follow-up visit, a repeat endometrial cytology was also negative. However, vaginal bleeding persisted, and the patient visited the clinic again a month ago. At this time, pelvic magnetic resonance imaging (MRI) was performed, which revealed irregularity and endometrial thickening, and the patient was referred to our institution—Nara Medical University, Kashihara, Nara, Japan—for further evaluation. Endometrial curettage was performed that revealed atypical cells with large nuclei and conspicuous nucleoli without gland formation, which appeared to be consistent with high-grade endometrioid carcinoma or UC. The level of tumor markers was not elevated: CA125, 17 U/ml; CA19-9, 9 U/ml; CA72-4, 2.9 U/ml; CEA, 1.1 ng/ml; and SCC, 0.9 ng/ml. Chest and abdominal contrast-enhanced computed tomography (CECT) revealed no metastatic lesions. Pelvic contrast-enhanced MRI showed multiple myomas and a 30 mm polyp-like mass projecting into the endometrial cavity without myometrial invasion. The patient underwent abdominal total hysterectomy, bilateral salpingo-oophorectomy, pelvic lymphadenectomy, para-aortic lymphadenectomy, and omentectomy. The surgical specimen of the uterus showed a 35 mm polypoid tumor developing from the uterine posterior wall. Microscopically, the polypoid tumor comprised well-differentiated endometrioid carcinoma, grades 1-2, and UC. The well-differentiated endometrioid carcinoma was confirmed on the surface of the endometrial polyp, and the coexisting UC showed a diffuse proliferation of atypical cells (). Pancytokeratin (AE1/AE3) was diffusely expressed in the differentiated carcinoma component and was focally expressed in the UC component. Estrogen receptor (ER) and progesterone receptor (PR) were well expressed only in the differentiated carcinoma component (). The UC component represented about 80% of the whole neoplasm. Endometrium invasion or lymph node (LN) metastasis was not observed. Based on these findings, the patient was diagnosed with DEC located on the endometrial polyp. The final Federation of Obstetrics and Gynecology (FIGO) stage was IA. The patient was treated with adjuvant chemotherapy (TC protocol: paclitaxel, 175 mg/m2 + carboplatin AUC 6, every three weeks, and six cycles). She has been disease-free for 15 months after the initial surgery.
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pmc-6176286-2
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Case 2. A 58-year-old woman (BMI, 22.9 kg/m2), who had been hospitalized for several months with a diagnosis of bipolar disorder, reported that she has been experiencing atypical vaginal bleeding for >1 year, which had worsened over time. An abdominal CECT showed a large pelvic mass, and she was transferred to our institution for further evaluation. Pelvic MRI revealed a bulky mass in the whole uterine corpus, which spread to the bladder and rectum. Chest and abdominal CECT revealed multiple LN metastases, which extended from the para-aortic to pelvic LNs. Endometrial curettage revealed the foci of atypical cells arranged in sheets with numerous mitotic figures. There was no sarcoma component, and the histological pattern represented that of only a carcinoma. ER and PR tumor cell were focally expressed. As tumor markers, CA19-9, CEA, and SCC levels had risen (CA19-9, 43 U/ml; CEA, 13.9 ng/ml; SCC, 80.4 ng/ml); CA125 and CA72-4 levels were within normal range (CA125, 12 U/ml; CA72-4, 2.5 U/ml). Although the pathological diagnosis remained uncertain, based on the overall findings, the patient was diagnosed with stage IVA uterine endometrial cancer. Because of the presence of mental disorder and poor general condition (performance status 4), best supportive care was selected as the optimal treatment. However, the patient died in three months. Autopsy revealed uterine tumor invasion to the bladder, rectum, and pelvic wall with the involvement of the greater omentum and small intestine. The metastases to the pelvic and para-aortic LNs were observed. Microscopically, endometrioid carcinoma (grade 2) and UC components were present. Pancytokeratin (AE1/AE3) was diffusely expressed in the differentiated carcinoma component and focally expressed in the UC component (). ER and PR tumor cells were expressed only in the differentiated carcinoma component. There were bone marrow hyperplasia and neutrophil infiltration in the lung and myocardium. The patient died of sepsis due to urinary tract infection secondary to the tumor invasion. The final diagnosis was DEC with FIGO stage IVB.
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pmc-6176294-1
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A 79-year-old male with a past medical history of hypothyroidism and benign prostatic hyperplasia presented with dry mouth for four months. Family history was significant for the presence of Crohn's disease and systemic lupus erythematosus (SLE) in his sister. He was initially referred to an oral surgeon who performed a lip biopsy two weeks prior to admission revealing nonnecrotizing epithelioid cell granulomas. The patient was prescribed a mouthwash solution containing diphenhydramine, nystatin, lidocaine, hydrocortisone, and tetracycline. He was referred to a rheumatologist. His xerostomia significantly worsened prior to his appointment with rheumatology so he decided to go to the emergency department (ED). He presented to the ED with generalized weakness and decreased oral intake secondary to mouth pain resulting in a 30-pound weight loss over four months. Physical exam was remarkable for dry oropharyngeal mucosa. Laboratory analysis revealed an elevated serum calcium of 12.71 mg/dL (reference range: 8.4–10.7 mg/dL), an increased ionized calcium of 1.9 mmoL/L (reference range: 1.10–1.30 mmoL/L), an increased creatinine level of 3.81 mg/dL (reference range: 0.7–1.3 mg/dL), a decreased parathyroid hormone level of 6.5 pg/mL (reference range: 15–65 pg/mL), and an increased erythrocyte sedimentation rate (ESR) of 55 mm/hr (reference range: 0–15 mm/hr). Despite adequate fluid hydration, his calcium level remained elevated. Further workup for hypercalcemia revealed undetectable parathyroid hormone-related peptide (PTHrp), mildly decreased 25-hydroxyvitamin D at 18.1 ng/ml (reference range: 20–100 ng/ml), and an elevated 1,25-dihydroxyvitamin D at 72 pg/ml (reference range: 18–64 pg/ml).
Further workup for hypercalcemia showed an elevated angiotensin-converting enzyme at 91 U/L (reference range: 18–53 U/L). Bence Jones protein revealed free lambda light chains in the urine. Serum protein electrophoresis showed an elevated gamma globulin level of 38% and an IgG monoclonal gammopathy with an M-spike of 1.47. Immunoglobulin free light chain revealed elevated kappa free light chains at 5.79 and elevated lambda free light chains at 14.1 with a kappa to lambda ratio of 0.4. A bone marrow biopsy was done and was sent for pathology.
Further workup for dry mouth showed positive antinuclear antibody (ANA) at a titer of 1 : 160, and the other rheumatologic workup was negative.
The constellation of findings prompted further workup for sarcoidosis. Chest radiograph (CXR) showed minimal hilar lymphadenopathy which was more pronounced on the right side, bibasilar infiltrates and mild bilateral pleural effusions. High-resolution computerized tomography (HRCT) of the chest showed prominent hilar densities, ground glass opacities, and bilateral pleural effusions (). He was started on prednisone 40 mg/day and noticed immediate symptomatic improvement. His calcium level normalized, and his acute kidney injury resolved. Bone marrow biopsy results revealed lambda-restricted plasma cell neoplasm with 5%–6% of bone marrow cellularity consistent with MGUS. The patient was discharged with a one-month follow-up from nephrology and oncology.
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pmc-6176296-1
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An 86-year-old retired male of African-Brazilian descent was admitted to the Clementino Fraga University Hospital for surgical correction of lumbar stenosis. He had a history of chronic arterial hypertension, stage 3 chronic kidney disease, benign prostatic hyperplasia, peripheral arterial disease, and arthrosis of the knees. He had long been treated with enalapril, hydrochlorothiazide, nifedipine, aspirin, simvastatin, finasteride, cilostazol, and tamsulosin. He had a smoking pack year equal to 30 but had quit smoking several years earlier.
In the immediate postoperatory period, he developed a hypertensive emergency and was treated with intravenous nitroglycerin. Soon thereafter, he developed cyanosis of the extremities, which was confirmed by pulse oximetry (SO2 = 79%), but not by arterial blood gas sampling (SO2 = 97%). He was then empirically treated with methylene blue considering the clinical suspicion of methemoglobinemia, but severe dyspnea ensued in close association with the beginning of treatment. His hemoglobin steeply decreased from 11.1 g/dL to 6.1 g/dL, but the physical exam revealed no evidence of bleeding or of liver enlargement. The patient was not aware of previous episodes of anemia. A laboratory work-up revealed an elevated reticulocyte count, macrocytosis, transient leukocytosis (leukocytes count = 14,000/mm3 with 60% neutrophils, 30% lymphocytes, 8% monocytes, and 2% eosinophils), normal platelet count (= 250,000/mm3), hemoglobinuria, and positive markers for hemolysis [LDH = 4701 U/L (normal < 250 U/L), total bilirubin = 1.6 mg/dL (normal range = 0.3-1.2 mg/dL), unconjugated bilirubin = 0.9 mg/dL (normal < 1 mg/dL), haptoglobin < 6 mg/dL (normal range = 44-215 mg/dL]. A Coombs test was negative. The peripheral blood smear was consistent with a diagnosis of hemolytic anemia (). A few days after exposure to methylene blue, the patient's methemoglobin was 4.5% (normal < 2%). Given the possibility that the hemolytic crisis had been triggered by a deficiency of G6PD, a measure of the activity of this enzyme was obtained, resulting in a value of 13.6 U/g Hb (normal > 6.7 U/g Hb). Because enzyme activity was assessed during the hemolytic crisis, a definite diagnosis of G6PD deficiency could not be confidently ruled out at the time.
The patient was treated with packed red blood cells and folic acid. A rapid clinical improvement, which was closely followed by normalization of the red blood cell count, was observed. Three months later, a follow-up measure of G6DP activity (5.5 U/g Hb) revealed a moderate degree of enzyme deficiency (the 10%-60% range), thus confirming the diagnosis of G6DP deficiency.
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pmc-6176298-1
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A 72-year-old man with history of localized esophageal carcinoma with a history of neoadjuvant chemoradiation and esophagectomy presented to a local hospital with dysphagia four months after the surgery. Endoscopy revealed a benign-appearing esophageal stricture at the site of the anastomosis, and biopsies confirmed benign tissue. He was treated with a series of esophageal dilations and temporary placement of a FC-SEMS by his surgeon. This stent was removed after 3 months of placement; however, the patient developed recurrent symptoms after several weeks. Given no evidence of cancer recurrence, a 100 mm × 23 mm Wallflex PC-SEMS (Boston Scientific, Natick, MA) was placed by his surgeon as an attempt for a permanent solution to the patient's dysphagia.
The patient developed recurrent dysphagia after 3 months. A CT chest with oral contrast demonstrated the PC-SEMS in appropriate position at the anastomosis but demonstrated evidence of circumferential soft tissue extending approximately 2 cm in length and 7 mm in depth nearly occluding the proximal side of the stent. An upper endoscopy confirmed the above finding, and biopsies which concluded this represent benign and hypertrophic tissue.
This patient was then referred to our institution for further management and consideration of stent removal. At the time of referral, the PC-SEMS had been in place for almost 5 months. Repeat endoscopy demonstrated a benign-appearing stricture in midesophagus, beyond which the standard 9.8 mm endoscope could not pass. An ultraSlim 5.5 mm gastroscope was then advanced through the stricture, and the stent was identified beneath the tissue ingrowth and extending across the anastomosis. The proximal edges of the stent were not visible and fully covered by tissue ingrowth (). Cryoablation of the tissue ingrowth was performed using the CryoSpray (TruFreeze, Lexington, MA) Ablation () for 20 seconds, followed by placement of a 125 mm × 23 mm Wallflex FC-SEMS (Boston Scientific, Natick, MA) within the previously placed PC-SEMS () in order to promote tissue necrosis and permit subsequent removal of both stents. This rescue FC-SEMS was removed after 2 weeks on repeat endoscopy, with less tissue ingrowth visible on the proximal end and with no stricture evident. An attempt was made to remove the PC-SEMS but was unsuccessful. Thus, a 125 mm × 23 mm Wallflex FC-SEMS was again placed within the embedded PC-SEMS. Endoscopy was repeated after 4 months with successful removal of the rescue FC-SEMS. Less hypertrophic tissue was seen at the proximal end of the PC-SEMS and the PC-SEMS was now able to be pulled away from the embedded stent's proximal margin mucosa. Given the distal end of the stent which was still embedded, the stent was unable to be removed safely. Thus, another 125 mm × 23 mm Wallflex FC-SEMS was placed within the PC-SEMS. A repeat endoscopy was performed at 6 months with successful retrieval of both the FC-SEMS () and PC-SEMS (Figures and ) with minimal resistance with the help of rat-tooth forceps under fluoroscopic guidance. The patient was symptom-free and was started on high dose oral proton pump inhibitor with slow taper to a low daily dose.
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pmc-6176301-1
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We describe the case of a 23-year-old Caucasian female affected with congenital tricuspid atresia and intact ventricular septum. She had a history of palliative surgery since first days of life but her percutaneous oxygen saturation (SpO2) level remained around 80% even though a Fontan procedure was performed at 12 years of age. Persistent desaturation was related to the presence of venous collaterals between the Fontan circulation and left atrium.
The patient admitted to Policlinico San Donato (San Donato Milanese, Italy) for hypertensive crisis, worsening dyspnea, and hemoptysis. There was no family history of relevant morbidities. On examination, her height was 175 cm, weight was 64 kg (BMI 17.7 Kg/m2), blood pressure (BP) was 160/85 mmHg, and SpO2 was 81% (room air). Electrocardiogram (ECG) showed sinus tachycardia (heart rate 101 beats/min), first-degree atrioventricular block (PR 220 msec), and right bundle branch block (QRS 140 msec). Chest computed tomography (CT) () incidentally detected a 6-cm mass in the left adrenal lodge.
The presence of a heterogeneous adrenal lesion, with hyperintense spots due to hematic content, was confirmed by abdominal magnetic resonance imaging (MRI) ().
Laboratory tests revealed increased levels of noradrenaline (NA) and its metabolites [plasma NA 5003.7 pg/ml, n.v. < 480 pg/ml; urinary NA 1059.5 µg/24 h, n.v. < 85.5 µg/24 h; urinary metanephrine 489 µg/24 h, n.v. < 320 µg/24 h; plasma adrenaline (A) 100 pg/ml, n.v.20-190 pg/ml; urinary A 15 µg/24 h, n.v.1.7-22.4 µg/24 h]. The patient reported no typical paroxysmal symptoms of catecholamine excess. Echocardiographic evaluation showed slight left atrial and ventricular enlargement, mild to moderate mitral regurgitation, and preserved systolic function (ejection fraction 65%).
The diagnosis of pheochromocytoma was confirmed by 123I-metaiodobenzylguanidine (123I-MIBG) scintigraphy showing abnormal accumulation of radioactive tracer in the left adrenal gland. A 18F-fluorodeoxyglucose positron emission tomography (18F-FDG-PET) performed in order to exclude any extra-adrenal uptake: no significant metabolic activity in the adrenal mass but intense uptake in supra- and subdiaphragmatic brown adipose tissue was detected, likely due to noradrenergic-stimulated glucose uptake ().
The patient underwent open left adrenalectomy after preconditioning with α-blockers (doxazosin) and, then, β-blockers (bisoprolol). Postoperative course was complicated by anemia due to hematoma formation in the left hypochondrium. Histopathological examination confirmed the diagnosis of pheochromocytoma with large hemorrhagic areas and scarce necrosis. No capsular or lymphovascular invasion was found. Immunohistochemistry revealed diffuse expression of chromogranin A, synaptophysin and neuron specific enolase, and S100 staining in sustentacular cells; Ki-67 was <5%. The P-PGL susceptibility genes VHL, RET, SDHA, SDHAF2, SDHB, SDHC, SDHD, MAX, and TMEM127 were analyzed for germline mutations and large deletions, via direct sequencing and multiplex ligation-dependent probe amplification methods; RET was only analyzed by direct sequencing. No aberration was found in these genes. Twelve months after surgery patient's BP and heart rate were under control and urinary NA and metanephrine levels were within the normal range. Plasma NA levels remained slightly increased (715 pg/ml n.v. 70-480), consistent with the hemodynamic changes in Fontan circulation [].
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pmc-6176302-1
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A 39-year-old woman was admitted to the ER with a right-sided drop-foot, fever, and pancytopenia. The patient reported night sweats during the last month and a 25 kg weight loss over the last year. On physical examination, enlarged, painless right cervical and axillary lymph nodes were found; the largest of which was 1 × 2 cm. A CT-scan revealed marginally enlarged axillary and abdominal lymph nodes as well as a marginally enlarged spleen. A bone marrow biopsy was performed and bilineage dysplasia was found, possibly suggestive of a myelodysplastic syndrome. The fever and anaemia responded well to broad-spectrum antibiotics and blood transfusions, respectively. An EBV-viremia (18.500 DNA copies/mL) was detected, and the patient was treated with acyclovir followed by rituximab. The decision to initiate acyclovir and rituximab was taken due to suspected virus-associated haemophagocytic syndrome. During this treatment, three separate episodes of hypothermia occurred with an interval of approximately two weeks between each episode.
The first episode occurred the day after rituximab infusion and was accompanied by moderate bradycardia, hypotension, and a prolonged QT interval. The patient was subjectively unaffected. However, the patient had a syncope-like episode a few days later. Subsequent ECG monitoring at the Department of Cardiology did not reveal any arrhythmias. The second episode of hypothermia occurred 13 days later just before a planned rituximab infusion. However, the patient had already received paracetamol as premedication prior to rituximab. The patient only experienced mild symptoms related to hypothermia, that is, moderate sweating, moderate hypotension, and insecure gait. Intravenous fluids were administered with clinical effect. During the third episode, an ear temperature as low as 32.8°C was recorded. The patient experienced profuse sweating and an ECG demonstrated bradycardia along with a borderline prolonged QT interval. The blood pressure reached a low point of 85/52 mmHg and the pulse was recorded as 50 bpm. Similar to the previous episodes, the patient was relatively unaffected, and no specific treatment in order to increase the body temperature was initiated. The third episode differed from the first two since neither rituximab nor paracetamol was given prior to the onset of hypothermia ().
A PET/CT was performed, revealing increased FDG uptake in enlarged right cervical and mediastinal lymph nodes and in focal areas of the spleen and liver. Furthermore, increased FDG uptake was found at multiple skeletal sites and in the bone marrow, but without CT correlate (). A surgical biopsy from a right cervical lymph node revealed classical HL, nodular sclerosis type. The bone marrow biopsy was without morphological and immunophenotypic evidence of lymphoma.
In the weeks following the last episode of hypothermia, three separate attacks with generalized seizures occurred. One of them was clinically described as a classic tonic-clonic Jackson-type attack, while the other two were less well characterized. An EEG showed focal activity compatible with epileptic state. Cerebral CT and MRI scans did not show any sign of intracranial or intraspinal tumors and did not provide any clues to clarify why the attacks occurred.
The patient was treated with 8 series of BEACOPP-14. The peroneal palsy which had remitted worsened again during chemotherapy, and it was therefore decided to continue without vinca alkaloids from series 3 and onwards. After 8 series the PET scan revealed a complete metabolic remission. No radiotherapy was added. Although no repeat bone marrow biopsy was done upon completion of the treatment schedule, all blood counts returned to normal making the initial diagnosis of a possible myelodysplastic syndrome unlikely. The patient received her last round of chemotherapy on December 2015. She was still in 1st complete remission and is regularly followed on an outpatient basis. No additional episodes of hypothermia have been registered so far.
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pmc-6176303-1
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A 70-year-old nonverbal female presented to the emergency department (ED) from home with her daughter who had noted that the patient has been acutely grimacing in pain from even the lightest palpation over her right flank. She had decreased urine output for the prior two days and her family had noticed an enlargement on the right side of her back one day prior to her ED visit. Of note, the patient presented with poor functional status, was totally bedridden, and was on no antibiotics. She also had a renal flow scan several months prior to her presentation, which revealed the absence of blood flow to the left kidney. A nephrectomy was ultimately rejected based on her medical history and current health status. The patient's medical history was significant for CVA with left hemiparesis, sacral stage 4 pressure ulcers, DM, HLD, failure to thrive, and asthma. Her past surgical history included a left double J-stent placement, an appendectomy, and a tracheostomy that was reversed.
On physical examination, the patient appeared to be in mild distress with a blood pressure of 93/50, heart rate of 116, and otherwise normal vital signs. Abdominal examination was remarkable for tenderness to palpation over the right flank with visible erythematous skin seen in the same area. Results of the initial laboratory tests were significant for a lactate of 2.9, white blood cell count of 18.4, hemoglobin of 7.1, platelet count of 933, albumin of 2.3, and potassium of 5.8, yet with a creatinine of 0.62. She underwent an IV contrast computed tomography (CT) scan of the abdomen and pelvis, which revealed a right hydronephrotic kidney that contained numerous large calcifications (Figures and ). The right previously placed ureteral stent was in satisfactory position. There was an extremely large fluid collection in the right retroperitoneum extending into the right flank consistent with leakage of urine from the obstructed right kidney ().
While, in the ED, urology was consulted and requested interventional radiology to drain the urinoma and to place a Foley catheter to monitor urine output, the attending physician, at the request of interventional radiology, performed a needle aspiration of the loculated fluid collection in the right flank via ultrasound and noted a small amount of purulent and urine-like fluid from two separate areas of collection. The patient was admitted to the intensive care unit for urosepsis, urinoma with abscess, symptomatic anemia, and failure to thrive, while, in the intensive care unit, the patient was evaluated by urology, infectious disease, gastroenterology, wound care, and palliative care.
Urology decided against emergent interventional radiology (IR) drainage as IR did not believe there was an acute need for drainage. It was postulated that the collection was more consistent with a urinoma without an apparent abscess thus not requiring emergent drainage. The patient also had been afebrile and it was reported that there were no fevers noted at home. It was discussed that emergent drainage would be performed if the patient acutely decompensated or became febrile. Additionally, the family refused surgical drainage because of her comorbid conditions. The patient was placed on empiric vancomycin and piperacillin/tazobactam.
The following day interventional radiology placed an 8 French pigtail drainage catheter and drained 610 ml of fluid. A subsequent IV contrast CT scan of the abdomen and pelvis showed the right retroperitoneal collection to be significantly decreased in size and nearly resolved. The contiguous component in the right flank soft tissues was slightly decreased with the drainage catheter in place. The patient was eventually discharged with the drain in place to hospice care.
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pmc-6176304-1
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A 54-year-old Caucasian female presented to our clinic with a two-year history of persistent hypocalcemia requiring multiple hospitalizations. Her symptoms included muscle cramps, tingling and perioral paresthesias. Her medical history was significant for HIV diagnosed four years ago, gastric bypass surgery done 15 years ago, hypertension, and COPD. She denied any history of prior neck surgery or radiation. She denied any history of hearing loss. She had no family history of autoimmune disease.
Her vital signs were stable with an unremarkable physical exam. Chvostek's and Trousseau's signs were negative. Pertinent medications included calcium carbonate, vitamin D3, calcitriol, atripla (efavirenz/emtricitabine/tenofovir disoproxil), hydrochlorothiazide, and inhaled budesonide/formoterol.
Laboratory testing showed total calcium of 5.7 mg/dL (normal range: 8.4-10.2 mg/dL), serum albumin 3.9 mg/dL, ionized calcium 2.7 mg/dL (normal range: 4.5-5.5 mg/dL), serum magnesium 1.7 mg/dL (normal range: 1.7-2.7 mg/dL), serum phosphate 6.3 mg/dL (normal range: 2.7-4.5 mg/dL), and intact PTH 7.6 pg/mL (normal range: 15-65 pg/mL). She had normal 25-hydroxy vitamin D 32 ng/mL (normal range: 30-100 ng/mL), 1,25 dihydroxy vitamin D 23 pg/mL (normal range: 18-72 pg/mL), TSH 1.2 μIU/L (normal range: 0.40-4.5 μIU/L), and creatinine 0.98 mg/dL (normal range: 0.5 -1.1 mg/dL). Absolute CD4 count was 629 cells/μL (normal range: 185-2273 cells/μL) with undetectable HIV-1 RNA viral load.
She was diagnosed with primary hypoparathyroidism. A serum sample was tested for anti-calcium sensing receptor (CaSR) antibodies [] and NALP5 antibodies [] to rule out autoimmune hypoparathyroidism and it was found to be negative; the CaSR antibody index was 1.09 (normal range: 0.57-1.38; upper limit of normal, 1.73) and the NALP5 antibody index was 1.12 (normal range: 0.62-1.93; upper limit of normal, 2.17). During subsequent clinic visits, doses of calcium supplements and calcitriol were titrated and she was started on magnesium oxide. She required calcium carbonate 2500 mg three times per day, calcium citrate 1900 mg twice per day, and calcitriol 0.5 mcg three times per day (). Her last corrected serum calcium level was 9.18 mg/dL. She was considered for treatment with recombinant human PTH but subsequently she was lost to follow-up.
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pmc-6176307-1
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A 65-year-old male with a BMI of 42 and uncontrolled type II diabetes mellitus came to our clinic with a fracture on the left tibial shaft. He was treated conservatively for 20 years. Radiographs showed a malunited fracture on the middle third left tibia in 20-degrees varus, 15-degrees apex anterior angulation with a 1 cm anterior translation of distal segment, 20-degrees internal rotation and 1.5 cm shortening. He also had severe tricompartmental osteoarthritis on the left knee (Figures and ). A CT scan confirmed the presence of malunion (). Blood sugar was controlled by an endocrine physician and a clamshell osteotomy of the tibia was planned.
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