_id
stringlengths 7
16
| description
stringlengths 55
95.2k
|
|---|---|
pmc-6211925-1
|
A 30-year-old female, with a medical history of headache for 3 years, was referred to our department. Symptoms including dizziness, nausea, vomiting, limb movement disorder, or other neurologic deficits were not present in this case. But her headache has been growing progressively severe in the past 3 months. Physical examination demonstrated a palpable hard lesion of the right frontal region. Preoperative computed tomography (CT) of the head showed a 5 × 3.7 × 4 cm sized, well-demarcated osteolytic frontal lesion with mottled ossified density, which also infiltrated the orbital roof (Fig. A–C). Enhanced magnetic resonance imaging (MRI) revealed a solitary frontal mass, which was hypointense on T1- and T2-weighted images, and enhanced heterogeneously (Fig. E–I). It was also found in the images the brain distortion caused by mass effect of the lesion. Based on the results of enhanced CT and MRI (Fig. D, G–I), the lesion was shown to be with sufficient blood supply. Initial differential diagnoses included hemangiopericytoma, atypical intraosseous meningioma, calvarial osteosarcoma, fibrous dysplasia, and histiocytosis. Then the complete surgical removal was subsequently performed. Intraoperatively, a reddish-white mass, covered with a thin layer of bone and densely adhered to the underlying dura, was observed in the right frontal region. Postoperative histologic analysis demonstrated a bone-forming tumor composed of diffusely trabeculae of woven bone, which was separated by richly vascular fibrous stroma, surrounded by osteoblasts, which ultimately confirmed the diagnosis of osteoblastoma (Fig. ). It was also observed in the stroma numerous thin-walled capillaries, occasional extravasation of blood and multinucleated giant cells. There was no radiologic evidence of recurrence in the 6 months follow-up.
|
pmc-6211931-1
|
An 82-year-old woman with severe cognitive impairment (Mini Mental State score 14/30) was admitted in our department because of anorexia, weight loss, fatigue, and mild night fever. She complained of chronic knee and wrist pain. On clinical examination, she had a left pleural effusion without crackles or clinical signs of heart failure. There was no joint effusion, nor synovitis. Biological data were as follow: C-reactive protein: 59 mg/L (N < 5), White blood cell count: 3.3 G/L with 8.2% eosinophils, hemoglobin was 10 g/d L, platelet count was 150 G/L. A High contrast thoraco-abdomino-pelvic CT-scan showed multiple millimeter lymph nodes in the mediastinum, and a left pleural effusion without parenchymal lesion. A thoracocentesis (200 mL) revealed an exudate with 3065 M/L of predominantly lymphocyte-white blood cells (59%) with eosinophilia (20%). Direct cytological examination showed Hargraves cells, that is, LE cells, characterized by homogenous nuclear material (hematoxylin body) encompassed by neutrophils. No malignant cells were found, and pleural fluid culture was negative for Mycobacterium tuberculosis. A few days later, antinuclear antibodies (ANA) with homogeneous pattern were found with a titer of 2560 (N < 80) in the serum, and 5000 in the pleural effusion. Anti-nucleosome antibodies were also present (91 UI/L [N < 20]), without antiphospholipid antibodies. There was no hypocomplementemia. Search for HIV, CMV, EBV, HBV, HCV was negative, leading to the diagnosis of systemic lupus erythematosus according to ACR criteria. Hydroxychloroquine (400 mg/d) and prednisone 0.5 mg/kg/day with slow tappering, allowed patient recovery within 1 week, without recurrence of pleural effusion. Six months later, the patient had no particular complains, and clinical examination was strictly normal, C reactive protein was < 5 mg/L and ANA titer was 320. During the following year, she experienced no recurrence.
|
pmc-6212298-1
|
An 88-year-old man was referred to our hospital for emergent massive hemoptysis. His medical history was remarkable for chronic heart failure, moderate mitral regurgitation, atrial fibrillation, and chronic kidney disease. He was undergoing treatment with apixaban and pilsicainide for atrial fibrillation. He was severely hypoxic (SpO2: 80 under O2 15 L/min by oxygen mask) and hypotensive (systolic blood pressure: 80 mmHg) on admission. In the emergency department, we performed intubation into the right main bronchus through guided bronchoscopy; this was followed immediately by right side one-lung ventilation as portable chest radiography showed consolidation in the left upper lung. Bronchoscopy showed that the trachea was almost obstructed by haemorrhage and haematoma. He experienced cardiopulmonary arrest immediately after the airway was maintained. However, spontaneous circulation was restored by cardiopulmonary resuscitation. Contrast computed tomography (CT) demonstrated an aortic aneurysm at the aortic arch, which penetrated the upper lobe of the left lung (Fig. A,B). We suspected that it would be difficult to perform emergent surgery because of the patient’s poor general condition. Furthermore, we believed that there was no indication for endovascular stenting due to the following reasons: (1) the root of the left brachiocephalic artery was close to the penetrating portion of the aneurysm, at a distance of 12 mm. Thus, there might have been a high risk of obstructing blood flow to the brachiocephalic artery; (2) a risk of aortic injury might have been induced by stenting because the aortic arch was highly calcified. He was admitted to the intensive care unit, and we controlled blood pressure using nicardipine and discontinued anticoagulation therapy and performed platelet and fresh frozen plasma (FFP) transfusion for haemostasis. Bleeding from the APF decreased gradually due to astriction by haematoma. On the 17th hospital day, we performed bronchoscopy for the suction of haematoma, except in the bronchi of the left upper lobe, and adjusted the intubation tube for conversion to bilateral lung ventilation. On the 18th hospital day, we performed endobronchial occlusion with EWS to prevent fatal hemoptysis despite the risk of atelectasis. We inserted the EWS into each target bronchus with haematoma, with consideration of the risk of re-bleeding due to the removal of the haematoma. EWS sizes were as follows: (B1 + 2a: 7 mm; B1 + 2b: 7 mm; B3b + c: 7 mm; and B3: 7 mm) (Fig. A). B1 + 2c did not undergo EWS insertion because this bronchus was not responsible for bleeding on CT findings. There was no massive hemoptysis after bronchial occlusion with the EWS; subsequent mild hemosputum was controlled by a haemostatic drug. The patient was successfully extubated on the 22nd hospital day and was discharged on the 47th hospital day without complications and free from oxygen. Radiography showed that EWS had promptly fixed each bronchus (Fig. B).
|
pmc-6212508-1
|
The authors report the case of a 45-year-old female who suffered a snowboarding accident and presented several days following the event complaining of persistent headache, nausea, vomiting, dizziness, and photic auras in the right eye. She visited an urgent care facility twice and during these visits, no imaging was obtained. A neurologist saw her 18 days after the accident. An MRI scan was ordered that showed multiple masses some of which harbored hemorrhagic components: a left frontal parafalcine, calcified 2.0 × 2.2 × 3.3 cm [anteroposterior (AP), transverse (TV), craniocaudal (CC)] mass with associated vasogenic edema, a 4.5 × 2.9 × 4.1 cm (AP, TV, CC) mass in the left frontotemporal convexity with another mass measuring approximately 2.3 × 2.0 × 1.9 cm (AP, TV, CC) located just superiorly, and a 2.6 × 2.9 × 3.9 cm (AP, TV, CC) mass in the left occipital lobe (Figure ). Additionally, there was an 8 mm left to right midline shift (Figure ). Her findings were most consistent with multiple meningiomas. There was also a possible vestibular schwannoma measuring 0.7 × 1.4 × 0.7 cm (AP, TV, CC) in the left internal auditory canal (figure not shown). Initially, she was thought to have NF2. Pre-surgical tumor embolization and a two-staged surgery were recommended. The patient had successful embolization of the left middle meningeal artery and left posterior meningeal artery.
The first surgical stage involved a left-sided craniotomy for resection of the frontal-parietal-temporal meningiomas; pathology reported WHO Grade I meningiomas with a low/moderate proliferation index (percentages of positive Ki-67 tumor nuclei: left occipital mass: 2–3%; midline frontal mass: 3–4%; and left frontal mass: 1–2%; Figure ). NF-2 blood testing (NEUROFIBROMATOSIS TYPE 2 SEQUENCING AND DELETION/DUPLICATION ANALYSIS IN Blood, UAB), which has a mutation detection rate in leukocytes of 93% was negative. This specific study detects truncating mutations (nonsense, frameshift, splicing mutations including deep intronic splice mutations), missense mutations, multi-exon deletions or duplications, and total gene deletions. Post-operative MRIs showed resection of the meningiomas in the left frontal/parietal/temporal convexity (Figure ). Seven months after her first-stage surgery, she underwent the second surgical stage: left occipital craniotomy for resection of a 2.9 × 2.7 × 4.2 cm (AP, TV, CC) mass (Figure ). A specimen from the left occipital mass was sent to pathology which classified it as meningioma, WHO Grade I with a Ki-67 of 2–3% (Figure ). Her most recent post-operative MRI scans at 16 months of follow up show multiple stable enhancing extra-axial masses compared to her immediate post-operative ones with no new lesions observed (Figure ).
In addition to having multiple meningiomas, she also had a soft tissue mass in her right palm and moles in her left axilla. MRI scans showed the mass to be approximately 11 × 10 × 20 mm (Figure ) with unclear pathology, and the patient was referred to plastic surgery. The patient underwent surgery to excise her cystic mass and pathology found it to be a benign nerve sheath tumor, consistent with Schwannoma with positive immunostain S-100. As for the moles on her left armpit, a shaved biopsy was obtained and pathology confirmed pigmented seborrheic keratosis, consistent with an atypical mole.
Because she was NF2 negative, further genetic testing was pursued for both somatic and germline mutations. Foundation Medicine, Inc. (Cambridge, Massachusetts, USA) tested her tumor tissue positive for SMARCB1 mutation (but not for NF2) while her blood genetic testing was negative for SMARCB1 mutation.
|
pmc-6212596-1
|
Patient 1 was a 61-year-old female who presented with a 5-cm anterior neck mass with retrosternal extension. She was surgically treated with a right thyroid lobectomy at another institution. The lesion was interpreted to be a poorly differentiated papillary thyroid cancer. Postoperative treatment consisted of sequential and combined chemoradiotherapy. Local recurrence in the region of the right thyroid occurred 20 months later, and radiotherapy was performed to relieve symptoms. Six years later, the patient had local recurrence again and was referred to our institution for further treatment. After curative wide local excision and central neck dissection, postoperative radiotherapy was given to the thyroid bed and bilateral cervical lymph node areas (55 Gy/16 fractions). Histological re-evaluation of the recurrent tumor specimens revealed CASTLE (pT4bN1M0). A third local recurrence was diagnosed 1 year later, and concurrent chemotherapy (paclitaxel 210 mg/m2 and cisplatin 40 mg/m2) and radiotherapy (50.4 Gy /28 fractions) were applied. The patient died from acute respiratory distress 1 month after the initiation of treatment.
|
pmc-6212596-2
|
Patient 2 was a 48-year-old male pathologically diagnosed with stage pT4aN0M0 CASTLE postsurgery. He presented with rapidly growing thyroid nodules with fixation to underlying structures. He underwent total thyroidectomy with central neck dissection. On exploration, the thyroid mass was seen extended to the left laryngeal nerve, strap muscle, trachea, and esophagus. Curative wide local excision was attempted on patients with gross residual local disease. Postoperative external beam radiation therapy (50.4 Gy/28 fractions) was given to the thyroid bed and bilateral cervical lymph node area. He remained well 2 years post therapy with no palpable neck disease.
|
pmc-6212596-3
|
Patient 3 was a 67-year-old female who presented with hoarseness and dysphagia for a few weeks. CT scans showed a 3.8 cm left thyroid tumor with superior mediastinal and tracheal involvements, i.e., stage pT4bN0M0. Complete resection of the tumor was successfully achieved by total thyroidectomy and central neck dissection. Postsurgical chemotherapy was applied (docetaxel 120 mg/m2 and cisplatin 30 mg/m2), followed by a course of intensity modulated radiotherapy (70 Gy at 2.5 Gy per fraction). She remains well 4 years after diagnosis.
|
pmc-6212660-1
|
An 8-year-old male patient reported to a dental clinic with the chief complaint of swelling in the upper right front tooth region since 1 year. Extraoral examination showed slight swelling was present on the right side of face, causing elevation of right ala of nose (). General examination, gross facial asymmetry, and other findings were noncontributory; there was no history of trauma.
The panoramic radiograph of the patient showed mixed dentition with tooth buds at different developmental stages. A large radiolucent lesion of 2 × 2 cm in size was found in the right side of face area in relation to periapical region of 54 and 55. Tooth bud of 14 was being pushed close to the region of floor of the orbit and tooth bud of 15 being pushed posteriorly ().
The borders of the lesion are ill defined. Coronal computed tomography showed a radiolucent lesion in relation to right maxillary sinus area with tooth bud being displaced close to the region below the floor of the orbit (). Considering the clinical and radiologic presentations, a provisional diagnosis of dentigerous cyst was determined.
The patient underwent surgery under general anesthesia (). A mucoperiosteal flap was opened; the entire cyst lining was enucleated along with the fibrous capsule () and was sent for histopathological examination. Histopathological examination revealed odontogenic epithelial lining of 6 to 9 cell thickness, which exhibited a wavy appearance.
The basal epithelial layer was composed of palisaded columnar cells. There was also presence of small satellite cystic islands of odontogenic epithelium seen in the fibrous connective tissue. Histopathological features were suggestive of KCOT ().
|
pmc-6212664-1
|
A 12-year-old male patient from Yemen () reported to the clinic at the Preventive Dental Sciences Department at the College of Dentistry, Jazan University, with a complaint of mobile teeth for the last 6 months. According to the patient’s parents, his deciduous teeth erupted normally, but exfoliated at the age of 3. By the age of 10, the patient had multiple permanent teeth extracted due to mobility and now complains of mobility of the remaining permanent teeth. On physical examination, bilateral hyperkeratotic lesions on the palm and soles were observed; however, no signs of keratosis were present on the knees and elbows (). Family history showed that his parents were relatives and that his brother was also exhibiting similar complications.
Intraoral examination had shown presence of permanent maxillary right lateral incisor, canine, first premolar, first molar, permanent maxillary left canine, second premolar, and first molar. In mandible, permanent mandibular right central incisor, lateral incisor, canine, first premolar, first and second molars, left central incisor, canine, first and second premolar, and first molar were present. All other permanent teeth were missing ().
Of these teeth, maxillary right first molar, left second premolar, first molar, mandibular right canine, first premolar, and first molar were showing varying degrees of flaring and mobility. Severe gingival inflammation associated with thick plaque accumulation and deep periodontal pockets was present. Dermatological examination had shown the presence of symmetrical, well-demarcated keratotic plaques on the palms and soles. On radiographic examination, alveolar bone loss associated with all the affected teeth was noted. Also, third molar buds were present in their bony crypts, with normal crown development and no associated bony changes. On consideration of the clinical and radiological features, a diagnosis of PLS was made. The treatment plan included oral hygiene modification, nonsurgical periodontal therapy, extraction of all the remaining mobile teeth, and insertion of maxillary and mandibular dentures. Consideration of dental implants will be considered after the age of 18.
|
pmc-6212664-2
|
The second case was the younger brother of the first patient who is an 11-year-old male (). The patient reported a similar chief complaint of mobile teeth and past dental history from his parents also discovered that his deciduous teeth erupted normally and exfoliated at the same age of his brother. On physical examination, bilateral hyperkeratotic lesions on the palm and soles were observed; however, no signs of keratosis were present on the knees and elbows ().
Intraoral examination had shown the presence of permanent maxillary right canine, first and second premolar, first molar, permanent maxillary left lateral incisor, canine, first and second premolar, and first molar. In mandible, permanent mandibular right central incisor, canine, first premolar, first molar, left central incisor, canine, second premolar, and first molar were present. All other permanent teeth were missing. Of these remaining teeth, many were showing varying degrees of flaring mobility. Furthermore, severe gingival inflammation associated with thick plaque accumulation and deep periodontal pockets was present. Dermatological examination had shown the presence of symmetrical, well-demarcated keratotic plaques on the palms and soles. On radiographic examination, alveolar bone loss associated with all the affected teeth was noted. Also, third molar buds were present in their bony crypts except for the lower left third molar, with normal crown development and no associated bony changes. On consideration of the clinical and radiological features, a diagnosis of PLS was made. The treatment plan included oral hygiene modification, nonsurgical periodontal therapy, extraction of all the remaining mobile teeth, and insertion of maxillary and mandibular dentures (). On consideration of the clinical and radiological features, diagnosis of this patient was also made as PLS.
|
pmc-6212666-1
|
A 5-year-old African girl presented to Dental Care Unit of the University hospital of Nantes for a general dental check-up, her sister having been previously diagnosed with LAP. Clinical examination showed no visible signs of gingival inflammation and no pain-related complaints were expressed by the patients.
Medical history of the patient revealed a GT associated with a slight anemia. She presented a complete primary dentition and the first permanent maxillary molars were partially erupted (). Oral hygiene was poor and plaque deposits were present around all teeth. Black stains were also noticed. Carious lesions could be found on the occlusal surface of multiple primary molars (#: A, J, L, K, and T). Radiographic examination showed severe horizontal bone loss around the four first primary molars (#: B, I, L, and S) (). Clinical examination of these teeth revealed the presence of severe periodontal attachment loss and slight mobility increase. Periodontal probing depths were assessed and pockets depth of 5 mm around affected teeth.
The treatment goal was to avoid further progression of the bone loss to the rest of the dentition and particularly permanent teeth. Therefore, it was decided to extract all the first primary molars affected by severe periodontitis, and to proceed on a thorough scaling and root planing on all the other teeth.
Due to the patient age and cooperation, her treatment was conducted under general anesthesia, and consisted of the extraction of the four affected teeth (). Management of carious lesions was also done at this occasion. She was injected slowly (5 minutes) with Eptacog alpha activated (rh-FVIIa, Novoseven®, Novo Nordisk, France) right before the surgery and then 2 hours after the first injection. All necessary means for blood hemostasis were also used during surgery:
Sutures (Vicryl 4.0)
Hemostatic sponge (Pangen®, Urgo, France)
Biological glue (Tissucol®, Baxter, France)
She also benefited from a tranexamic acid (Exacyl® Sanofi-Aventis, France) prescription, started the evening of the surgery and continued for 10 days, 100 mg three times a day for 10 days.
Extracted teeth showed no signs of root resorption. After surgery, she was treated with systemic antibiotics for 7 days with two daily prescriptions of amoxicillin (50-100 mg/kg/day). No antibiotic-related side effects were reported and the parents supervised medication intake over the whole period.
One week after the surgery, the patient did not complain of any pain or discomfort. Satisfactory wound healing was achieved without complications. Intraoral hygiene was adequate, and parents were reinstructed to watch it.
Both parents were also screened for periodontal disease. The mother exhibited a slight gingivitis and was referred for periodontal treatment. The older sister (OS) and brother were also screened, and although the 3-year-old brother presented no signs of periodontal disease, the 8-year-old sister showed periodontal breakdown with no diagnosed contributory medical history.
The OS had mixed dentition with the first permanent molars, the permanent mandibular incisors, and the central maxillary incisors fully erupted. Oral hygiene was satisfactory. Radiographic (Fig. 4) and clinical examination permitted the establishment of LAP diagnosis. No sign of bone loss around permanent teeth could be detected. There was also a shallow carious lesion on the 84. Proper dental and periodontal management was delivered.
|
pmc-6213486-1
|
A 71-year-old woman consulted the Respiratory Center of Matsusaka Municipal Hospital. The patient was being treated with amlodipine because of arterial hypertension. Lung adenocarcinoma with ALK arrangement was diagnosed based on clinical and pathological findings. Therapy with crizotinib (500 mg/day) was associated with marked tumor shrinkage and clinical improvement (A–C). Parameters of kidney function were normal before the initiation of crizotinib. Three weeks following crizotinib administration, the blood level of creatinine increased from 0.73 mg/dL (pre-treatment value) to 1.21 mg/dL and remained at similar levels thereafter, but there were no abnormal findings in the kidneys upon computed tomography CT (D). Eleven months after starting crizotinib treatment, the blood level of creatinine increased further (1.68 mg/dL) and multiple (>3) renal cysts were detected by CT examination (E). Multiseptated renal cysts were detected by CT thirteen months after initiation of crizotinib (F). Ultrasound study showed cystic formations, normal renal size and normal blood flow in the kidneys. Laboratory analysis of the cream-colored liquid obtained by ultrasound-guided cyst aspiration showed no cancer cells and microbial culture was negative. Urine analysis showed a mild proteinuria. Crizotinib was stopped and alectinib was started instead for the control of lung tumor. The blood level of creatinine decreased to 0.86 mg/dL after three weeks and the renal cysts regressed after three months of crizotinib withdrawal (G).
|
pmc-6213993-1
|
Our patient was a 4-year-old Italian boy who visited our pediatric hospital for incidental head trauma with a linear fracture of the right parietal bone and a small hematoma. He presented with a Glasgow Coma Scale of 15/15 and normal neurological testing. His weight was 21 kg (>97th percentile), height was 113 cm (>97th percentile), and head circumference was 54.5 cm (>97th percentile). The patient exhibited dysmorphic facial appearance with trigonocephaly, frontal bossing, large ears, prominent chin, and high palate with dental malposition. Physical examination of the skin found one “café-au-lait” spot on the right hemithorax (2 cm × 0.5 cm) and an irregular skin lesion with a rubbery consistency and fibrosis (3 cm × 4 cm) on the right femoral side. He also exhibited syndactyly of the second and third right toes and learning difficulties. He was born at full-term via caesarean section because of macrosomia. His birth weight was 4.050 g (>97th percentile). His parents were healthy and unrelated. His older brother was also healthy and had normal development. His grandfather died at the age of sixty to chronic lung disease ().
|
pmc-6214163-1
|
A 52-year-old Han Chinese woman who worked as a teacher was presented to our emergency department complaining of headache and vomiting accompanied by postural changes. She had no respiratory symptoms and denied other discomfort. Computed tomography (CT) of her chest showed multiple nodules and masses in her right lower lung lobe (Fig. ). Laboratory data, including results of routine blood tests and tumor markers (carcinoembryonic antigen [CEA] 4.1 ng/ml), were all normal. Finally, she was diagnosed with posterior circulation ischemia and received symptomatic treatment. She did not take the abnormalities in her lung seriously and declined to undergo further examination.
After nearly 7 months, the patient came to our respiratory outpatient department and underwent enhanced CT so that we could observe the changes in her lung, which showed scattered multiple nodules and masses in her right lateral basal and posterior segments, more serious than the previous time (Fig. ). Hospitalization was recommended for further examination and treatment. She had cough as her only respiratory symptom and denied sputum, fever, chest pain, wheezing, malaise, weight loss, or other symptoms. She had not recently traveled or had contact with pigeons’ droppings or with soil, and she had no smoking or alcohol consumption history. Her family members included a healthy husband and a daughter. Her medical history included thyroid adenoma resection 13 years earlier. She had not taken any medicine before she was admitted to our hospital.
Physical examination revealed slightly decreased breath sounds at the right base upon auscultation. The result of the neurological examination was normal. On admission, her pulse was 106 beats/min, blood pressure 130/70 mmHg, and temperature 36.6 °C. Laboratory data, including results for blood cell count, platelet count, renal and liver function, C-reactive protein (CRP), procalcitonin, urinalysis, and stool routine and tumor markers, were all normal, except that CEA was 9.0 ng/ml, higher than the previous measurement. According to the patient’s CT results, we considered that she might have pulmonary bacterial infection and prescribed moxifloxacin and ceftizoxime as empirical treatment. After 2 weeks of antibiotic therapy, another CT examination was performed to evaluate the therapeutic effects. Unfortunately, the lesions shown on the previous CT studies had not resolved, and patchy consolidation was reported, suggesting that another diagnosis, such as pulmonary cryptococcosis, secondary pulmonary tuberculosis, or malignant tumor, should be taken into consideration. After that, a tuberculosis infection T-cell spot test (T-SPOT.TB; Oxford Immunotec, Marlborough, MA, USA) and tests for autoimmune antibodies were performed, but the results were all negative. A histopathological examination of percutaneous lung biopsy from the right posterior segment revealed granulomatous inflammation, and periodic acid-Schiff (PAS) staining showed red-colored yeast walls, suggesting pulmonary cryptococcosis (Fig. ). The patient was then treated with fluconazole 0.2 g twice daily, and her condition was monitored with regular CT examinations.
During 6-month treatment with fluconazole, the patient underwent CT examination a total of four times, showing great improvement in her lesion of the right lateral basal segment, but the lesion in her posterior segment remained almost the same as before (Fig. ). Two months after her drug withdrawal, she had surgery for a femoral neck fracture with cannulated screw internal fixation as an incidental event. CT performed as a routine preoperative examination showed that the multiple nodules and masses in her right posterior segment still had not resolved.
Another 2 months after the operation, she was admitted to our respiratory department for further examination. An enhanced CT study showed that the lesion in her right posterior segment had increased, though the lesion in her right lateral basal segment was decreased (Fig. ). She denied any clinical symptoms, and her laboratory test results were normal. This extraordinary phenomenon led us to think about another diagnosis in this case. Another histopathological examination of percutaneous lung biopsy from the nodule in the right posterior segment was performed. Finally, the result, as we suspected, was adenocarcinoma (Fig. ).
Two days later, the patient turned to our department of thoracic surgery. Thoracoscopy-assisted radical surgery of the right lower lobe was performed. Postoperative pathology showed the adenocarcinoma in her right lower lobe and metastasis in groups 2, 4, and 9 lymph nodes, suggesting stage IIIA (pT1bN2M0) cancer. Four cycles of chemotherapy (pemetrexed 800 mg and carboplatin 500 mg) were administered. Illness evaluation was stable disease. In consideration of the metastasis in the mediastinal lymph nodes, the patient received radiation treatment (50 Gy in 25 fractions) after chemotherapy. The patient’s CEA reduced to 1.12 ng/ml after that. She was regularly followed until January 2018, when a CT examination showed multiple nodules in her right middle lobe. She then received gefitinib 250 mg daily, and the nodules had disappeared in May 2018 when she came to a visit for disease assessment. Gefitinib has been continued.
|
pmc-6214173-1
|
A 5 years old girl (Fig. b, c) (sister of case 1) presented with recurrent infections, including 2 episodes of meningitis at 11 months and 2 years, several episodes of eczema herpeticum and oral thrush. She also had recurrent episodes of upper respiratory tract infections and otitis media. She developed a generalized skin rash at 2 months of age. Her serum IgE was elevated (> 2000 IU/ml) and she had eosinophilia (1000/µl, normal = 450/µl). Her serum immunoglobulins, isohemagglutinin titre, lymphocyte subsets and NBT assay were normal. The anti typhoid Vi vaccine was not done. The National Institutes of Health (NIH) score was 29 (Table ).
A heterozygous mutation with a nucleotide exchange of 1145 G to A in exon 13 leading to an amino acid change R382Q in the DNA binding domain identified in the STAT3 gene.
|
pmc-6214173-2
|
A 9 year old girl presented with recurrent infections from infancy. This included umbilical sepsis and a generalized skin rash during the neonatal period, recurrent skin abscesses since 6 months of age and recurrent episodes of otitis media. She has retained 2 primary teeth. Chest X ray revealed of a right upper lobe pneumatocele. She was diagnosed with the β thalassaemia trait. Her serum IgE was elevated (> 2000 IU/ml), while her eosinophil count was mildly increased (500/µl, normal = 450/µl). Her serum immunoglobulins, isohemagglutinin titre, anti typhoid Vi antibody titre, lymphocyte subsets and NBT assay were normal. The National Institutes of Health (NIH) score was 37 (Table ).
A heterozygous mutation with a nucleotide exchange of 1909 G to A in exon 21 leading to an amino acid change V637M in the SH2 domain identified in the STAT3 gene.
|
pmc-6214173-3
|
A 7 year old girl presented with recurrent infections from the neonatal period, including septicemia on day 9 after birth with S. aureus isolated from blood, recurrent skin abscesses which were drained on 5 occasions, 3 episodes of pneumonia, several episodes of otitis media and chickenpox which was complicated by post varicella pneumonia at 7 years of age. She also developed a rash on the scalp and face in the neonatal period. Her chest X ray showed multiple pneumatoceles. She has had a pericardial effusion, and a peripancreatic abscess diagnosed by ultra sound. Both were aspirated. She had retained 2 primary teeth. She died at 7 years 5 months following a brief respiratory tract infection. Her serum IgE was elevated (> 2000 IU/ml), while her eosinophil count was increased (8650/µl), normal = 450/µl. Her serum immunoglobulins, isohemagglutinin titre, lymphocyte subsets and NBT assay were normal. The anti-typhoid Vi vaccine was not performed as the patient died before the post vaccine sample was collected. The National Institutes of Health (NIH) score was 59 (Table ).
A heterozygous mutation with a nucleotide exchange of 1144 C to T in exon 13 leading to an amino acid change R382W in the DNA binding domain was identified in the STAT3 gene.
|
pmc-6214173-4
|
An 11 year old boy presented with recurrent abscesses since 4 months of age, including skin, dento-alveolar and lung abscesses. He developed a pyo-pneumothorax at 3½ years, and the aspirated pus grew P. aeruginosa. He had also developed a skin rash at the age of 6 months. He had a greenstick fracture of the right ulna at 6 years. His serum IgE was elevated (> 2000 IU/ml). His eosinophil count, serum immunoglobulins, isohemagglutinin titre, anti typhoid Vi antibody titre, lymphocyte subsets and NBT assay were normal. The National Institutes of Health (NIH) score was 28 (Table ).
A heterozygous mutation with a nucleotide exchange of 1909 G to A in exon 21 leading to an amino acid change V637M in the SH2 domain identified in the STAT3 gene.
The laboratory investigations and STAT3 mutations detected in our patients are presented in Table . STAT3 mutations are presented in figure. Table shows a comparison of our patients, with those from the west [, ] India [], ethnic Chinese from China [, ], Hong Kong [] and Taiwan [], and Japanese patients [].
|
pmc-6214646-1
|
A 71-year-old Korean man presented from home with his family members for episodes of agitation, delusions, and confusion occurring intermittently over the past few weeks. The patient had no history of head injury, neck pain, recent stressors, travel or new medication. His past medical history was significant for diabetes, which was managed by lifestyle modifications. Family history was negative for any psychiatric history. At the time of the interview, the patient denied any headaches, chest pain, weight loss, abdominal pain, or dysuria. According to the patient's family, his behavior changed over the last three weeks and was marked with “making things up,” where he made bizarre, grandiose statements that he was a "billionaire" and a "lawyer,” among other inaccurate statements. The family also reported the patient underwent a personality change with increased irritability, aggressive outbursts towards neighbors and family (e.g., he punched a family member), and gathering/saving trash. Further, his family also reported episodes of forgetfulness along with his personality change.
On initial examination, the patient had no insight; he stated that he had no psychiatric problems, that he was “richer than Bill Gates,” and that he could “buy a new house every month.” The patient also stated that his family did not understand him, and he would have to move to California where he has “many friends.” It is notable that, despite these claims, he oriented to time, place, and person during the interview. Upon initial assessment, the patient was admitted to the medical floor to rule out any underlying medical condition given his age at presentation as well as the sudden onset of symptoms. Clinical laboratory assessments included a drug screen, N-methyl-DA receptor antibodies, vitamin B12, folate, and syphilis screen along with thyroid studies, comprehensive metabolic panel, and complete blood count. The results of all laboratory assessments were either normal or within reference ranges. Radiologic studies performed included a computerized tomography (CT) scan and magnetic resonance imaging (MRI) of the patient’s head. The MRI showed a signal abnormality in the cerebral hemispheric white matter consistent with a chronic microvascular change (Figure ). The CT scan showed no acute abnormalities (Figure ). The psychiatry consultation-liaison followed the patient during medical admission. Although the presentation was consistent with bipolar disorder, the psychiatry consultation-liaison recommended a medical evaluation. Once the patient was medically cleared, the patient was started on aripiprazole and transferred to the inpatient psychiatric unit for further assessment and treatment.
Upon admission to the inpatient psychiatric unit, the patient appeared to respond well to aripiprazole with a reduction in symptoms. Therefore, his dose was increased. After the patient reported concerns of poor sleep, we started him on trazodone. However, within the first few days of his hospital course, the patient became increasingly delusional, grandiose, and threatening towards others. As his symptoms did not resolve over the next few days, the patient’s aripiprazole dose was reduced and then discontinued. The patient was then started on risperidone. As recommended by the neurology consultation, his care team performed a diagnostic lumbar puncture, which revealed no pathological findings. On risperidone, the patient’s mood continued to appear stable, his delusions lessened in intensity, and he functioned well on the unit. The patient was discharged home with follow-up instructions at an outpatient psychiatric care center.
|
pmc-6214677-1
|
A 51-year-old Bolivian male with a past medical history of long-standing rheumatoid arthritis which was treated with methotrexate and steroids 10 years prior to his presentation when he noticed an enlarged spleen. He was diagnosed with Felty’s syndrome and was treated with corticosteroids. Later, he was found to have a decreased white cell count which was treated with filgrastim. Several months before hospitalisation, the patient experienced increasing fatigue, night sweats and weight loss of 10 Ibs and was admitted to the hospital after developing nausea. There was no history of fever, joint pain or skin rash.
On physical examination, vital signs included a blood pressure of 126/77 mmHg, heart rate of 93 bpm, respiratory rate of 12/minutes and temperature of 36.5 °C. Abdominal examination revealed a flat abdomen with a total liver span of 10 cm and a splenomegaly of 15–16 cm below the left costal margin. There was no lymphadenopathy in the cervical, supraclavicular or axillary areas. Laboratory findings were significant for leukopenia (white blood cell 0.58 × 109/L), anaemia (Hgb 8.5 g/dL) and thrombocytopenia (platelets 54 × 109/L), i.e., a pancytopenia.
A computerised tomographic scan of the chest, abdomen and pelvis showed a massively enlarged spleen measuring 12.3 × 21.2 × 30.1 cm. There was a poorly defined mass in the spleen consistent with lymphomatous involvement. The liver was also enlarged measuring 18.2 × 20.2 × 21.5 cm. Bone marrow biopsy revealed small to intermediate sized T lymphocytes in an interstitial and intrasinusoidal distribution involving approximately 30% of the cellularity of a 98% cellular marrow and mild reticulin fibrosis; flow cytometry revealed T lymphocytes that were positive for CD3, CD7, CD2, CD45 and TCR-γδ and negative for CD5, CD4, CD8 and TCR-αβ. Chromosomal abnormalities were not detected. Based on these findings, a diagnosis of HSTCL was made.
|
pmc-6214689-1
|
A 90-year old female patient was admitted to the Emergency department (ED) of our hospital in December 2017 after trauma injuries. Following initial examination and due to nature of injuries, she was transferred to our dislocated traumatology unit where further diagnostic examinations were performed.
As a part of diagnostic processing, samples were referred to our laboratory for routine haematology, coagulation, biochemistry, blood gas and urine analysis.
Peripheral blood for CBC, collected by venipuncture in 3.5 mL Vacuette® tube (K3EDTA, Greiner Bio-One, Kremsmunster, Austria), was analysed on Sysmex XT-1800i haematology analyser (Sysmex Corporation, Kobe, Japan). The results on admission are presented in , Sample 1. Red blood cell count and haematocrit (Hct) values were surprisingly low and did not correlate with the haemoglobin (Hb) concentration. Consequently, RBC indices were spuriously increased, especially MCH and MCHC, while Plt and WBC count seemed valid. Additionally, the RBC results were flagged by the analyser. Because of flags which indicated RBC agglutination and the obviously erroneous RBC results, the tube was visually checked for clumps and the presence of micro-aggregates was established. After centrifugation, haemolysis of the sample was noted. According to our laboratory protocol, these results were not released to the clinician and a new sample was requested from the ward.
A new whole blood sample was delivered to our laboratory. After visual inspection of the sample for clumps, we excluded their presence and CBC testing was repeated. The test results were almost equal to those measured in the first sample (, Sample 2). Again, low RBC and Htc with high MCH and MCHC values were observed. The analyser’s flags were the same. Blood smear was prepared, using the May-Grünwald-Giemsa stain (Merck, Darmstadt, Germany), and examination under the light microscope (Olympus BX43, Olympus, Tokyo, Japan) revealed clusters of RBCs (). At this point, the presence of CAs was suspected and the tube was incubated at 37 °C for 30 minutes. The CBC results after incubation seemed corrected (, Sample 2a). Clusters of RBCs were not observed in the blood smear prepared from the warmed sample (). According to our intralaboratory procedure based on Sysmex’s document for flagging interpretation, we centrifuged the warmed sample for 10 min at 1800xg and replaced the supernatant plasma with the same volume of particle-free commercial diluent (Cellpack®, Sysmex Corporation, Kobe, Japan) (). This procedure is sometimes required in cases with high CAs titers. The sample was well mixed and CBC test was run again. The results obtained were comparable to those obtained by incubating the sample at 37 °C (, Sample 2b).
To confirm the presence of CAs, we performed some additional analyses. Direct and indirect antiglobulin (Coombs) test using anti-IgG and anti-C3 antibodies were performed using MONOGnost® monoclonal polyspecific anti-human globulin reagent (Biognost, Zagreb, Croatia) and the results were positive for direct and negative for indirect antiglobulin test. The results indicated in vivo sensitization of patient RBCs with IgG and/or C3 component of complement. Furthermore, the concentrations of IgG, IgA, IgM, C3 and C4 component of complement in patient’s serum were determined on the Abbott Architect c8000 automated biochemistry analyser (Abbott Laboratories, IL, USA). Only concentration of IgM was above the upper reference limit which indicated that CAs in this case might be IgM class, such as usually found in CAD ().
To prevent future analytical problems related to the presence of CAs in the patient’s sample, we informed the clinical staff on the importance of immediate analysis of this patient’s sample (i.e. immediate sample transport after collection was instituted for this sample alone). The results of haematology parameters determined during the rest of the patient’s hospitalization period are presented in . There were no further analytical problems when the sample was immediately transported to the laboratory. On the 3rd day of hospitalization, to correct for anaemia, the patient received two doses of RBC concentrate.
|
pmc-6214886-1
|
A retired 76 year-old Caucasian man, BMI 27, in July 2017 was referred to OUR INSTITUTION with a 2-year history of persistent abdominal pain, resistant to analgesics (Paracetamol and Ketoprofen), irregular bowel habits and rectorrhagia. He reported a 1-year history of iron-deficiency anemia (≈7 g/dl), treated with blood transfusions and investigated with upper and lower endoscopy. The patient had a medical history significant for type 2 diabetes mellitus, treated with Metformin, and small cell carcinoma of the bladder, treated with radical cystectomy and orthotopic ileal neobladder, radiotherapy was not performed. In 2003 he was diagnosed an incisional hernia and underwent open prosthetic repair with a composite mesh (Composix™ E/X Mesh, Bard) implanted in intraperitoneal position.
At admission to our Unit, a physical exam showed a well-healed midline laparotomy incision with no evidence of hernia. As completion to previous endoscopic procedures, a CT scan was performed showing entero-enteric fistulae and migration of prosthesis into adherent intestinal loops (, ). Informed consent had been previously given by the patient who was treated by a high volume experienced surgeon. The patients underwent laparotomy and a large mass of about 25 cm of diameter, consisting of adherent ileal loops, was found (). Prosthetic material penetrating the bowel was detected, resulting in a natural by-pass between the intestinal loops, which explained the absence of canalization-related symptoms. A dual intestinal resection was performed. An inflammatory process involving the rectus muscles made the abdominal wall repair very challenging. A 30 × 30 cm absorbable mesh (Vycril®-Ethicon) was used to reconstruct the posterior fascia of the rectus muscles. A transversus abdominis release (TAR) could not be used due to the critical conditions of the posterior components of the abdominal wall.
According to VHWG grade III, an appropriately shaped, not cross-linked, 20 × 30 cm biological implant (SurgiMend®, Integra LifeScience) was positioned in the retromuscular site. A Prevena™ Incisional System (KCI) was used to protect the skin and removed after 6 days. A small dehiscence of the lower third of the surgical wound was found and treated with V.A.C.® Therapy (KCI) for one week, and then with advanced wound care. The patient was discharged on the 23rd postoperative day. The 3-month clinical examination showed the surgical wound well healed. After 9 months the patient is still in good health, with complete resolution of previous anemia.
|
pmc-6215343-1
|
We present the case of a 52-year-old Mexican man who worked as an office employee and lived in a suburban area of the city of Colima, Mexico. He and his wife denied recent trips outside the city. Zoonosis was absent. He was not physically active and did not have an adequate diet. Pathological antecedents revealed liver cirrhosis, diagnosed 12 years ago, alongside esophageal varices that had been treated with sclerotherapy 7 years prior. He also had a diagnosis of essential hypertension, diagnosed 20 years ago. His treatment prior to hospitalization included 20 mg of propranolol every 12 hours, which was used to treat his essential hypertension, esophageal varices, and to reduce his portal hypertension.
He arrived at our institution “IMSS General Hospital Zone 1, Colima” in the early afternoon (day 1, see Table ). He began to experience extreme pain localized in his right foot, 16 to 18 hours prior to admission, with a local pain scale of 10/10. He denied any recent forms of punctures to the overlying skin (including animal and insect bites).
On physical examination during admission, he was somnolent, oriented in person and space, but not oriented to time. His vital signs were: arterial pressure (AP, systolic/diastolic) 67/49, mean AP (MAP) 55, heart rate (HR) 88, respiratory rate (RR) 16, and body temperature 36 °C. He presented hepatopathy facies and spontaneous eyelid opening, his oral mucosa was dry, and his neck showed jugular engorgement grade I. Both hemithorax were slightly hypoventilated with no adventitious sounds. Precordial was rhythmic with low intensity sounds and no murmurs were heard. Peristalsis was present in his abdomen but low in intensity and there was no hepatosplenomegaly. His upper extremities were symmetrical, eutrophic, with no signs of edema; he moved his upper extremities freely without any limitation, no asterixis was present, and a force scale of 4/5 was seen. During exploration of inferior extremities, there was a clear asymmetric pattern. His right lower extremity was volume augmented with signs of edema ++ including large and small bullae formation with serohematogenous liquid inside involving most of his right foot and ankle (Fig. ). The pedal pulse was present but weak and had a local elevated temperature on palpation. His movements were markedly limited due to extreme pain. Deep vein thrombosis maneuvers were performed and were not present. Plantar reflexes were also not seen.
A peripheral intravenously administered high-dose double scheme of antibiotics (clindamycin plus ceftriaxone), crystalloid fluids, and corticosteroids was initiated. Laboratory studies were performed soon after admission: hemoglobin (Hb) 12.6 g/dl, hematocrit (Htc) 40.3%, mean corpuscular volume (MCV) 103.9 fL, white blood cells (WBC) 39,400/mm3, neutrophils 37,460/mm3, platelets 104,000/mm3, thrombin time (TT) 26.7 seconds, international normalized ratio (INR) 2.15, partial thromboplastin time (PTT) 42.8 seconds, glucose 61 g/dL, urea 102.72 mg/dL, creatinine 1.8 mg/dL, total bilirubin (TB) 3.4 mg/dL, direct bilirubin (DB) 2.6 mg/dL, indirect bilirubin 0.8 mg/dL, albumin 1.5 g/dL, alanine aminotransferase (ALT) 35 U/L, aspartate aminotransferase (AST) 58 U/L, P 7.1 mEq/L, Ca 8.4 mEq/L, Cl 106 mEq/L, K 6.4 mEq/L, Na 129 mEq/L, Mg 1.72 mEq/L, lactate dehydrogenase (LDH) 420 U/L, and C-reactive protein (CRP) 16.07 mg/L. Correction of hypoglycemia and electrolyte imbalance was initiated. Deep vein thrombosis was ruled out by clinical assessment and Doppler ultrasound. Aspiration of bulla liquid was obtained and sent to a laboratory for cultivation (positive to Cedecea lapagei, results returned on day 2). Interdisciplinary consultation with angiology was carried out (no indications were added). He was classified with a Sequential Organ Failure Assessment (SOFA) score of 11, Acute Physiology and Chronic Health Evaluation (APACHE) II of 22 points (42.4% mortality rate), and a Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score of 11 points (high risk > 75% of having necrotizing fasciitis) [].
In the late afternoon (day 1) his somnolence and disorientation persisted, he was hypotensive with an AP of 80/40 with a MAP of 55, and he was unresponsive to fluid resuscitation. His hypoglycemia persisted despite vigorous treatment. No fever was present and his skin was pale. His left lower limb had ascending progression of serohematogenous bullae proximal to the patella. His extreme pain continued. Since our patient’s MAP was persistently below 65 mmHg despite intravenously administered fluid therapy, an anterior jugular central venous catheter was placed and we decided to include norepinephrine 8 mg in 0.9% 1000 cc physiological solution passing at a rate of 8 ml/hour, categorizing the case as septic shock []. Concomitantly he had developed acute kidney injury (AKI) “Kidney Disease: Improving Global Outcomes” (KDIGO) stage II.
During the night (day 1), abundant large fetid serohematogenous bullae and edema continued to advance up his right thigh with persistent generalized hypoperfusion. Because of previous mentioned conditions, it was decided to change antibacterial therapy to intravenously administered imipenem and clindamycin. Bullae fluid culture results and antibiogram were not available at that time. Norepinephrine was boosted to maintain MAP above 65 mmHg. Intensive care unit (ICU) was called in for valorization to admit our patient. Unfortunately, due to the lack of space in the ICU, this idea was dismissed. Since rapid deterioration was evident, new laboratory studies were ordered (approximately 10 hours after initial blood test): Hb 10.1 g/dL, Htc 32.7%, MCV 107.5 fL, WBC 11.6/mm3, platelets 35,000/mm3, TT “Does not coagulate”, PTT “Does not coagulate”, glucose 30 g/dL, urea 115.5 mg/dL, creatinine 2.4 mg/dL, K 6.4 mEq/L, Na 130 mEq/L, LDH 406 U/L, and CRP 220 mg/L. His SOFA score was 16.
Necrotizing fasciitis was our primary diagnosis due to clinical and laboratory findings (LRINEC score of 12 points). Even though the diagnosis was very likely, the lack of a biopsy study meant that we could not confirm such a diagnosis. Multiple organ dysfunction secondary to septic shock was also diagnosed. The differential diagnosis of animal or insect bite was dismissed due to lack of exposure history and lack of puncture wound.
During the next morning (day 2) he maintained the same general conditions. Norepinephrine was boosted up again (MAP 50). Soon after, he entered cardiopulmonary arrest and cardiopulmonary resuscitation was performed. Unfortunately he died later that morning.
|
pmc-6215387-1
|
A 40-year-old man presented with straining at micturition and progressive thinning of urinary stream for the past 1 year. The patient had a history of urethral stricture disease and underwent OIU 5 years ago. Retrograde urethrogram revealed a short segment mid-bulbar urethral stricture (). The patient underwent uneventful day care OIU performed under spinal anesthesia. OIU was performed with cold cutting knife at 12 o'clock position and 18F silicon catheter placed. Following catheter removal after 7 days, the patient had profuse bleeding per urethra. The patient was admitted in emergency services and worked up. All hematological and coagulation profiles were normal. Bleeding stopped after perineal compression. 18F Foley catheter was reinserted and patient kept on observation for 6 hours during which period there was no fresh bleeding episode. Catheter was again removed after 48 hours following which the patient had repeat profuse urethral bleed. CT angiography was carried out in view of recurrent profuse bleeding, which showed right bulbourethral artery pseudoaneurysm of dimensions 9 × 8 × 8 mm (). The patient was transfused 3 U of packed red blood cells as hemoglobin dropped from 10.4 to 6.2 g/dL. As angioembolization facility was unavailable due to technical reasons, we sought for USG-guided compression repair of urethral artery pseudoaneurysm. Pseudoaneurysm was localized with colour Doppler, and perineal focused USG compression was performed for about 25 minutes (). The patient was kept on observation for 48 hours in hospital; did not have any fresh bleeding episode. On close follow-up, the patient had no recurrence of bleed. Per urethral catheter was removed after 2 weeks and the patient was voiding well. A repeat CT angiography showed resolution of pseudoaneurysm ().
|
pmc-6215535-1
|
A 30-year-old woman was referred to our department with a 2-week history of sudden vision loss in the left eye. Her medical history was notable for fever and general malaise 2 weeks earlier. On examination, her best-corrected visual acuity was 20/20 in the right eye and 20/500 in the left eye. Pupils were equally round and reactive to light with no relative afferent pupillary defect. Results of anterior segment examination were unremarkable, and there were no vitreous cells in either eye.
Dilated fundus examination of the left eye showed foveal granularity surrounded by diffuse deep yellow-white retinal discoloration with satellite slightly indistinct, multifocal deep yellow-white dots. There also were blurred disc margins with peripapillary whitening (Fig. ). The fundus of the right eye was unremarkable. Fundus autofluorescence (FAF) imaging of the left eye revealed multiple, coalescent, punctate hyperautofluorescent lesions associated with focal small hypoautofluorescent areas (Fig. ). Fluorescein angiography (FA) showed early confluent patchy areas of choroidal hypofluorescence and late punctate hyperfluorescence with a “wreath-like” configuration around the fovea and optic disc leakage (Fig. , ). Indocyanine green angiography (ICGA) showed in the early and intermediate phase well-demarcated geographic areas of choroidal hypofluorescence in the posterior pole extending beyond the clinical limits of the yellowish retinal lesions that became more visible and more confluent in the late phase. Large choroidal vessels were visualized within these hypofluorescent areas, excluding any masking effect. There also were associated peripapillary hypofluorescence and optic disc staining indicating severe inflammation (Fig. , ). Swept-source OCT (SS OCT) showed outer retinal layer changes including disruption of the ellipsoid zone, irregularities of the RPE, and accumulations of hyperreflective material resting on the RPE and extending anteriorly through the interdigitation zone, ellipsoid zone, and outer nuclear layer toward the inner retina. SS OCT also showed hyperreflective dots in the inner choroid and choroidal thickening (subfoveal choroidal thickness of 370 μm in the left eye vs 250 μm in the fellow unaffected eye) (Fig. ). Swept-source OCTA (DRI OCT Triton plus; Topcon) showed confluent extensive geographic areas of loss of signal in the choriocapillaris that colocalized with the ICGA hypofluorescent areas, highly suggesting a choriocapillaris ischemia (Fig. ). All imaging findings in the right eye were unremarkable.
Results of physical examination were normal. Laboratory tests, including a complete blood count, chemistry panel, serological test for syphilis, C-reactive protein, and erythrocyte sedimentation rate, were performed, all of which were within normal limits or negative. A chest X-ray was also unremarkable. Tuberculin skin test showed a 20-mm induration, and the result of QuantiFERON-TB Gold test was positive.
The patient received oral prednisone initiated with a dose of 1 mg/kg/day and then progressively tapered. The patient was also given isoniazid 300 mg/day and rifampin 600 mg/day for 3 months to treat latent tuberculosis.
Nine months after initial presentation, visual acuity had improved to 20/20. The white retinal dots had completely resolved, leaving some areas of RPE depigmentation on FAF, FA, and ICGA (Fig. –). SS OCT showed complete resolution of abnormal findings with a recovery of a quite normal outer retinal and choroidal aspect (Fig. ). OCTA demonstrated markedly improved flow deficits of the choriocapillaris (Fig. ). The ocular findings remained unchanged over a further follow-up period of 12 months.
|
pmc-6215577-1
|
A 35-year-old man was admitted at the oculistic emergency room for a right exophthalmos accompanied with severe pain, periorbital swelling, redness, and mucopurulent discharge. The patient, HIV and HCV positive for 11 years, had a history of drug and alcohol addiction, including habitual marijuana smoking. Clinical examination revealed severe nonreducible exophthalmos with bulb dislocated medially and inferiorly, associated with eyelid chemosis and decreased vision in the right eye (1/10). The motility of the globe in all gazes was restricted by mechanical proptosis. Fundus examination revealed optic disc swelling associated with venous congestion. A brain MRI was performed in order to evaluate the involvement of the orbit and the brain. The examination showed an intraorbital mass that was inhomogeneously hypointense on T2-weighted images and hypointense on T1-weighted images and showed intense and inhomogeneous contrast enhancement. The mass was located in the lateral orbit cavity extending to the ethmoid and maxillary sinuses, with bony destruction, encasing and displacing the medial rectus and superior oblique muscles, optic nerve, ocular globe, and vascular structures ().
On the basis of these findings and considering patient's history of HIV related diseases and drug addiction, few diagnosis have been hypothesized that included neoplastic diseases, e.g., lymphoma, and infections, in particular mycotic origin, such as aspergillosis, considering the known association with marijuana smoking. Prior to beginning any treatment, the patient was subjected to a biopsy of the orbital mass. At histology H&E stain of the tumor showed a monomorphic proliferation of plasmablastic cells that was suggestive of plasmablastic lymphoma. The patient died within a week following diagnosis for a heart attack, even before further treatment could be initiated.
|
pmc-6215578-1
|
A 74-year-old male with history of stage III sigmoid adenocarcinoma 15 years ago treated with sigmoid colectomy followed by adjuvant 5-fluorouracil (5-FU) chemotherapy presented to his local hospital with acute worsening of epigastric pain associated with nausea and vomiting. On physical examination, the patient was febrile at 38.5°C, tachycardic, and normotensive. Abdominal examination revealed tenderness in the right upper abdomen and rigidity of the abdominal wall with positive Murphy's sign. Laboratory testing revealed a hemoglobin level of 11.5 g/dl and a white cell count of 16/μl with 80% neutrophils, and other tests were within normal range (which included liver enzymes, bilirubin, LDH, lipase, and amylase).
CA19-9 was elevated at 4945 IU/ml, and the CEA level was measured at 24.11 μg/l.
Abdominal ultrasound revealed a sludge and irregular thickness of the gallbladder.
The patient was started on intravenous broad-spectrum antibiotics immediately. Laporascopic cholecystectomy was performed the day after admission. Unfortunately, the postoperative course was complicated by a septic shock and required ICU admission for few days (). The initial pathology of the gallbladder showed a moderately differentiated adenocarcinoma of unknown primary possibly due to gall bladder primary. Further investigations revealed a cecal mass with regional retroperitoneal lymphadenopathy.
The patient was referred to our hospital where he had a biopsy of the latter mass, and the histopathology result was consistent with a moderately differentiated adenocarcinoma of colonic origin. A comprehensive pathological review of the gallbladder specimen was performed, and reexamination and further immunohistochemical analysis including epithelial cytokeratins 7 and 20 (CK7 and CK20) and homeobox protein-2 (CDX-2) were done. Tumor cells isolated from the specimen were positive for CK20 and CDX-2 and negative for CK7.
Our patient was confirmed to have metastatic disease from colon primary; therefore, he was started on palliative capecitabine with significant symptomatic improvement reported after two cycles. He continues to tolerate chemotherapy.
|
pmc-6215580-1
|
A 40-year-old G5P3103 at 12 weeks and 3 days with a history of two prior cesarean sections and known heterotopic pregnancy consisting of cesarean scar pregnancy and intrauterine pregnancy () presented ten days after successful selective reduction of cesarean scar pregnancy with potassium chloride (KCl) injection in the ultrasonography unit.
The patient reported two days of fevers prior to her presentation with new onset vaginal bleeding. After her initial visit after selective reduction, she was treated with Nitrofurantoin for a urinary tract infection at urgent care. She presented two days after urgent care to our emergency department (ED) with pain and ultrasonographic evaluation that demonstrated no fetal heartbeat and discharge home for follow-up with her provider for management options. Three days after ED visit, she represented with new onset fevers, chills, back pain, and scant vaginal bleeding. She denied any significant past medical history and her previous surgical procedure included gastric bypass surgery, two cesarean sections, and an endometrial ablation for heavy menses.
On physical examination, the patient was febrile, tachycardic, hypotensive, and being in septic shock. She had scant dark blood in the vaginal vault, a 16 week size uterus with fundal tenderness. Ultrasound confirmed presence of no fetal cardiac activities and presence of high vascular flow to the myometrium surrounding the cesarean scar pregnancy ().
The patient was counseled on septic abortion and she underwent a complete infectious disease workup, including blood cultures, urine cultures, and chest x-ray, and was started on broad-spectrum antibiotics (ampicillin, gentamicin, and clindamycin). Initial urine culture demonstrated no growth and initial blood culture grew Enterococcus faecalis. The patient desired uterine preservation and underwent bilateral uterine artery embolization using absorbable gelfoam and scheduled dilation and curettage under sonographic guidance the following day. The procedure was complicated with intraoperative hemorrhage of 1000cc that resolved with uterotonic medications and blood transfusion. Final pathology was consistent with products of conception. Hospitalized postoperatively, she continued to have daily low- grade fevers while on antibiotics with persistent daily positive blood cultures with Enterococcus faecalis. Antibiotic regimen was changed based upon the sensitivities to ampicillin and ceftriaxone, a normal echocardiogram ruled out endocarditis, and repeat transvaginal ultrasound demonstrated a large amount of heterogeneous avascular material in the lower uterine segment. The postoperative serum beta-hCG was 896 IU/L. CT scan of the abdomen and pelvis demonstrated a large high-density material within the endometrial cavity of lower uterine segment ().
With concerns for persistent bacteremia, failed antibiotic therapy, and retained materials within the uterine cavity, the patient underwent a total abdominal hysterectomy and bilateral salpingectomy. The specimen was bivalved to show the entire uterine cavity with large amounts of blood clots, adherent placental tissue as well as a very thin anterior uterine segment ().
Final pathology demonstrated products of conception with associated chronic and acute inflammation. Uterine culture obtained at hysterectomy demonstrated growth of Enterococcus faecalis, confirming the uterus as source of bacteremia. The patient had resolution of her fevers and negative blood cultures after the hysterectomy. Intravenous antibiotics were discontinued on postoperative day four and transitioned to a two-week course of Augmentin. She was discharged home with a two-week office follow-up. At her postoperative check and six-week visit she recovered fully.
|
pmc-6215581-1
|
An 18-year-old African American female with a history of hyperthyroidism presented to our facility with fever, chills, body aches, significant cervical lymphadenopathy, facial edema, and a progressive (eventually generalized) macular morbilliform rash. She reported that elevated transaminases had been noted during a recent visit with her endocrinologist. Her CBC included a WBC count of 8,600 mm3, hemoglobin of 12.4 g/dl (MCV 77.8 fL), and platelet count of 261,000 mm3. Ferritin was markedly elevated (1229.6 ng/mL); serum iron, TIBC, and iron saturation were decreased, suggestive of anemia of chronic disease. AST was elevated to 127 units/mL, and ALT was elevated to 239 units/mL. Hemoglobin electrophoresis revealed normal adult hemoglobin. A rapid HIV test was nonreactive. PCR testing of peripheral blood was negative for EBV and HHV-6. Multiple blood cultures were negative.
Abdominal ultrasound showed splenomegaly and enlarged porta hepatis lymph nodes. A PET scan revealed diffuse hypermetabolic lymphadenopathy involving cervical, supraclavicular, axillary, pelvic, and inguinal nodes, as well as findings consistent with malignant infiltration of the bilateral kidneys and spleen (). Although the possibility of a drug reaction had been in the differential diagnosis prior to the imaging studies, the degree and extent of the imaging abnormalities raised clinical concern for a malignant process.
Due to the concern for malignancy, axillary lymph node and bone marrow biopsies were performed. Examination of the bone marrow showed that it was appropriately cellular for age (∼80%), with maturing trilineage hematopoiesis, polyclonal plasmacytosis, and eosinophilia. Scattered small T-cell aggregates were present. Flow cytometric immunophenotyping did not detect any abnormal lymphoid populations. No evidence of malignancy was identified.
Histologic examination of the lymph node revealed mostly preserved, but significantly distorted, nodal architecture with expansion of the paracortex by a mixed infiltrate of small lymphocytes, eosinophils, histiocytes, plasma cells, and scattered large atypical lymphoid cells, including occasional Reed–Sternberg-like cells. Secondary follicles were largely absent (Figures and ). A few apoptotic bodies and pigment-containing histiocytes were identified.
Immunohistochemical stains for CD20, PAX5, CD79a, OCT2, and BOB-1 highlighted the B-cell population in the cortex that was largely confined to primary follicles. CD23 highlighted irregularly expanded follicular dendritic cell meshworks. CD3 highlighted numerous T cells in the paracortex and interfollicular areas. CD15 highlighted granulocytes. CD30 highlighted scattered large immunoblasts, including rare Reed–Sternberg-like cells (); no sheets of positive cells were seen. MUM1 was positive in plasma cells, predominantly in the medullary cords and sinuses. ALK immunostaining and EBV in situ hybridization (EBER) were negative.
The lymph node findings, including architectural distortion, expanded follicular dendritic cell meshworks, eosinophilic infiltrate, and proliferation of large CD30-positive lymphoid cells raised concern for a malignant process such as T-cell lymphoma or classic Hodgkin's lymphoma; however, the morphologic and immunophenotypic features were not entirely typical for those diagnoses. Molecular analysis of the lymph node did not detect any monoclonal IGH or IGK gene rearrangement or T-cell receptor gene rearrangement.
Two days after the lymph node biopsy was performed, the patient's WBC count had risen to 24,100/mm3. Examination of the peripheral blood smear revealed neutrophilia, atypical lymphocytes, and mild relative eosinophilia, with an increased absolute eosinophil count of 1,400/mm3. On further review of the patient's history, it was noted that the patient had received a course of minocycline to treat folliculitis, beginning approximately five weeks prior to admission and ending four days prior to admission.
After consideration of all of the available information, a diagnosis of DIHS was made. The minocycline was discontinued permanently. Following treatment with prednisone, the patient's rash, leukocytosis, and lymphadenopathy gradually resolved.
|
pmc-6215582-1
|
A 26-year-old male patient with no remarkable medical and social history presented with a five-day history of a cough and fever. Before coming to our hospital, he went to a clinic and received amoxicillin, which resulted in no improvement of his symptoms. He visited another hospital three days before presenting to us and was hospitalized with a diagnosis of bacterial pneumonia. Although he received tazobactam/piperacillin, his clinical symptoms showed deterioration. Two days later, he was admitted to our hospital.
His vital signs were as follows: temperature, 38.1°C, heart rate of 112 beats/min, blood pressure of 98/60 mmHg, respiratory rate of 24 breaths/min, and saturation of peripheral oxygen of 94% with a reservoir mask of 6 L/min. On physical examination, weak respiratory sounds and coarse crackles were heard in the lower left chest.
His initial white blood cell count was 6,800/μL, hemoglobin was 14.0g/dL, platelet count was 133,000/μL, and C-reactive protein level was 34.30 mg/dL (). Arterial blood gas (reservoir mask of 6 L/min) showed that the pH was 7.44, PCO2 was 41.1 mmHg, PO2 was 69.2 mmHg, HCO3− was 27.2 mmol/l, and BE was 2.8 mmol/L. Among the viral and bacterial rapid tests performed, only the Mycoplasma antigen tested positive.
Chest radiography indicated an infiltrating shadow in left middle lung field and right lower lung field. A chest CT indicated lobar pneumonia in the lower left lobe and an infiltrating shadow in the left whole lung lobe and the lower right lobe (). Based on these findings, the patient was diagnosed with severe mycoplasma pneumonia.
Azithromycin 500 mg/day and prednisolone 30 mg/day were initiated. After admission, his respiratory condition worsened until a reservoir mask 15 L/min was required. We then changed the reservoir mask to a nasal high-flow oxygen (40 L/min, FiO2:0.8) and increased the prednisolone to 60 mg/day. In case of macrolide resistance, we additionally administrated minocycline 200 mg/day. On hospital day 4, the patient's respiratory status and inflammatory markers on laboratory findings improved. On hospital day 20, he was discharged.
|
pmc-6215584-1
|
A 7-year-old Syrian boy with war-related burn injury was referred to our hospital for reconstructive surgery for burn scars and contractures on his face, neck, and body. A consultation with anesthesia department was held by plastic and reconstructive surgery clinic for the preanesthesia evaluation. Patient was conscious and oriented on examination. He had severe scar contractures involving neck, face, anterior chest, and both shoulders leading to restricted mouth opening, no neck extension, and stooped posture with chin and chest fused together by scars and the neck and head contracted in flexed position. The width from upper incisor to lower teeth was approximately 15 mm and Mallampati class was 3, while thyromental and sternomental distance could not be evaluated due to neck and head being contracted in flexed position. Cardiac, thoracic, and laboratory investigations revealed normal findings. Detailed history of the patient obtained from the parents by the help of a translator revealed that the child had been posted for the reconstructive surgery in another university hospital, while the operation was cancelled due to failure to maintain mask ventilation even after pain relief and induction of anesthesia. The previous anesthesiologist had given two attempts after induction of anesthesia but failed at intubation. Then child was awakened. The day after, he was transferred to our hospital for difficult airway approach and the operation. Awake FFB nasal intubation was planned because of the past history of “cannot intubate and cannot oxygenate” scenario. The necessity and details of the procedure were explained to the patient and his family by the help of a translator. After a 6-hour fasting period, the patient was admitted to our intensive care unit (ICU), accompanied by a family member and translator. Following the routine (NIBP, HR, StO2) monitorization (Nihon Kohden, Japan), patient has been informed again about the details and steps of the procedure with the help of the translator. Premedication and sedation were not applied because of the patient's status. During the initial trial phase, nasal drop of xylometazoline 0.1% was instilled for vasoconstriction in both nostrils. Three puffs of 10% lidocaine were implemented for topical anesthesia. Through a nasal cannula, oxygen was administered at 5 L/min through the left side. Tip of the fiberoptic bronchoscope (FOB, 2.8 mm, Karl Storz-Endoskope, Germany) was inserted into the contralateral nostril. Endoscopy was performed. When the vocal cords were visible, the trial procedure was ended. It was explained to the patient and his family that the same procedure would be repeated on the day of surgery as followed by intubation and induction of general anesthesia. On the day of operation, two days after the initial trial, patient was taken to the surgery room and monitored (Infinity Delta Dräger, Lübeck, Germany) routinely (NIBP, HR, SatO2). A nasal drop of xylometazoline 0.1% was instilled for vasoconstriction. Three puffs of 10% lidocaine spray were implemented for topical anesthesia. It directly sprayed onto the mucosa of the mouth, pharynx, and tongue. Through a nasal cannula, oxygen was administered at 5 L/min through the left nostril. Endoscopy was performed through the right nostril. Two ml of 2% lignocaine was sprayed through the FOB on to the glottis after the vocal cords were seen. The FOB's tip was then passed into the trachea through the laryngeal opening and was stopped just above the carina. Lubricated 5.0 nasotracheal tube was railroaded over the FOB. After three ventilations, position of nasotracheal tube was confirmed by the FOB. Successful tracheal intubation had been achieved while maintaining spontaneous ventilation and was monitored by capnography. Propofol, fentanyl, and rocuronium were used for induction of general anesthesia via intravenous route and maintained with remifentanil 0.1 μg/kg/min and sevoflurane in oxygen (Primus workstation Dräger, Lübeck, Germany). The operation lasted for approximately four hours. The contractures on neck and left axilla were released and graft was placed. The intraoperative course was uneventful. The patient was extubated after complete recovery of consciousness, adequate spontaneous breathing, preventive reflex, and muscle strength [] ().
|
pmc-6215586-1
|
A 12-year-old boy reported to our institute with a chief complaint of swelling on the left side of the face for the past six months (). The patient was apparently normal 6 months back after which he noticed the swelling. The patient's general, family, and medical history was not contributory.
Extraorally, there was a presence of diffuse swelling involving the left side of the face, extending superiorly to the left zygomatic arch, inferiorly to the lower border of the mandible, anteriorly to the corner of the mouth, and posteriorly to the retromolar portion of the mandible. There was no obliteration of nasolabial folds seen. On palpation, the swelling was hard, noncompressible, and nonreducible along with diffuse expansion of the middle and lower 3rd of the facial region.
Intraoral examination revealed a swelling involving the retromolar trigone and molar regions with obliteration of the mucobuccal fold. On palpation, the lesion was firm to hard in consistency and nontender with expansion of left buccolingual cortical plates.
Radiological examination of orthopantomogram showed the presence of unilocular radiolucencies extending from the last erupted molar to retromolar region ().
Based on the above clinical and radiological findings, provisional diagnosis of unicystic ameloblastoma was given. Incisional biopsy was carried out, and the specimen was sent to the Department of Oral Pathology and Microbiology. The gross specimen measured around 1 × 0.6 × 0.4 cm to 0.2 × 0.2 × 0.1 cm in dimension, which was firm in consistency, creamish brown in colour, and irregular in shape. The histopathological examination showed sheets of odontogenic islands lined by tall columnar cells enclosing stellate reticulum-like cells along with squamous metaplasia in the connective tissue stroma extending into the peripheral epithelium. (Figures and ). These features suggested the diagnosis of acanthomatous ameloblastoma. IHC Ki-67 also showed mild positivity in fewer areas ().
Further radiological investigations proceeded with CT scan neck (plain and contrast) and CT Brain (). Serial axial sections of the neck were studied from the nasopharynx up to the thoracic inlet before and after intravenous contrast. The findings revealed that an expansile lytic lesion of size 5.2 × 3.4 × 6.5 cm (AP × Trans × CC) with a largely preserved peripheral cortical rim (with few areas of dehiscence), and no matrix calcification was seen arising from the left mandibular ramus involving the coronoid process but sparing the condyle (superior limit approximately 5 mm from the condyle). On contrast administration, heterogeneous enhancement with multiple nonenhancing foci (likely necrotic areas) was noted. Also, an area of few air pockets was seen within. Serial axial sections of the brain were studied. The findings revealed an expansile lytic lesion arising from the left mandibular ramus, with relative preservation of the surrounding cortical rim (with few areas of dehiscence) and no focal space occupying the lesion. There were no metastatic disseminations evident on these imaging findings which conclude the conventional type of ameloblastoma.
Surgical treatment was planned under general anaesthesia. Extraction of 74, 34 and hemimandibulectomy of the left mandible was carried out. The resected mandible was reconstructed by free fibular graft. The weight and measurement of the resected specimen was 87 gms and 9 × 6 × 4 cm, respectively. Histopathological examination of the resected specimen revealed anastomosing follicular islands of the odontogenic epithelium lined by tall columnar cells enclosing stellate reticulum-like cells. Squamous metaplasia of the stellate reticulum-like cells was observed. Areas of cystic degeneration of the stellate reticulum-like cells were also seen. A reactive change of lymphoid tissue was seen. Based on the clinical, radiological, and histopathological examination, a final diagnosis of acanthomatous ameloblastoma was made (Figures –). A three-month postoperative follow-up of the patient showed good prognosis with no recurrence.
|
pmc-6215614-1
|
A 75-year-old woman was admitted to the hospital with abdominal pain, nausea and vomiting for 3 days. She did not drink alcohol, and there was no clinical or biochemical evidence of primary liver disease or coagulopathy. Physical examination revealed mild tenderness in the right upper abdominal quadrant. Laboratory tests revealed that the percentage of neutrophils (N%) was 80.3% (50–70%), alanine aminotransferase (ALT) was 192 U/L (< 64 U/L), aspartate aminotransferase (AST) was 66 U/L (< 64 U/L), γ-glutamyl transpeptidase (γ-GT) was 197 U/L (< 47 U/L), and all other laboratory parameters were normal (e.g., haemoglobin and platelet counts, prothrombin time, and renal function). An abdominal computerized tomography (CT) scan demonstrated dilatation of the extrahepatic bile duct with a stone at the lower CBD and sludge in the gallbladder. (Fig. ) Bile duct cholangiopancreatography revealed a dilated CBD (10 mm in diameter) with a round filling defect (8 mm in diameter) (Fig. ). Balloon dilation (10 mm in diameter) of terminal CBD after a 5-mm long sphincterotomy for extraction of the stone was uneventful. Unfortunately, she presented with cholangitis and a significant increase in the percentage of neutrophils (94%) and cholestatic parameters (total bilirubin 111.1 μmol/L (2–18 μmol/L), direct bilirubin 81.3 μmol/L (< 7 μmol/L), ALT 465 U/L, AST 538 U/L, and γ-GT 634 U/L) after 3 days.
A high-density image of the middle CBD with a markedly dilated biliary tree was revealed on the second CT (Fig. ). Thus, ERCP was repeated. A long filling defect was noted in the dilated common bile duct (Fig. ), and a blood clot (maximum diameter 35 mm × 10 mm) was extracted with a basket (Fig. ). Then, an endoscopic nasobiliary drainage (ENBD) tube was inserted into the CBD to ensure continued biliary drainage. Two days later, her temperature returned to normal, and abdominal pain was relieved. Histopathological examination revealed massive red blood cells with white blood cells and tissue necrosis (Fig. ). After the treatment, she recovered and was discharged without any other complication.
|
pmc-6215629-1
|
A 68-year-old man with left vision deterioration presented to our outpatient clinic in August 2011. His best corrected visual acuity (BCVA) was 20/20 in the right eye and 20/40 in the left eye. Anterior segment examination was unremarkable. Dilated fundus examination revealed red-orange lesions in the macula associated with RPE damage. IA (Spectralis HRA, Heidelberg Engineering, Heidelberg, Germany) revealed a BVN and polyps. OCT (Carl Zeiss Meditec, Dublin, CA) disclosed significant subretinal fluid with RPE detachment. Based on these findings, a diagnosis of PCV was made and IVRs were initiated in a pro re nata (PRN) regimen. After 42 anti-VEGF injections, 7 initial IVRs and subsequent 35 IVAs, over 5 years and 7 months, his left vision remained at 20/40, but OCT showed persistent subretinal fluid and RPE detachment. As IA demonstrated a polyp associated with the BVN (Fig. ), his treatment strategy was switched to combination IVR and PDT according to the EVEREST II study []. IA 3 months subsequent to PDT confirmed complete regression of polyp, with the BVN persisting without apparent regression (Fig. ).
In this patient, the first OCT-A (PLEX Elite 9000; Carl Zeiss Meditec, Dublin, CA) image (Fig. ) was acquired 9 months prior to combination therapy and disclosed the BVN and polyp in the corresponding locations as indicated by IA (Fig. ). Before PDT incorporation, the eye received 9 additional IVRs, but OCT-A after each injection showed no apparent changes in the BVN or polyp (Fig. ). Based on these findings, the treatment strategy was switched to combined therapy of IVR and PDT. Standard full-fluence PDT was performed 3 days after the 43rd IVR, followed by PRN-IVR for subretinal or intraretinal fluid. In full fluence PDT, patients were infused with verteporfin (6 mg/m2). Fifteen minutes after the start of infusion, PDT at standard fluence (light dose, 50 J/cm2; dose rate, 600 mW/cm2; wavelength, 689 nm) was applied to the eye for 83 s. The laser spot size was derived by adding 1000 mm to the greatest linear dimension. Thus, both BVN and polyps were included in the verteporfin PDT treatment area. The effects of combination therapy on the BVN and polyp were investigated by frequent OCT-A performed at every visit to our outpatient clinic. As early as 1 week after PDT, OCT-A revealed complete regression of both the BVN and polyp (Fig. ). Right vision improved from 20/40 to 20/30. The BVN showed gradual reperfusion at 1 month (Fig. ) and 2 months (Fig. ), and had virtually restored its original appearance at 3 months while the polyp remained closed (Fig. ).
We confirmed the changes observed in en face OCT-A images using corresponding OCT-A cross-sectional scans. The en face OCT-A data acquired just before PDT showed the polyp as an aneurysm-like dilatation of the BVN (Fig. ). A cross-sectional OCT-A (Fig. ) image of the PED (the space between Bruch’s membrane and the RPE layer) contained a toal of 3 major flow signals corresponding to the polyp and 2 branches of the BVN, respectively. One week after PDT when en face OCT-A demonstrated complete regression of the BVN and polyp (Fig. ), the residual space between Bruch’s membrane and the RPE layer on cross-sectional OCT-A suggested that the BVN was occluded but the vessel structures were still preserved (Fig. ). The en face OCT-A taken 3 months after PDT demonstrated a very similar BVN to that beforehand except for the absence of the polyp (Fig. ). Cross-sectional OCT-A confirmed that the flow signals corresponding to branches of the BVN were indeed restored but the polyp remained absent (Fig. ). At 3 months after PDT, IA also visualized the BVN, which showed a near-identical appearance as that before combination therapy, along with absence of the polyp (Fig. ).
|
pmc-6215629-2
|
A 65-year-old woman presented with left vision deterioration in July 2017. Her BCVA was 20/20 in the right eye and 20/22 in the left eye. Anterior segment examination was unremarkable. Dilated fundus examination revealed orange nodular lesions in the macula associated with exudative changes. IA uncovered a BVN and polyps. OCT disclosed subretinal fluid with PED. Based on these findings, a diagnosis of PCV was made and she began IVA treatment. After 5 consecutive monthly IVAs, OCT showed persistent subretinal fluid and PED, and so we switched her to combination therapy of IVR and PDT according to the EVEREST II study []. En face OCT-A just before PDT revealed a fan-shaped BVN and a polyp (Fig. ), and corresponding cross-sectional OCT-A at the plane of the polyp demonstrated dome-shaped PED containing flow signals (Fig. ). Two weeks after PDT, OCT-A showed a trace of the BVN and complete regression of the polyp (Fig. ). Cross sectional OCT-A revealed flattened PED that was devoid of flow signals (Fig. ). Left vision improved from 20/60 to 20/20. En face OCT-A at 2 months indicated that the BVN had largely restored its original shape but the polyp was still absent (Fig. ). In corresponding cross-sectional OCT-A, the PED had mildly restored its height, with no apparent restoration of flow signals (Fig. ). The patient was lost to follow-up after her 2-month visit.
|
pmc-6215636-1
|
A 45-year-old Moroccan man of low socio-economic status, a farmer by profession, with no particular personal or family medical history and without any medications prior to diagnosis, presented to our emergency department with gross hematuria as the main symptom associated with urinary frequency. He had a history of tobacco smoking (24 pack-years) and did not consume alcohol.
His vital signs were: body temperature 37.5 °C, blood pressure 120/70 mmHg, and pulse 88 beats per minute. His physical examination revealed minimal lower abdominal pain without any mass, with a strictly normal neurological examination.
Laboratory data only revealed an acute anemia (hemoglobin, 8.5 g/dl) requiring four units of packed red blood cells transfusion. No other anomaly in the laboratory data was found. A urine culture was negative. An abdominal ultrasound revealed a huge mass (70 × 60 mm) on the posterior wall of the urinary bladder with no hydronephrosis (Fig. ). Cystoscopy revealed a large endoluminal mass arising from the retrotrigonal region.
A transurethral endoscopic resection of his bladder for hemostatic and biopsy purposes was performed under general anesthesia. His postoperative course was uneventful. Microscopic examination of the resected specimen showed small round cells and occasional “tennis-racket” shaped cells with acidophilic cytoplasm, the nuclei were hyperchromatic, the matrices were myxoid and richly vascularized, dense cellularity was present near the surface of the epithelium of the bladder (Figs. and ). Immunohistochemical studies showed that these cells expressed myogenin, desmin, and vimentin (Figs. , , and ). The final diagnosis was embryonal botryoid RMS.
A thoracoabdominal computed tomography (CT) scan revealed no distant metastasis.
Our patient initially received three cycles of neoadjuvant chemotherapy using vincristine, actinomycin D, and cyclophosphamide (VAC), and later underwent a radical cystectomy associated with extended pelvic lymph node dissection with transileal urinary diversion (Bricker type). No lymph node metastasis was found and the margins of the resection were also negative. Our patient was free of disease 24 months after treatment.
|
pmc-6215653-1
|
A 6-year-old girl with a history of multiple IgE-mediated food allergies, atopic dermatitis, and a remote history of asthma presented to the emergency department with urticaria, coughing, and wheezing. Symptoms began minutes after entering a ginseng store that was selling powdered American ginseng products. She did not have any respiratory symptoms or ingest anything prior to entering the store. Parents suspected she inhaled some powdered ginseng. This was her first known exposure to ginseng. On physical examination, she was afebrile with normal blood pressure for age. Respiratory examination confirmed increased work of breathing and decreased air entry with wheezing bilaterally. On dermatologic examination, she had urticaria on her chest. She was treated in the emergency department with salbutamol, dexamethasone and diphenhydramine. Symptoms resolved shortly after treatment, and the patient was referred to our Allergy Clinic.
In the Allergy Clinic, parents provided a history of wheezing with viral infections between ages two and four, with intermittent inhaled corticosteroid and salbutamol use. There had been no exacerbation of respiratory symptoms in over a year. She had confirmed food allergies to peanuts, tree nuts and fish, and had outgrown egg and wheat allergies. She was found to be sensitized to tree pollen.
Skin prick testing (SPT) with American ginseng powder dissolved in water was positive with a 13 × 12 mm wheal. Spirometry was normal (FEV1 107% predicted). The family declined an oral challenge to ginseng, given the severity of her initial reaction. Basophil activation test (BAT) showed a dose-dependent increase in expression of CD63 on basophils in response to American ginseng extract, but not Korean ginseng extract (Fig. ). No changes were observed in a non-atopic control, and minimal changes were observed in an atopic control that was tested with American ginseng extract.
It was concluded that this patient had an anaphylactic reaction to American ginseng. She was advised to strictly avoid all ginseng products and carry an epinephrine autoinjector at all times.
|
pmc-6215653-2
|
A 3-year-old boy with asthma and atopic dermatitis was referred to our Allergy clinic with a history of recurrent ocular pruritus, tearing, and conjunctivitis. There were no associated nasal or respiratory symptoms. His ocular symptoms consistently occurred minutes after entering his grandparents’ herbal product store, on days when American ginseng was being ground. Symptoms typically resolved within 24 h of leaving the store, and did not occur when other herbs were being ground. There were no other identifiable triggers, and no seasonality to his symptoms. He was regularly ingesting soup boiled with small amounts of ginseng root without adverse reaction.
SPT with American ginseng powder dissolved in water was positive with a 13 × 7 mm wheal. SPT to common environmental aeroallergens was positive for dust mite. The family declined BAT due to needle phobia. An oral challenge was performed using American ginseng powder. Parents were asked to bring in 50 g of powdered ginseng [], however, only brought in 3 g as they felt this quantity was too large. The powder was mixed with water, and ingested in increasing quantities. Parents stopped the challenge at 2 g, stating this was the maximum they would ever use in soup. He was monitored for 1 h following the challenge, and did not develop any signs of IgE-mediated allergy. The family was advised that it was likely safe for him to continue ingesting small amounts of ginseng in soup, but to avoid the grandparents’ store as much as possible, particularly on days when ginseng was being ground. He was given a prescription for olopatadine 0.1% eye drops for ocular symptoms as needed.
|
pmc-6215663-1
|
A 42-year-old woman, married and with two children, was referred to our hospital outpatient gastrointestinal clinic for a 4 months’ history of post-prandial heartburn with frequent regurgitations, fatigue and change in bowel movements (4–5 soft stools). At the moment of the first consult, a written informed consent on the publication of personal information was obtained from the patient. She had experienced unintentional weight loss of 3 kg in about one month despite normal or even increased food intake. In her clinical history no previous significant gastrointestinal symptoms were present. The patient’s older sister had been diagnosed of coeliac disease at age 20. Her personal and family history was otherwise unremarkable. She first underwent biochemical investigations including immunoglobulin A (IgA) anti-tissue transglutaminase antibodies (anti-tTG) and stool parasitological and cultural analysis. Serological testing showed normal IgA levels and negativity for anti-tTG levels and antiendomysial antibodies (Ema). The patient’s ferritin was 33 mg/l (n.v. 30–400 mg/l), serum folate was lower than 5 nmol/l (n.v. > 7 nmol/l), haemoglobin level was normal as well as white blood cells and platelet count. The result of stool analysis was negative for parasites and ova. Since symptoms persisted, she was then referred for an upper GI endoscopy. Esophageal and gastric mucosa did not show any macroscopic alterations. Duodenal folds were normally represented as well as mucosa. Multiple gastric antrum and corpus-fundus mucosa biopsies were taken along with biopsies from the bulb and second part of duodenum (at least four).
Gastric biopsies showed a Helicobacter Pylori (HP) pan-gastritis while duodenal mucosa showed villous atrophy (Fig. ) associated with an increase in intraepithelial T lymphocyte (IEL) numbers up to more than 40 IEL/100 epithelial cells (EC), recognized by CD3 immunostaining (Fig. ). The histologic features were consistent with a diagnosis of coeliac disease Marsh type 3b [, ]. She was informed of the result and advised to begin gluten-free diet (GFD). After 4 weeks of GFD the patient didn’t experience any improvement of symptoms, and bowel movements with abdominal pain increased to around 6–7 daily; she was advised to prompting repeat further stool examinations. Genetic evaluation for alleles HLA specific for coeliac disease was also requested. At the same time, in order to re-evaluate initial diagnosis, duodenal biopsies were reviewed and a careful study of the duodenal mucosa showed the presence of scattered crescent-shaped randomly oriented trophozoites of Giardia on the luminal surface of the duodenal wall (Fig. ). The organisms were minute, easily overlooked or mistaken for detached intestinal epithelial cells or erythrocytes. Subsequently, results of the second stool examinations revealed the presence of G. lamblia with findings of trophozoites and cysts. The patient was consequently treated with metronidazole, 500 mg twice daily for 6 days, showing a prompt response with a reduced frequency of diarrhea in the following days. The genetic results showed DQA1*03 and DQB1*03:02 alleles codifying for HLA-DQ8, otherwise compatible with CD diagnosis. In the following weeks, the patient intermittently maintained a GFD, but observed no difference in her well-being. Stool examination was repeated 2 months after the end of antibiotic therapy with negative results. After about 2 months of GFD she was invited to discontinue it. She subsequently performed eradication therapy for HP with triple therapy of metronidazole, tetracycline and bismuth (Pylera®).
Around 6 months later, the patient did not complain gastrointestinal symptoms. In January 2017 she repeated serological tests and a follow-up esophagogastroduodenoscopy. No alterations were found in haemoglobin, folic acid, cholesterol, triglycerides and antibodies (anti-tTG and Ema) levels. Multiple duodenal biopsies were performed, showing normal histologic appearance of the mucosa with normal finger-like villi, no evidence of increased IEL numbers and complete absence of crescent-shaped Giardia trophozoites (Fig. ). The gastric biopsies showed resolution of active HP gastritis. The patient showed no clinical signs of CD and the conclusion was that the clinical scenario could be fully explained by giardiasis.
|
pmc-6215674-1
|
A 67-year-old female patient with chagasic megaesophagus and megacolon, without myocardiopathy was submitted to a Heller myotomy for achalasia in 2000 and endoscopic dilatation in 2015. Patient was admitted to the Nutrology Ward in May 2016 with protein-calorie malnutrition associated with achalasia and using enteral nutrition. In July 2016, the patient underwent a Serra-Doria surgery. In the third postoperative day she presented an important abdominal distension. She was submitted to a new surgical intervention, and then a terminal ileum perforation was detected, leading the surgeon to perform an enterectomy with side-to-side anastomosis.
The next day after the surgery (4th postoperative day) the patient presented a decreased level of consciousness (Glasgow coma scale = 8), hypotension and hypoxemia. She was submitted to orotracheal intubation and transferred to intensive care unit (ICU). On ICU admission the patient was sedated and hydrated. The body temperature was 38.5 °C, arterial blood pressure = 104 × 55 mmHg, the heart rate was 101 beats per minute and respiratory rate = 14 breaths per minute. The cardiac auscultation was normal, and respiratory auscultation was compromised with adventitious sounds such as rales and crackles especially in the left pulmonary base. A distended abdomen was observed.
Laboratory findings revealed hemoglobin at 8.9 g/dL and hematocrit of 28%. The white blood cells were increased (20,300/mm3), with the left shift until myelocytes (7%) with presence of anisocytosis and neutrophils with abundant toxic granulation. The platelets count was 203,000/mm3, urea = 64 mg/dL, creatinine = 0.96 mg/dL, sodium = 160 mmol/L and potassium = 4.2 mmol/L. The C reactive protein was 19.39 mg/dL. At admission, the Acute Physiology and Chronic Health Evaluation II (APACHE II) for the patient was 33 (death risk of 75%). Blood samples were collected and empiric antibiotic treatment was initiated with cefepime and metronidazole focused for intra-abdominal infection. The patient showed a gradual worsening level of consciousness and septic shock with refractory hemodynamic instability unresponsive to fluid or drugs resuscitation. The patient died three days after ICU admission.
|
pmc-6215960-1
|
A 29-year-old man with Crohn’s disease and ileitis experienced upper abdominal pain. A small bowel series identified a longitudinal ulcer and subsequent abdominal computed tomography (CT) showed an ileal stricture and an urachal tumor mass that extended into the umbilicus (a, b). He was referred to our hospital for surgery because of a fistula from the stenotic ileum to the urachal tumor. Intraoperatively, it was found that the urachal remnant connected to the urinary bladder (a, b), and the Crohn’s disease-related intestinal lesion had formed a fistula to the urachus. The patieint underwent a partial ileal resection, urachal resection, and partial cystectomy (a–c). There were non-caseating epithelioid granulomas in the muscularis propria of the small intestine with inflammation of all layers, and these findings are characteristic of Crohn’s disease. There was extensive neutrophil infiltration and inflammation beneath the epithelium of the urachus because of exposure to foreign substances (a, b). The patient had an uneventful recovery and was healthy with no recurrence at 8 years of follow-up.
|
pmc-6215960-2
|
A 43-year-old man was diagnosed with Crohn’s disease and ileitis when he was at 33 years of age. At 41 years of age, he experienced fecal discharge from his umbilicus, pneumaturia, and fecaluria with frequent urination. A small bowel series and barium enema study showed an ileal stricture, and an ileorectal fistula. The patient was referred to our institute for surgery at 43 years of age. A small bowel series showed an ileal lesion with a fistula to the rectum and cecum, but not to the umbilicus. Abdominal CT indicated an ileal lesion involving the urachus, with abscess formation. Because the patient had severe pain, cystoscopy could not be performed. Intraoperatively, it was found that the patient had a periumbilical inflammatory lesion extending to the bladder through the urachal remnant and the longitudinal ulcer of the ileal lesion (a, b). The patient underwent urachal curettage, partial ileal resection, and partial cystectomy. The patient showed severe inflammation of the bladder, a decreased bladder capacity of 150 ml after the cystectomy, and frequent urination. He was administered infliximab after the surgery and was free of recurrence at 6 years of follow-up.
|
pmc-6215962-1
|
A 61-year-old woman was admitted to a hospital due to obstructive jaundice. Extensive EC was found by diagnostic imaging, and she was subsequently scheduled for brachytherapy since the tumor was found to be unresectable. Seven years prior at the same hospital, she underwent multi-organ en bloc resection for advanced gall bladder (GB) carcinoma involving the distal stomach and right side transverse colon (). Extended cholecystectomy, distal gastrectomy, and right hemi-colectomy with loco-regional lymphadenectomy were also performed. Despite not receiving adjuvant chemotherapy, she had remained without tumor relapse. She was referred to our institute for a second opinion.
Obstructive jaundice was resolved by percutaneous transhepatic biliary drainage (PTBD) via the left lateral sector of the liver at the previous hospital. Enhanced abdominal computed tomography (CT) and PTBD cholangiography showed wide stenosis of the intra- and extra-hepatic bile duct (a and b). Cholangioscopy and intraductal ultrasonography (IDUS) showed a papillary tumor with wall thickness and stenosis with no involvement of the adjacent right hepatic artery (RHA) (a and b). Endoscopic biopsy performed at the stenotic lesion and non-stenotic bile duct at the confluence of the anterior and posterior sectional branches was negative. Although invasive adenocarcinoma was diagnosed, cancer infiltration was not observed at the non-stenotic bile duct epithelium. Since neither distant nor node metastasis was observed and liver function reserve was sufficient for hemi-hepatectomy, we planned for HPD despite the possibility of tumor recurrence from GB cancer.
There was no peritoneal dissemination, liver metastasis, or distant node metastasis by laparotomy. Although there was a postoperative adhesion in the upper abdomen, the front of a superior mesenteric vein (SMV) was found and pancreaticoduodenectomy (PD) was performed (a and b). During exfoliation of the hilar bile duct, adhesion to the surrounding main vessels became severe, particularly in the neighboring RHA (a); however, this was considered non-tumor invasion by macroscopic findings. The anterior sectorial arterial branch was partially injured and was repaired with 8-0 polypropylene sutures (b). After anastomosis, arterial flow increased compared with dissection. Left hepatectomy with transection of the right hepatic duct was also performed at the lesion. Thus, R0 resection was performed without tumor exposure at the dissected plane (). Pancreatojejunostomy, hepaticojejunostomy, and jejuno-jejunostomy were also performed. The total operating time was 685 min (including 45 min for arterial repair), and blood loss was 1200 mL, which did not require blood transfusion.
Microscopic findings showed papillary adenocarcinoma with stromal and pancreatic invasions that did not extend to the dissected surface. Lymph node metastasis was found on the pancreatic surface. R0 resection was also histologically confirmed. The postoperative course was uneventful without severe damage to the liver, and the patient was discharged at day 14. Four months after HPD, she remains without tumor recurrence or complications.
|
pmc-6215971-1
|
A 43-year-old man with pituitary gigantism from the age of 16 secondary to a GH-functional PA. He underwent microsurgical trans sphenoidal surgery for resection of a PA in 1990 and adjuvant radiotherapy given in 2011 (radiotherapy scheme, original radiology and pathology unavailable). In 2016, almost 27 years after the initial surgery, he presented progressive decrease in vision on the left eye for six months. He also complained of excessive perspiration and sweating and had a past history of carpal tunnel syndrome. He was found to have gigantism features as extreme physical size (2.07 mts height) and pigmentation around eyes, neck and flexures.
In ophtalmologic examination, a left ptosis with both exophthalmos and upward/inward limitation on left eye movements was achieved, and in the Goldmann’s test perimeter an ipsilateral amaurosis and right hemianopia (Octopus 900 Haag Streit Inc., Bern, Switzerland) ().
Random serum GH level was 0.071 ng/ml (0.003–0.97 ng/mL) with a normal GH concentration during the Oral Glucose Tolerance Test (OGTT) with a GH-nadir of 0.13 ng/ml (the gold standard for diagnosis is that GH excess fails to suppress serum GH level to less than 1 ng/ml after OGTT). The Insulin-like Growth Factor-1 (IGF-1) 84.3 ng/ml (64–210 ng/mL); the rest of hormones are listed and shown in .
On brain Magnetic Resonance-Imaging (MRI) (Siemens 3.0 T magnetic resonance scanner and a 32-channel head coil) revealed a large policystic selar lesion with extensive osteophytic reaction and invasion of the ipsilateral orbital apex associated with fibrous dysplasia (see A and B) with a T2-weighted hypointense and enhancing selar mass lesion with Calcium intensity on the Gradient echo sequences (GRE) (C). Also the skull CT-scan (Siemens SOMATOM Sensation 64-slices) revealed an osteolytic lesion on the right orbital apex with thickness of the diploe (E) and pituitary gland with a calcified rim around the tumor (D, F and G). There was no personal or family history of prior endocrine disease.
A second surgical resection was performed in 2016 by a left orbitozygomatic approach for tumor removal with no acute complications (A–C). Three days after the resection, the patient presented sudden unresponsiveness with an asymmetrically larger left pupil and rostrocaudal deterioration due to an ischemic stroke on left Anterior Cerebral Artery (ACA) and Middle Cerebral Artery (MCA), performing an urgent ipsilateral decompressive craniectomy. Unfortunately after surgery the patient continued unresponsive, leading to his death.
After the informant consent was obtained, brain autopsy was performed. Neuropathologic examination of the pituitary lesion removed in 2016 (first neuropathology report was unavailable) was characterized macroscopically by a 40 × 40 mm mass with grayish outer surface and inner yellowish-irregular zones thickened by “sand-like” calcifications (A and B). Histologically micro-hemorrhage with ossification areas within a thick pseudocapsule and multiple calcifications with a diffuse desmoplastic stromal component even within blood vessels were observed (C and D). Immunohistochemistry stains were positive for Growth Hormone and osteoconine (F–G). Brain autopsy showed perivascular granular-dystrophic calcifications in thalamus, hypothalamus and basal ganglia (D, H–J).
|
pmc-6215972-1
|
A 60-year-old Caucasian male patient was brought to the resuscitation bay of our Emergency Department after a syncopal episode and was noted to be in shock. His initial observations include Heart Rate 126/min, Blood Pressure 102/79 mmHg, Respiratory rate 32/min, temperature 36.8C and oxygen saturation of 98% on 2 L of nasal prongs. The patient had a Glasgow Coma Scale of 14 and was clinically dehydrated. Abdominal examination revealed a distended abdomen that was tender in the left upper quadrant with no evidence of peritonism. Laboratory tests showed polycythaemia with Haemoglobin of 189 g/L and white cell count of 20.3 × 109/L with preserved renal and hepatic functions. Arterial blood gas analysis was consistent with normal anion gap metabolic acidosis with a pH of 7.28 and lactate of 4.5. The patient responded to initial fluid resuscitation.
Further history was obtained: he had a 2-day history of progressively worsening upper abdominal pain associated with multiple episodes of dark coloured emesis and loose bowel motions. The pain was exacerbated by oral intake. He had a background of ulcerative colitis diagnosed at the age of 40 which required one course of oral steroid with no further follow up or treatment.
As the patient remained in a stable state, a CT abdomen was performed which demonstrated a long segment of superior mesenteric vein (SMV) thrombus extending into the portal vein. This resulted in venous engorgement with associated thickening of jejunal wall and extensive mesenteric stranding suggestive of intestinal ischemia. There is also a moderate amount of free fluid with no evidence of intramural gas or perforation (, ).
Acute mesenteric venous thrombosis was diagnosed and management options were explored. Although the CT appearances were sinister and suggestive of intestinal ischemia, the patient had no signs of peritonism. Thus, decision was made for conservative management. Heparin infusion was commenced, nasogastric tube was inserted and the patient was placed on bowel rest. Central venous line, arterial line and a urinary catheter were inserted and the patient was transferred to the intensive care unit (ICU).
Serial abdominal examinations were performed. Over the subsequent 24 h, the patient remained clinically stable and showed improvement in his metabolic acidosis. His abdominal examination was consistent with ileus with no peritoneal signs. Total parenteral nutrition was commenced in anticipation to delays in re-establishing enteral nutritional intake.
The patient was stepped down from ICU on day 6, his abdominal tenderness improved and showed signs of resolving ileus. Episodes of melaena were observed with no haemodynamic changes and with stable haemoglobin levels. Oral intake was slowly introduced and gradually upgraded. He was subsequently discharged on day 17 post admission after transitioning onto oral anticoagulation with warfarin.
The patient was followed up in the outpatient clinic one month after discharge. He had returned to his baseline function with no gastroenterological symptoms. Extensive investigations were performed to assess for underlying causes of the patient’s SMV thrombosis. These included thrombophilia and myelodysplasia screens, malignancy screens and an echocardiogram. Unfortunately, no specific causes were detected. His initial polycythaemia also resolved after adequate fluid replacement and was attributed to severe dehydration on presentation. A follow up colonoscopy was performed which showed no evidence of mucosal inflammation or features to suggest inflammatory bowel disease.
|
pmc-6216046-1
|
This case details a 54 year old woman who was referred to our clinic by her general practitioner for investigation of bilateral breast pain that she had for 6 months.
Her past medical history included hypertension for which she took a single anti-hypertensive. She had no familial history of breast or gynaecological malignancy. On clinical examination pathological nodes were palpated bilaterally in the axillae. There was left sided nipple inversion with a palpable mass in the upper outer quadrant of approximately 3 cm diameter.
On examination of the right breast there was skin tethering of the nipple and 3 masses were palpated, the largest being in the upper inner quadrant at 5 cm diameter.
Ultrasound and mammography of bilateral breasts demonstrated advanced bilateral breast cancer with axillary node metastases.
The right breast had a large lesion consistent with primary breast cancer at 12 o’clock measuring 44.3 mm in diameter. An additional 7 smaller lesions were distributed through the right breast consistent with satellite lesions. The largest pathological node in the right axilla measured 42 × 30 mm.
In the left breast at the 2 o’clock position there was a lesion consistent with breast carcinoma measuring 31.2 mm. The largest node in the left axilla measured 13.1 × 10.2 mm.
There was skin thickening of both breasts consistent with oedema, the right side was most pronounced. Core biopsies from lesion on right breast at 12 o’clock and the left breast at 2 o’clock demonstrated invasive carcinoma. , : (H&E x10) Left/right core biopsy show invasive carcinoma, no special type
The hormone receptor status was identified with right breast lesion being ER negative whilst the left breast lesion was ER positive.
, , : (x10) Positive staining for ER and PR. HER2 Immunohistochemistry score 3 + .
, , : (x10) Negative staining for ER and PR. HER2 Immunohistochemistry score 3 + .
The patient was presented at an oncology multidisciplinary team meeting.
Subsequent staging scans were arranged which included a CT of head, neck, abdomen and pelvis, a nuclear medicine bone scan and a PET scan.
The CT scan showed no evidence of metastatic disease. The only finding was the bilateral breast malignancies with bilateral pathological axillary lymph nodes, with the right being most pronounced.
There was mild FDG avid ill defined ground glass pulmonary opacity in the left lower lobe of her lung, this was though to represent an inflammatory or infective process given its absence on the CT scan. A follow up CT scan was recommended to ensure resolution.
There was no evidence of bony metastases on the nuclear medicine bone scan.
The patient was referred to medical oncology to commence neo adjuvant chemotherapy and hormone therapy. Pending the response to the therapy the patient will be considered for bilateral mastectomy and axillary clearance.
|
pmc-6216048-1
|
A 50 year old female patient previously healthy has undergone an abdominal ultrasound demanded by her primary care physician when her routine checkup blood test showed a slightly elevated level of liver enzymes with no other lab abnormalities. This ultrasound showed right adrenal lesion of 9 cm of diameter. An Abdominal MRI was then done and revealed a soft tissue necrotic encapsulated mass of 10 × 9 cm of right adrenal gland origin. She was completely asymptomatic and the physical exam was strictly normal. Endocrinological evaluation was done with normal hypothalamic-pituitary-adrenal axis function and no hyper secretion of catecholamines. The patient was considered to have a non-secreting right adrenal mass for which an adrenal scan was done and showed a well encapsulated 10 × 9 × 7 cm heterogeneous right adrenal mass with areas of necrosis and calcifications without local invasion (). The decision of right laparoscopic trans peritoneal adrenalectomy was taken with the patient. The surgery was done under general anesthesia after central and arterial lines insertion, the patient was on left decubitus position. 5 trocars were inserted as follows: The first 12-mm port was inserted at the lateral border of the rectus abdominis muscle just above the level of the umbilicus to accommodate the camera. Two subcostal 11 mm ports were also placed; one in the midclavicular line and the other in the lateral border of the rectus abdominis muscle. The forth 5-mm subcostal trocar was inserted in the anterior axillary line to retract the liver and the fifth 5 mm one was inserted in the epigastrium and used specially for aspiration and irrigation. After liver retraction, the peritoneum along the lateral aspect of the IVC was incised to expose the IVC just below its intrahepatic course. The duodenum which was diverted by the mass was mobilized. Dissection was next carried inferiorly by incising the peritoneum along the lateral edge of the vena cava to the superior edge of the renal vein. Dissection of the mass was subsequently carried out with special care at the medial aspect where we found that the wall of the IVC and the renal vein were very adherent to the mass which had a lot of small vessels that were oozing during all the time of the surgery (). In addition, the mass was extended posterior to the vena cava and we could not do a medial retraction of the IVC since the tumor was adherent to it. In front of these facts, we decided to convert to open surgery by a sub-costal incision (between two trocars) that allowed us to remove the mass safely (). A drain was put in the retro peritoneum at the end of the surgery, the operative time was 200 min, the blood loss was 850 cc and no transfusion was done. The drain was removed at the second post-operative day and the patient was discharged uneventfully on the sixth day after surgery.
Histologically, the tumor consisted of spindle cells with alternating areas of compact hypercellularity with irregular streams and without atypia or mitosis (). This tumor was completely compressing and reducing the adrenal gland that was laminated but intact without histological abnormalities (). Immunohistochemical analysis demonstrated negative CKAE1-AE3, synaptophysine and chromogranine. In contrast to these results, S-100 and CD68 (PGM1) staining were diffusely positive across the tumor (). Thus, the evidences corresponded to a benign schwannoma ().
|
pmc-6216069-1
|
A 36 years old woman with a suspicious diagnosis of cervical carcinoma in a tertiary hospital several days before visited our hospital. The patient had a regular menstrual cycle. Her childbearing history was G4P1A3, and she gave the birth by cesarean section in 2005. She was diagnosed with hypertension for five years and took medicine regularly, with blood pressure being controlled in normal condition.
She complained about vaginal bleeding after copulation for Six months, accompanying with constipation, diameter-thinning stool and an increase of vaginal discharge. She also complained that she had a mild pain sometimes in the low back and pelvis, without obvious association with menstrual cycle. So she came to a tertiary hospital specialized in gynaecology and did some examination according to the doctor’s advice. Pelvic ultrasound showed a 6.0 × 4.3 cm hysteromyoma and a 4.7 × 3.9 cm posterior cervical mass, with the ovaries found no abnormality. Enhanced pelvic computed tomography (CT) showed a 3.5 × 3.9 cm vaginal occupying lesion invading the rectum () and a 5.8 × 4.7 × 5.3 cm hysteromyoma. The doctor gave a suspicious diagnosis of cervical carcinoma.
Then the patient came to our hospital for further diagnosis and treatment. Additional examination was done. Examination of tumor markers showed that the serum level of CA125 was 87.9U/ml, 2.5 times of the normal upper limit, with squamous cell carcinoma antigen (SCC), CEA and CA199 within the range of normal value. Physical exam found that cervix uterus was thickened, with irregular shape and locally Protruding nodules. Vaginal and cervical biopsy only showed chronic inflammation. Colonoscopy examination found a mass at the rectum 4 cm from the anus () and biopsy indicated spindle-cell-like mesenchyma-derived tumor, with the immunohistochemical result not supporting the diagnosis of interstitialoma. Supplementary PET-CT examination showed an increase of 18FDG uptake of the rectal mass (), with the standardized uptake value(SUV) as 4.7. PET-CT reported that it was a submucous tumor, with interstitialoma being suspicious. Then another biopsy examination of the rectum mass was done and the diagnosis result was rectal endometriosis supported by the immunohistochemical result.
Multidisciplinary team, including oncologists, surgical specialists, gynecologists and pathologists, was established to discuss the complex condition. The pathologist supported the diagnosis of rectal endometriosis according to the immunohistochemical result. All doctors shared a common thought that there is a possibility that both rectal carcinoma and rectal endometriosis were existent at the same time, taking the small insufficient biopsy specimen into account. If a radical operation was performed, treating it as a malignant tumor, considering that the rectal mass was only 4 cm away from the anal verge, the patient could not save the anal function, and colostomy would be existent permanently, reducing the quality of life. After a heated discussion, a consensus that partial resection should be operated to confirm the histopathological diagnosis and then to determine the further treatment was achieved. Eventually, the final diagnosis was confirmed as rectal endometriosis and gynecological management was suggested.
Thereafter, the patient was discharged, seeking medical treatment in a tertiary hospital specializing in Gynecology. Gonadotrophin-releasing hormone antagonists was injected subcutaneously once per month for six months, with all the symptoms gradually disappeared. Then, a ring that slowly releases levonorgestrel was put into the uterine cavity, and it should be used until menopause according to the instruction.
|
pmc-6216074-1
|
A-23-year-old female brought to our hospital with ACL rupture caused by car accident. The patient had a history of knee dislocation with an open wound and rupture of popliteal artery. The patient underwent open reduction surgery of her right knee joint by orthopaedic surgeon and popliteal artery repair by vascular surgeon. The reduction of the knee joint went good, but the popliteal artery repair was failed because there was leakage, the distal popliteal artery was damage and the angiography () showed the blockage below the popliteal artery. Data showed that limb revascularizations in both military and civilian populations were mostly done by autogenous bypass. Popliteal artery injury with early identification of limbs at risk, shown to be beneficial to do: early four compartment fasciotomy, temporary intra-luminal shunting, definitive repair of concomitant venous injuries [], because there was good distal vascularity from the collateral artery of the patient and there was no sign of ischemia with the popliteal artery blockage then the patient underwent popliteal artery ligation 1 week later.
The patient received non-operative treatment for her ACL rupture. After 2 years, she did not get a good improvement to her knee and the patient still felt a giving way sensation and unstability of her knee. Then the patient transferred to our hospital for ACL reconstruction, but the surgery required a special consideration in the technique of ACL reconstruction because of the history of popliteal artery ligation ().
|
pmc-6216080-1
|
A 58-year-old male presented (day −120) with symptoms of congestive heart failure that developed over a 2-week period. Transthoracic echocardiography was notable for a 30% ejection fraction, moderate to severe aortic regurgitation and severe mitral regurgitation. Because his symptoms were refractory to medical therapy, he underwent transesophageal echocardiography for anticipated valvular surgery, which revealed 4–6 mm mobile echodensities on the mitral valve. The patient denied any constitutional symptoms, fevers or chills, weight loss, or skin lesions. He was born in Mexico and had exposure to livestock as a child. He had lived in the USA for the past 28 years and worked as a gardener in the greater Los Angeles area with no travel outside the city for more than 20 years. He denied any exposure to pets, history of homelessness, or sick contacts. His blood cultures were negative, as was serologic evaluation for Brucella, Bartonella, and Coxiella. The patient was prescribed a 6-week course of ceftriaxone and doxycycline. During this time, he underwent aortic and mitral valve surgery with bioprosthetic placement. Bacterial sequencing using broad range PCR primers (completed by the University of Washington department of laboratory medicine) of his native valve was negative for bacterial pathogens. Pathology of the valve reported papillary fibroelastoma.
The patient returned one month following valve surgery (day −70) with fevers, chills and reported night sweats for 3 weeks. He had completed his prior antibiotics. Empiric therapy for presumed bacterial prosthetic valve endocarditis was initiated. Transesophageal echocardiography demonstrated normal hemodynamics of his prostheses, a small 0.4 cm mobile echodensity was noted on the mitral valve. His EKG showed a new third-degree AV block. However, 3 blood culture sets prior to antibiotic administration were negative, and the patient was asymptomatic without documented fevers in the hospital. Since he had only 1 major criterion (positive echocardiogram for possible “vegetations”) plus one minor criterion (underlying valvular disease) by the Duke Modified Criteria , it was felt that he likely had noninfectious vegetative endocarditis perhaps due to post-valvular surgery thrombus. Empiric antibiotic therapy was stopped and the patient remained clinically stable and afebrile over subsequent days of monitoring. A pacemaker was placed.
One month later (day 0), the patient presented with 3 weeks of fevers, chills, night sweats, and dyspnea on exertion. He reported no cough, new skin lesions, and the sternal wound was asymptomatic. He was afebrile and his examination was notable for a generalized frail appearance, a new diastolic murmur appreciated best at the heart apex radiating to the axilla and bibasilar rales. He had leukocytosis of 15,500/mm3 and elevation of his creatinine to 0.88 mg/dL (baseline 0.55 mg/dL). Blood cultures were drawn, and patient was started on empiric vancomycin, rifampin, and gentamicin for presumed prosthetic valve bacterial endocarditis.
A transesophageal echocardiogram demonstrated new bulky vegetations of up to 3.3 cm on the mitral valve encasing the leaflets, with a 0.8 cm highly mobile component, with sparing of the aortic valve and pacemaker leads. The infection extended to the aorto-mitral curtain and aortic root (). Wedge-shaped densities, probably infarcts, of the spleen and kidneys were visible by abdominal CT scan; in addition, a 2.9 cm occipito-parietal intraparenchymal hemorrhage was seen on head CT scan. By day 3 of hospitalization, the admission blood cultures remained sterile, although the patient started spiking fevers. After a multi-disciplinary conference, he was started empirically on liposomal amphotericin B 5 mg/kg daily. The following day, the serum BDG was reported as > 500 pg/ml (negative cutoff < 59 pg/ml), and the serum GM EIA index value was elevated at 2.24 (negative cutoff index < 0.5). As a result, the patient was administered voriconazole 4 mg/kg IV twice daily in addition to liposomal amphotericin B 10 mg/kg/d. He was also continued on vancomycin and cefepime. The rifampin was discontinued due to the concern that it would lower the serum voriconazole levels. Valvular surgery was not performed due to worsening of the intraparenchymal hemorrhage. On day 11, fungal balls were observed in the patient's admission blood culture bottles (). The mold that subsequently grew on solid media showed phenotype consistent with Aspergillus and identification was later confirmed as Aspergillus fumigatus (). With development of transaminitis and acute kidney injury, the liposomal amphotericin B, vancomycin and cefepime were discontinued, and micafungin 100 mg IV daily was added to the voriconazole. Unfortunately, although the patient's organ failure improved, surgical intervention was determined to be too high-risk. On day 17, the patient died from a presumed massive stroke. Serum obtained on day 16 showed a BDG > 500 pg/ml and GM of 2.63. An autopsy was not obtained.
|
pmc-6216084-1
|
A 45 year old lady came with right lower abdominal pain radiating to right lower limb over 4 months. There were no other significant abdominal complaints. Patient did not have any contact with tuberculosis. Clinical examination was normal. Abdominopelvic ultrasonography showed 4 × 3 cm solid mass in right iliac fossa. Contrast enhanced CT of the abdomen () showed homogeneous, vascular and well defined retroperitoneal solid mass overlying right iliacus muscle; suggestive of a benign neurogenic tumour. At laparotomy, a vascular tumour overlying the right femoral nerve was excised.
Histopathology () showed atrophic germinal centre with vessel traversing through it: “lollypop appearance”. The expanded mantle zone shows concentric rings of small lymphocytes: “onion skin appearance”- suggestive of hyaline vascular variant of Castleman disease.
All biochemical investigations and PET CT were normal. Patient is on follow up for 6 months and is doing well.
|
pmc-6216084-2
|
A 33 year old male came with rapidly growing left inguinal lymph node mass, low-grade fever, loss of weight and severe debility over 2 months. There was no contact with tuberculosis. Examination showed: 6 × 8 cm size painless, firm, left inguinal lymph node mass; small bilateral axillary and cervical lymphadenopathy. Liver and spleen were not palpable. There was pitting edema of left leg.
Fine Needle Aspiration Cytology- was inconclusive; negative for tuberculous bacilli.
Histopathology of excision biopsy specimen () showed florid reactive follicular pattern with hyperplastic germinal centre, focal inter-follicular predominance of plasma cells and marked vascular proliferation suggestive of plasmacytic variant of Castleman disease.
Immunohistochemistry- CD-20, CD-10, BcL-2 confirmed the reactive follicular architecture. CD-3 & CD-13 B highlighted the predominance of T-lymphoid and plasma cells respectively in the interfollicular zones. CD-23 highlighted the follicular dendritic network within germinal centres. Opinion: Plasma cell variant of Castleman disease with no features of malignancy identified.
18 FDG PET-CT (Non-contrast): Metabolically active left internal iliac, external iliac (), left inguinal, bilateral cervical, left axillary lymph nodes () were noted.
|
pmc-6216091-1
|
A 65-year-old man referred to the emergency department for evaluation of the lower extremity swelling associated with pain for four days. Physical examination demonstrated an overweight man (BMI 29.6 kg/m2) with extensive pitting edema of the left lower limb from the groin to the knee joint with calf tenderness. Color Doppler ultrasound revealed an extensive DVT involving common iliac, external iliac and common femoral vein as well as superficial femoral down to popliteal vein. He was treated by bed rest, elevation with bandaging of left leg, 6000 IU of low molecular weight heparin subcutaneously twice a day and further evaluation was performed to find the underlying etiology. Investigations including hematological, immunological, biochemical, lipid profile, protein S and protein C were normal. Abdominal and pelvic ultrasound (US) showed incidental finding of severe left hydroureteronephrosis with almost lost of cortical thickness, for that abdominal and pelvic computed tomography (CT) scan revealed marked left-sided hydroureteronephrosis and an impacting stone measuring (18 × 10 × 10 mm) at the level of L5/S1 () with signs of DVT affecting left iliac and femoral vein below the above mentioned region (). Next day percutaneous nephrostomy was performed to decompress the hydronephrotic kidney. He was kept as an inpatient for one week under observation then after discharged home on oral anticoagulation in the form of rivaroxaban 20 mg daily. Six weeks later, color Doppler US showed complete recanalization of the superficial femoral, popliteal as well as the proximal segment of deep veins of the leg but common iliac, external iliac and common femoral veins and proximal superficial femoral vein were still partially thrombosed. Under spinal anesthesia, left ureterorenoscopy showed an impacted stone at the level of iliac vessel pulsation causing edema and external compression of the iliac vessels. Through pneumatic lithotripsy, the stone was fragmented and JJ stent inserted (). Next day the patient discharged home and continued on taking his antithrombotic treatment (rivaroxaban 20 mg). The JJ stent was removed 3 weeks later. Three months after that, Doppler US showed complete recanalization of iliac vessels.
|
pmc-6216529-1
|
A 58-year-old male patient with no other known disease presented to our clinic with blurred vision for the last 2 months. His best corrected visual acuity was 20/100 in both eyes. Pupils were isochoric and light reflexes were present bilaterally. There was no afferent pupillary defect. Slit-lamp anterior segment examination was normal and intraocular pressure values were within normal limits. Fundus examination revealed RPE changes in the macula of both eyes.
Irregular hyperfluorescent areas were observed in both eyes in the early and late phases of FFA (Heidelberg retinal angiograph 2) ().
On ICGA, both eyes were found to have dilated choroidal vessels in the early phase and appearance consistent with plaque CNV in the late phase ().
Fundus autofluorescence revealed hyperautofluorescent spots were seen in the central fovea and superonasal to the fovea ().
Bilateral subretinal fluid, shallow irregular PED, and pachy-veins were observed on spectral domain OCT (Heidelberg). Subfoveal choroid thickness was 307 µm in the right eye and 254 µm in the left. Pachy-vein thickness was measured as 285 µm in the right eye and 206 µm in the left ().
OCT-A (RTVue XR “Avanti”, Optovue, Fremont, California, United States of America) imaging revealed tangled hyperreflective neovascular network compatible with type 1 CNV in the choroid slab of both eyes. The selected CNV area was 4.671 mm2 in the right eye and 3.533 mm2 in the left. The flow area through the selected CNV area was 2.847 mm2 in the right eye and 2.211 mm2 in the left. The largest diameter of the selected CNV area was 1.26 mm in the right eye and 1.28 mm in the left ().
|
pmc-6216534-1
|
A 24-year-old female patient presented with complaints of blurred vision for 2 weeks. According to the patient’s history, she had attempted suicide 2 weeks earlier by taking 15 tablets of pregabalin (Lyrica, 300 mg; Pfizer, Tadwort; United Kingdom) and was brought to the emergency department of another center with loss of consciousness and seizures. According to the patient’s discharge report, her blood pressure was 100/60 mmHg, heart rate was 165/minute, respiration rate was 34/minute, and body temperature was 36.8 °C in the initial examination done in emergency services. Hemogram and biochemical values were within normal limits. Arterial blood gas analysis done during follow-up in intensive care showed pH: 6.79, PaO2: 45 mmHg, PaCO2: 55 mmHg, HCO3: 7.9 mmol/L, and BE: -33.6 mmol/L. Blood drug level was not analyzed. The patient exhibited deep metabolic acidosis and convulsions and was treated with intravenous hydration, 20 ampules of NaHCO3 and 0.05 mg/kg midazolam (Dormicum, Roche). After treatment, arterial blood gas analysis showed pH: 7.41, PaO2: 145 mmHg, PaCO2: 31.8 mmHg, HCO3: 18.8 mmol/L, and BE: -3.3 mmol/L. On day 3 of follow-up, the patient’s general condition was improved and she was conscious and alert. She had developed blurred vision during this time, and was referred to the ophthalmology department upon discharge. Ophthalmologic examination revealed bilateral serous exudative macular detachment, upon which the patient was referred to our clinic for further examination and treatment.
On examination in our clinic, her best corrected visual acuity (BCVA) was 20/40 in the right eye and 20/50 in the left eyes. Anterior segment examination was normal. Intraocular pressure was within normal limits. Foveal reflex was absent bilaterally on fundoscopic examination (). Fundus fluorescein angiography revealed foci of hypofluorescence in the posterior pole starting in the early phases and continuing in the late phases (). Optical coherence tomography (OCT) images obtained in the other center and in our clinic showed subretinal fluid in both eyes (). Based on the patient’s history and examination findings, the serous macular detachment was believed to be a result of pregabalin intoxication. Treatment was started with topical nepafenac 0.1% (Nevanac Alcon, Forth Worth, Texas, United States of America) 3 times a day. The subretinal fluid was totally resolved after 1 month of treatment (). Topical treatment was discontinued. On examination 3 months after her initial presentation, BCVA was 20/20 in both eyes and no subretinal fluid was evident on OCT.
|
pmc-6216535-1
|
A 28-year-old male patient presented with diplopia that was more pronounced in downgaze and had developed after a motorcycle accident a year earlier. The patient exhibited a chin-down head position and had 20/20 visual acuity and normal anterior and posterior segment examination findings in both eyes. He had minimal V pattern esotropia with -1 limited depression in adduction on the right and -2 limited depression in adduction on the left, and +1 IO hyperfunction bilaterally (). Double Maddox rod test revealed 20 degrees of extorsion () and fundus photograph revealed +3 extorsion (). Bilaterally reduced SO muscle function was observed on Hess screen test (), while binocular visual field test revealed single vision in the superior visual field (). Based on ophthalmic examination findings, the patient was diagnosed with bilateral SO palsy and underwent modified HI with adjustable suture technique in the right eye and modified HI procedure in the left eye (). On postoperative day 1, double Maddox rod test revealed 5 degrees of extorsion and the suture was adjusted to eliminate this remaining torsion. On postoperative day 3, the patient’s head position was improved, he was orthotropic in primary gaze, and fundus photography showed +1 intorsion. At postoperative 4 months, the patient was orthotropic with no limitation or torsion in any gaze position, and maintained straight gaze (). There was no torsion in fundus images. Extraocular muscle functions were normal in the Hess screen test and his field of single vision in binocular visual field testing had expanded ().
|
pmc-6216535-2
|
A 53-year-old male patient presenting with diplopia stated that his complaint had started after a traffic accident 6 months earlier. He had 20/20 vision in both eyes and normal biomicroscopic and fundoscopic examination findings. The patient exhibited a chin down head position and had torsional diplopia as well as V-pattern esotropia of 12 prism diopters on downgaze. He had limited depression in adduction (-2) in both eyes, but no IO hyperfunction. Double Maddox rod test revealed 20 degrees of extorsion and fundus photography revealed +2 extorsion. Bilateral SO muscle hypofunction was observed in Hess screen test and binocular visual field testing revealed diplopia on downgaze. The patient was diagnosed with bilateral SO palsy based on examination findings, and the modified HI surgery was performed in both eyes. At postoperative week 1, the patient showed improved head position, fundus images showed no extorsion, and 2 degrees of extorsion were observed in the double Maddox rod test. Hess screen test showed normal SO muscle function bilaterally and slight IO hypofunction. Diplopia on downgaze was not detected in binocular visual field testing. At postoperative 4 months, the patient exhibited normal head position and was orthotropic in primary gaze. No torsion was observed in double Maddox rod test and fundus images. The patient described slight diplopia on upgaze. Hess screen test showed normal SO function with -1 hypofunction in the IO muscles. No additional intervention was done.
|
pmc-6216535-3
|
A 58-year-old female patient reported developing double vision following a traffic accident 1 year earlier, and that later her right eye gradually developed an upward deviation. Her vision was 20/20 in both eyes and her anterior segment examination and fundoscopy findings were normal. She exhibited a left head tilt. In primary gaze position, hypertropia of 14 prism diopters at distance and 12 prism diopters at near was measured in the right eye. Depression in adduction was -2 limited and IO hyperfunction was not observed in the right eye. Double Maddox rod test revealed 10 degrees of extorsion and +2 extorsion was measured on fundus photography of the right eye. Hess screen test revealed reduced SO muscle function in the right eye and binocular visual field testing revealed diplopia on downgaze. She was diagnosed with right SO palsy and modified HI surgery with 5.5-mm SR recession was performed. At postoperative 1 week, the patient showed improved head position and extorsion. Minimal hypertropia was observed on the right eye in primary gaze position, while Hess screen test revealed improved SO muscle function in the right eye and binocular visual field testing demonstrated reduction in the area of diplopia on downgaze. Examination findings at the first postoperative month showed no further changes, and the patient continued follow-up in a different city.
|
pmc-6216536-1
|
A 36-year-old male patient presented to our clinic with a complaint of sudden, painless vision loss in his left eye for 1 week. His history was unremarkable except for raw meat consumption. Best corrected visual acuity (BCVA) was 20/20 and 20/125 and intraocular pressure was 16 mmHg and 14 mmHg in his right and left eyes, respectively. Anterior segment examination was normal bilaterally. Pupillary light reflexes showed relative afferent pupillary defect in his left eye. The optic nerve head was edematous with indistinct margins and star-like macular exudates were detected in left fundus examination (). In addition, spectral-domain optical coherence tomography (SD-OCT) (Topcon 3D-OCT 2000 Corporation, Tokyo, Japan) showed subretinal fluid in the macula. Right fundus examination was normal. Visual evoked potential was consistent with delayed conduction and Humphrey visual fields showed an inferior arcuate scotoma in the central 20 degrees in the left eye.
Etiological investigation was conducted, including complete blood count, biochemical, viral, bacterial, and parasitological serological tests. Detailed evaluation was performed, including chest x-ray and quantiferon test for tuberculosis, lysozyme and angiotensin converting enzyme level analysis for sarcoidosis, and relevant serological tests for cat-scratch and Lyme disease, along with consultations for rheumatologic and neurological diseases. Cranial magnetic resonance imaging and laboratory tests were all in normal range except Toxocara immunoglobulin (Ig) G seropositivity with increased avidity (ELISA and Western Blot) and elevated total IgE (Total IgE = 140 IU/mL) without eosinophilia.
Intravenous methylprednisolone therapy (1 g daily for 1 week) was administered with a preliminary diagnosis of neuroretinitis. After 1 week, BCVA in the left eye increased to 20/30. Considering his history of raw meat consumption, the neuroretinitis was thought to be related to Toxocara infection, and oral albendazole treatment (400 mg twice daily) was given in addition to the maintenance corticosteroid regimen for 1 month.
After 1.5 months, BCVA in the left eye was 20/20 and clinical signs including optic nerve head edema and macular exudates had completely resolved. The subretinal fluid in the macula had also disappeared on SD-OCT ().
|
pmc-6217712-1
|
A 66-year-old woman presented with polyuria and polydipsia for 3 weeks prior to evaluation by her primary care physician. Because of the concern for diabetes insipidus, the patient underwent MRI of the brain with and without contrast. Scans showed a right temporoparietal brain lesion ~4.5 cm × 4 cm in size (Fig. ). The patient underwent surgical resection, and the neurosurgeons achieved gross total resection with an absence of visible disease on contrast-enhanced MRI. A diagnosis of GBM was made and testing determined the tumor to be methyl guanine methyl transferase (MGMT) hypermethylated, EGFR amplified and EGFRviii positive.
The patient opted to enroll in a clinical trial that uses 3D spectroscopic MRI [] to monitor the metabolic response of patients to an experimental histone deacetylase inhibitor (HDACi), belinostat, concurrent with TMZ and radiation therapy. Pan-isoform HDACi’s like belinostat are hypothesized to have a synergistic effect with TMZ for radiosensitization of tumor cells; belinostat has more blood–brain barrier penetration than other HDACi’s []. A post-resection sMRI scan suggested the presence of residual non-enhancing disease. She received 60 Gy radiation in 30 fractions over 6 weeks in conjunction with belinostat and TMZ, and appeared to have stable disease per MRI 1-month post-radiation (Fig. ).
One month later, the patient experienced linguofacial swelling and hives after her first cycle of adjuvant TMZ. Recognizing this to be an allergic reaction, an extensive desensitization regimen was performed to no avail. The patient was taken off the clinical study and alternative chemotherapeutic agents were considered. Since the patient could not tolerate TMZ and refused to try other alkylating agents, and noting that her tumor exhibited mutated EGFR, she was started on erlotinib, an EGFR inhibitor used primarily in treatment of non-small cell lung cancer []. Concurrently, she began use of Novocure’s Optune, a device which generates low intensity TTFs via a scalp-mounted transducer array []. The patient has tolerated the treatment well and her complaints thus far have been left arm paresthesia while playing stringed instruments, as well as scalp, face and arm irritation from the Optune device. She is on a regimen of 7× 150 mg tablets of oral erlotinib weekly. Per imaging and clinical course, she appears to have stable disease 9 months post-radiation (Fig. ).
|
pmc-6217764-1
|
A 34-year-old Caucasian man was injured due to an explosion in May 2017 at a combat mission in East Ukraine (Donetsk region). Our patient’s status was critical at admission. He was diagnosed with a penetrating blind wound of the abdomen, penetrating damage of the duodenum, colon, and marginal damage of the inferior vena cava, and acute hemorrhage. As a result of shelling after the booby trap explosion, our patient presented with wounds and fractures of both lower extremities. Our patient was in sopor at admission; other neurological parameters were not evaluated. Parameters of his vital functions (e.g. heart rate, blood pressure, oxygen saturations, etc.) were unknown at the time of admission, because they were not included in the official medical records at the first medical aid stage at the battlefield.
First medical aid was provided immediately at the place of injury by medical military personnel: a tourniquet was placed on his right lower extremity; intramuscular injection of 2 mg of butorphanol, 100 mg of doxycycline, 0.5 ml of anatoxin against Clostridium tetani. Both lower extremities were immobilized by using the ladder splint. Subsequently, our patient was urgently evacuated by ambulance to the nearest hospital and was provided with qualified medical aid.
Qualified surgical aid was provided at the Central District Hospital of Toretsk City. Our patient was admitted to the hospital within 55 minutes of his injury, according to the principle of the “golden hour”. Antibiotic therapy was continued with administration of doxycycline, 100 mg. He was diagnosed with a penetrating wound of the abdominal wall (the dressing on the abdomen was impregnated with intestinal contents and dark blood) and severe hemorrhagic shock due to continuing internal bleeding. Our patient underwent urgent laparotomy. During the operation we detected such injuries as: a perforating missile wound of the transverse colon with torn edges, a perforating missile wound of the horizontal part of the duodenum (the third part), a focus of primary traumatic necrosis at the head of the pancreas, diffuse fecal peritonitis in reactive phase. There was approximately 1800 mL of blood in the retroperitoneal space, hence a large and tense hematoma was diagnosed. The hematoma was aspirated, and further revision of the retroperitoneal hematoma site revealed the source of bleeding, which was a marginal wound of the infrarenal part of the inferior vena cava. Vascular suturing was performed to achieve persistent hemostasis. Colloid and crystalloid solutions, along with blood units were infused to maintain arterial blood pressure. To treat hemorrhagic shock our patient underwent resuscitation by transfusion of three doses of 270 mL of red cell mass, 2 doses of 250 mL of fresh frozen plasma, infusion of colloid and crystalloid solutions. Taking into account the critical status of the patient, the decision was made to minimize surgical interventions of the intestinal injuries according to the principles of damage-control tactics. Thus, intestinal wounds were sutured and a nasojejunal intubation was performed. Resection of necrotic focus at the head of the pancreas was not carried out. In order to stabilize systemic hemodynamics, after abdominal wall closure a 30-minute intraoperative pause was taken to continue intensive infusion therapy. Then we began surgical interventions to treat the extremity wounds. After tourniquet removal, bleeding from the right tibial artery was revealed without signs of critical limb ischemia. To stop the bleeding, ligation of the right posterior tibial artery was performed. Fractures of the left tibia were treated by extrafocal osteosynthesis using a rod external fixation device at the stage of specialized surgical aid on May 31, 2017 (second day after injury).
Specialized surgical aid was provided at the Military Medical Clinical Center of the North Region in Kharkiv. Upon admission, our patient underwent ultrasonography (US) of the abdominal and pleural cavities to check for possible complications. Further treatment modalities at the stage of specialized surgical aid included wound redressing, infusion therapy by colloids and crystalloids, ceftriaxone 4 g/day, metronidazole 1 g/day, parenteral nutrition by protein-based and medium chain triglycerides mixture, pantoprazole 40 mg/day, metoclopramide hydrochloride 20 mg/day, drotaverine hydrochloride 80 mg/day, enoxaparin sodium 80 mg/day, ferric(III)-hydroxide polyisomaltosate 200 mg/day), two transfusions of red blood cells and fresh-frozen plasma, albumin 20 g/day, infusion and vitamins (B1, B12), physiotherapy. Thoracic X-ray was not performed, since a whole-body CT scan is routinely performed for all hemodynamically stable patients with projectile and blind gunshot wounds according to the protocol of Military Medical Department of Ministry of Defense of Ukraine beginning July 2014.
The US scan showed no dilatation of the inferior vena cava. Blood flow was retained, and a small volume of fluid in the right pararenal fatty tissue was identified. To our surprise, CT analyses revealed a foreign body of metallic density of 9.5 × 6.5 mm in the middle lobe branch of the right pulmonary artery (Fig. ). Considering the absence of entrance wounds at the chest, a migration of the metal fragment was suspected from the inferior vena cava at the time of suturing, considering the embolus type of the foreign body. Severe trauma and damage control tactics were taken into consideration, thus we decided not to remove the metal fragment immediately, but to postpone its removal until after stabilization of his abdomino-skeletal injuries.
Our patient started enteral feeding through nasojejunal tube on June 1, 2017 (the third day after his injury). Drainage tubes from the site of the inferior vena cava and pelvic region were removed, one at the horizontal part of the duodenum remained. On June 6, 2017 (the eighth day after his injury) a bile leakage was detected from the remaining drainage tube and a contrast CT scan of his abdomen was performed. Analyses of the CT scan showed accumulation of contrast in the front of the descending part of the duodenum within an area of 6 × 5 × 6 mm without clear boundaries; in the same area a limited fluid cluster of 32 × 38 × 34 mm was identified with air bubbles and without clear boundaries, suggesting an intra-abdominal abscess.
On June 7, 2017 (the ninth day after his injury), our patient was transported to the National Military Medical Clinical Center of Ministry of Defense in Kyiv in order to apply more treatment modalities. After transportation, our patient underwent relaparotomy. At revision in the right upper quadrant of his abdomen, an infiltrate was identified and dissected. While undergoing dissection, approximately 50 mL of fecal content was released and two necrotic areas (25 × 30 mm and 15 × 40 mm) with perforations were identified in the transverse colon at the site of the previous sutures (Fig. ). Further revision revealed leakage of pancreatic juice and bile from the sutured areas of the duodenum. His duodenum was re-sutured followed by a right hemicolectomy and a side-to-side ileotransversoanastomosis. Taking into consideration the presence of local peritonitis, posttraumatic pancreatitis, and pancreatic necrosis, we decided to disconnect the stomach and the sutured zone of the duodenum from the food passage. For this purpose, an antecolic gastroenteroanastomosis with a Braun anastomosis was performed, and a nutrition tube was inserted into the efferent jejunal loop of the Braun anastomosis. Furthermore, we revealed suture insufficiency in the duodenum and leakage of pancreatic juice. Considering such intraoperative findings, we decided to perform a right hemicolectomy. We did not consider performing intestinal reconstruction by a two-stage procedure with the open abdomen because of our previous experience in the treatment of gunshot-related peritonitis after the application of high-energy weapons in patients that were injured during armed conflicts in East Ukraine. In addition, the choice of surgical approach was frequently decided intraoperatively, including in this case.
Biliary decompression was achieved by an ultrasound-guided transhepatic cholecystostomy in the right upper quadrant as an additional method for biliary decompression, and to prevent the formation of a duodenal fistula. On the second day after relaparotomy, an external duodenal fistula occurred and healed on the 29th day after the duodenum was disconnected from the food passage. On the fifth day (July 7, 2017) after relaparotomy, a suppuration of the laparotomy wound occurred. Also, a right-side upper-lobe nosocomial pneumonia and unspecified reactive hepatitis were diagnosed and treated. In the postoperative period after the relaparotomy, our patient received the following therapy: doripenem monohydrate 1.5 g/day, gatifloxacin 400 mg/day, fluconazole 100 mg/day, nadroparin calcium 2850 IU, pantoprazole 40 mg/day, epidural block, enzymes, physiotherapy, and enteral feeding.
On July 20, 2017 (the 52nd day after his injury), our patient was discharged from the surgery ward and transferred to a military rehabilitation center in the Military Medical Clinical Center of Occupational Pathology of Personnel in Irpin City. Our patient was administrated physiotherapy, exercise therapy, massage, wound redressing, symptomatic medications for the multi-fragment fractures of both shins. Bone callus formation in the fracture sites was detected (Fig. ). Blood tests results were available at this stage (performed on August 27, 2017 – the 60th day after injury) for the following parameters: red blood cell (RBC) count of 4.6 × 1012/L, hemoglobin (Hb) level 127 g/L, white blood cell (WBC) count 8 × 109/L, platelets 208 × 109/L, erythrocyte sedimentation rate (ESR) 14 mm/h, total bilirubin 10.4 μmol/L, serum glucose 6 mmol/L, alanine transaminase (ALT) 584 U/L, aspartate transaminase (AST) 200 U/L, creatinine 89 μmol/L, total protein 66 g/L, serum urea 4.7 μmol/L, and amylase 31 U/L.
During the 2.5 months after the injury, the fragment that migrated after the booby trap explosion in the right mid-lobe artery did not present any clinical manifestations. Meanwhile, our patient was diagnosed with bullous lung disease of the upper lobe of the right lung, thus the removal of the fragment was performed after our patient had completed the rehabilitation course. Our patient was admitted to the Department of Thoracic Surgery at the National Military Medical Clinical Center (Kyiv). A chest X-ray and CT scan showed no changes in the right mid-lobe pulmonary artery at the location site of the embolus from the booby trap fragment (Fig. ). On August 17, 2017 (the 80th day after injury), our patient underwent a right-sided thoracotomy in the fifth intercostal space. On revision of the right lung, a firm metal object with a diameter about 10 mm was identified in the basal region of the middle lobe. The mediastinal pleura was incised, the branches of the right upper pulmonary vein were identified. The right pulmonary artery and the mid-lobe artery were taken to traction sutures (Fig. ). The booby trap metal fragment was identified in the lumen of the branch of the right mid-lobe artery at the medial segment, next to the bifurcation of the lobar artery. The wall of the artery was cut above the location of the metal fragment and a metal object 8 × 6 mm in diameter was visualized tightly fixed to the wall of the vessel (Fig. ). Analyses of the shape and kind of the embolus showed features of a piece of metal wire, which was a hand-made element that acted as additional shrapnel and was manually added to the conventional F1 hand grenade, to achieve a higher crippling effect against military personnel. Further, the distal lumen of the right mid-lobe artery was thrombosed, while the blood supply and aeration of the middle lobe of the right lung were preserved due to the collaterals. The embolus bed was washed with antiseptic, followed by ligation of the proximal and distal parts of the artery. An atypical resection of the upper lobe of the right lung was performed considering the presence of the multiple lung bullas up to 15 mm in the upper lobe of the right lung. After thoracotomy, our patient was administrated for ciprofloxacin 600 mg/day, ertapenem 1 g/day, and amikacin sulfate 1 g/day.
The bone fractures of both extremities were consolidated, external fixation devices were removed on the 168th day after the injury (Fig. ). Our patient was discharged from the hospital in a satisfactory condition and then was retired from the military service (Fig. ).
The follow-up of our patient as well as past medical, environmental, family and employment history are not available, which is a limitation. Our patient associated himself with weak alcohol consumption and moderate smoking for 10 years, which is very common in Ukrainian population.
|
pmc-6217864-1
|
A 64-year-old uncircumcised, paraplegic male presented with a two-month history of an asymptomatic, enlarging penile lesion with episodic spontaneous bleeding. The patient had been confined to a wheelchair for the past 15 years due to transverse myelitis and was wearing a diaper secondary to overflow urinary incontinence. He reported a monogamous relationship with his wife and currently was not sexually active because of erectile dysfunction. The patient denied a history of smoking and sexually transmitted diseases. Past surgical history was significant for a transurethral resection of the prostate for benign prostatic hyperplasia. Physical exam revealed a 2.5-cm cauliflower-like nodule occupying 40% of the glans penis (Figure ). The lesion expressed a white discharge and was nontender. It involved the distal urethrae creating a hypospadias from the tip of the glans to the corona. The inguinal lymph nodes were not palpable.
Skin biopsy was performed, and histopathology demonstrated exophytic papillary architecture with markedly irregular epidermal acanthosis (Figure ). Well-differentiated, bulbous projections of squamous cell proliferation with characteristic round, pushing deep margins were found pressing against the dermis and submucosa (Figure ). High-power magnification revealed nuclear polymorphism, dyskeratosis with keratin pearls, and mild cytologic atypia (Figure ). No dermal or submucosal invasion was seen. These histological features were consistent with VC. In situ hybridization was positive for HPV 6, 11, 31, and 33 and negative for HPV 16 and 18 (Figures -). Venereal disease research laboratory (VDRL) and human immunodeficiency virus (HIV) screening were negative. The patient underwent a partial penectomy. Follow-up cystourethroscopy 10 months later showed no evidence of recurrence.
|
pmc-6217865-1
|
A 50-year-old male with a known history of coronary artery disease (CAD), hypertension, and alcohol abuse was brought into the emergency department (ED) in an obtunded state by emergency medical services. En route to the hospital, he had an episode of a generalized tonic-clonic seizure (GTCS), which subsided with the administration of intravenous (IV) midazolam.
Initial presentation to the ED revealed a patient with fluctuating consciousness and a Glasgow Coma Scale (GCS) score of 11/15. Vitals included a temperature of 36.6 C, a blood pressure of 178/107 mm Hg, a heart rate of 92 beats per minute, oxygen saturation of 90%, and a respiratory rate of 20 breaths per minute. The physical examination was unremarkable while a neurologic exam was limited due to sedation. Cough and gag reflexes were intact.
Initial laboratory investigations are presented in Table .
Considering a low serum osmolality of 231 mEq/L, a low random urine osmolality of 161 mOsm/kilogram of water, and a lack of prior medications that could be attributed to the decreasing levels of serum sodium, his hypovolemic hyponatremia was attributed to psychogenic polydipsia and/or poor oral solute intake. The patient was initially treated with an infusion of two liters of normal saline (NS) and 200 mL of hypertonic saline. The serum sodium rose to 112 mEq/L with this infusion in the first 24 hours. An appropriate rise in serum sodium was observed in the ensuing four days, at a rate of 8 mEq per 24 hours. During the course of his medical intensive care unit (MICU) stay, the patient remained somnolent and visibly encephalopathic, which led to a self-extubation on the fifth day of his admission. He maintained a near-normal oxygen saturation on room air and was, therefore, continued on oxygen via a nasal cannula.
The patient’s altered mentation was initially attributed to multifactorial etiologies, which included intensive care unit (ICU) delirium, acute hypoxic encephalopathy, and electrolyte abnormalities. An appropriate sodium correction and a lack of hypoxic episodes prior to or after self-extubation ruled out the differentials, explaining his altered mentation. Despite adequate treatment, a persistent somnolence prompted a brain magnetic resonance imaging (MRI) scan whose findings were consistent with osmotic pontine demyelination. Imaging revealed edema and swelling in the pons, which was consistent with a subacute injury related to osmotic demyelination. Edema involving the internal/external capsules, thalamus, and amygdala as well as enhancement involving the subcortical white matter in the temporal lobes were also observed and attributed to a likely related subacute osmotic injury (Figure ).
The patient continued to have difficulty in efficiently managing his oral secretions secondary to his neurological deficits and eventually aspirated with resultant findings of aspiration pneumonia in the ICU setting. He was treated with ampicillin-sulbactam for the duration of this predicament, five liters of oxygen via nasal cannula and frequent oral/pharyngeal suction. A subsequent neurology and palliative care consult concluded that the best approach moving forward was for the patient to undergo aggressive physical, occupational, and speech therapies over the next eight weeks and to observe for signs of improvement. The patient is currently followed in the outpatient department (OPD) with frequent follow-ups while further decisions regarding goals of care will be made after the resolution of these intensive therapeutic strategies.
|
pmc-6217868-1
|
A 22-year-old homeless male with no known past medical history presented with a complaint of epigastric abdominal pain and non-bloody bilious vomiting for last two days. He was taking amphetamine and cocaine but quit nine months ago. He admitted recent regular Spice/K2 use. He starts taking Spice/K-2 one week prior to the onset of symptoms. Physical examination was unremarkable except mild ictrius. Abdominal examination was normal. Aspartate aminotransferase (AST) 712 IU/L, and alanine aminotransferase (ALT) 1764 IU/L were elevated. Total bilirubin and direct bilirubin were 3.8 mg/dl and 1 mg/dl, respectively. Hepatitis B surface antigen (HBsAg), anti-hepatitis C virus (HCV), antinuclear antibodies (ANA), anti-smooth muscle antibodies (ASMA), and anti-liver-kidney microsome-1 antibodies (ALKM-1) were negative. Blood alcohol level was undetectable, and urine toxicology was positive for cannabinoids and barbiturates. Blood work ruled out viral, autoimmune, metabolic and other toxic etiologies of liver injury. Therefore, patient acute liver injury was deemed to be secondary to Spice/K2 toxicity. N-acetyl cysteine initiated for imminent acute liver failure with clinical and biochemical improvement observed over a course of one week. The trend of liver enzymes during hospitalization is shown in Table .
The patient discharged in stable clinical condition and on follow-up visit after four weeks he reported no further symptoms and liver function tests were within normal range. The patient counseled about abstinence from drugs of abuse, and he agreed to consider joining the outpatient drug rehabilitation program.
|
pmc-6217869-1
|
A 72-year-old man with a past medical history of abdominal aortic aneurysm repair, prostate cancer, ischemic stroke with residual left hemiparesis, and significant peripheral vascular disease complicated with an above-the-knee amputation presented to our departments following an abdominal CT for possible bowel obstruction with a 3.1-cm x 2.6-cm solid renal tumor (Figure ). The renal tumor was enhancing and located centrally at the inferior pole of the left kidney (Figure ).
This patient was referred to interventional radiology because he was deemed a poor surgical candidate due to his underlying comorbidities. Due to the central location and the high probability of irreversible cryoablation of the renal collecting system, the decision was made to proceed with bland arterial embolization. This was done with the expectation that the tumor would shrink enough to allow a safe window for cryoablation.
Conventional angiography was performed which demonstrated enhancement of a well-defined vascular, renal mass (Figure ). Super selective catheterization using a microcatheter of the feeding vessels allowed for selective bland embolization of the tumor with 100-300 microns polyvinyl alcohol particles (Figure ). Post-embolization angiography showed a cessation of vascular flow to the tumor (Figure ). Follow-up CT two months post-embolization showed a 30% reduction in tumor size (Figure ).
Three months following initial embolization, the patient underwent cryoablation of the renal tumor. Two cryoprobes were inserted into the center of the tumor under CT guidance (Figure ), and two freeze cycles were performed (10 minutes each) interspersed by a thaw cycle of eight minutes. Intraoperative images demonstrated complete coverage of the residual tumor by the ice ball (Figure ). The patient tolerated the procedure well and had no immediate post-procedure complications. The renal collecting system was not affected by the cryoablation procedure. A follow-up CT four years after the cryoablation showed no residual malignancy (Figure ).
|
pmc-6217871-1
|
We report a case of a 63-year-old female with a medical history significant for a gastric bypass surgery (initial: 40 years ago, revision: 20 years ago), provoked deep venous thrombosis/pulmonary embolism 20 years ago, hypothyroidism, gastroesophageal reflux disease, and chronic low back pain, who presented to the hospital complaining of abdominal pain that started gradually 3 weeks ago and the associated symptoms including anorexia, nausea, and vomiting.
She underwent an abdominal computed tomography, which showed a marked distention of the gastric remnant with irregular thickening within the antro-pyloric region and the post-surgical changes of gastric bypass procedure showing a patulous segment of small bowel within the left mid-abdomen at the presumed jejunojejunal anastomosis.
Emergency esophagogastroduodenoscopy was done, which was unremarkable because of the inability of the scope to pass through the anastomosis following the bypass surgery. She underwent balloon-assisted retrograde enteroscopy, which showed a gastric bypass with a normal-sized pouch, an intact staple line, and gastro-jejunal anastomosis characterized by healthy appearing mucosa (Figure ). The examined portion of jejunum was normal. A large frond-like villous mass was found at the pylorus with no evidence of bleeding (Figure ). It was approached in a retrograde fashion and biopsied with cold forceps. The gastric remnant could not be evaluated, as the pyloric mass was obstructing the lumen (Figure ).
Pathology work-up was expedited, and the results indicated adenocarcinoma in situ in the antro-pyloric region. Carcinoma in situ is an early stage of gastric cancer and falls under the category of Tis or stage 0; it is characterized by an intraepithelial tumor that does not invade the lamina propria as compared to the T1 stage tumor that invades the lamina propria, submucosa, and muscularis mucosa []. Her-2/neu testing was deferred because of the lack of definitive invasion.
|
pmc-6217873-1
|
A 21-year-old Pakistani male presented with unresolved, high spiking fever for four weeks, associated with episodes of moderate headache, mild, dry cough, generalized body ache, arthralgia and later, an erythematous rash over his back and limbs. The fever reportedly developed a month before the current hospital visit while the patient was on vacation in his hometown, in the rural area of Jacobabad. It began as episodes of high grade fever and progressed to a more continuous pattern with chills accompanying both phases. There were multiple bouts of fever through the days and nights which would only partially and temporarily respond to acetaminophen or ibuprofen. There was no previous history of similar fever break outs. The arthralgia was mostly confined to the knee and ankle joints. Upon first presentation to a healthcare facility in the nearby small city of Sukkur, the patient was hospitalized for fever and treated with intravenous antibiotics (ceftriaxone and meropenem) along with paracetamol for pain and artemether for suspected malaria. The various lab investigations conducted, including those for the malarial parasite, hepatitis B surface antigen, hepatitis C antibody and HIV 1 and 2 antigens, all came out normal, as did his X-ray chest, echocardiography, and ultrasound of the abdomen. After a two-week stay and unsettled fever, the patient was referred to a healthcare center in Karachi with tertiary healthcare facilities where he was treated with further antibiotics as a potential case of enteric fever, but to no relief.
During this third presentation, the examination revealed a weak looking, fatigued male to us with a fever of 39.0°C. The erythematous rash over his back and limbs, noted first upon this third presentation, had possibly gone unnoticed before due to his dark colored skin. There were no other remarkable findings from the other systems, including no sore throat or synovitis. Hematological investigations showed elevated neutrophils (82%), a total leukocyte count touching the upper limit of normal, disturbed liver function tests with elevated alanine transaminase (124 U/L), and a remarkably high level of C-reactive proteins (13.5 mg/dl). Renal and coagulation profiles were normal, as were blood and urine cultures and the chest X-ray. The anti-cyclic citrullinated peptide, antinuclear antibody (ANA), and rheumatoid factor (RF) were all negative as well. As all these systemic investigations continued to come out normal, the diagnosis of exclusion kept increasing the chances of this being a case of AOSD. Based on this suspicion, the serum ferritin levels were carried out and found to be markedly elevated (2698.00 ng/mL). Additionally, the ultrasound of the abdomen revealed benign looking, small lymph nodes (para-aortic and mesenteric) that were not noticeable on the previous scan, as well as mild hepatosplenomegaly.
Based on his clinical features and review of the laboratory evaluations, the differentials considered this time were: a resistant infection like that by cytomegalovirus (rooted out by the lack of a sore throat and no monocytosis in the laboratory reports), thyroiditis (excluded due to the absence of an enlarged and tender thyroid), lymphoma, and Still’s disease. Although serum ferritin is raised in both lymphoma and AOSD, a diagnosis of lymphoma usually follows findings of more significant lymphadenopathy and hepatosplenomegaly, variable weight loss and raised lactate dehydrogenase (LDH) levels on investigations, along with other signs and symptoms. The absence of the aforementioned, coupled with the patient’s immediate response to the first dose of steroids with the settling of fever and improved wellbeing, all pointed towards AOSD and away from lymphoma—further confirmed by the Yamaguchi criteria [].
The patient was treated with oral prednisolone in tapering doses, starting with 60 mg per day. As mentioned, he responded to the very first dose with the settling of fever. He went home and returned after two weeks, in an absolutely normal state and did not have a recurrent bout of fever. He was advised to come back in case of any relapse.
|
pmc-6217876-1
|
The liver graft was from a young male patient, 20 years old, who weighed 57 kg, was 1.78 meters tall, and had a body mass index (BMI) of 18 kg/m2. He was admitted to a general hospital with a subarachnoid hemorrhage and intracranial hematoma on the left side, and he suffered brain death. He had a previous pathological history of SCA and was treated for his anemia with several blood cell therapies and a splenectomy when he was 16 years old. He was receiving ceftriaxone, meropenem, and vancomycin when he was submitted to donor surgery, five days after the neurosurgery. He was under low doses of vasopressors: norepinephrine (0.18 mcg/kg/min) and vasopressin (0.02 mcg/kg/min). The best suitable recipient was chosen by balancing the risk of a hematological disease or thrombotic risk factors associated with the recipient remaining on the waiting list and either dying or dropping out of the list. All laboratory analysis and liver function of the donor were normal. After all analysis and arguments discussed with the transplant team (surgeons, hepatologists, and infectologists), as risk of using SCA graft, probabilities of developing disease, and a few case reports in the literature, on the other hand, the benefits of being an excellent hepatic graft option, the recipient and her family were informed of all risks and probabilities, and a unanimous informed consent decision was made to receive the donor liver and follow with the transplant. The donor surgery was fine and was not associated with any complications. Both of the deceased patient's kidneys and liver were donated to three different recipients in different centers.
The liver recipient was a 37-year-old woman, and her blood type was the same as that of the donor. She weighed 54 kg and was 1.65 meters tall. Her BMI was 19.8 kg/m2. She was diagnosed with hepatitis B virus (HBV) cirrhosis and hepatocellular carcinoma (HCC) according to the Milan criteria (2 tumors each with diameter ≤ 3 cm, without extrahepatic and major vessel involvement). Model for end-stage liver disease (MELD) score was 18 and Child-Pugh-Turcotte (CPT) classification was B7. The serum alpha-fetoprotein (AFP) value was increasing recently to the transplant (>200 ng/ml). In the waiting list for liver transplant, the patient performed 3 transarterial chemoembolization (TACE) sessions, initially with completely treated areas but afterwards showing partial treatment (progression). The liver allograft weighed 1.495 kg. The cold ischemia time was 8 hours and 30 minutes, and the warm ischemia time was 38 minutes. The transplantation was performed on September 27th, 2016. The patient received four units of red blood cells, three units of platelets, and eight units of plasma during the surgery. The liver biopsy protocol included preclamping and prerevascularization biopsy with mild siderosis and steatosis, hepatocyte ballooning, rare canalicular cholestasis, and moderate sinusoidal congestion with predominance of drepanocytes ().
The postrevascularization biopsy revealed mild ischemia reperfusion injury (grade 2), including apoptosis of hepatocytes and a minor neutrophilic sinusoidal infiltrate. Kupffer cell erythrophagocytosis and scarce sickle cells were still seen in a biopsy performed on the following day (), along with some ischemic areas. The immunosuppression protocol included corticosteroids every day after the anesthetic induction for 6 months. In terms of the glucocorticoid withdrawal regimen, basiliximab 20 mg was administered on the anesthetic induction and on the fourth postoperative day; mycophenolate mofetil and tacrolimus were administered from the 4th day on (due to the institutional protocol and literature we initiated the onset of tacrolimus later due to acute renal failure and using protocol with corticosteroids and basiliximab). The tacrolimus dose (0.10-0.15 mg/kg/day administered twice a day) was adjusted based on the liver blood tests and the blood level 6-10 ng/ml was maintained during this first year.
The LT recipient also received antibiotics based on the medical profile of the liver donor: vancomycin and meropenem for seven days plus Amicacina for two days. Furthermore, she received hepatitis B immunoglobulin (HBIG) for seven days and then monthly for a year. She still receives entecavir (ETV) daily for preventing hepatitis B recurrence. As part of the LT protocol, she was submitted to a Doppler ultrasound of the vascular graft anastomosis investigation on the first postoperative day, and no abnormalities were noted. The follow-up of the vascular imaging examination will depend on clinical and laboratory data. Despite her good postoperative evolution, she developed acute kidney failure and she required hemodialysis for three weeks. The cause of acute renal failure and hemodialysis was due to liver transplantation procedure and the baseline renal function somewhat altered. Her recovery of renal function was complete, and she did not require renal replacement therapy anymore. She was discharged from hospital on the 37th postoperative day. Postoperative chemotherapy was not necessary due to good evolution and postoperative oncologic control image (CT) with absence of recurrence in the transplanted liver and decrease in the serum tumor marker (AFP <10 ng/ml). A long-term follow-up of 18 months revealed a good evolution, with normal liver blood tests.
This study was approved by the Institutional Review Board and accomplished the entire requisite for studies in humans according to the guidelines of the 1975 Declaration of Helsinki.
|
pmc-6217878-1
|
A 27-year-old Chinese woman, gravida 3, para 2, body mass index (BMI ) 17.20 kg/m2, visited our clinic with eight-week pregnancy in a prolapsed uterus on 4th of September 2013. Pelvic examination revealed stage 3 pelvic organ prolapse (POP), with point C as the leading edge using the Pelvic Organ Prolapse Quantification (POPQ) examination (Aa+3, Ap+3, Ba+6, Bp+6, C+6, D+2, gh 4.5, pb 2, tvl 9 ). Her prolapsed uterus could be restored to pelvic cavity within bed rest. It was more serious while standing or walking. Hospitalization was recommended for this pregnant woman, but she refused and she waited at home for delivery.
Her previous pregnant record was as follows: a dead female baby was induced at the 30th week of gestation during her first vaginal delivery in 2003, puerperium was uneventful, and two days after delivery, she was discharged in good health. She had her second vaginal delivery, after 38+3rd week of gestation and seven-hour labor in 2007; a 2800 g alive baby boy was delivered, with Apgar scores of 10/10. Pelvic examination revealed stage 3 POP using the POPQ examination (Aa+3, Ap+3, Ba+6, Bp+6, C+6, D+2, gh 4.5, pb 2, tvl 9) at the 36+3rd week of gestation in her second pregnancy. No special examination or treatment was executed before and after childbirth. However, the prolapsed vaginal mass was spontaneously restored after childbirth.
The woman presented to our hospital again with premature rupture of membrane (PROM) in labor at 39+6th week of gestation with an irrestorable uterine prolapse for 8 months on the 8th of May 2014. Pelvic examination revealed stage 4 POP using the POPQ examination (Aa+3, Ap+3, Ba+9, Bp+9, C+9, D+5, gh 4.5, pb 2, tvl 9 ) and it revealed that prolapsed uterus was in size of 20×20 cm, pink, hyperaemic, and edematous but not ulcerated. The cervical canal did not subside, internal orifice of cervix did not open, amnionic vesicle has been broken, and regular contraction was seen. A series of transabdominal ultrasonographic examinations showed a normally developing fetus in the longitudinal position in the uterine cavity, isthmus uteri was 64 mm and it was partially extruded outside the vulva which was protruding from the perineum about 64×68 mm, and the boundary was still clear, and with cervical oedema. Emergency caesarean delivery was decided and an alive boy baby weighting 2480 g, with Apgar scores of 10/10, was delivered. We used Magnesium Sulfate Solution to nurse the prolapsed uterus. Three days postpartum, the prolapsed uterus was in size of 10×10 cm. On the seventh day postpartum, the prolapsed uterus was in size of 7×5 cm, and it was restored inside the pelvic cavity after manual reposition. Pelvic floor three-dimensional ultrasound indicated that residual urine was 40 ml, cervical length was 5.6 cm and internal orifice cervix was dilated, bladder neck displacement was 15 mm, posterior angle of bladder was 180 degree, and hiatus of levator antimuscle was 32 cm2. She was discharged on the eighth days postpartum. A telephone postpartum follow-up on the 14th day showed that there was no lump prolapse when the patient was standing or walking. But when the abdominal pressure increased, such as when squatting and defecating, prolapsed vaginal mass could be palpable, with size of 2 cm × 1 cm. 42 days after childbirth, she refused regular postpartum examinations for personal reasons.
|
pmc-6217878-2
|
A 33-year-old Chinese woman, gravida 2, para 1, BMI 20.70 kg/m2, noticed a protrusion in size of 2 × 1 cm from her vagina at 13th week of gestation in 2015. Her first pregnancy resulted in one uncomplicated spontaneous vaginal delivery in 2009; the newly-born baby weighted 3000 g. There was neither history of pelvic trauma or prolapse, nor any stress incontinence during or after the first pregnancy.
The protrusion was not sensible while resting but rather palpable after moving. She visited our outpatient clinic at her 15th week of gestation in 2015 and complained worsened uterine prolapse. Pelvic examination revealed stage 3 POP, with point C as the leading edge using the POPQ examination (Aa+3, Ap+3, Ba+6, Bp+6, C+6, D+1, gh 5, pb 1, tvl 10 ). A no. 5 ring pessary in size of 7×7 cm (see ) was applied to keep the uterus inside the pelvic cavity after manual reposition. The gravid uterus persisted in the abdominal cavity after removing at the 30th week of gestation because it became larger. An alive healthy baby boy of 2680 g was delivered after four-hour labor at 39+3 week's gestation on the 5th of October 2015. She was discharged three days postpartum with complete resolution of the uterine prolapse. A follow-up postpartum examination after 42 days revealed evidence of uterine prolapse and a no. 3 ring pessary in size of 5×5 cm has been applied to keep the uterus inside the pelvic cavity after manual reposition until now. At the time of reporting, pelvic examination of this woman revealed stage 3 POP, with point C as the leading edge using the POPQ examination (Aa-2, Ap-2, Ba-1, Bp-1, C+2, D-3, gh 5, pb 1, tvl 10). Pelvic floor four-dimensional ultrasound indicated that bladder neck mobility was slightly increasing, posterior wall of the bladder was slightly bulged, and anterior vaginal wall was slightly prolapsed in anterior compartment. Stage 2 uterus prolapse was seen in middle compartment, the levator animuscle was not broken, and hiatus of levator animuscle was normal in posterior compartment (see ). Follow-up is on-going.
|
pmc-6217880-1
|
A 45-year-old woman was admitted to our breast surgery unit because of a palpable breast lesion in March 2014. After core biopsy she was diagnosed with breast cancer and received systemic neoadjuvant chemotherapy of cyclophosphamide (500mg/m2)-docetaxel (75mg/m2)-pharmorubicin (90mg/m2) regimen every 3 weeks. Computed tomography (CT) scan performed at the diagnosis of cancer and ultrasonography test before each cycle of chemotherapy showed no hepatic abnormality (). After 4 courses of chemotherapy, a 3 × 3cm slightly hypoisoechoic hepatic lesion was identified in left lateral lobe on routine ultrasonography (USG) (). On subsequent plain CT scan, the lesion is homogeneous and isointense compared to surrounding liver parenchyma (). After enhancement, rapid homogeneous enhancement of lobular-shaped nodule was seen in the arterial phase with a slightly hypodense central scar (). The lesion is isodense without signs of central scar in the portal venous phase (). Abdominal magnetic resonance imaging (MRI) showed that the lesion is slightly hypointense on T1-weighted image with the central scar appears more significant hypointense () and slightly hyperintense on T2-weighted images (). After enhancement, there is strong homogeneous enhancement of the lesion except for the central area during the arterial phase () and slightly hyperintense in the portal venous phase () compared to the normal liver tissue in fat-saturated T1-weighted image.
Both the patient's liver function serum values and tumor makers were within normal range and she had a negative hepatitis virus serology at the time when hepatic nodules were diagnosed. Besides, she had no history of smoking, drinking, or medication use and had no relevant family history. As the imaging findings could not be used to rule out the possibility of hepatocellular carcinoma, a histological examination was recommended. However, the patient refused to take liver biopsy due to personal reasons. Considering CT and MRI indicate the typical presentation of FNH, modified radical mastectomy was conducted afterwards and the patient was pathologically staged as pT2N2M0-GIII. The subsequent chemotherapy was 2 courses of the same regimen. The follow-up USG and CT showed that the lesion had been stable until the time of the report. She had been disease-free for more than 4 years.
|
pmc-6217881-1
|
Case 1 is for a female patient aged 20 at symptom onset. Despite a positive family history of angioedema attacks, a long history of recurrent peripheral and abdominal attacks, and more than 100 laryngeal attacks, the patient was only diagnosed with HAE at age 50 (). Typical symptoms included mild peripheral edema of the limbs typically lasting 3 days. Over time skin swelling extended to the abdominal and thoracic walls and head. Some of face edema was followed by aphonia and suffocation. Hours before the onset of an HAE attack, she was always progressively adynamic and these symptoms resolved gradually when the edema became evident. Initially, painful abdominal attacks were rare; however, the patient began to experience weekly abdominal attacks preceded or followed by peripheral edema. The patient repeatedly received hydrocortisone, antihistamines, and epinephrine for the treatment of attacks, all of which were ineffective. Specific treatment with C1-INH concentrate, icatibant, or fresh frozen plasma (FFP) was never received in the ED. Following some of these attacks, the gallbladder, appendix, and left ovarium were surgically removed.
The fatal attack, occurring at age 52, started with dysphagia, quickly followed by dysphonia and dyspnea. The patient was admitted to a small regional hospital and 6 hours after symptom onset underwent respiratory arrest whilst waiting to receive FFP. Resuscitation attempts were unsuccessful and the autopsy revealed laryngeal edema.
Evaluation of the case reveals several failings in the patient's treatment: firstly the severity and type of angioedema attack were not correctly recognized or assessed by the emergency physician. Secondly, the ED did not contact the HAE reference center upon admission of the patient to gather details of any previous history of attacks. Finally, the airway was not secured and emergency measures, such as cricothyrotomy, were not prepared in advance nor performed. As a consequence, life-saving measures and effective drug therapy were not provided early enough to prevent the patient's death.
|
pmc-6217881-2
|
A male patient aged 3 at symptom onset was diagnosed with HAE at age 7 (). Initial symptoms included facial swelling and painful abdominal attacks, occasionally associated with vomiting and/or diarrhea. Most of these attacks were followed in 2–3 days by swelling of a hand, leg, or genitalia. The patient had a positive family history of HAE; his brother, father, grandfather, and one paternal aunt had a history of attacks of recurrent peripheral edema and his grandfather suffered a fatal laryngeal attack aged 67 years. Prior to diagnosis, the patient had an appendectomy at age 6 following an abdominal attack. After diagnosis, the patient received prophylactic treatment with tranexamic acid.
The fatal attack, occurring at age 11, started with facial edema, followed by progressive dysphagia, dysphonia, and dyspnea. He was admitted to a small local hospital and treated for allergic laryngeal edema with repeated doses of corticosteroids and epinephrine, despite his mother advising the treating physician that these treatments had previously been ineffective. Respiratory arrest occurred 3 hours after admission and neither tracheotomy nor intubation was attempted. Autopsy confirmed obstructive laryngeal edema.
Evaluation of the case reveals several failings in the patient's treatment; although the emergency physician was made aware of the nature of the edema, hereditary angioedema was not recognized by the treating physician. The patient's family history and previous history of attacks were also not considered. Consequently, the patient was misdiagnosed and incorrectly treated for allergic edema. Effective treatment was not given and life-saving measures were neither prepared nor attempted.
|
pmc-6217881-3
|
A male patient aged 8 at symptom onset was diagnosed with HAE at age 57 (). Initial symptoms included abdominal attacks recurring every two weeks and the first laryngeal attack occurred at age 16. In the 8 years prior to his death, the patient experienced one laryngeal attack per year; one led to suffocation with loss of consciousness and four required intubation. The patient had been treated with FFP on several previous occasions with a fair response. In the 6 months prior to his death, he had been symptom-free.
The fatal attack, occurring at age 59, started with dysphonia. Attack severity progressed rapidly; the time from symptom onset to respiratory arrest was 20 minutes. Resuscitation measures were undertaken in the hospital; however, this was only after irreversible hypoxic brain damage had occurred. The patient did not have access to on-demand therapy for treatment of attacks at home.
Evaluation of the case reveals that the course of laryngeal edema can be extremely short. It is therefore imperative that patients have appropriate home treatment available, although this may not always be effective for rapidly progressing attacks. Therefore EDs should be aware of the potential for HAE attacks to progress rapidly so that emergency measures to keep the airway safe and/or cricothyrotomy can be urgently administered.
|
pmc-6217881-4
|
Case 4 is for a male patient aged 22 at symptom onset (). The patient was not previously diagnosed with HAE despite experiencing repeated peripheral and abdominal attacks. The patient also had a positive family history; five family members with confirmed HAE had died from laryngeal edema.
The fatal attack occurred at age 42; the patient awoke with the sensation of a lump in his throat and gradually developed dysphagia. After approximately 8 hours, he became dysphonic but refused to attend the ED and was treated at home with corticosteroids by his wife, a nurse. One hour later, he could not swallow and extreme breathlessness followed so the patient attempted a self-tracheotomy. An ambulance was called, and while waiting for it, the patient lost consciousness. Cardiac massage was performed by the attending ambulance crew and 5 mg of epinephrine was administered. As the patient was not intubated because no physician was in attendance, his wife attempted to perform a tracheotomy which was unsuccessful. Upon arrival at the ED, an electrocardiogram indicated electrical activity but the patient remained in respiratory arrest and later died. HAE diagnosis was confirmed retrospectively when the patient's daughter, with similar peripheral edema and abdominal symptoms, was diagnosed with low C1-INH levels.
Evaluation of the case reveals the challenges of treating an undiagnosed HAE patient. Despite previous repeated swelling attacks and a family history of HAE and fatal laryngeal edema, the lack of sufficient education and awareness by the patient and emergency medicine crew led to the mismanagement of the fatal episode. Notably, there was a long delay in calling emergency services and they were who in turn were not prepared to administer measures to keep the upper airway open, perform an emergency cricothyrotomy, or be able to provide effective treatment for the HAE attack.
|
pmc-6217882-1
|
A previously healthy 57-year-old man presented to the emergency department with fever and progressive altered mental status for 5 days. Three days before admission, he was diagnosed with influenza A at a clinic, for which oseltamivir was prescribed. Upon arrival at the emergency department, his vital signs were unstable: respiratory rate, 40 breaths/min; heart rate, 153 beats/min (irregular); blood pressure, 96/70 mmHg; body temperature, 39.9°C; and Glasgow Coma Scale (GCS), 11/15 (E3V3M5). On physical examination, an oral mucosa was dry and coarse crackles in the left lung were documented, but there were no heart murmur and no lower edema. Blood examination results were as follows: white blood cell count (10100/μL); hemoglobin (15.7 d/L); platelet count (12.8 × 103 /μL); C-reactive protein (36.82 mg/dL); creatine kinase (3181 IU/L); procalcitonin (19.58 ng/mL); and brain natriuretic peptide (123 pg/mL). Urinary Legionella antigen test was positive, while rapid influenza A and B antigen tests were both negative. Arterial blood gas analysis yielded the following findings: pH 7.54, pCO2 25.8 mmHg, pO2 81.2 mmHg (O2 10 L/min reserver mask), HCO3- 21.1 mmol/L, and lactate 2.0 mmol/L. A consolidation was observed in the left lung field by chest X-ray and chest computed tomography (CT). Electrocardiogram initially documented atrial fibrillation (AF), while echocardiogram revealed left ventricular ejection fraction (LVEF) of approximately 30%. Due to the presence of Legionella, pneumonia, and qSOFA of 3 points, it was diagnosed with Legionella pneumonia and septic shock. He was transferred to the intensive care unit (ICU), intubated, and started with mechanical ventilation management and intensive care. His progress in the ICU is shown in .
Tazobactam/piperacillin and levofloxacin for Legionella pneumonia and peramivir for suspected influenza pneumonia were initiated intravenously. For septic shock, noradrenaline at 0.06 μg/kg/min, hydrocortisone at 200 mg/day, and intravenous immunoglobulin were started. In view of AF, landiolol was started at 1 μg/kg/min and then increased to 10 μg/kg/min. Moreover, due to the low ventricular contractility, additional dobutamine at 3 μg/kg/min was initiated. The patient was then given polymyxin B-immobilized fiber and continuous hemodiafiltration (CHDF) for acute kidney injury (AKI) and suspected endotoxin shock (endotoxin level was later observed to be high, at 139.7 pg/mL). On day 3 of hospitalization, we performed cardioversion several times for AF and there was a temporary return to normal sinus rhythm (SR); however, it immediately returned to AF. Transthoracic echocardiography showed that LVEF worsened to 15%, and the left ventricular end-diastolic/systolic diameter (LVDd/Ds) was increased to 61/55 mm. On the night of day 3, metabolic and respiratory acidosis progressed due to circulatory failure (pH 7.138, pO2 70.4 mmHg, pCO2 68.4 mmHg, BE -8.7 mmol/L, lactate 2.4 mmol/L) (ventilator mode: pressure control ventilation: FiO2 70%, RR 20/min, PEEP 9 cmH2O, PC 15 cmH2O). Thus, the patient was diagnosed with cardiogenic shock due to septic cardiomyopathy. This required introduction of VA-ECMO and catecholamines were discontinued. Subsequently, the acidemia ameliorated and hemodynamic circulation stabilized. AF reverted to normal SR on day 5. And on day 7, LVEF recovered to 60%. Therefore, it stabilized hemodynamics by infusion, a little catecholamine, and VA-ECMO was discontinued on day 8.
While VA-ECMO was in progress, the patient developed progressive jaundice; this appeared to be a complication of VA-ECMO because of mechanical hemolysis (on day 7, total/direct bilirubin: 13.3/9.6 mg/dL, LDH 3077 IU/L, Hb: 8.1 g/dL). Therefore, after VA-ECMO withdrawal, we expected that total bilirubin will be decreased, but no improvement was observed (max total/direct bilirubin: 19.8/15.8 mg/dL). Cholecystitis was detected via an abdominal echography. Following percutaneous transhepatic gallbladder drainage performed on day 11, the bilirubin level declined. Subsequently, on day 15, the patient's general condition stabilized, and he was extubated. However, CHDF was switched to hemodialysis (HD) as the patient's anuric state due to AKI persisted. After 16 days, the patient was discharged from the ICU.
An arteriovenous fistula was created as a continuation of maintenance HD was necessary. Although, in the ICU, the patient had been on initiated enteral nutrition (EN), he was unable to tolerate EN due to intestinal dysfunction advancement. Therefore, he was initiated on total parenteral nutrition. Long-term rehabilitation intervention was also required due to ICU-acquired weakness (ICU-AW), because of long-term sedation and muscle relaxation during extracorporeal support. After 70 days, dialysis was discontinued; the patient was able to eat independently at approximately 90 days. He was transferred to a recovery hospital on day 108 and underwent complete social reintroduction at 5 months after discharge from our hospital.
|
pmc-6217883-1
|
A 37-year-old African American man with a history of type 1 diabetes and sickle cell trait was referred to the Gastroenterology service for ERCP/EUS to evaluate jaundice. He presented with right upper quadrant (RUQ) abdominal pain with associated nausea and vomiting ongoing in the past ten days. He denied the use of tobacco, alcohol, or other illicit drugs. The patient reported several female sexual partners in the past six months. Physical exam revealed scleral icterus and RUQ abdominal tenderness. Laboratory studies were notable for alanine aminotransferase (ALT) of 59 U/L, aspartate aminotransferase (AST) of 39 U/L, total bilirubin of 11.4 mg/dL, and alkaline phosphatase (ALP) of 657 U/L. His hepatitis A antibody, hepatitis B surface antigen, hepatitis B surface antibody, and hepatitis C antibody were negative. HIV-1 and HIV-2 antibodies were negative. Serum autoimmune markers, alpha-1 antitrypsin, iron profile, and ceruloplasmin were also negative. Antimitochondrial antibody was positive and smooth muscle antibody was weakly positive. Lactate dehydrogenase and haptoglobin levels were within normal limits.
Liver ultrasound showed mild hepatic fatty infiltration without biliary obstruction or stones. Magnetic resonance cholangiopancreatography (MRCP) was negative for biliary or pancreatic ductal dilation. RPR returned positive with a reflex titer of 1:64. Treponema pallidum IgG was sent for confirmation and it was reactive. Liver biopsy demonstrated chronic hepatitis with normal hepatic architecture, Kupffer cell hyperplasia, hepatic cholestasis and ductal proliferation (). Iron stain was positive. Periodic acid-Schiff and Periodic acid-Schiff-diastase stains were negative for alpha 1 anti-trypsin granules. Warthin starry stain was negative. Immunochemical stain for Treponema pallidum revealed no organisms. These findings were suggestive of syphilitic hepatitis. Patient had a reported allergy of pruritus to penicillins in the past. The allergist was consulted and patient underwent a challenge with oral penicillin and tolerated it well, no reaction was noted. The patient was given a single dose treatment: penicillin G 2.4 million units intramuscular route (IM) once. He showed symptomatic improvement subsequently, including resolution of nausea/RUQ abdominal pain. He was discharged to be followed up outpatient. Follow-up visit in two weeks revealed improvement in serum biochemical tests with ALT of 45 U/L, AST of 39 U/L, ALP of 298 U/L, total bilirubin 1.7 mg/dL, and absence of abdominal pain.
|
pmc-6217886-1
|
A 36-year-old male presented to the outpatient department with a history of low backache for the past one year, associated with typical claudication symptoms, left-sided unilateral numbness, and paresthesia of the foot. He gave no history of radicular pain, but he complained of progressive reduction in claudication distance. He had experienced a sudden deterioration in gait along with urinary incontinence, following a trivial traumatic fall, one week before presentation. There was no history of constitutional features. On examination, he had a bilateral high-stepping gait due to foot drop. Symmetrical weakness of both the L4 and L5 roots (MRC grade 0/5) and partial weakness of the S1 root (MRC grade 3/5) was noted with nondermatomal sensory disturbances. Deep tendon reflexes of the lower limbs were absent bilaterally. Though anal tone was normal, saddle anesthesia was present. Postvoidal ultrasonogram of the urinary bladder revealed a residual urine volume of 250 ml, thus confirming a neurogenic bladder.
Plain radiography of the lumbar spine did not show any gross feature of instability and was inconclusive (). Magnetic resonance imaging (MRI) of the lumbar spine revealed a solitary intraspinal posterior epidural lesion of 1.8 × 1.5 × 0.5 cm at the L2-L3 level with heterogeneous signal intensities and adjacent epidural fatty hypertrophy contributing to severe canal stenosis (3 mm). The cauda equina was severely compressed and was almost not visible (). Owing to the heterogeneous signal intensities, computerized topography (CT) was performed which revealed the presence of an osseous lesion attached to the right L2 inferior articular process causing severe secondary canal stenosis (). Considering the recent-onset neurological deficit, the patient was advised surgical decompression and excision biopsy at the earliest. The patient was positioned prone on a Relton Hall frame under general anesthesia. A standard midline posterior approach was employed and L2 and L3 lamina were exposed. The spinous process was removed and using a motorized burr, a rectangular trough was created surrounding the lesion. The lamina was thinned out using a burr to avoid further insult to the dural sac, and then using a Kerrison ronguer, laminectomy was completed all around the lesion under microscopic guidance (). A small osteotome was used to remove the attachment on the right side which required partial removal of the facet joint. The lesion was then held, and the adherent soft tissues beneath the lesion were removed, resulting in en bloc removal and complete decompression of the cauda equina. Fusion was performed, as the procedure involved partial facet joint resection. The lesion was sent for histopathological examination. There were no adverse events or postoperative complications. The patient was mobilized with bilateral orthoses, and bladder training was initiated.
At 4 weeks, there was an improvement in his urinary symptoms, and by 12 weeks partial neurological recovery (MRC grade 3/5 motor power) in bilateral L4 and L5 was observed. The S1 root power increased by one grade (MRC grade 4/5). His gait improved thereafter, and he was able to return to his normal activities by 6 months with further neurological improvement by 1 grade in all roots.
The gross specimen measured 2 cm × 1.5 cm × 1.5 cm. It was greyish white in color and firm to hard in consistency (). It had a well-defined capsule and had the feel of particulate materials on cut sections. Histopathological examination revealed the presence of bone, cartilage and ligamentous tissue, and zones abutting all these composed of mature adult-type encapsulated adipose tissue (Figures and ). This confirmed the diagnosis of benign osteolipoma.
|
pmc-6218189-1
|
A seventy-year-old male, with atrial fibrillation and remote prostate cancer, presented with a left-sided foot drop, which had developed three weeks prior. He presented complaining of low back pain and radicular symptoms prominent in the left fifth lumbar (L5) nerve root territory, with pain in the anterolateral aspect of the distal leg and numbness along the dorsum of his foot. The physical examination was significant for a significant weakness in the left tibialis posterior, extensor hallucis longus, and anterior tibialis, presenting as a foot drop and a weakness in foot inversion. All other muscles were normal in strength, particularly the quadriceps, hip adductors, iliopsoas, and tibialis posterior. Sensory findings included decreased pin-prick detection in the L5 dermatome. Osteotendinous patellar and Achilles tendon reflexes were normal and present bilaterally. A spine exam was notable for normal alignment and range of motion with no spinal or paraspinal point tenderness. He had a markedly positive ipsilateral straight-leg raise test at under thirty degrees, no pain with hip or knee passive range of motion, no trochanteric sensitivity, and no Tinel’s sign at the fibular head.
Magnetic resonance imaging (MRI) findings revealed a large, left-sided paracentral extruded L2-3 disc with lateral recess and foraminal stenosis and mass effect on the ventral aspect of the thecal sac (Figures -). Other disc levels showed degenerative changes including minor lateral recess stenosis at L3-4 and, notably, a normal L4-5 disc (Figure ). Serology was unremarkable. Although the patient’s clinical presentation and imaging did not classically correlate, it was felt that the L2-L3 disc was the etiology of the patient’s L5 radiculopathy, and a left L2-3 microsurgical discectomy was performed. There were no complications postoperatively, and he had immediate pain relief. At the six-week follow-up, his foot drop was near normal, sensation was intact with minimal paresthesias, and he remained pain-free. At the one-year follow-up, he experienced full resolution of his foot drop and has regained full function and sensation and remains pain-free.
|
pmc-6218463-1
|
A 55-year-old female with a history of aggressive periodontitis was referred for full mouth rehabilitation. The remaining teeth had to be removed due to excessive mobility. The patient had been wearing complete dentures since the age of 25.
Despite the considerable amount of bone loss and unwillingness to undergo any major bone graft procedures, the patient demanded fixed prostheses.
Considering the presented situation, the All-on-4 protocol was selected for full mouth reconstruction. Four regular platform (RP) implants (Replace Select; Nobel Biocare, Göteborg, Sweden) with the diameter of 4.3 mm and the length of 13 mm were inserted in the canine and second premolar areas of each jaw, using the prescribed All-on-4 guide (All-on-4, Nobel Biocare, Göteborg, Sweden). After four months, the uncovery surgery was performed. Two weeks later, the open tray technique with splinted impression copings was used for the impression procedure. After trying in the screw-retained metal framework on multiunit abutments (Nobel Biocare, Göteborg, Sweden) in the mouth and evaluating their passive fit, they were returned to the laboratory for tooth set-up at the previously established vertical dimension of occlusion. After the delivery of the metal-resin (hybrid) prostheses and adjusting the occlusion (), the patient was followed for complications which mostly were chipping of the pink acrylic resin.
After seven years, the most distal implants on the left side of both jaws had to be removed due to bone loss. Since the patient demanded new and more aesthetically pleasing fixed prostheses, it was decided to replace the failed angulated implants with straight ones (10 mm in length) at a little more distal position ().
Since the crown height space in either jaw was more than 15 mm, the Toronto implant-supported prosthesis was chosen as the new treatment plan [,]. After impression making using the splinted open tray technique and establishing the vertical dimension of occlusion, multiunit abutments (Nobel Biocare) with proper gingival heights were used. Acrylic resin patterns (GC Corp., Tokyo, Japan) of the mesostructures were fabricated and tried in the mouth to verify the impression accuracy and the passive fit (). After casting the mesostructure patterns with nickel-chromium (Ni-Cr) metal alloy (Wiron 99, Bego, Bremen, Germany), they were tried in the mouth. However, despite confirming the accuracy of the acrylic resin patterns, the cutting and soldering of the mandibular metal mesostructure were necessary to achieve the desired passive fit (). As with the usual Toronto framework design, parallel individual abutments were made using acrylic pattern resin, and were then cast with metal ().
The modified ridge lap design was used for the tissue side of the frameworks to ensure esthetics and tissue health. Individual metal-ceramic crowns were fabricated using Ni-Cr metal alloy and porcelain (EX3, Kuraray Noritake Dental Inc., Okayama, Japan) with shades of A1 and A2 for cervical and incisal/occlusal halves, respectively. To mimic the gingival tissue color, a pink porcelain (EX3, Kuraray Noritake Dental Inc., Okayama, Japan) was applied to the mesostructures (). The crowns () were tried in the mouth, and the group function occlusion was used as the occlusal scheme. As the final stage, the screw abutments were fastened in the mouth to 35-newton centimeter (Ncm). Before cementing the crowns, the abutment screw holes were filled with Teflon tape (SITCO, Fujian, China).
Eugenol-free temporary cement (Temp-Bond® NE, Kerr Corp., Orange, CA, USA) was used for the crowns adjacent to the abutment screw holes [], while all the other crowns were cemented using zinc phosphate luting agent (Harvard; Harvard Dental International GmbH Margaretenstr, Hoppegarten, Germany; ). A maxillary night guard was also fabricated for the patient to reduce the risk of porcelain fracture by distributing the forces (). The patient was instructed to regularly perform oral hygiene measurements including using an interdental brush and super floss as well as the conventional brush and floss. The patient was asked to return for regular follow-ups which were every three months during the first year, and then every six months.
Twelve months after the delivery of the prostheses, chipping of the pink porcelain was observed in the mandibular anterior region (). Also, during the next six months, the same problem happened in the maxillary anterior region (). To recover the framework from the mouth, the crowns placed near the abutments’ access holes were removed using a crown remover instrument. After removing the remaining crowns from the mesostructure using the heat from a porcelain oven (Zirkonofen 700 Ultra-Vakuum, Zirkonzahn, Germany), the pink porcelain was applied to the defective parts, and the prostheses were delivered to the patient as described before.
|
pmc-6218697-1
|
A 39-year-old male patient presented to the emergency room due to pain and swelling in the dominant right wrist, following an in-car traffic accident. His physical examination revealed limited and painful movement in the right wrist and tenderness over the radial and ulnar styloids. No ne- urovascular deficits were detected. The patient did not have a history of any disease. A non-displa- ced radial styloid fracture and a minimally displaced fracture of the ulnar styloid were observed on the anteroposterior and lateral X-ray view of the wrist (). The patient underwent MRI in the emergency room, as a requisite of a M.D. thesis planned to be published later (). The radi- ology department of our hospital reported findings of “rupture and retraction in the APL tendon” in the MRI report. Based on these findings, the previously intended surgical treatment of the non-disp- laced fracture with closed fixation was switched to open reduction and fixation.
Following general anesthesia, the patient was operated via the longitudinal dorsal approach over the anatomical snuff box. A single EPB and two APL tendons were observed in the 1 st dorsal compartment, as reported in 70% of the cases in the literature []. Following gentle traction with a tendon hook, the musculotendinous avulsion was visualized on the proximal aspect of the tendons (). The radial styloid fracture of the patient was fixed using a cannulated compression sc- rew (Medartis®; Germany) (). The tendons were cut off the musculotendinous juncture and the tendon stumps were left free beneath the forearm fascia for a probable tendon transfer in the fu- ture. The patient was postoperatively followed with a palm-based thumb spica splint for three week and then hand and wrist exercises were performed to the extent the patient could tolerate. The pati- ent exhibited no limitation of movement or functional loss throughout the two-year follow-up peri-od.
The abductor and extensor muscle strengths of the operated and non-operated thumbs were objectively measured at the second year follow-up. Ethical approval had not been applied by authors for this study. The measurements of isometric muscle strength were performed using a digital hand dynamometer MicroFet 2 (Hoggan Health Industries, Draper, UT), used safely in numerous studies, and the muscle strengths were recorded in Newtons (N) () [, , ]. Patients were verbally encouraged and asked to exert full force on the dynamometer for 5–10 seconds. The process was repeated five times with two-minute intervals of rest[,,].
The paired sample t-test was used in comparing the extensor and abductor muscle strengths of both thumbs. The mean extensor force exerted by the affected thumb was 13.48+-0.36 N and 13.44+-0.36 N for the left thumb. The mean abductor force exerted by the affected thumb was 9.3+-
2.23 N and 12.22+-0.9 N for the left thumb. No statistically significant difference was found in the extensor and abductor muscle strengths between the two thumbs (p > 0.05).
|
pmc-6218698-1
|
A 25-year-old Mexican female with family history of ovarian cancer at her maternal side and personal history of a mass on her right breast clinically and radiologically diagnosed as fibroadenoma 2 years ago. The same mass had a growth of 2 cm so an incisional biopsy was performed at another institution. This was positive for mucinous carcinoma, and the patient was referred to our institution for treatment and follow up.
At our institution in the biopsy slides revision, we found a tumor composed of mucinous lakes with numerous tumor cells of medium to large size with a moderate amount of eosinophilic cytoplasm confirming the presence of pure mucinous carcinoma. We could not identify the tumor size and surgical margins by no prior radiological and pathological report. []. The tumor was classified as a Low grade tumor (G1) based on the Scarff Bloom Richardson scale. We did not observe perineural or vascular invasion. On immunohistochemistry the results were 100% and 90% positive for estrogen and progesterone receptors respectively with high intensity, and HER2 receptors negative.
On physical examination a surgical injury was seen. Upon palpation, this zone felt indurated, edematous and presented as a rough irregular texture. No other lesions were found. An ultrasound was performed, showing avascular distortion and a heterogeneous mass with microcysts (white arrows) []. MRI showed a hypointense irregular mass that indicates a mild enhancement in contrast sequences and a hyperintense sequence in SPAIR. The evaluation of the lymph nodes was not suspicious for metastasis.
With the radiological suspicious of residual tumor and pathological findings accomplished at our institution a partial mastectomy and a sentinel lymph node biopsy was performed. Pathological findings showed a residual mass of 25 mm of pure mucinous carcinoma with negative surgical margins. Sentinel lymph node was negative for metastasis.
Molecular screening with EndoPredict clinic showed a score of 2.7 classifying it as a low risk, reason why this patient was treated with tamoxifen because her positivity to receptors and low risk showed in molecular assay. The clinical and pathological stage IB was confirmed.
|
pmc-6218744-1
|
A 6-month-old male initially presented to his pediatrician to be evaluated for an episode of gross hematuria. The parents denied any history of fever, trauma, or any other associated symptoms. A renal ultrasound was performed which showed a right lower pole, predominantly solid, well-defined lesion with multiple small cystic components, measuring 3.2 x 1.8 x 2.6 cm, in addition to a small 2.1 mm linear calcification with shadowing (). An MRI of the abdomen was performed with and without intravenous contrast that showed a right lower pole lesion with multiple T2 cystic components, measuring 2.8 x 2.5 x 1.8 cm (). The cystic components were noted to be hypointense and nonenhancing on the postcontrast sequence with mild enhancement of the intervening septa.
Upon referral to our clinic, physical examination was unremarkable. The patient's abdomen was soft, nondistended, and nontender to palpation without any discernable palpable masses or hepatosplenomegaly. The patient's white blood cell count was 10,800/uL, hemoglobin was 12.0 g/dL, creatinine was 0.44 mg/dL, urinalysis was positive for blood, and urine culture was negative for infection. Hepatic function panel and electrolyte panel were within normal limits. The mass did not have hormonal function. A laparoscopic right radical nephrectomy was recommended.
The patient was taken to the operating room and was given intravenous antibiotics for prophylaxis. After general anesthesia was induced, an orogastric tube and urinary catheter were inserted. The patient was placed into a modified left lateral decubitus position with the right flank up (). Care was taken to pad all joints and the patient was secured to the operating table. A 5 mm port was placed at the umbilicus via open Hassan technique. Two other 5 mm working ports were placed under visualization in the left upper quadrant and subxiphoid. An additional left upper quadrant 5 mm port was placed for liver retraction.
First, the abdominal cavity was completely inspected. Next, the peritoneum was opened at the hepatic flexure outside of the colon. The colorenal ligaments were then incised over the kidney from the level of the liver down to the level of the inferior pole of the kidney. This allowed for complete reflection of the colon medially. We then began dissection inferiorly and medially and proceeded to skeletonize the ureter. The ureter was then tented up anteriorly and underneath the lower pole of the kidney. We proceeded to dissect out the renal hilum using a LigaSure™ device and suction. We skeletonized the hilum, which appeared to have a single artery and vein. We then created a plane in between the adrenal gland and the upper pole of the kidney. Using the LigaSure™ device, we took down the upper pole attachments, incising the hepatorenal ligaments all of the way to the lateral wall. The posterior attachments were also taken down using the LigaSure™ device. We proceeded to deploy a JustRight™ 5 mm device across the renal artery and vein en bloc.
The kidney was freed in its entirety. We then proceeded to divide the ureter with the LigaSure™ device. Under vision we placed a 12 mm trocar along the right border of our previously marked Pfannenstiel incision in order to deploy a 10 mm EndoCatch™ bag. We then extended our incision medially along the Pfannenstiel mark, allowing us to remove the specimen intact in the EndoCatch™ bag. All fascial defects were closed ().
Pathologic macroscopic analysis of the specimen revealed a 1.4 x 1.3 x 0.8 cm white, indurated mass, 0.3 cm from the renal capsule (). Microscopically the tumor was composed of an ossified core containing epithelioid cells with abundant cytoplasm surrounded by a spindle cell component with small, oval nuclei, and scattered mitoses (). The tumor appeared well-circumscribed based on submitted sections. Margins were negative for invasion. The spindle cells showed moderate nuclear positivity for WT-1. Both populations were negative for AE1/3, desmin, synaptophysin, and CD99. While differential diagnosis included a blastemal predominant Wilms Tumor and congenital mesoblastic nephroma, the tumor was determined to be most consistent with a rare ossifying renal tumor of infancy.
|
pmc-6218747-1
|
At initial presentation, the patient was a 57-year-old female with body mass index (BMI) of 25.6 kg/m2 and medical history including controlled hypertension and asthma. Past surgical history consisted of three unremarkable caesarean sections. Her chief complaint was that of progressive right hip pain and radiographs demonstrated advanced osteoarthritis (). After failing conservative measures, the patient underwent elective right total hip arthroplasty (). Her surgery was uncomplicated with 200 cc of estimated blood loss. The patient had a preoperative hemoglobin of 13.5 g/dL and 13.3 g/dL on postoperative day one. Postoperative platelet count was also within normal limits at 231,000/mcL. She had an unremarkable hospital course and was discharged on postoperative day one.
The patient presented to the emergency room seven days after discharge with significant fatigue and shortness of breath with minimal exertion over the past day. She reported feeling nauseous with several vomiting episodes. Her husband also described one episode of confusion and word finding difficulty the previous day. What particularly alarmed the patient was the development of a yellowish appearance of her skin and eyes. The patient denied any complaints of her right hip.
Physical examination revealed a well-healing right hip surgical incision without evidence of hematoma, erythema, or drainage. She was noted to have scleral icterus, jaundiced palms, and scattered petechiae and purpura throughout her bilateral arms. Labwork demonstrated severe thrombocytopenia with a platelet count of 6,000/mcL and microcytic anemia with a hemoglobin of 5.4 g/dL and mean corpuscular volume (MCV) of 76 fL. Schistocytes were observed on peripheral blood smear. The hematology service was consulted given these abnormalities and high suspicion for TTP. The patient was transferred to the medical intensive care unit, and emergent plasmapheresis and steroids were initiated. Three units of packed red blood cells were transfused with appropriate rise in hemoglobin. The patient was found to have ADAMST13 activity of <3% (ref. range 68–163%) and positive ADAMST13 inhibitor titer confirming the diagnosis of TTP. A total of six plasmapheresis treatments were performed over the subsequent seven days. Following plasmapheresis treatment, the patient's platelet count steadily rose to a level of 468,000/mcL on the sixth day after readmission. Platelet transfusion was held as she demonstrated no clinical evidence of active bleeding during hospitalization. On subsequent days after initiation of treatment, the patient reported feeling much improved. Throughout her presentation, the patient maintained adequate urine output with creatinine level within normal limits suggesting no renal dysfunction. Laboratory values significantly improved (), and she was discharged on day six after readmission. At subsequent clinic follow-ups, the patient has been found to be doing very well with good ambulatory status, healed surgical wounds, and hematologic values within normal limits.
|
pmc-6218748-1
|
A 60-year-old postmenopausal African woman presented to the emergency department with a 6-month history of progressive bilateral breast pain and mass associated with itchiness. There was no history of fever, chills, discharge, or trauma and no previous breast surgery. Family history was negative for breast cancer. The patient had a history of diabetes mellitus, hypertension, and cardiomyopathy.
On presentation, the patient was alert and oriented, with a temperature of 37°C, a pulse of 110/min, and blood pressure of 110/70 mmHg. Breast examination revealed a bilateral 7.5∗6 cm hard, fixed mass in the periareolar area with erythema and peau d'orange without discharges or palpable axillary lymph node. The rest of the examination was within normal.
A mammogram revealed bilateral diffused skin thickening edematous parenchyma with vascular calcification (Figure ) and 1.4∗0.8 cm hypoechoic lobulated irregular mass at the right breast (BIRADS 3) (Figure ). Bilateral core biopsies from both masses were taken.
The histopathology result showed necrotic acutely inflamed fibrofatty tissue ().
On follow-up, i.e., one week later, the patient presented with bilateral malodorous breast discharge at the biopsy site. On physical examination, both RT and LT breasts showed necrotic tissue with pus discharge and no crepitus and with palpable apical axillary lymph nodes.
Her laboratory results revealed leukocytes of 10.85∗10 mg/dL and elevated glucose of 148 mg/dL. She started on intravenous ceftriaxone and was taken to the operating theater for bilateral debridement and incisional biopsy as inflammatory breast cancer was suspected. Microscopic examination of specimens showed necrotic fibrofatty mammary tissue and foci of chronic inflammation. Two weeks later, the patient continued to have a nonhealing ulcer with foul-smelling discharge and expanding necrotic tissue. NF was suspected and the patient underwent bilateral simple mastectomy with primary wound closure by a stapler.
The histopathological examination of the specimens revealed an extensive cutaneous necrosis involving the epidermis, dermis, and subcutaneous fat with thrombus and necrosis of blood vessels () constant with necrotizing fasciitis. Postoperatively, she had an uneventful recovery and was discharged home after 3 days. Follow-up visits were arranged, and the patient was found to be completely healthy with a well-healed wound.
|
pmc-6218749-1
|
A 58-year-old Caucasian male presented with progressively decreasing central vision in both eyes over the past five years. He also complained of mild night blindness. There was no significant medical history and no family history of retinal disease. Best-corrected visual acuity was 20/60 in the right eye and 20/200 in the left eye with mild nuclear sclerotic cataracts. Fundoscopic examination revealed large areas of macular atrophy centered on the fovea surrounded by reticular pseudodrusen. Peripapillary atrophy was also present along with scattered areas of peripheral pavingstone degeneration OU (Figures and ). There was no intraocular inflammation and a recent electroretinogram (ERG) was within normal limits. Fundus autofluorescence (FAF) showed well-defined areas of atrophy OU and sparing of the central fovea in the right eye (Figures and ). Spectral domain OCT (SDOCT) (Heidelberg Spectralis OCT, Heidelberg, Germany) revealed outer retinal and choriocapillaris atrophy and reticular pseudodrusen (Figures and ). OCTA (Angioplex, Carl Zeiss Meditec, Dublin, CA) demonstrated marked absence of choriocapillaris flow (Figures and ). The retinal vasculature was mildly attenuated on OCTA as well. These vascular changes were not readily apparent on fluorescein angiography. Genetic testing for the A3243G and C1QTNF5 mutations was negative.
|
pmc-6218751-1
|
An 18-year-old male, nursing home resident, presented to the hospital with labored breathing and acute respiratory failure. The patient at baseline was nonverbal with a Glasgow coma scale (GCS) of 10/15. Approximately a year prior to presentation, the patient had suffered from traumatic brain injury after a fall. He underwent large left-sided craniectomy with a bone flap and placement of VP shunt at an outside hospital at the time. At initial presentation to our hospital, the patient was started on broad-spectrum intravenous antibiotics to cover for pneumonia. On day 3, the patient was noted to have sinus bradycardia with heart rate in the 40 s and low blood pressure at 90/60. On examination, the patient had a GCS of 8/15 with unequal pupils. Emergent CT head revealed sinking skin flap syndrome with paradoxical brain herniation and 19.9 mm midline shift (). The patient was placed in Trendelenburg position and transferred to the intensive care unit for close monitoring. An urgent neurosurgery consult was also obtained, and cranioplasty was advised but the family deferred. The patient's VP shunt was adjusted to increase the intracranial pressure. A repeat CT scan after 5 days revealed stable midline shift with no interval changes and improvement in mental status ().
|
pmc-6218752-1
|
A 68-year-old male with past medical history significant of end-stage renal disease on hemodialysis, hypertension, hyperlipidemia, diabetes mellitus type 2, cirrhosis, hepatitis C, chronic obstructive pulmonary disease, and benign prostate hyperplasia presented to the emergency department with a 2-week progressive dyspnea. In addition, the patient complained of generalized weakness, subjective fevers, and abdominal pain in the right upper quadrant. The initial blood tests showed a normal complete blood count, no renal function abnormalities, and no electrolyte abnormalities and aspartate transaminase (AST) of 19 IU/L, alanine transaminase (ALT) of 5 UI/L, albumin of 2.9 g/dL, total bilirubin of 1.4 mg/dL, and prothrombin time of 15.6 seconds. Hepatitis B surface antigen and human immunodeficiency virus (HIV) 1 and 2 antibodies were negative. CXR was obtained and revealed a large left pleural effusion. Diagnostic thoracentesis revealed an exudative pleural fluid per Light's criteria and negative cytology. Ascites was noted on physical exam, and the patient underwent a diagnostic paracentesis that revealed a serum ascites albumin gradient of <1.1, polymorphonuclear cell count <250/mm3, and negative culture. Repeat paracentesis was performed to rule out malignancy or tuberculosis as the cause of ascites. No malignant cells were found, adenosine deaminase (ADA) activity was 3.4 IU/L, and Ziehl–Neelsen stain, Lowenstein–Jensen cultures, and polymerase chain reaction (PCR) amplification were negative for mycobacteria. Decision for abdominal computed tomography was made, which revealed peritoneal carcinomatosis with multiple subcentimeter lesions in the liver, spleen, and adrenal glands. Interventional radiology was consulted, and an omental biopsy was obtained which showed necrotizing granulomatous inflammation with rare acid-fast bacilli (AFB). Repeat biopsy was done for tissue culture, and this was positive for Mycobacterium tuberculosis complex. He was started on treatment with rifampin, isoniazid, pyrazinamide, and ethambutol immediately after biopsy, based on very low resistance rates of M. tuberculosis complex in our area. This patient's mycobacterial isolate was sent to the local Department of Health laboratory, to confirm antimicrobial susceptibility. The patient's AFB sputum cultures have remained negative. The patient's systemic symptoms improved days after treatment was started.
|
pmc-6218753-1
|
A 71-year-old man, originating from Vietnam, was referred to a tertiary care endocrine surgery center with incidentally found severe hypercalcemia of 3.65 (reference range, 2.20–2.62) mmol/L during routine follow-up for atrial fibrillation. His only symptoms of hypercalcemia on further questioning were fatigue and polyuria. The patient's history included the left leg dystrophy due to childhood polio, hypertension, atrial fibrillation, chronic kidney failure (stage IIIB), and dyslipidemia. He had no family history of parathyroid or other endocrine disease, and he had no prior neck irradiation.
On physical examination, a palpable mass was noted on the right lower neck without associated palpable lymphadenopathy. Flexible nasolaryngoscopy showed normal vocal cord movement, suggesting no invasion into the recurrent laryngeal nerve. Laboratory results showed markedly elevated levels of calcium at 3.3 mmol/L, PTH at 271 (1.4–7.6) pmol/L, alkaline phosphatase (ALP) at 446 (40–150) U/L, and phosphate of 1.06 (0.8–1.4) mmol/L. Ultrasound of the neck showed a complex, predominantly cystic nodule with solid components inferior of the right thyroid lobe measuring 4.1 × 3.6 × 3.1 cm ().
Sestamibi scan ((99m)Tc) displayed a dominant right-sided neck lesion with minimal uptake of technetium. Additionally, two well-defined lucent bone lesions, located in the right clavicle and scapula, showed increased tracer uptake (). Subsequent CT scans of the neck, thorax, and abdomen and a bone scintigraphy confirmed the presence of two previously defined osseous lesions along with two additional lucent and sclerotic lesions in the sternum and 7th rib. Differential diagnosis of the bone lesions included both metastasis as well as brown tumors secondary to long-standing hypercalcemia related to hyperparathyroidism. We did not evaluate the lesion with FNA preoperatively.
Due to the clinical suspicion of parathyroid cancer, en bloc parathyroidectomy with the right thyroid lobectomy and ipsilateral level VI lymphadenectomy was planned. Rivaroxaban, a direct Xa inhibiting anticoagulant indicated for atrial fibrillation, was stopped 5 days prior to surgery. The evening before the scheduled surgery, the patient presented to the emergency department with symptomatic hypocalcemia including perioral, finger, and toe numbness. Laboratory results showed profound hypocalcemia with a corrected calcium level of 1.85 (2.32–2.62) mmol/L, phosphate of 0.61 (0.8–1.4) mmol/L, and a decrease in PTH level to 87.5 (1.4–7.6) pmol/L ().
Repeated ultrasound showed further avascular solid components of this parathyroid mass but no changes in size of the lesion. Parathyroid tumor autoinfarction was suspected with concomitant hungry bone syndrome. The surgery was postponed, and the patient was treated with high-dose calcium supplementation and activated vitamin D. After eucalcemia was achieved, the originally planned surgical intervention was performed.
Rapid intraoperative PTH assays showed a significant drop of PTH after the removal of this mass (baseline intraoperative PTH, 134 pmol/L; 10-minute sample, 13.8 pmol/L). During the surgery, we used intraoperative nerve monitoring (IONM) for assessment of the right recurrent laryngeal nerve. The nerve signal was not lost throughout the operative dissection, and there were no adhesions to the recurrent laryngeal nerve from the parathyroid lesion. Postoperative movement of vocal cords was normal, calcium levels remained stable, and the patient was discharged 1 day after surgery.
The right hemithyroidectomy specimen identified a 4.0 cm mass located at the right perithyroidal region. The mass was an infarcted enlarged cellular parathyroid gland with cystic degeneration (). There was no evidence of angioinvasion or malignant invasive growth to suggest the morphological diagnosis of parathyroid carcinoma. Given the clinical suspicion for malignancy and underlying infarction, this lesion was further assessed with immunohistochemical biomarkers () [–]. The lesion was positive for PTH (confirming the parathyroid origin) and was negative for galectin-3 and PGP9.5. There was no loss of expression for parafibromin, Rb, p27, and bcl-2. The MIB-1 labeling index was 1%. The mitotic activity was 0.6 per 10 high-power fields (based on 3 per 50 high-power fields). The immunoprofile also did not suggest malignancy. While no atrophic rim of parathyroid tissue was identified, submitted additional parathyroid tissue from the right neck and significant drop of intraoperative PTH were suggestive of a single gland disease, consistent with an infarcted parathyroid adenoma [].
At the 6-month follow-up, calcium and PTH levels remained improved and no persistence or recurrence of disease was suspected.
|
pmc-6218754-1
|
A 37-year-old female presented to our clinic with restricted movement of the left elbow for a duration of 9 months after history of trauma. The patient was previously treated by the local bonesetter. On physical examination, the elbow was stiff in 40 degrees of flexion. Disruption of the three-point bony relationship between the olecranon tip and medial and lateral epicondyles was revealed. The olecranon tip was prominent posteriorly with tenting of the triceps tendon. No neurologic deficit was observed. The X-rays showed the posterior elbow dislocation with displaced radial head fracture (). The CT scan demonstrated the irreparable radial head fracture with indented articular surface as well as the deformation of the fracture ends. A tip fracture of the coronoid process (Reagan-Morrey type I) was noted ().
The surgical treatment with open reduction of the elbow with radial head replacement was planned. In this study, the custom-made anatomical radial head prosthesis was chosen over the commercial radial head prosthesis due to limited availability of the commercial prosthesis in our country. In order to fabricate the patient-specific prosthesis, a high-resolution CT scanning (Philips Brilliance 64 CT scanner, Cleveland, OH; voxel size 0.45 × 0.45 × 0.45 mm, 120 kV, 150 mAs, pitch 0.6) of both elbows was performed and reconstructed into 3D images. Subsequently, 3D image of the affected side was aligned with the mirrored 3D image of the contralateral bone by registration of the radial tuberosity and diaphysis to identify the correct anatomic profile of the reconstructed part (). The aligned 3D images were then processed through image processing techniques and computer-aided design (CAD) to construct a 3D prosthesis model. With this technique, accurate preoperative planning for the position of additional bone resection and extent of radial neck restoration can be established (). The stem configuration of the prosthesis was designed in conforming to the alignment of the intramedullary canal while the stem length was determined to achieve a cantilever quotient of 0.5. To aid in filling of bone cement, a free space of 1 mm between the prosthesis stem and the bone was also maintained.
Once the reconstruction of computerized radial head prosthesis was completed, a STL file was generated for fabrication of resin-customized implant using the stereolithography (SLA) technique. This 3D-printed resin model was directly used as a master pattern for investment casting. The resin model was first embedded with a high-temperature resistance ceramic. Subsequently, it was heated up to a temperature range of 450°C–1000°C to obtain a ceramic mold for titanium casting. Finally, the customized titanium implant was produced by investment casting in a clean environment. The hand polishing was performed to smooth the prosthetic surface. The roughness of the final polished prosthesis is in a range of 20 ± 10 micrometers. The complete manufacture of the prosthesis from the original CT took a total of 10 days. Note that the impressed mark in line with the radial tuberosity was specifically designed on the surface of the prosthesis to guide for the rotation alignment (). The 3D resin models of the proximal radius representing the cutting line were also fabricated using the 3D printing technique for intraoperative guidance ().
The patient was operated under general anesthesia and was placed on the ordinary surgical table in supine. The injured arm rested on the support. A tourniquet was placed proximally on the arm.
The incision was made on the posterior aspect of the elbow, beginning at the midline 7 cm proximal to the olecranon, curve the incision laterally around the olecranon and continue farther distally along the line of proximal ulna for 7 cm. The ulnar nerve and posterior interosseous nerve were identified and isolated with elastic sling. The shortened triceps bound down by fibrous tissue to the humerus was incised and lengthened using a Speed V-Y muscleplasty technique []. The contracted capsule and collateral ligaments were cut. Dense fibrous tissue filled up the olecranon; the coronoid fossae was carefully excised to avoid peeling off of the underlying cartilage. A large articular bone defect on the medial trochlea was observed. The radial head fragment and the fracture ends were exposed. Severe cartilage damage of the radial head with the metaphyseal bone loss was noted. The radial neck was resected with the microsagittal saw according to the preoperative planning. Cancellous bone in the intramedullary canal was removed using the bony curette, and the canal was irrigated with saline solution. A bone chip harvested from the radial head had been inserted into the canal as a cement restrictor. An acrylic cement (Palacos® radiopaque bone cement 1 × 40 g Single, 40.8 g methyl acrylate copolymer, 20 ml methyl methacrylate monomer 0.5 g gentamicin, Zimmer Dover, OH) was mixed and applied into the canal. The radial head prosthesis was introduced into the canal with the impressed mark aligned with radial tuberosity until the prosthetic neck fully seated on the cutting cortex (). Excess cement was cleared from the prosthesis-bone junction. The collateral ligament and triceps aponeurosis were repaired. Concentric reduction of radioulnar, radiocapitellar, and ulnohumeral joints through the entire range of motion was assessed under clinical examination and fluoroscopy. Fixation of the coronoid fracture was not performed as the elbow stability though a functional range of motion was sufficiently restored with the radial head replacement and collateral ligament repair.
Postoperatively, the arm was immobilized in a posterior splint at 90°. Active-assisted range-of-motion exercises were initiated in 10 days after the surgery. We did not use any medications or irradiation as prophylaxis against heterotopic ossification.
At the latest follow-up, 24 months after surgery, the elbow extension was 28°, flexion was 145°, pronation was 80°, and supination was 90° (). Hand grip power of the injured side averaged 95.4% of the normal side (27.6 kg for injured arm and 28.3 for normal arm). The Mayo Elbow Performance Index (MEPI) increased from 20 points at the preoperative day to 85 points, and the patient-based Disabilities of the Arm, Shoulder, and Hand (DASH) was reduced from 88.33 points to 28.33 points. The patient was satisfied with the cosmetic and functional outcomes. The last follow-up X-rays demonstrated the concentric elbow joint, but the narrowing of the radiocapitellar joint space is noted. The radiolucency around the bone-cement interface was apparent, but no progression was seen compared with the X-rays done at 12 months postoperatively. The proximal bone resorption at the radial neck and capitellar osteopenia were observed ().
|
pmc-6218755-1
|
A 47-year-old African American male with poorly controlled diabetes mellitus type 1 presented with lower back pain intensifying over several weeks. The patient was born and raised in Ohio but moved away to join the army. While in the military, he was stationed in Germany and Korea. He had moved to Arizona approximately a year prior. The patient had complaints of six months of progressively worse chronic nonproductive cough, night sweats, and significant weight loss. He denied fever, headaches, confusion, gait abnormalities, new joint pain, and skin rashes. Physical examination was grossly unremarkable. The white blood cell (WBC) count was 7,800/μL. Chest X-ray revealed bilateral hilar lymph nodes along with likely granuloma at the right middle lobe. Computed tomography of the chest revealed subcentimeter bilateral pulmonary nodules and middle mediastinal and right hilar lymphadenopathy. He underwent bronchoalveolar lavage with transbronchial lymph node biopsy. The pathology was consistent with granulomatous inflammation and fungal elements consistent with extensive coccidiosis. His cocci serology IDCF qualitative was positive, and the cocci IDCF titer was 8. His HIV test was negative. He was diagnosed with pulmonary coccidioidomycosis and started on oral fluconazole 400 mg daily.
The patient had returned to the Emergency Department complaining of worsening lower back pain over the past few weeks. He denied bowel or bladder incontinence and numbness. Physical examination revealed tenderness at the lower back. X-ray of the lumbosacral spine did not show osteomyelitis. Magnetic resonance imaging (MRI) of the lumbar spine without contrast revealed acute-to-subacute superior endplate compression deformity of the L3 vertebral body. Also noted was approximately 10–20% loss of the vertebral body height. No evidence of epidural abscess, spinal canal compromise, neural foraminal narrowing, or spinal cord compression was noted. CT-guided needle biopsy was performed confirming osteomyelitis due to C. immitis (Figures and ). Fluconazole was switched to itraconazole for better bone penetration. Unfortunately, the patient had significant weight gain and lower extremity swelling. Therefore, itraconazole was switched to posaconazole. He subsequently had gradual improvement of lower extremity swelling and weight loss. Subsequent MRI of the lumbar spine with and without contrast revealed T2 hyperintensity within the disc extending into the inferior endplate of L2 and superior endplate of L3. Findings were related to discitis and osteomyelitis. There was also a compression fracture of the superior endplate of L3 with approximately 50% height loss. Finally, there was a mass-like ventral epidural enhancement to the left of the midline with associated mass effect on the thecal sac related to developing an epidural abscess (). Neurosurgery was consulted, and the patient underwent L2-L3 laminectomy, L2-L4 posterior spinal fusion, and evacuation of the epidural abscess. The pathology report also confirmed to be positive for Coccidioides spherules (Figures and ). He underwent retreatment with intravenous liposomal amphotericin B (AmBisome) 5 mg/kg of ideal weight for several weeks along with continuing oral posaconazole for life. His repeat cocci IDCF titers decreased from 8 to 2. The patient denied axial pain or radicular pain. His spine remained stable, and he was neurologically intact.
|
pmc-6218756-1
|
A 64-year-old African American man, resident of a correctional facility, was transferred from an outside hospital for further management of liver failure. He was initially admitted at an outside hospital when routine blood tests showed leukocyte count 24,000/µL, platelet count 123,000/µL, serum sodium level 127 meq/L, aspartate aminotransferase (AST) 169 U/L, alanine aminotransferase (ALT) 116 U/L, alkaline phosphatase (ALP) 230 U/L, total bilirubin 17.7 mg/dL, and direct bilirubin 13 mg/dL. His past medical and surgical history was significant for decompensated alcohol-induced cirrhosis with untreated chronic hepatitis C; recurrent ascites; hypertension; schizophrenia; cholecystectomy; and appendectomy. He is a current smoker (40 pack-year) but quit drinking alcohol and using intravenous drugs 6 years ago. The patient complained of mild diffuse abdominal pain with several episodes of watery, nonbloody, nonfoul-smelling diarrhea. He denied fevers, chills, vomiting, melena, or hematochezia. He also reported an unintentional weight loss of 18 pounds over the last 6 months. The rest of the review of the system was negative.
On examination, he was alert and oriented, with temperature 37.3 degrees Celsius, pulse 113/min, blood pressure 103/71 mmHg, respiratory rate 18/min, and saturation 96% on room air. He had icteric sclera. Heart and lung exam was normal. Abdomen was mildly distended, nontender, and tympanic with no shifting dullness. Extremities were warm, with left foot and leg-pitting edema and severe tenderness without erythema. On direct questioning, the patient recalled hurting his left leg 8 days prior to admission while removing his boots, followed 3 days later by swelling, pain in the left foot and ankle that progressed to the leg. No skin lesions or wounds were noted.
His admission labs were significant for a leukocyte count of 14,700/µL, hemoglobin 12.1 g/dL, platelet count 137,000/µL, sedimentation rate 20 mm/hr, C-reactive protein 16mg/dL (normal range 0–0.5 mg/dl), AST 161 U/L, ALT 99 U/L, ALP 177 U/L, total bilirubin 15 mg/dL, direct bilirubin 10.7 mg/dL, albumin 1.5 g/dL, and INR 1.54. HIV antibodies were negative. Hepatitis C virus (HCV) RNA was 184,000 copies/mL.
Six hours after admission, the patient developed worsening tachycardia (125/min) with hypotension. He was given 2 liters of intravenous normal saline boluses and started on vancomycin and cefepime empirically. Blood cultures grew a mucoid strain of K. pneumoniae, in both aerobic and anaerobic cultures within 8 hours of collection, resistant only to ampicillin. Vancomycin was discontinued, and further serotype testing was not performed at that time.
A diagnostic paracentesis revealed ascites leukocyte count 358/mL with 66% neutrophils, and peritoneal fluid cultures were negative. A transthoracic echocardiogram was normal. A CT scan of the chest was done showing mild lower lung atelectasis. A triple-phase CT scan of the liver showed an enlarged left hepatic lobe with a shrunken right lobe with cirrhotic morphology, a small right hepatic lobe cyst, and moderate ascites. Magnetic resonance imaging of the left foot and leg without contrast revealed intraosseous abscess in the second metatarsal and marrow edema within the cuneiforms and second through fourth metatarsal bases suggestive of osteomyelitis (Figures –). There was extensive edema within the subcutaneous soft tissues of the leg and dorsum of the foot, with infiltration of subcutaneous fat compatible with cellulitis (Figures and ).
The patient's hospital course was complicated by encephalopathy requiring transient intubation for airway protection and acute kidney injury secondary to sepsis and hepatorenal syndrome requiring hemodialysis. The patient sepsis and bacteremia resolved; his mental status improved but remained in renal failure. He was discharged to a correctional facility to complete 8 weeks of intravenous ceftazidime.
|
pmc-6218791-1
|
We present the case of a 40-year-old woman with a history of systemic sclerosis, diagnosed 3 years ago. She arrived without treatment, due to a poor toleration of the medication metrotexate. She requested medical help in different opportunities for unspecific symptoms for 3 months including; nausea, vomiting, dizziness, asthenia and loss of weight. She didn't demonstrate any improvement and arrived with an uncertain diagnosis. Our institution observed symptoms, showing a decline in her renal function (creatinine 1,6 mg/dl and Uremic nitrogen blood BUN 41,3), with a urine test showing hematuria 28 xc, associated with hypertension 214/140 mmHg. Initially she was treated for an infection, showing rapid renal deterioration to creatinine 6,67 mg/dl and BUN 96,77 mg/dl with oliguria and overload. We started conventional treatment with IECA and calcium channel blockers, the patient showed no response, on the contrary her renal functiondeclined to the point that RRT was needed (). At the same time, the patient developed a deep thrombocytopenia and anemia, showing smear schistocytes in the blood, elevated Lactate Dehydrogenase (LDH), and consumption of haptoglobin (Figures -.) We ruled out Thrombotic Thrombocytopenic Purpura (TTP) because ADAMST 13 was normal; we also dismissed different immunologic disease. Kidney histopathology showed Thrombotic Microangiopathy (TMA), therefore we started plasma exchange getting slight improvement. Our patient showed a dramatic decline in renal, hematologic, and cardiac functions, therefore we decided to initiate treatment using C5 blocker with previous vaccination against encapsulated bacteria, resulting in an improvement in platelet count and red cells within the first week. 6 months later the patient showed full renal recovery and as a result RRT treatment was no longer needed.
Given the dramatic morbidity and mortality of this disease, in particular in the context of our incomplete understanding of its roots, we believe that to present this case may be relevant as it shows a successful outcome that may lead to new ways to approach the research needed to develop more suitable methods and treatments.
|
pmc-6218792-1
|
An 85-year-old man visited our hospital for treatment of sustained ventricular tachycardia (VT), which caused an unstable hemodynamic state. His medical history was significant for acute myocardial infarction (AMI) 2 weeks prior, and a drug-eluting stent was implanted in the left main trunk. On admission, the patient was asymptomatic. His vital signs were as follows: blood pressure, 144/78 mm Hg; pulse rate, 80 beats/min; respiratory rate, 16 breaths/min. His oxygen saturation was 99% in room air, whereas his laboratory test showed the following results: sodium, 140 mmol/L; potassium, 4.5 mmol/L; creatine kinase, 28 U/L; creatine kinase-muscle/brain, 9 U/L. Electrocardiography showed sinus rhythm with T-wave inversions in the I, aVL, and V1-V6 leads. Chest radiography revealed mild widening of the cardiac silhouette and right pleural effusion (). After the admission, he presented with sustained VT. Intravenous amiodarone was administered and CPR was performed, but amiodarone was ineffective in terminating VT. Hence, emergency coronary angiography (CAG) was performed for suspected myocardial ischemia. Intra-aortic balloon pumping and percutaneous cardiopulmonary support were established before emergency CAG because of his unstable hemodynamic state. CAG revealed 90% stenosis at the middle segment of the left descending artery, thus, ad hoc percutaneous coronary intervention (PCI) was performed. During the PCI, his serum hemoglobin level decreased from 11.1 to 3.0 g/dL. Chest radiography revealed pleural effusion in the right lung, which was not present on admission. Noncontrast CT was performed after emergency PCI, which revealed right hemothorax without any chest wall fractures. A right chest tube was placed, and 1.8 L of pleural effusion was drained over 6 hours. Despite blood transfusion of 12 units of red blood cells and 12 units of fresh frozen plasma, his serum hemoglobin level decreased, suggesting persistent blood loss. To identify the source of the bleeding, contrast CT was performed, which revealed small anterior mediastinal hematoma and bloody pleural effusion (). Three-dimensional contrast CT demonstrated an extravasation at the branch of the right IMA (Figures and ). The patient's deteriorating hemodynamic condition precluded thoracotomy or embolization to stop the bleeding. He subsequently died on the next day of hospitalization.
|
pmc-6218800-1
|
A 52-year-old man presented with upper abdominal pain for more than 3 months and weight loss of 3 kg in 10 days in July 2015. His performance status was 1 according to the criteria of the Eastern Cooperative Oncology Group (ECOG). The blood count analysis results were as follows: white blood cell (WBC) 23.99 × 10∧9/L, neutrophil (NEUT) 86.1%, and absolute neutrophil count (ANC) 20.64 × 10∧9/L. The tumor marker test results were as follows: serum carcinoembryonic antigen (CEA) 177 ng/dl, carbohydrate antigen-724 (CA-724) 20.34 IU/ml, and carbohydrate antigen-153 (CA-153) 31.59 IU/ml. Bone marrow biopsy of the granulocyte series demonstrated obvious active hyperplasia, and megakaryocytic and erythrocytic series were active and proliferous. There was no evidence of bone marrow metastasis.
Upper gastrointestinal endoscopic findings showed 3 cm × 2 cm ulcers at the anterior wall of the lesser antral curvature with no apparent active bleeding (Figures and ). Histopathological examination revealed a well differentiated tubular adenocarcinoma (). An abdominal computed tomography (CT) scan demonstrated irregular wall thickening on the lesser curvature side of the gastric antrum with mass formation with a large ulceroinfiltration as well as multiple metastases to neighboring lymph nodes (Figures –). The CT scan also revealed metastatic lesions in liver segment 4 () and evidence of hypodense liver metastatic lesions in the left lobe of the liver (). The CT scan also showed multiple liver metastases lesions that ranged in size from 1.0 to 4.3 cm over the entire liver (Figures and ).
A clinical diagnosis of stage IV (cT3NxM1) advanced gastric cancer was made according to the 7th American Joint Committee on Cancer (AJCC) system. Trastuzumab plus cisplatin-based chemotherapy has been recommended as the first-line standard treatment regimen for the patients with HER2-positive advanced gastric cancer according to the 2015.V3 gastric cancer guidelines of the National Comprehensive Cancer Network (NCCN). Since the result of HER2 status testing in this case was negative, it was not necessary to use the drugs targeting HER2, such as trastuzumab, for this patient.
On the basis of the abovementioned findings, we administered EOX combination chemotherapy. For each cycle, intravenous infusion epirubicin (50 mg/m2) was administered on day 1, followed by an intravenous drip of oxaliplatin (130 mg/m2) for 2 hours on day 1. Oral capecitabine (625 mg/m2) was administered twice daily for 3 weeks. This regimen was repeated every 3 weeks.
In October 2015, after completion of three cycles of chemotherapy, an abdominal CT scan showed that the mass of the gastric antrum had decreased to less than 3.3 cm (Figures and ), and metastatic lesions of neighboring lymph nodes that had been previously observed had disappeared (). The scan showed that the liver metastatic lesions had almost disappeared, with the exception of lesions in the left lobe of the liver, which measured less than 1.5 cm in size (Figures and ). After an additional three cycles of chemotherapy, another abdominal CT scan was performed in December 2015. In December 2015, after completion of six cycles of chemotherapy, endoscopic findings showed improvement in the gastric antrum mass. This result showed that the liver metastatic nodules that had been previously observed were no longer present (Figures and ). The scan showed that the gastric mass had almost disappeared (). Follow-up endoscopy showed that the ulcer lesion had disappeared and was replaced by scar tissue ().
Radical distal gastrectomy with Roux-en-Y with a residual stomach and jejunum anastomosis, as well as a D2 lymphadenectomy, combined with obvious metastatic liver lesions resection was performed in January 2016, resulting in complete removal of the primary tumor and any suspicious lymph nodes. Pathological findings after surgery showed no tumor cells detected in the gastric primary lesion. Metastases to perigastric lymph nodes were observed in none of 18 resected lymph nodes, suggesting pathological complete remission. The final pathological stage was ypT0N0M0. Following the operation, we planned to administer postoperative adjuvant chemotherapy with another two cycles of EOX regimen again.
The side effects and toxicities were evaluated every regimen cycle. During EOX treatment, the patient presented some adverse events such as mild gastrointestinal reaction, grade 1 neuritis, and grades 1-2 hematological toxicities that were considered tolerable. Mild gastrointestinal reactions, including grade 1-2 nausea and vomiting, were the most common EOX-related toxic effects.
Every 3 months, an abdominal CT scan was performed. There was no evidence of recurrent tumor up to February 2018. The patient had maintained CR for more than 27 months after surgery, with a 33-month overall survival (Figures and ). The patient still had no tumor recurrence up to the time of this case history article submission.
|
pmc-6218875-1
|
A 55-year-old woman, with HIV/HCV (genotype 3a) coinfection diagnosed in 1991, and previous intravenous drug user, has been followed by our outpatient department since 1998. The patient had been treated with antiretrovirals since 1991, with a good immunovirologic response. In 2005, she received anti-HCV therapy with pegIFN and ribavirin for 24 weeks, but the HCV infection relapsed after treatment discontinuation. Hereafter, she regularly followed her antiretroviral therapy and had an HIV-RNA load persistently < 50 copies/mL and stable clinical condition. Despite this, in summer 2007, she developed two consecutive episodes of acute kidney injury (AKI) associated with cholestatic acute hepatitis. At the time of her first hospital admission, her antiretroviral therapy (ART) with tenofovir disoproxil fumarate 245 mg q.d., lamivudine 300 mg q.d., and fosamprenavir 700 mg b.i.d. was stopped, in the suspicion of possible renal damage. Blood tests revealed acute kidney failure, with a serum creatinine level of 6.3 mg/dL associated with blood urea nitrogen 67 mg/dL and uric acid 10.6 mg/dL. Other notable results were hyperbilirubinemia (19.8 mg/dL, normal range 0.20 – 1.20 mg/dL), hepatic failure, with a coagulative deficit (prothrombin time 25), and metabolic acidosis. Anti-smooth-muscle-antibodies (ASMA) were positive (160 : 1), while antinuclear antibodies (ANA), antibodies directed against proteins that bind to nucleic acids (ENA), antibodies directed against phospholipids and antineutrophil cytoplasmic (ANCA), adrenal cortex antibodies (ACA), antimitochondrial antibodies (AMA), and anti-liver-kidney microsomal (ALKM) antibody were negative. Urinalysis showed proteinuria, hematuria, and pyuria. Abdomen ultrasound showed a liver enlargement without focal lesions, a modest ascites, and enlarged kidneys with an increment of arterials resistances. The patient’s management required hemodialysis, albumin, vitamin K, and human plasma. At the same time, the patient started methylprednisolone therapy (250 mg daily, then reduced to 40 mg daily). After clinical and chemistry improvement, the patient was discharged with oral steroid treatment (prednisone 25 mg daily), which was subsequently tapered. Three months later, after steroid discontinuation and ART still discontinued, a new onset of AKI (serum creatinine levels 6.0 mg/dL) required hospital admission. A kidney biopsy was performed showing tubule-interstitial nephritis and focal glomerulosclerosis (). Blood tests confirmed the presence of ASMA antibodies as well as negativity for ANA, ENA, p-ANA, c-ANCA, LKM (liver-kidney microsomal), ACA, and AMA. Then, methylprednisolone 40 mg daily was restarted with success (Serial serum creatinine levels are reported in ). At discharge, oral prednisone (37.5 mg daily) was prescribed, and it was tapered over a period of 4 weeks. In January 2008, considering the progressive improvement of renal function (creatinine 1.0 mg/dL), the consultant nephrologist suggested stopping steroid treatment and starting maintaining treatment with mycophenolate mofetil (MMF), 1,000 mg b.i.d. Following the starting of MMF, the patient exhibited negative ASMA antibodies.
She also restarted ART with abacavir 300 mg b.i.d., lamivudine 150 mg b.i.d., and lopinavir/ritonavir 400/100 b.i.d.. MMF was continued with good adherence and tolerance until November 2009, when, after 2 years of good clinical condition, it was discontinued. Four weeks after the suspension of MMF, the patient experienced a new deterioration of liver and renal function that required the reintroduction of MMF, at a dosage of 500 mg b.i.d., and new hospital admission (). In the following years, she continued ART, switching to darunavir/ritonavir (800/100 mg), etravirine 200 mg × 2, and maraviroc 150 mg × 2 and continued MMF. In June 2012, a new episode of cholestatic hepatitis developed, without renal involvement. A liver biopsy was obtained showing coexisting chronic HCV-related hepatitis and autoimmune hepatitis. Immunosuppressive therapy was enhanced with steroidal therapy (1 mg/kg), but she refused to continue. Thus, she was put on an increased dosage of MMF, of 1,000 mg b.i.d.. This therapy was continued in the following years with improvement of renal and hepatic parameters. In September 2015, the patient received anti-HCV treatment with sofosbuvir 400 mg q.d. plus daclatasvir 60 mg q.d. for 12 weeks without ribavirin. Before the starting of HCV treatment, maraviroc was stopped to avoid expected drug-drug interaction. Sustained virological response (SVR) was achieved, and, 3 months later, in agreement with the nephrologists, the patient halved the MMF dosage, and stopped it after other 12 weeks. Subsequent blood tests showed a permanent negativity of HCV-RNA load, with creatinine and hepatic enzymes persistently within the normal range (creatinine persistently < 0.95 mg/dL, and bilirubin < 1.20 mg/dL) and negative ASMA antibodies 12 months after MMF suspension. Since the patient no longer needed to keep an NRTI-sparing regimen, a single-tablet-regimen with rilpivirine/emtricitabine/tenofovir disoproxil fumarate was reintroduced.
|
pmc-6219025-1
|
A 55-year-old Japanese man who had returned from Nigeria was admitted to our hospital with a persistent high fever for 2 days. Blood smears revealed parasitemia with 0.05% of the erythrocytes containing ring forms (Fig. ). The polymerase chain reaction (PCR) detected P. falciparum. Upon presentation, he had dysarthria, expressive aphasia, urinary incontinence, and truncal ataxia; symptoms suggestive of cerebellar ataxia. He looked dazed, and he was unable to use appropriate words in conversation. Glasgow Coma Scale score was 13 (E4V4M5). He had no other signs or symptoms of severe malaria. Hematological investigations revealed a white blood cell count of 5.8 × 103 cells/μL, hemoglobin level of 18.8 g/dL, hematocrit level of 50.0%, and platelet count of 54 × 103/μL. The total bilirubin, aspartate transaminase, alanine transaminase, lactate dehydrogenase, creatine kinase, serum creatinine, and C-reactive protein levels were elevated to 2.1 mg/dL, 407 IU/L, 175 IU/L, 1354 IU/L, 1593 IU/L, 1.54 mg/dL, and 6.56 mg/dL, respectively. A serum test revealed hyponatremia with a sodium level of 123 mEq/L. A computed tomography (CT) scan of the brain did not show any remarkable findings. He had no past medical history, nor had he received any vaccination in the past 5 years. On day 1 of the illness, the patient was started on oral artemether/lumefantrine treatment 3 times every 8 h at home. Since the medication was past the expiration date, we restarted another course of artemether/lumefantrine upon admission. On day 3 of the illness (day 2 of hospitalization), the parasites were undetectable by a blood test, though the neurological symptoms persisted even after the patient became afebrile on day 4. MRI of the brain demonstrated a high-signal lesion in the splenium of the corpus callosum on diffusion-weighted images with a decreased apparent diffusion coefficient (Figs. –). Analysis of the cerebrospinal fluid upon recovery of the platelet count on day 10 revealed a slightly increased total cell count (6.3 cells/μL), and normal levels of protein and glucose. While renal function recovered by day 7, neurological symptoms gradually improved by day 12, and hyponatremia improved by day 21. Brain MRI on day 16 showed complete regression of the splenic lesion (Fig. ). The patient was diagnosed with mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) due to malaria.
|
pmc-6219057-1
|
A 3-year-old girl was referred to the neurogenetics clinic, National Research Center, Egypt because of the delayed milestones of development and unusual facies. She was the offspring of a non-consanguineous marriage with no similarly affected family members. The pregnancy and delivery histories were uneventful, however small head and dysmorphic facies were noted at birth. Delayed milestones and failure to gain weight were noted since early life. Seizures were developed at the age of 9 months as myoclonic and the focal seizures were fairly controlled on a combination of valproate and levetiracetam. Evaluation of the motor and mental developmental milestones was remarkably delayed; she could only sit supported, had impaired cognitive functions with obvious autistic features, had the inability to maintain holding objects, and didn’t acquire any speech skills. Her main anthropometric measurements revealed head circumference 40 cm (−6 SD), length 79 cm (−3.6 SD) and weight 7.200 kg (−3 SD). Clinical examination showed dysmorphic facies including, round face with full cheek, narrow forehead, thick bow shaped eyebrows, hypertelorism, long smooth philtrum, downturned corners of the mouth, low set ears, retro-micrognathia and short neck (Fig. : a and b). She had bilateral simian creases, vascular markings on the palm, tapering fingers, and clitoromegaly on genital assessment. Neurological evaluation showed hypotonia with elicited reflexes. Table shows a comparison of the main clinical presentation, involving cytobands, size of 1q deletion, smallest region of overlap (SRO) in the previously reported patients with pure 1q43q44 submicroscopic deletion and our patient.
Electroencephalogram showed frontotemporal epileptogenic focus. CT brain displayed abnormal gyral pattern, hypogenesis of corpus callosum and bilateral deep Sylvian fissure (Fig. ). Echocardiogram, fundus examination, abdominal and renal ultrasonography revealed no abnormalities. Psychomotor assessment using Stanford Binet International Scale method showed profound retardation.
This study was carried out in compliance with the Declaration of Helsinki and approved by the National Research Centre Ethical Research Committee.. Informed consent was obtained from the parents for genetic testing and publication of this case report.
|
pmc-6219087-1
|
In the summer of 2007, a 25-year-old Japanese woman (height 161 cm, body weight 80 kg, body mass index 30.8 kg/m2) felt weakness in both upper limbs and could not raise both arms while washing her face. Suspected neuromuscular symptoms included eyelid ptosis, trismus when gargling, and inability to stand due to weakness in lower extremities. She was immediately hospitalized in the internal medicine department at Kyoto University for further evaluation. No particular family and personal medical history were reported. Bilateral eyelid ptosis, masticatory muscle fatigue, and diffuse weakness of all limbs, denoting Osserman IIA classification, were noted on physical examination. A Tensilon test was positive, and the serum acetylcholine receptor antibody (anti-AChR) level was markedly elevated (32 nmol/L), which was strongly indicative of MG. Electromyography showed a waning phenomenon. In addition, a computed tomography (CT) scan revealed a thymoma. After evaluating these clinical findings, she was diagnosed as having MG. According to the Myasthenia Gravis Foundation of America (MGFA) scale, her muscular weakness was categorized as class IIIa. At the time of initial diagnosis, in July 2007, she did not prefer surgical resection of the thymoma, and received orally administered anti-cholinesterase inhibitor (pyridostigmine 60 mg/day). However, her symptoms did not improve. She was therefore given a corticosteroid (oral methylprednisolone up to 30 mg/day) in addition to the anti-cholinesterase inhibitor. Although symptoms of MG were initially relieved, at the beginning of the year 2010, clinical recurrence of MG was observed. The serum anti-AChR level was markedly elevated at 46 nmol/L. She was readmitted to our hospital with weakness of the extremities and worsened ptosis. She received systemic steroid pulse therapy, and the first course comprised 1000 mg of methylprednisolone administered intravenously for 3 days. Finally, in April 2010, she underwent thymectomy performed via a trans-sternal approach using video-assisted thoracoscopic surgery. During the perioperative period, she received intravenous corticosteroid pulse therapy three times, amounting to a total of 12,000 mg methylprednisolone equivalent. Myasthenic weakness improved MGFA IIa; administration of corticosteroid was gradually tapered to 20 mg/day as a maintenance dose. Her serum anti-AChR level was decreased to 11 nmol/L.
At the end of the year 2014, when she was 32-years old, sudden right hip arthritis occurred. She was referred to our orthopedic department during the next 2 months. We suspected a case of steroid-induced ONFH because she received repeated systemic corticosteroid pulse therapy for MG. There were abnormal signs in magnetic resonance imaging of her bilateral hip joints. Thus, she was diagnosed as having bilateral stage 3A, type C (type C1 in right hip, type C2 in left hip) ONFH (Fig. ) classified based on the 2001 revised Japanese Investigation Committee guidelines []. After bilateral ONFH diagnosis, she complained of continuous and hip joint pain on both sides, stronger on the left than on the right. Therefore, she was scheduled for a left primary THA initially. In October 2015, an elective left THA was performed via an anterolateral approach with our patient in a lateral position using the cementless THA system (R3 acetabular cup, SL-PLUS stem, and 28 mm Oxinium head on highly cross-linked polyethylene; Smith & Nephew). The cementless cup was first placed targeting 20° anteversion using the manufacturer’s cup inserter and 40° abduction by aligning the jig to the longitudinal axis of the body. Using the CA technique, stem anteversion was coordinated with cup anteversion targeting a CA angle of 50 ± 10°. During surgery, we confirmed that there was no tendency of dislocation by simulating dislocation of the hip under general anesthesia (maximum flexion with maximum internal rotation for simulating posterior dislocation, and maximum external rotation with maximum extension for simulating anterior dislocation). We confirmed proper alignment of the prosthesis by evaluating postoperative radiographs. In the analysis of postoperative X-ray and CT scans after left THA, the cup inclination angle, the cup anteversion angle, and the stem anteversion angle were 37°, 13°, and 35°, respectively. The resulting CA angle was 48° (Fig. ). Her postoperative course was normal, and following rehabilitation therapy including conventional training of daily living, standing up from the floor, sitting straight, and sitting cross legged, she became ambulatory and was discharged.
Three months after surgery, due to sudden occurrence of myasthenic weakness in her lower extremities while resting on a bed, she tended to raise her left limb up using both hands for sitting up. With her left leg in a figure-of-four position, she experienced sudden-onset severe pain and locking of left hip movement. She was diagnosed as having anterior dislocation following THA (Fig. ) and treated with closed reduction under sedation at the emergency department of another hospital. One year postoperatively, her University of California, Los Angeles (UCLA) activity score for her left hip improved to 4 from a preoperative activity score of 3 and her Harris Hip Score (HHS) improved to 81.0 points in her left hip compared with a preoperative HHS of 49.0 points. She was followed up as an out-patient but right hip pain developed gradually. At the end of the year 2016, an elective right THA was performed as was done earlier on her left side. Postoperative radiographic assessment using CT images after right THA showed that the cup inclination angle, the cup anteversion angle, the stem anteversion angle, and the CA was 42°, 27°, 24°, and 51° respectively (Fig. ). No postoperative complications were observed. We instructed her to exercise care during passive movements of her left leg, which may worsen her MG condition. No recurrence of dislocation in either hip has been observed since. Clinical scores in her right hip improved (UCLA activity score, 5; and HHS, 81.0 points) compared with those preoperatively (UCLA activity score, 4; HHS, 65.0 points) at the final follow-up. She returned to a normal life and has been able to walk long distances without a cane. We are carefully following her up as an out-patient.
|
pmc-6219210-1
|
A 53-year-old, non-diabetic, Chinese gentleman, presented with multiple episodes of bilateral lower extremities edema in 2012. There was no prior history of weight loss, skin rash, heart failure symptom or numbness over the lower extremities. Physical examination revealed a well-built gentleman with an elevated blood pressure reading of 160/90 mmHg. There was bilateral lower limbs edema but no organ enlargement was noted. Blood investigation showed normal full blood count, mildly impaired renal function with serum creatinine of 202 μmol/L and hypoalbuminemia without raised globulin level. Connective tissue disease screening was negative. 24 h urinary protein collection revealed proteinuria of 9 g per day Ultrasonography scan of the kidneys showed normal renal parenchymal echogenicity with bilateral kidneys’ size measuring at 9.5 cm and 9.6 cm respectively. Renal biopsy carried out showed AL amyloidosis with no evidence of free light chain deposition (as evidenced by the Congo red staining and negative for other specific staining). Extensive workup to look for other features of primary amyloidosis failed to show any association with systemic involvement (bone marrow aspiration and trephine biopsy, skeletal survey, echocardiogram, rectal biopsy was all reported negative). The only positive results was from the serum electrophoresis whereby it demonstrated the presence of IgG lambda paraprotein < 0.2 g/L migrating towards beta zone without any evidence of immunoparesis. The urine electrophoresis showed albuminuria of 14.1 g/L with mixed IgG lambda paraproteinuria of < 0.15 g/L. He was subsequently referred to hematologist for an opinion and was treated with 2 cycles of CTD (cyclophosphamide, thalidomide and dexamethasone) and VTD (bortezomide, thalidomide and dexamethaosone) with no resolution of the nephrotic syndrome. A repeated renal biopsy performed 3 years later showed no histological difference as compare with the first biopsy. A repeated systemic amyloidosis workup again showed inconclusive result. Bone marrow aspiration and biopsy repeated showed normal cellularity with presence of 2–3% plasma cells likely to be reactive in nature. Flow cytometry result showed 6% lymphocytes and 0.5% plasma cells with no aberrant plasma cells detected. He remains in overt proteinuria (7.8 g–10 g/dl) with a slowly creeping serum creatinine.
|
Subsets and Splits
No community queries yet
The top public SQL queries from the community will appear here once available.