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pmc-6195922-1
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A 19-year-old multigravid female was visiting our private clinic for a routine antenatal transabdominal ultrasound check at 16 weeks gestation. Two foetuses joined at the chest and abdomen were identified by two-dimensional ultrasound, and further confirmation was made by three- and four-dimensional ultrasound. There was blood incompatibility between the parents (the husband was A+ whereas the mother was A–). Anti-D was previously administered after delivery of her first child, a single viable healthy male baby. She had no history of abortion or miscarriage. Her height was 165 cm, weight 63 kg, looked normal, was neither anaemic nor hypertensive and had no history of chronic diseases.
The last menstrual date of this twin pregnancy was unknown. The mother did experience normal foetal movements. She had no vaginal discharge or hyperemesis gravidarum. Her abdomen was slightly large for the date of pregnancy. There was no family history of twins.
A Voluson 730 ultrasound machine from GE Healthcare (Waukesha, WI) was used. The transabdominal ultrasound was performed and two conjoined female foetuses were detected; each foetus was with a single head and a pair of arms and legs. The twins were joined at the lower chest and the upper abdomen. Only one functional foetal heart was observed centrally between both the foetuses with slight tendency towards one of the foetuses ( and ). Single anterior placenta was seen with the umbilical cord and a normal amount of liquor. The colour Doppler study showed two aortic arches emerging from the single functional heart (). On the basis of these findings, a diagnosis of dicephalic thoraco-omphalopagus conjoined twins was made ().
The parents were informed of the malformation and the likely outcome if the twins survived after delivery. They decided to terminate the pregnancy and refused further evaluation and investigation. A CS was performed at the request of the parents in the hospital and the delivery of viable conjoined twins aged 19 weeks was achieved without complication ( and ). The conjoined twins died a few minutes after delivery.
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pmc-6195923-1
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A 32-year-old male with no significant past medical history presented to our facility with a history of worsening pain and swelling in the medial aspect of his proximal left lower extremity over the past several years. Anteroposterior (AP) and “frog leg” lateral radiographs of the left lower extremity revealed a dense lesion consistent with dense matrix, adjacent to the medial aspect of the femur (). An MRI of the left lower extremity revealed that the lesion was of extraskeletal origin, arising from the adductor magnus, without any involvement of the femur (, ). The solid heterogeneous mass was located in the posterior compartment of the proximal-to-mid thigh and measured 9.6 × 7.3 × 13 cm in the transverse, AP and vertical dimensions, respectively. Multiple tiny hypointense foci, which corresponded to the calcific density noted on the prior plain radiographical studies, were noted centrally. The mass was otherwise of intermediate soft-tissue intensity on T
1 weighted images and moderately hyperintense to muscle on T
2 weighted images. Following intravenous administration of gadolinium, moderate enhancement of the mass, excluding the central portion, was observed. Superiorly and inferiorly, the margins of the mass were indefinite, and the mass demonstrated peripheral vasculature, which was most prominent at the cephalad and caudad margins. The mass was centred within the adductor magnus muscle fibres that were displaced around the mass. Anterolaterally, the mass was very closely approximated to the posterior cortex of the femur and the fascial margins that separated the quadriceps and the posterior compartments (Supplementary videos). No obvious signal abnormality was noted within the femur to indicate invasion or primary osseous origin. Post-excision pathology confirmed the presence of mesenchymal chondrosarcoma.
In the interval, surveillance imaging for metastatic disease with CT scan of the chest, abdomen and pelvis was performed. At 18 months, multiple bilateral non-calcified pulmonary nodules concerning for metastasis were identified (). The most prominent pulmonary nodule was present within the posterior right base, measuring 2.3 cm in the greatest dimension and abutting the pleura (). With regard to the abdominal and pelvic series, a new right adrenal lesion appearing as a hypodense pedunculated mass measuring 1.6 cm in the greatest dimension was noted. There was no evidence of osseous metastases.
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pmc-6195924-1
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In February 2014, an 11-year-old male presented with a history of recurrent respiratory tract infections and suspected tuberculosis following a case within his family. On physical examination, he appeared to be in good health. Chest auscultation did not reveal any specific pathological heart or lung sounds. Abdominal objectivity was negative. His parents did not refer any other relevant medical history about him. Tuberculin skin tests resulted positive and a chest X-ray showed a dishomogeneous parenchymal consolidation in the left lower lobe (). Suspecting active tuberculosis, a preliminary CT scan of the thorax was performed showing a heterogeneous consolidation with some cystic masses containing mixed fluid and air in the left lower lobe posterior segment, not in communication with the respiratory tract, and a probable expression of a dysplastic parenchymal area ( and ). These findings were suggestive of pulmonary sequestration, including a differential diagnosis of congenital pulmonary airway malformation, because of the presence of a cystic component within the consolidation. The injection of contrast medium showed an artery arising from the descending thoracic aorta that divided into two 1.2 cm after its exit, with both branches extending to the dysplastic area; therefore, a diagnosis of intralobar sequestration with associated aspects of bronchial atresia was made; superinfection and trapping of contiguous parenchyma coexisted. Just below the emergence of the previously anomalous vessel, another artery was detected that crossed the midline to achieve a healthy parenchyma in the right pulmonary base, configuring a pattern of aberrant systemic artery feeding a normal lung (–). Other congenital anomalies that appear to be related to pulmonary sequestration were absent; in particular, pulmonary venous drainage was regular through the pulmonary veins, there was no communication between the bronchus and the oesophagus, and no diaphragmatic defects or other gross pulmonary anomalies were identified either. Our patient responded well to antitubercular antibiotic therapy and his clinicians, together with his family, decided to keep him under control with clinical follow-up, avoiding surgery for the moment.
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pmc-6195930-1
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A 70-year-old male presented to the emergency department with pain and swelling in his right groin 11 days following a pulmonary vein isolation procedure with unguided femoral venous puncture for atrial fibrillation (AF). His past medical history included AF, radical prostatectomy and melanoma. He was on rivaroxaban for anticoagulation, which had been held prior to the procedure and subsequently restarted the day after the procedure.
The procedure was performed at another centre, and on two occasions afterwards, he had attended the treating centre, and twice had undergone a duplex scan of the femoral vessels that had revealed no collection or pseudoaneurysm. He attended the emergency department with severe pain and leg swelling three times, but was discharged with normal bloods and the knowledge of two negative duplex scans.
On the fourth visit, his haemoglobin had dropped from 14.2 g l−1 to 10.7 g l−1 over 6 days. A repeat duplex in our radiology department () revealed a large haematoma with evidence of a deep pseudoaneurysm. A CT angiogram revealed a pseudoaneurysm of a posterior right common femoral branch artery (). A subsequent angiogram revealed active bleeding at the site () and the feeding artery was coiled by interventional radiology (). The patient was admitted for observation for 24 h and discharged with no further problems. 2 months on, he remained well.
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pmc-6195934-1
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An 11-month-old male infant was admitted with high-grade fever and symptoms and signs of a lower respiratory tract infection. A chest radiograph revealed normal lung fields and an abnormal cardiac silhouette, with a prominent bulge along the left cardiac border (). This led to further evaluation by a transthoracic echocardiogram (TTE), which revealed a large left paracardiac blood-filled sac with sluggish flow (). This sac occupied most of the field of view on the echo windows, making it difficult to assess the extent of the lesion and its relationship to the left atrium (LA). A CMR was then performed for further characterization of the lesion, in particular to determine its extent and effects on cardiac function.
A non-contrast CMR was performed on a 3 T scanner under oral sedation with chloral hydrate syrup. The scan time was 30 min. After administration of chloral hydrate, the infant was allowed to sleep in his mother’s arms, wrapped in his own blanket. Once sedated, the infant was transferred to the MRI scanner, where vectorcardiographic leads were applied. He was scanned using a 32-channel cardiac coil placed around his blanket. In our experience, it is highly comforting to the child to be examined while wrapped in the same fabric they are used to at home or in the ward. It not only helps sedate them more easily, but also maintains the sedation for the 30–40 min taken to complete the study.
We obtained axial three-dimensional (3D) whole-heart images using respiratory-gated spoiled gradient (SPGR) sequence and cine images in standard cardiac planes using free-breathing steady-state free-precession (SSFP) sequences. The former provided volumetric data that was reconstructed in various planes to define the location, morphology and extent of the lesion. The latter was used for evaluating the ventricular function and blood flow within the lesion. The CMR depicted a 7-cm large, predominantly intrapericardial dog ear-like protrusion arising from the LA () with slow swirling flow within (). It was diagnosed as a congenital LAAA compressing the left superior pulmonary vein (). It was seen adhering to the adjoining left ventricle (LV) and causing a mild mass effect on the LV in the form of a flattening of its basal lateral wall (). The superior aspect of the aneurysmal sac showed a small septum, which was suspected to be the site of a pericardial defect owing to a small extrapericardial herniation of the aneurysm (). The mitral valve was unremarkable. No other congenital cardiac defect was seen. The biventricular function was normal. The CMR findings were confirmed on complete surgical resection of the aneurysm.
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pmc-6195939-1
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A 14-year-old male presented to the emergency department with two episodes of seizure preceded by a history of headache for 1 month and diminution of vision in both eyes for 5 days. His past history was significant for appendicectomy 3 years ago and severe abdominal pain 2 months ago. On examination, the patient was conscious, oriented and comprehending, with no neurological deficits. His blood pressure was 170/110 mmHg and other vitals were normal. A fundoscopic examination later revealed Grade 4 hypertensive retinopathy.
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pmc-6195940-1
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A 70-year-old male was admitted to our hospital complaining of abdominal pain, nausea and vomiting for 3 days. The pain was accompanied by a loss of appetite, with no gas or faecal discharge within the previous 24 h. His past medical history revealed appendectomy. His vital signs were within the normal range and physical examination revealed distention of the abdomen with right lower quadrant tenderness. Laboratory tests were normal with the exception of a mild leukocytosis (11,800 cells mm3). Abdominal plain film showed findings of mechanical bowel obstruction and an abdominal CT was requested. The CT scan showed mildly distended ileal loops with fluid levels, clasped at the level of a fibrous connection between the MD and the abdominal wall. A cluster of collapsed bowel loops, approximately 50 cm, was herniated throughout this orifice, configuring an internal hernia (). No signs of infarction were observed on the CT scan ().
The patient was immediately referred for surgery. Laparoscopic exploration revealed distension of the small bowel, confirming the presence of an internal hernia throughout an orifice caused by adhesion of the MD to the abdominal wall. The fibrous band was released, the incarcerated bowel was normalized and the MD was sectioned by a laparoscopic linear stapler (). The entire small bowel was then carefully inspected and placed in its correct position. The patient was discharged after 5 days without postoperative complications.
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pmc-6195964-1
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A 62-year-old woman presented at the ophthalmology department of hospital with a 1-week history of left eye pain and blurred vision. The ophthalmologist performed ophthalmic fundoscopy and optical coherence tomography (OCT) on the patient (Fig. and Fig. ). The diagnosis was metastatic carcinoma in the eye. The diagnostic evaluation was completed with PET-CT, which confirmed central lung cancer in the lower lobe of the right lung with ocular metastasis (Fig. ). Brain MRI showed no obvious abnormality. Pathological findings of bronchoscopic biopsy indicated non-small cell lung cancer (NSCLC) in the lower segment of the right lower lobe of the right lung with features of squamous cell carcinoma (Fig. ). After consultations with an MDT, including specialists from the surgery, internal medicine, ophthalmology, radiotherapy and imaging departments, the patient underwent right lower lobe resection and lymph node dissection in December 2016. Postoperative pathology led to the diagnosis of right lung squamous cell carcinoma of T2aN1 stage. In February 2017, the patient underwent an eye examination, which indicated that the ocular lesions were enlarged (Fig. ). The patient received 4 courses of gemcitabine plus cisplatin regimen from February to May 2017. Her eye symptoms improved after 2 courses of chemotherapy (Fig. ) and disappeared completely after 4 courses (Fig. ). The progression-free survival (PFS) duration of the patient was 11.9 months until the emergence of brain metastasis, which was treated with cranial radiotherapy. The patient was followed up for 16.5 months (March 2018) after surgery, and the ocular lesion was still well controlled without any specific ocular treatment.
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pmc-6195983-1
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A 38-year-old Japanese woman with no history of smoking was referred to our institution for cough and dyspnoea on exertion. She had been diagnosed with sporadic LAM via a surgical lung biopsy (SLB) 2 months prior to her first visit to our institution. She had a medical history of stillbirth. Schirmer test and serum anti-SS-A antibody test results were both positive. At the age of 35 years, she was diagnosed with SjS in accordance with the 2012 American College of Rheumatology Criteria []. She was also diagnosed with APS in accordance with the 2006 Sydney APS Classification Criteria []. At the time of LAM diagnosis, her levels of serum autoimmune antibodies were as follows: RF, 68 IU/mL; anti-dsDNA antibody, 24 IU/mL; anti-cardiolipin antibody, 11 IU/mL; anti-SS-A antibody > 500 U/mL; and anti-SS-B antibody < 7.0 U/mL.
Diffuse, thin-walled cystic lesions were observed on HRCT (Fig. ). An SLB was performed at segment 6 of the right lower lobe. The lung tissues exhibited spindle cell nests in the interstitium. Further examination revealed that these LAM cell nests were positive for alpha-smooth muscle actin (αSMA), human melanoma black-45 (HMB45), oestrogen receptors, and progesterone receptors. Formation of lymphoid follicles (lymphoid cell aggregates) was observed in multiple areas of lung tissue (Fig. -).
The patient received no medication for LAM or decreases in respiratory function. At the initial and 6-month follow-up visits, her percent predicted forced vital capacity (%FVC) values were 87.2% and 82.0%, her percent predicted forced expiratory volume in 1 s (%FEV1) values were 49.1% and 46.5%, and her percent predicted diffusing capacity of the lung for carbon monoxide (%DLco) values were 26.0% and 18.9%, respectively. She underwent lung transplantation 51 months after the first visit to our institution.
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pmc-6195983-2
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A 61-year-old Japanese woman with no history of smoking was referred to our institution for dyspnoea on exertion. The patient had been diagnosed with sporadic LAM via SLB 2 months prior to her first visit to our institution. She had a medical history of pneumothorax. Autoimmune antibody tests were negative at the initial visit, although she tested positive for anti-SS-A antibody (28.4 U/mL) 38 months after the first visit. She received a diagnosis of SjS based on 2012 American College of Rheumatology Criteria [].
HRCT revealed diffuse, thin-walled cystic lesions (Fig. ). SLB was performed from the lingular segments of left upper lobe and left lower lobe. Proliferation of LAM cells was observed in the interstitium, while immunostaining experiments revealed that the LAM cell nests were positive for αSMA and HMB45. Cystic lesions were observed within the lung tissue, along with some lymphoid follicles and lymphoid cell infiltration in the peribronchiolar regions (Fig. -).
Sirolimus treatment was initiated 30 months after the first visit. The patient remained alive at the 6-year follow-up, with no further decreases in pulmonary function (%FVC: 141.2%, %FEV1: 101.0%, %DLco: 61.2%). Administration of sirolimus did not affect the course of SjS.
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pmc-6195983-3
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A 48-year-old Japanese woman with no history of smoking was referred to our institution due to the presence of abnormal shadows on chest radiographs. She was diagnosed with sporadic LAM via a SLB 4 months after her first visit to our institution. She had a medical history of uterine myoma and diffuse goiter. Lip biopsy revealed infiltration of lymphocytic cells, and serum anti-SS-A antibody test results were positive (12.1 U/mL). The patient was diagnosed with SjS in accordance with the 2012 American College of Rheumatology Criteria [].
HRCT revealed diffuse, thin-walled cystic lesions (Fig. ). Although a transbronchial lung biopsy (TBLB) was performed, it did not lead to the diagnosis of LAM. SLB was performed at segments 4 and 8 of the right lung. Cystic lesions of up to 8 × 6 mm in size were observed within the lung tissues, along with proliferation of LAM cells in the interstitium. Immunostaining experiments revealed that LAM cells were positive for HMB45, αSMA, and oestrogen receptors (Fig. , ). Lymphoid follicles with germinal centres in the walls of membranous bronchioles (500 × 500 μm) and chronic interstitial pneumonia with a subpleural focus were observed within at 2.0 × 2.5 mm area using a microscope. Honeycombing and band-like infiltration of lymphoid cells was observed within a visceral pleura measuring 150 × 2500 μm.
She received no medication for LAM, and no further decreases in respiratory function were observed at the 8-month follow-up (%FVC: 98.8%, %FEV1: 83.4%, %DLco: 117.5%).
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pmc-6195983-4
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A 44-year-old Japanese woman with no history of smoking was referred to our institution for dyspnoea at rest and subsequently diagnosed with sporadic LAM via TBLB. She had a medical history of RA, which was diagnosed in accordance with 1987 American College of Rheumatology Criteria [] and treated with bucillamine, methylprednisolone, and salazosulfapyridine. Levels of serum autoimmune antibodies were as follows: RF 46 IU/mL, ANA 1:160 (speckled).
HRCT revealed diffuse, thin-walled cystic lesions and right pleural effusion (chylothorax) (Fig. ). TBLB was done performed in the upper and lower portions of the right lung. LAM cell populations with eosinophilic cytoplasm encompassing the wall of the dilated lymphatic vessel were observed in TBLB specimens. Immunostaining experiments revealed that these LAM cells were positive for αSMA, oestrogen receptor, progesterone receptor, and HMB45 (Fig. , ).
She received no medication for LAM. No long-term follow-up data regarding respiratory function and LAM were obtained due to her difficulty in visiting the hospital. Her initial values were as follows: %FVC: 79 8%; %FEV1: 72.8%; %DLco: 35.1%. Her survival one month after the first visit was confirmed.
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pmc-6195983-5
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A 49-year-old Japanese woman with no history of smoking was referred to our institution for dyspnoea at rest. She was diagnosed with sporadic LAM based on histological examination of a retroperitoneal tumour (lymphangioleiomyoma) 4 months prior to the first visit. She had a medical history of SLE, uterine myoma, and pneumothorax. SLE was associated with pleurisy, proteinuria, and psychosis. She was diagnosed with SLE in accordance with the updated 1997 American College of Rheumatology Criteria [, ]. SLE was treated with prednisolone. Serum levels of autoimmune antibodies were as follows: ANA 1:80 (homogeneous, speckled); anti-dsDNA antibody, 7.6 U/mL.
HRCT revealed diffuse, thin-walled cystic lesions (Fig. ). A retroperitoneal tumour measuring 12.5 × 8.4 cm in size was resected. Histological examination revealed a lymphangioleiomyoma testing positive for αSMA and HMB45.
She received no medication for LAM and was treated with prednisolone (5 mg/day) for SLE. However, no decreases in pulmonary function were observed during the 12 years between her initial and most recent visit (%FVC: 102.0%, %FEV1: 85.4%, %DLco: 86.6%).
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pmc-6196007-1
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An 8-year-old female presented with a history of gradually increasing swelling and pain in the scapular region. The MRI scan showed a large expandable mass involving the subscapularis muscle (Fig. ). A staging study did not reveal evidence of spread of the disease. The biopsy revealed the diagnosis of extraskeletal Ewing Sarcoma. The patient received neoadjuvant chemotherapy as per the existing hospital protocol. Before surgery, all image data were imported and surgical planning was made on a 3D tumour model. The scapular prosthesis was designed according to images of the contralateral site. During surgery, the lesion was exposed and a subtotal scapulectomy with excision of the involved muscles with a tumour-free margin was completed. The prosthesis was implanted and fixed with nonabsorbable suture to the residual acromion, and created a fundamental fulcrum for the functionality of the system. Residual muscular tissues then were fixed to the small holes on the prosthesis, and the LD flap was created as described above.
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pmc-6196007-2
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A 45-year-old woman was diagnosed with subscapularis muscle synovial sarcoma in 2006 (Fig. ). After a proper staging study was completed, an autograft of the scapula after cryotherapy with liquid nitrogen was performed. After surgery, the patient received adjuvant chemotherapy. Five years after surgery, massive resorption of frozen graft and osteosynthesis failure were observed, which necessitated revision surgery. The patient underwent reconstruction with massive bone allograft. Another complication occurred 4 years later which involved a fatigue fracture of the acromion with pain and loss of function. We decided to perform a salvage procedure with a custom-made prosthesis, according to the data of the contralateral side. Due to the absence of the acromion, the implant was not anchored to the bone, but only to residual muscles and ligaments. Moreover, the lack of soft tissue because of multiple surgeries makes the LD flap particularly suitable and effective in that case.
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pmc-6196019-1
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Our patient was a 15-year-old Han Chinese boy who presented with fever, weakness, pallor, edema, dyspnea, and gradually increasing abdominal girth for 2 weeks. His parents denied consanguineous marriage. His medical history was not significant. His one older sister and two younger sisters were all healthy. He was delivered after full-term normal pregnancy. His growth and development were markedly slow after birth, but his school records were excellent. His spleen was resected due to unexplained enlargement at the age of 9. He had a history of multiple prolonged hospital admissions for pyrexia of unknown origin, without reaching a clear diagnosis.
The results of an initial assessment on admission were: he looked unwell, height 135 cm, weight 30 kg, and no sign of secondary sex characters. He had fever with a temperature fluctuating between 37.2 and 39.7 °C, pallor, wakefulness, distress, and clarity of mind. There was no jaundice, petechiae, or fresh rash on skin. Superficial lymph nodes were palpable in inguinal region. His eyelids were swollen, no cyanosis, pharynx without congestion, and bilateral tonsils III° large. There was no thyroid enlargement. Trachea in midline. There were audible harsh breath sounds and crackles bilaterally. His heart beat was 114/minute with normal rhythm, no murmur was heard. He had a distended abdomen with hepatomegaly; liver palpable 10 cm below costal margin with firm consistency and free from tenderness. He had a huge scar located in the left upper quadrant (Fig. ). Murphy sign (−); shifting dullness sign (+). There was marked pitting edema in his lower extremities. A neurological assessment was normal. The results of other systemic examinations were essentially normal.
Initial and subsequent laboratory tests and imaging revealed the following results:On routine blood tests conducted over several days, white blood cell (WBC) count was more than 20 × 109/L with normal classification, whereas hemoglobin (Hb) and platelets (PLT) decreased continuously (Fig. ). Liver enzymes and bilirubin were increased slightly to moderately with hypoproteinemia: lactate dehydrogenase (LDH) 301 U/L, aspartate transaminase (AST) 45 U/L, alkaline phosphatase (ALP) 218 U/L, total bilirubin (TBIL) 72.9umol/L, albumin (ALB) 29 g/L, but a kidney function test and urine analysis were unremarkable. Prothrombin time (PT) and activated partial thromboplastin time (APTT) were prolonged slightly; D-Dimer increased moderately. Other positive indicators were: procalcitonin (PCT) fluctuated around 0.2 ng/ml; antistreptolysin (ASO) was 259 IU/mL; Widal’s test TH 1:160, TO 1:320; tumor marker CA-25 was 384.0 U/mL, CA724 was 38.78 U/mL; ferritin was 550.58 ng/mL; hepatitis E virus antibody IgG(+); and Legionella pneumophila serum antibody IgM(±). The tests of thyroid function, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), CD64 index, T-lymphocyte subsets, and glucose-6-phosphate dehydrogenase (G6PD) were normal. Coombs test, interferon-γ release assay, antinuclear antibodies (ANAs), hepatitis A virus (HAV)\hepatitis B virus (HBV)\hepatitis C virus (HCV)\human immunodeficiency virus (HIV) antibody test, and herpes simplex virus (HSV)\cytomegalovirus (CMV)\Epstein–Barr virus (EBV)-deoxyribonucleic acid (DNA) test were negative. Blood and fungal cultures were negative. Bone marrow smear showed infectious bone marrow image and occasional atypical lymphocytes. Ultrasound revealed grossly enlarged liver (right oblique diameter 164 mm) with normal echotexture, gallbladder wall swelling, left renal calculi, massive peritoneal effusion, but portal vein and common bile duct had normal diameters. There was slight pericardial effusion with normal pulmonary arterial pressure. Enhanced computed tomography (CT) scans showed severe pulmonary interstitial fibrosis with infection, multiple lymph node display, liver shape irregularity, and no obvious abnormality in abdominal angiography (Fig. ).
After receiving therapies including anti-infection, anti-virus, atomization inhalation, diuresis to eliminate swelling, liver-preserving and gallbladder protection, human serum ALB, immunoglobulin and blood transfusion, the symptoms of our patient improved, and his peak body temperature decreased (Fig. ). However, it was difficult to explain the illness with “one etiology”; it was assumed that it was probably a kind of rare genetic metabolic disease associated with infection. To confirm the diagnosis, pathological sections of the spleen were borrowed from another hospital, where the boy underwent splenectomy 6 years previously.
Hematoxylin-eosin (HE) staining of the spleen sections showed that red pulp was occupied by a large number of Gaucher cells, splenic trabecula disappeared, and some splenic nodules remained. The results of specific stains were: Rf (+) and periodic acid–Schiff (PAS) (−). The results of immunohistochemical stains were: CD68 (+), Lyso (+), Vim (+), KI-67 (+<5%), Pan-CK (−), EMA (−), HHF35 (−), CD31 (−), CD34 (−), LCA (−), and S-100 (−) (Fig. ). Final diagnosis was GD (type 1). However, our patient’s family declined to test beta-glucosidase levels, gene mutation site, or purchase imiglucerase (enzyme replacement therapy). Finally, he was discharged from hospital after his temperature had declined to normal.
During a subsequent 6-month telephone follow-up, he said that he was able to continue with his school life, and do some mild physical activities; he no longer had a fever. A routine blood examination at a local hospital showed mild anemia, and a CT scan showed no significant progress of pulmonary infection. After raising enough money, his family would consider using imiglucerase.
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pmc-6196130-1
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A 42-year-old male presented to the emergency department with a left-sided pleural effusion in the setting of a two-month history of intermittent fevers, dry cough, and unintentional weight loss. His medical history consisted of recurrent venous thromboembolism secondary to a prothrombin gene mutation and recurrent renal calculi. He was a smoker (20 pack-year history). On arrival to our institution, the patient was febrile (38.0°C) with dull percussion over the left lower chest and reduced breath sounds in the same region on auscultation. Abdominal examination demonstrated mild left flank tenderness with no palpable masses or tenderness elsewhere. The remainder of the physical examination was unremarkable.
Laboratory studies showed an elevated white cell count (12.2 x 109/L) with a predominant neutrophilia (10.20 x 109/L), microcytic anaemia (haemoglobin 85 g/L), and an elevated C-reactive protein (CRP) (283 mg/L). Blood and urine cultures were negative.
Plain chest radiograph demonstrated a moderate-sized left pleural effusion (Fig. ). A computed tomography (CT) pulmonary angiogram was performed given his past history, demonstrating no pulmonary embolus and normal lung parenchyma. A bedside lung ultrasound confirmed a moderate-sized, simple effusion and an elevated left hemidiaphragm. Analysis of aspirated pleural fluid demonstrated an exudative effusion with a pH of 7.367, total protein of 51 g/L, glucose of 5.6 mmol/L, and lactate dehydrogenase of 239 U/L and was negative for malignant cells on cytology. Culture of this pleural fluid was also negative. Pleural fluid analysis did not suggest empyema or complicated parapneumonic effusion.
The patient was commenced on empiric i.v. broad-spectrum antibiotics (tazobactam/piperacillin) with no clinical improvement in symptoms after five days. The patient continued to experience persistent fevers and a persistently elevated CRP.
CT scan of the abdomen and pelvis was performed to investigate other potential sources of infection (Fig. ). This demonstrated a chronic left pyelonephritis with a multiloculated appearance of the renal calyces, suggestive of XGP. There was also a large staghorn calculus obstructing the renal pelvis, with additional abscesses at the hilum of the spleen and inflammation of the tail of the pancreas.
CT-guided drainage of the splenic abscess and pyelonephritis was subsequently performed—both were positive for Proteus mirabilis sensitive to amoxicillin, ceftriaxone, and gentamicin. The patient was subsequently treated with ceftriaxone for a total duration of four weeks.
In addition, the patient was managed with a left total nephrectomy. Histopathology of this tissue confirmed the diagnosis of XGP, demonstrating severe pyelonephritis with chronic inflammatory infiltrates and xanthomatous cells. The patient’s postoperative recovery was uneventful.
He was discharged from hospital and remains well on routine follow up.
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pmc-6196253-1
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A 74 year old male with the diagnosis of probable Menière's Disease (MD) according to the recent Bárány Society diagnostic criteria for MD (), presented 5 years of progression of recurrent vertigo attacks with concomitant ear fullness and tinnitus. The patient also has a mild broad-frequency bilateral hearing loss according to the 1997 Bureau International d'Audiophonologie (BIAP) criteria. Medical treatment with betahistine (24 mg/12h) and on demand sulpiride (50 mg) has achieved a good control of his vertigo and related symptoms. During these 5 years the patient presented periodically at a tertiary neurotology clinic to monitor his progression of probable MD.
Apart from the mild hearing loss, no significant visual impairment or other alterations were found during these 5 years on the patient otoneurotologic examination: normal ocular movements, smooth pursuit, and saccadic movement were found, along with absence of ocular misalignment on skew deviation test and no spontaneous nystagmus. Other neurological examinations and cerebral magnetic resonance images (MRI) were also normal for this patient.
The patient's semicircular canal function was monitored during the 5 years of development of recurrent vertigo using vHIT ICS Impulse™ devices (Otometrics A/S, Taastrup, Denmark). The vHIT explorations were always performed by a senior neurotologist, but because of the 5 year time period, different ICS Impulse™ hardware devices and software versions were used for the patient's examination: For the 2013 examination ICS Impulse™ hardware device with FireWire connection and software version 2.0 was used; for the 2015 examination the hardware was changed to ICS Impulse™ universal serial bus (USB) hardware device with software version 2.0, for the 2016 examination the software was updated to 3.0, and for the 2018 examination version 4.1 was used. The data collected during these years were exported and re-analyzed with ICS Impulse™ 4.1 version, this was made to avoid possible analysis bias in the data presented in this paper because of possible differences on gain calculation methods between the different ICS Impulse™ versions used to collect the data over time. For all vHIT tests using these different vHIT devices, the patient showed an increased aVOR gain in both horizontal canals (Figure ). Note that the aVOR gain calculated by (version 4.1) Impulse™ software is the ratio of the area under the desaccaded eye velocity to the area under the head velocity during the impulse, and so is an area gain.
The minimum aVOR gain value was measured for the horizontal left canal in 2016 (gain of 1.11), and the maximum aVOR gain value of 1.65 was measured for the right horizontal canal in 2016. During the 5 years of testing a significantly enhanced eye velocity, with resulting enhanced aVOR gain, was always found. The value of aVOR gain fluctuated, both for tests on the same side and also one side with respect to the other. The side with the higher gain changed from the left side in 2013 to the right side in 2018.
In 2018 we also performed oculomotor tests including the visual-vestibular interaction (VVOR) test (), by using the same vHIT testing equipment to obtain precise eye-movement recordings. In these oculomotor tests no position or tracking errors were found. For the saccadic eye movement test, high-velocity saccadic eye movements were recorded (Figures -).
For VVOR testing (Figures -), a bilateral enhanced eye velocity was observed. Mathematical analysis of the desaccaded VVOR eye velocity response () showed a positive VVOR gain value of 1.35 for the right side and 1.4 for the left side measured at ~1.8 Hz stimulation frequency. This shows that enhanced eye velocity on high velocity vHIT was accompanied by enhanced eye velocity on low-velocity VVOR testing.
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pmc-6196253-2
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A 45 year old female with the diagnosis of 10 years' progression of definite () bilateral type I Menière's Disease, according to the Lopez-Escamez classification (), with recurrent vertigo and hearing loss attacks symptomatically controlled with sulpiride (50 mg) on demand, was referred to the cochlear implants unit of a tertiary hospital center as a possible candidate for cochlear implantation, because of profound hearing loss (1997 BIAP) in the left ear and fluctuating moderate-severe (1997 BIAP) hearing loss in the right ear. The most recent vertigo episode occurred 10 days prior to her visit to the cochlear implant unit. No visual impairment or other alterations were found on otoneurotologic examination; normal ocular movements, normal smooth pursuit, and saccadic movement, absence of ocular misalignment on skew deviation test and no spontaneous nystagmus were also found, despite the recency of the last vertigo attack. Other neurological examinations and standard cerebral MRI were also normal for this patient.
In addition to the same tests as for case I, because of the bilateral MD diagnosis, the patient had received 3 months prior a 3 Tesla MRI examination of the inner ear using the HYDROPS MRI sequence (): this sequence is based on the digital subtraction of images produced by the different time diffusion of gadolinium along the inner ear fluids. This MRI showed bilateral cochlear and vestibular endolymphatic hydrops with left side hydrops predominating, as can be observed in Figure .
The instrumental vestibular testing was performed by the same senior neurotologist using a vHIT ICS Impulse™ USB hardware version with software version 4.1: this device was a different unit from the device used in case I. Figure shows enhanced vHIT eye velocity responses for both sides, with an aVOR gain value of 1.14 on right horizontal canal function test and 1.05 for left side. In this case, the vHIT calibration was repeated four times with similar enhanced gain values obtained, and a fifth calibration was done with the default system calibration parameters, also yielding similar aVOR gain values. The VVOR test was also performed on this patient using the vHIT ICS Impulse™ device, finding an enhanced eye velocity response during the VVOR test with a measured VVOR gain () value of 1.39 for left side and 1.35 for right side.
For both cases presented in this paper, the patients gave written consent to publish the results obtained from their clinical examinations and instrumental tests.
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pmc-6196439-1
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A 21-year-old woman presented to our hand and peripheral nerve clinic 4 months after attempted Nexplanon® removal from her left arm. The patient reported that neither she, nor her nurse practitioner (NP), was confidently able to feel the Nexplanon® before the attempted removal. Imaging studies to confirm location of the Nexplanon® were not performed. The patient remembered a small incision being made at the site of insertion after local anesthetic was injected. The provider was not immediately able to find the Nexplanon®, but after exploring the local area, did grasp another structure in her arm. The patient felt an “electric shock” sensation that radiated down to her medial elbow as the NP pulled. No further attempts to remove the Nexplanon® were undertaken.
Immediately after the removal attempt, the patient had complete numbness in her small and ring fingers. She returned for follow up to her NP. As months progressed, the numbness did not improve, and she began to notice wasting of her hand muscles and weakness in her grip. At almost 4 months after the attempted removal, her NP ordered a nerve conduction study which showed 50% loss of ulnar nerve function.
On presentation to the office, she had classic signs of severe, chronic ulnar nerve injury: wasting of the ulnar nerve-innervated intrinsic muscles of the hand, a claw position of the ring and small fingers, and dense numbness in an ulnar sensory nerve distribution (Fig. ). On examination of her arm, there was a well-healed incision with surrounding scar tissue from the extraction attempt. The Nexplanon® was not palpable. X-rays showed the radio-opaque Nexplanon® at the junction of the proximal and middle thirds of the humeral shaft with the most distal end 16.5 cm proximal to the medial epicondyle (Fig. ).
Given the patient’s history, physical exam, and nerve tests, timely surgical intervention was recommended. In the OR, the upper extremity surgery team used fluoroscopy to mark the location of the Nexplanon® (Fig. ). On surgical exploration, the Nexplanon® was found deep to the brachial fascia of the arm and in direct contact with the ulnar nerve. Less than five millimeters away was the undamaged brachial artery--the main blood supply to the arm, forearm, and hand. The Nexplanon® was removed using microsurgical instruments.
Because of the patient’s dense ulnar nerve palsy, the ulnar nerve at the level of the attempted extraction was also explored. Dissection showed that the ulnar nerve had been severely damaged at this level (Fig. ). Nerve injury can take many forms; this patient’s injury was a neuroma-in-continuity whereby the ulnar nerve was still one solid, longitudinal structure, but contained an abnormal portion, filled with scar tissue and damaged nerve fascicles that could not conduct electrical signal. The surgical team confirmed the neuroma’s inability to conduct via intra-operative electrical stimulation. The appearance of her neuroma-in-continuity was classic: the neuroma was fusiform in shape and felt thickened and hard, unlike the proximal and distal, soft and pliable uninjured nerve (Fig. ).
The upper extremity surgeons treated the neuroma-in- continuity with microsurgical resection, followed by reconstruction. After the damaged, scarred nerve was removed, there was a 3 cm gap between healthy sections of the ulnar nerve (Fig. ). The patient retained three uninjured nerve fascicles which made up less than 20% of the normal diameter of the nerve. The healthy fascicles were dissected free and preserved (Fig. ). To bridge the nerve gap, the patient’s sural nerve was harvested from her lower leg, cut into 3 cm long segments and bundled together to recreate the caliber and fascicles of the resected ulnar nerve (Fig. ). This cabled sural nerve autograft was sutured into place using a surgical microscope and 9–0 Nylon sutures.
At 7 months after nerve reconstruction, the patient had weak grade 2/5 function of ulnar innervated muscles. She still had dense numbness in an ulnar nerve distribution. She did have an advancing Tinel’s sign on physical exam and reported intermittent paresthesias in an ulnar nerve distribution in her hand.
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pmc-6196447-1
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A 70-year-old retired Caucasian man, a former electrical society employee, presented to our hospital with moderate dyspnea on exertion (slight limitation of physical activity; New York Heart Association [NYHA] class II). He was asymptomatic for anginal pain, palpitations, or syncope. His past medical history included well-controlled hypertension, inguinal hernioplasty, and right total hip replacement for arthritis secondary to hip dysplasia. His family history revealed an unspecified heart condition in his father, who had died aged 55 years old. He denied smoking, drinking alcohol to excess, and use of recreational drugs. He did not have diabetes or hypercholesterolemia. He was receiving therapy with ramipril.
The patient’s vital signs at presentation were blood pressure 140/80 mmHg, pulse 58 beats/min with regular rhythm, and body temperature 36.0 °C. His body mass index was 30.8 kg/m2.
He appeared alert, oriented, and cooperative. His lungs were clear to auscultation and percussion bilaterally; no cardiac murmurs were appreciated. His abdomen was globose and nontender to palpation. His bowel sounds were normal in quality and intensity in all areas.
Mild lower extremity pitting edema was noted, but neither cyanosis nor clubbing was present.
Cranial nerves III–XII were intact; the results of motor and sensory examination of the patient’s upper and lower extremities were normal. The patient’s reflexes were normal and symmetrical bilaterally in both extremities.
The patient’s laboratory findings were unremarkable, except for a brain natriuretic peptide value of 1441 pg/ml (white blood cell [WBC] count 8.08 × 103/μl, neutrophils 6 × 103/μl, red blood cell [RBC] count 4.63 × 106/μl, mean corpuscular volume 88 fl, mean corpuscular hemoglobin concentration 32 g/dl, hemoglobin 13.9 g/dl, platelets 218 × 103/μl, creatinine 1.11 mg/dl, glomerular filtration rate 65 ml/min/m2, total protein 6.5 g/dl, urea 50 mg/dl, Na+ 142 mmol/L, K+ 3.8 mmol/L, Cl− 98 mEq/L, glucose 77 mg/dl, bilirubin 0.58 mg/dl, alkaline phosphatase 67 U/L, alanine aminotransferase 19 U/L; normal urinalysis, with traces of RBCs, WBCs, and bacteria).
Transthoracic echocardiographic examination demonstrated a marked left ventricular concentric hypertrophy, with regular ventricular cavity size. The patient’s segmental/global kinetics were preserved, with borderline normal ejection fraction (55%); an expanded left atrium and a mild mitral regurgitation were noted.
Therefore, cardiac magnetic resonance (CMR) imaging was performed to investigate a suspected hypertrophic cardiomyopathy (Signa HDxt Echospeed 1.5-Tesla magnetic resonance imaging [MRI] scanner; GE Healthcare, Milwaukee, WI, USA). After administration of intravenous contrast material, late gadolinium enhancement (LGE) consistent with chronic subendocardial ischemia was revealed. As collateral evidence, CMR also showed a plausible coronary artery anomaly (Figs. and ).
Consequently, the patient underwent coronary computed tomographic angiography (CCTA) (Brilliance iCT 256-slice scanner; Philips Healthcare, Cleveland, OH, USA). A preliminary scan for scoring the amount of coronary calcium was obtained, and the Agatston score was calculated (0). Iodinated contrast media (Iopamiro 370; Bracco Imaging, Milan, Italy) was injected into an antecubital vein.
Nitroglycerin 0.3 mg was sublingually administered immediately before contrast injection. The patient was in sinus rhythm with a heart rate of 75–80 beats/min, unvaried after two 5-mg doses of intravenous metoprolol, so retrospective gated CCTA was performed, and reconstruction of cardiac phases from 40% to 78% of the R-R interval was done.
For proper scan timing, we used a bolus-tracking technique with an ROI placed in the ascending aorta. When the attenuation values in the selected area rose to a preset threshold (150 Hounsfield units), the system automatically played a short, prerecorded breath-hold instruction to the patient, and the scan was automatically commenced.
The following imaging parameters were used for data acquisition: 256 × 0.625 detector collimation, 270-ms gantry rotation time, 100-kV tube voltage and 618-mAs tube current-time product, and field of view of 18 cm with a matrix of 512 × 512. The dataset was reconstructed with a model-based iterative reconstruction, and the images were postprocessed and analyzed on an external workstation (Extended Brilliance Workspace; Philips Healthcare).
CCTA imaging clearly revealed that the LCA originated from the pulmonary artery and then bifurcated into the left anterior descending artery and the left circumflex artery. The RCA arose from the right coronary sinus, as normal. The RCA and LCA appeared markedly dilated and tortuous (Figs. and ). Dilated intercoronary vessels along the epicardial surface of the heart and dilated bronchial arteries corresponded to the collateral pathways of the LCA with the RCA and with systemic vessels, respectively (Fig. ). The retrograde flow from the anomalous coronary artery to the main pulmonary artery was well depicted (steal phenomenon). The coronary arteries were smooth with no evidence of atherosclerotic disease. Several calcifications at the papillary muscle were observed.
After CCTA, invasive coronary angiography (ICA) was performed. First access was made through the radial artery, then another access was attempted through the femoral vein to confirm the origin of the LCA from the pulmonary artery (Fig. ).
The risk/benefit ratio for surgery or surveillance with medical treatment was carefully analyzed; owing to the patient’s age, signs of mild chronic myocardial ischemia, and only slight limitation of physical activity, a therapy for ischemic cardiomyopathy (including bisoprolol 1.25 mg/d, furosemide 25 mg/d, canrenone 25 mg/d, ramipril 1.25 mg/d, apixaban 5 mg twice daily) was administered, and periodic surveillance was started. In our center, we do not have a standardized follow-up protocol for adult patients with ALCAPA syndrome. In this specific case, our patient underwent a cardiological visit, based on clinical examination and transthoracic echocardiography, 6 months after ICA. The patient was still symptomatic for dyspnea caused by slight physical activity (NYHA class II); an echocardiogram demonstrated stable chamber measures and ejection fraction (left ventricular ejection fraction, 55%), and mitral regurgitation was still moderate. The estimated pulmonary artery pressure was 55 mmHg.
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pmc-6196470-1
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A 69-year-old female patient diagnosed with acral lentiginous melanoma of the left foot, pT3a pN2a cM0 R0, stage IIIA, was treated by amputation of the first toe with complete resection. Four years later, she developed histologically confirmed multiple in-transit metastases requiring recurrent excisions. The disease continued to progress, with cutaneous and lymph node metastases. She was enrolled in a clinical trial combining pembrolizumab and T-VEC (talimogene laherparepvec, a GM-CSF-expressing oncolytic HSV-1 virus) administered by seven intralesional injections []. The patient had no known prior autoimmune disorders and had no neurologic manifestations prior to the CPI treatment.
Four months after the first dose of pembrolizumab, the patient developed several vitiligo lesions, followed by abnormal sensation with tingling and numbness of fingers and hands suggestive of bilateral carpal tunnel syndrome 1 month later. Symptomatic treatment was proposed, but neurological manifestation worsened progressively. At this time, the patient was considered in complete remission and denied having any sicca syndrome, leading to the discontinuation of pembrolizumab after having received eleven administrations.
Between eight and 10 months after the first dose of pembrolizumab, she developed painful tingling sensations in the hands and feet with pseudoathetoid (waver) movements in the arms and hands and a progressive unsteady gait. She was admitted to the hospital for worsening neurologic symptoms and was first seen by the neurologist.
On examination, she had a loss of sensation of the right face, generalized absence of deep reflexes, and loss of vibration and positional senses in the index and big toes. Nerve conduction studies revealed the absence of sensory nerve action potentials (SNAPs) in the upper and lower limbs and reduced compound muscle action potentials for the median and ulnar nerves but not for the peroneal and tibial nerves. The brain MRI revealed enhancement of the right trigeminal Gasser’s ganglia and its mandibular branch (Fig. ). The cerebrospinal fluid (CSF) analysis showed a high level of protein (1317 mg/l, normal: < 460) and pleocytosis (92 leukocytes/μl, normal: < 5), with negative cultures, suggestive of aseptic meningitis as CSF PCR testing was negative for herpes-simplex types 1 and 2, varicella-zoster, cytomegalovirus, Epstein-Barr virus, HHV-6 and polyoma JC-virus. Extensive work-up excluded infection with HIV, Borrelia burgdorferi, syphilis and hepatitis viruses. A diagnosis of sensory neuronopathy (SNN) was considered probable based on the pattern of her neuropathy with a Camdessanche’s score of 11 (normal: < 6.5) []. The patient was at this time restricted to a wheelchair, unable to stand and walk, and the overall neuropathy limitations scale (ONLS) was 9 out of 12 (disability in both arms preventing all function, requires wheelchair to travel 10 m, but able to stand up and walk 1 m) []. The patient received intravenous pulses of methylprednisolone (1 g/d) for 5 days and improved progressively over 2 weeks, with an ONLS score passing from 9 to 5 (moderate disability in the upper limbs; walks with unilateral aid for 10 m). She was discharged to a rehabilitation center.
One month later, the patient was readmitted to the hospital for severe ataxic relapse, with an ONLS score of 11, and was restricted to her bed. Investigations were completed by an 18FDG-PET-CT that found no signs of melanoma recurrence. Blood testing revealed an important inflammatory syndrome with CRP levels at 18 mg/l (normal: < 10); erythrocyte sedimentation rate > 110 mm/h (normal: < 20); hemolytic anemia hemoglobin at 98 g/l (normal: 117 to 157), with haptoglobin at 0.1 g/l (normal: 0.3 to 2.0), LDH at 286 U/l (normal: 135 to 214), total bilirubin at 67 μmol/l (normal: 0 to 21), lymphopenia at 0.8 G/l, an elevation of the total IgG levels to 29.9 g/l, (normal: 7.00 to 14.50) and presence of autoantibodies such as antinuclear antibodies (ANAs): anti-SSA (52 kd at 48 CU, normal level: < 20, and 60 kd at 108 CU, normal level: < 20). A panel of 45 antibodies involved in neurologic paraneoplastic syndromes was negative, including antineuronal (anti-HU and anti-Yo) and antiganglioside antibodies (such as GQ1b). The following analyses were negative or normal: antineutrophil cytoplasmic antibodies (ANCAs): c-ANCA proteinase (PR3), p-ANCA myeloperoxidase (MPO) and atypical ANCA (x or a-ANCA); rheumatoid factor; blood complement C3/C4; creatine kinase; urine spot with proteinuria and creatininuria; creatinemia and serum electrolytes. Of note, ANAs were negative in the serum sample that had been collected prior to the initiation of pembrolizumab. A biopsy of the accessory salivary glands (ASGB) showed abnormal interstitial sclerosis with a focus of > 50 lymphocytes/4 mm2 (Chisholm and Mason’s score of 3 out of 4) (Fig. ). The findings above led to a diagnosis of an induced Sjögren’s syndrome (6 points according to the 2016 ACR/EULAR classification criteria for Sjögren’s syndrome) [] associated with peripheral nervous system impairment.
After this relapse, an empirical “upfront” treatment with pulses of intravenous methylprednisolone at 1 g/d and immunoglobulins at 0.4 g/kg (both for 5 days) were introduced in combination with acyclovir (which was stopped after negative HSV PCR results in the CSF). In the absence of clinical improvement, a second-line treatment was initiated with cyclophosphamide at 15 mg/kg (one dose) in association with oral prednisone at 60 mg/d. Once the neuro-Sjögren diagnosis was established, cyclophosphamide was replaced with rituximab administered at 375 mg/m2 per dose once a month (for 4 administrations at weeks 0, 2, 6 and 10). The decision to change the therapy was made based on the similar efficacy of rituximab and cyclophosphamide for the treatment of the neurologic manifestations of Sjögren’s syndrome and to minimize the risk of T cell suppression and melanoma recurrence.
The clinical improvement was then rapid, with a progressive amelioration of the ONLS score from 11 to 5. The biological parameters improved in parallel, such as normalization of the levels of hemoglobin, total bilirubin, IgG and erythrocyte sedimentation rate (Fig. ). The brain MRI showed a marked regression of the enhancement of the trigeminal nerve (Fig. ).
After six monthly rituximab infusions, we observed the total disappearance of B-cells in a second salivary gland biopsy and normalization of the biological markers, but the patient had persistent neurological deficits, with the absence of deep reflexes, loss of sensation in the distal part of the extremities, and imbalance when walking with bilateral aid for 10 m.
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pmc-6196561-1
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Our patient was a 24-year-old Australian female with a clinical history of atopy with childhood asthma, eczema and allergic rhinitis, and lymphocytic enteritis complicated by B12 deficiency diagnosed age 23. Genetic studies revealed the patient is HLA DQ2/8 negative and her enteritis was successfully treated with oral budesonide. She also manifested immunodeficiency with recurrent lower respiratory tract infections (age 23), vulval/cervical intraepithelial neoplasia (age 22) and melanoma (age 24), some of which preceded her immunosuppressed state. The family history was notable for combined variable immunodeficiency (CVID) in her brother and maternal aunt, both with autoimmune features, and her mother had alopecia (Fig. a; Tables , ). Despite the above characteristics, the patient and her family had no formal diagnosis but had been referred for review by a specialist immunologist.
Five years prior to her current presentation our patient was diagnosed with RRMS at age 19 in the setting of recurrent optic neuritis and demyelinating lesions on MRI (Table ). She had progressed through several lines of RRMS therapy including dimethyl fumarate, fingolimod and natalizumab. Dimethyl fumarate was discontinued due to lymphopaenia (age 20) and fingolimod was ceased due to immune thrombocytopenic purpura (ITP) (age 22), a reported complication of fingolimod therapy []. Natalizumab therapy was also discontinued, due to presumed natalizumab-induced interstitial lung disease (age 22) []. This diagnosis was supported by bilateral patchy nodular infiltration with ground glass opacities and interlobular septal thickening on high resolution computed tomography, bronchoscopy and biopsy that showed no granulomatous inflammation or features of malignancy. The patient was commenced on daclizumab therapy.
Following 3 months of daclizumab therapy, our patient presented to hospital with an exacerbation of lymphocytic enteritis and was given a diagnosis of combined immunodeficiency with hypogammaglobulinaemia, reduced B cells and switched memory B cells with absent pneumococcal vaccine responses; T cell lymphopenia with reduced naïve T-cells (Table ). The patient initially declined regular intravenous immunoglobulin (IVIg) infusions to treat hypogammaglobulinaemia.
Six months following commencement of daclizumab therapy, our patient presented to hospital with a 1-week history of nausea, vomiting, tachycardia and high fevers (> 39 °C). Further assessment revealed an absolute neutropaenia (0.0 × 109/L) and elevated C-reactive protein. No clear infective cause was found despite numerous investigations, including blood, bone marrow, urine and stool cultures. Bone marrow biopsy revealed an agranulocytosis with an apparent maturation block at the myeloblasts stage but no features of hemophagocytosis and Epstein–Barr virus (EBV) in situ hybridization was negative (Fig. b–d).
Daclizumab was suspected to be the underlying cause of agranulocytosis and was ceased. However, there was minimal improvement for several weeks despite cessation of daclizumab and treatment with broad spectrum antibiotics and antivirals, IVIg and the neutrophil granulopoiesis stimulant, granulocyte-colony stimulating factor (G-CSF). On commencement of T cell immunosuppressive agents including systemic corticosteroids and methotrexate, the patient made a rapid recovery with resolution of fever and agranulocytosis within 3 weeks (Fig. e, f). The patient has not received any further daclizumab therapy and remains well on low dose prednisolone and methotrexate.
Whole-exome sequencing was performed in the proband and her brother. A novel heterozygous missense variant in CTLA4 was identified (c.410C > T; p.Pro137Leu in exon 2 of CTLA4; Fig. a). Their mother was also confirmed to carry the mutation, and the aunt is awaiting clinical testing. This novel variant affects the CTLA-4 protein at a highly conserved amino acid in an essential functional domain and is classified as ‘likely pathogenic’. No additional mutations were identified in our patient.
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pmc-6196570-1
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A 35-year-old Arabic female, who was not known to have any medical illnesses, presented to the emergency department (ED) of a secondary hospital for the third time with the same complaint: a throbbing headache for the past 2 days that did not respond to analgesics (Table ). The migraine-like headache was in the left frontal area, moderate in severity, and sometimes severe enough to interrupt her sleep. It was continuous with no relieving or exaggerating factors. Her headache was accompanied by two episodes of vomiting. She denied having a migraine in the past or any similar kind of headache. There was no history of fever, photophobia, or change in behavior or personality. No history of abnormal movement or loss of consciousness was reported. Furthermore, she had no history of weakness or loss of sensation, or gate or posture abnormalities. She denied diplopia, vision loss, or dysphagia. There was no previous history of venous thromboembolism (VTE) or any hematological diseases in her family. There was a positive family history of stroke at a young age. Other systematic reviews were unremarkable.
On physical examination, the patient walked to the observation area in the ED. She looked a bit tired, hypoactive, and in mild pain distress. She was hemodynamically stable, and her vital signs were temperature 37 °C, blood pressure 117/68 mm Hg, and pulse rate 71 beats per minute. Her respiratory rate was 18 per minute, and oxygen saturation was 99% in room air. General examinations revealed no neck rigidity, the cranial nerves were intact, and there was no proptosis. Her chest was clear, and her abdomen was soft and not tender. Neurologically, she was completely conscious, alert, and oriented to time, place, and person. Her Glasgow Coma Scale (GCS) was 15/15 with no focal neurological deficit, and the power in all four limbs was 5/5, with normal reflexes, and equally reactive pupils. The Brudziniski and Kerning signs were negative. The Babinski reflex went down. Fundus examination was not performed.
She had been seen twice before in the ED by two junior general practitioners who diagnosed her condition as migraine. They attributed her migraine to an anemic state and oral contraceptive pills, which were taken recently to regulate her menstrual cycle. They therefore discharged her after prescribing oral analgesics and diclofenac injection. During her third visit, an emergency consultant raised the suspicion of CVT based on the following red flags: recurrent visits to ED, no previous migraine in the past, headache interfered with sleep, and recent use of oral contraceptives. Plain computed tomography (CT) of the brain was performed initially and showed hyperdense signs in the left transverse sinus and straight sinus (Fig. ). Early in the morning, a brain venogram was conducted which confirmed left transverse sinus thrombosis, inferior sagittal sinus, straight sinus, and deep venous thrombosis in the confluence sinuses (Figs. , and ).
During the therapeutic interventions, the patient was referred to the neurologist on call who initiated a therapeutic dose of low-molecular weight heparin (LMWH; enoxaparin) 1 mg/kg subcutaneously. The patient was admitted to a regular ward in good condition. Twelve hours following the ED visit, in the evening, her level of consciousness deteriorated. She became agitated, confused, disoriented, and talked only a few words inappropriately. Her GCS dropped to 12/15 (eye opening 4, verbal response 4, motor response 4) and both her pupils were reactive equally to light measuring 4 mm.
She was evaluated by a critical care physician on call who intubated her electively and moved her to the intensive care unit (ICU) for monitoring. He advised urgent CT brain scan and to initiate phenytoin 900 mg intravenously. Two hours later, the plain CT showed a newly developed third ventricular hemorrhage and deep venous thrombosis in the vein of Galen and left transverse venous sinus thrombosis (Fig. ).
Her neurologist saw her 12 hours later in the morning round who attributed the deterioration in her condition to the increase in intracranial pressure. The neurologist advised to discontinue phenytoin and continue enoxaparin in the same therapeutic dose (60 mg subcutaneously). Acetazolamide (500 mg intravenously) followed by 250 mg twice daily was started. Moreover, a neurosurgeon, who was consulted, evaluated her 10 hours later and agreed to proceed with an extraventricular drain (EVD) to relieve the early hydrocephalus. However, despite EVD insertion, a CT scan of her brain showed an increase in brain edema with an acute increase in the intraventricular pressure and transtentorial herniation resulting in hydrocephalus and left thalamic ischemia (Figs. and ). A multidisciplinary team from neurology, neurosurgery, and ICU decided to perform bi-frontal craniotomy as brain decompression surgery (Fig. ). One day following the craniotomy, she developed left pupil dilatation, and an urgent CT brain scan showed a large extracranial (epidural) hemorrhage in the left frontoparietal area, with generalized brain edema and periventricular ischemia (Figs. and ). Her condition required urgent evacuation, and enoxaparin was switched to low therapeutic range heparin at a dose of 40 mg subcutaneously daily. A few days later, a follow-up brain CT scan revealed newly developed ischemic areas in the left parietal and occipital lobes and a significant reduction in the hemorrhage. Furthermore, during her ICU stay, she developed surgically induced meningitis and multiple chest infections. The patient ended up in the ICU on tracheostomy with mechanical ventilation, in a permanent vegetative state and in need of long-term care.
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pmc-6196575-1
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A 50-year-old male patient with aortic dissection originating just above the aortic valve and extending down to the common iliac arteries (Stanford A, Figure ) underwent immediate surgery with repair of the ascending aorta in moderate hypothermia under cardiopulmonary bypass. The arterial line was inserted in the right axillary artery, the vent was placed in the right upper pulmonary vein, and two-stage venous line was inserted through the right auricula. The intact aortic valve was resuspended. Extracorporeal circulation was suspended after induction of moderate hypothermia (25°C) and the aortic cross-clamp from the ascending aorta was removed. The false lumen was then glued and an open distal anastomosis to a prosthetic graft was constructed. Then, extracorporeal circulation was resumed, systemic circulation was deaired and the patient was warmed.
Early on the first postoperative day, CT of the aorta was requested because of marked elevation of lactate-dehydrogenase (129 μkat/l) and signs of acute renal insufficiency (creatinine 292 μmol/l). CT showed good postoperative result in the ascending aorta, but large amount of air in the branches of the superior mesenteric artery up to the arcades was found (Figure ). There was absolutely no air in the portal-venous system including the liver. The bowel loops were not distended, and there were no signs of bowel paralysis.
At midnight on the first postoperative day, a biphasic CT scan of the abdomen was requested due to elevated intra-abdominal pressure (18 mmHg). The CT showed distribution of the intra-arterial gas more into the periphery and into the wall of the bowel loops that still did not display signs of ileus (Figure ). The next morning, surgical exploration was performed due to increasing intra-abdominal pressure as a sign of imminent abdominal compartment syndrome. An extensive bowel resection from oral ileum down to the splenic flexure had to be performed due to extensive necrosis of the bowel. Shortly after the operation, the patient became hypotensive with signs of overwhelming vasoparalysis and died on the third postoperative day of multiple organ dysfunction syndrome (MODS) with systemic inflammatory response syndrome (SIRS).
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pmc-6196765-1
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The patient was a 78-year-old Chinese gentleman who presented with fever, cough, diarrhoea and vomiting, of 1 day duration. Significant comorbidities included ischaemic heart disease (IHD), chronic obstructive pulmonary disease (COPD), bronchiectasis, peptic ulcer disease, hypertension and hyperlipidaemia.
Other than bilateral crepitations in the lung, the physical examination was otherwise unremarkable. Initial investigations were significant for a mild leucocytosis, acute kidney injury and mild hypokalaemia (Table ). Chest and abdominal radiographs were unremarkable. Treatment was initiated for an infective exacerbation of bronchiectasis. Blood cultures and stool microbiology studies were sent off, and intravenous Co-amoxiclav and hydration were started.
Blood culture initially was reported as positive of gram negative rod. An ultrasound of abdomen was done. It showed no intra-abdominal abscess. Over the next few days, stool and blood cultures came back positive for non-typhoidal Salmonella species sensitive to ampicillin, ceftriaxone and cotrimoxazole. Salmonella serovar was not specified due to the hospital laboratory protocol. A computed tomography (CT) aortogram (Fig. ) was done to look for aortitis as our patient has significant atherosclerosis. It did not show aortitis; however, an enlarged (11–12 mm in diameter), fluid filled appendix with hyperenhancement of the wall, minimal adjacent fat stranding and small mesenteric nodes in the right iliac fossa, was found. This was consistent with acute inflammation of the appendix and likely reactive lymphadenopathy. There was no evidence of abscess or perforation. A surgical and radiological consult was obtained, both agreed that the findings were in keeping with acute appendicitis. The decision was made to proceed with conservative management in view of poor premorbid status and multiple comorbid conditions.
The patient completed 2 weeks of intravenous ceftriaxone. Repeat blood culture did not show persistent salmonella bacteraemia. Acute kidney injury resolved and the patient was discharged well.
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pmc-6196789-1
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A 9-year-old girl was presented to our hospital on referral from a peripheral hospital with a one-week history of darkish discoloration of both feet and her left hand and a six-day history of a purulent feculent discharge from an exploratory laparotomy wound. She had presented to the referring hospital eight days earlier, with a two-week history of high-grade fever and a one-week history of a generalized abdominal pain and diarrhea. She had an exploratory laparotomy at the referring hospital. Intraoperative findings include a feculent intraperitoneal collection and a single perforation on the antimesenteric border of the terminal ileum. An intraoperative diagnosis of perforated typhoid enteritis was made, and the perforation was closed in two layers. One unit of blood was transfused postoperatively. On the first day of postsurgery, the patient developed darkish discoloration of the left hand and both feet which were associated with pain. She was not a known sickle cell disease patient and had no past histories suggestive of intermittent claudication. On the second day of postoperation, a purulent discharge from the lower aspect of the laparotomy wound was noted, and there was darkening of the feet and duskiness of the distal one-third of both legs. Due to the progressive worsening of the patient's condition, she was referred to our hospital on the seventh day of postoperation.
At presentation to our unit, she was acutely ill looking, febrile with a temperature of 38.1°C, dehydrated, not pale, and anicteric. Her respiratory rate was 30/minute with reduced air entry and coarse crepitations in both lower lung fields. She had a pulse rate of 130/minute, which was regular and of moderate volume. Only first and second heart sounds were heard. Her abdomen was full, and did not move with respiration, and had a midline dressing soaked with a feculent fluid. There was a complete wound dehiscence of the abdominal wound, with both edges being necrotic. There was no bowel evisceration. Both feet were dark and cold, with duskiness of the skin of the distal one-third of both legs (). There was loss of sensation in the feet and absent dorsalis pedis, anterior and posterior tibial pulses bilaterally. Popliteal pulses were palpable bilaterally. The left hand was dark, dry, and shriveled up to the wrist. The radial pulse was however present. The right upper limb was normal. A diagnosis of complete wound dehiscence, fecal fistula with peripheral gangrene of both feet and left hand in a patient who had surgery for typhoid intestinal perforation, was made. The patient was commenced on intravenous (IV) fluid resuscitation and intravenous antibiotics (ceftriaxone and metronidazole). She was placed on nil per os (NPO) and nasogastric decompression of the stomach. A consult was sent to the orthopedic surgery team who advised that the gangrene be allowed to demarcate before any decision could be taken on the limbs.
Hematological investigations after fluid resuscitation showed a hematocrit of 23.9% and a white blood cell count of 18.9 × 109/l and a hemoglobin genotype of AS. She was transfused with 200 ml of packed cells, in preparation for a wound exploration. She was however noted to be making scanty urine, and due to her unstable clinical condition, the surgery was postponed while IV fluid resuscitation and local wound care continued. By the fourth day of admission in our unit, the left upper limb gangrene had extended to the distal forearm, while on the lower limbs, the gangrene had extended to the middle aspect of the legs. The patient's condition continued to deteriorate in spite of efforts at resuscitation. She died on the eighth day of admission in our unit.
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pmc-6196789-2
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A 4-year-old boy was presented to our unit on referral with a three-week history of high-grade fever and a five-day history of generalized abdominal pain and abdominal distension. There was an associated history of headaches and body weakness. He had no history of jaundice. He had several episodes of vomiting which was initially none bilious, but later became bilious. There was an associated history of passage of diarrhea stools two days before presentation, although the patient had not passed stools on the day of presentation. He had no history of passage of melena or of hematochezia. His parents complained that he had been passing scanty urine for about five days before presentation. He had been receiving medications from the referring hospital for about two weeks before presentation.
At presentation, he was chronically ill looking with a toxic facie, febrile (temperature 38.5°C), pale, dehydrated, anicteric, and had no pedal edema. His respiratory rate was 28/minute; he had reduced air entry on both lung bases posteriorly. His pulse rate was 128/minute, which was regular but of small volume. Only first and second heart sounds were heard. Abdominal examination showed a distended abdomen which did not move with respiration. He had generalized tenderness with guarding. The bowel sounds were absent. A digital rectal examination showed an empty rectum with a full and tender rectovesical pouch. An initial assessment of a generalized peritonitis was made. The patient was placed on NPO with nasogastric tube for gastric decompression. He was commenced on IV fluid resuscitation and broad spectrum IV antibiotics (ceftriaxone and metronidazole) and was worked up for surgery. Initial laboratory investigations showed a hematocrit of 24% and hypokalemia (2.5 mmol/l) and a serum urea of 10 mmol/l. After an initial fluid resuscitation and correction of serum potassium, he had 300 ml of whole blood transfused. He had an exploratory laparotomy on the second day of admission. At surgery, about 600 ml of a feculent peritoneal fluid was drained, and a single perforation on the antimesenteric border of the terminal ileum was closed in two layers. An intraoperative diagnosis of perforated typhoid enteritis was made. Blood and tissue cultures were not done. He was continued on IV fluids and the same empiric antibiotics after the surgery.
On the third day of postoperation, the abdominal wound had a purulent discharge from the distal end. The wound was opened and stitches were removed from the site of drainage, with the institution of daily wound dressing. By the fifth day of postsurgery, a dark patch was noticed to have developed in the skin over the right iliac fossa and measured about 5 cm in its widest diameter. At about the 7th day of postoperation, the dark dry patch had extended to the right hypochondrium, and a similar patch had appeared along the edges of the abdominal incision and the left iliac fossa. A diagnosis of anterior abdominal wall gangrene was made. On the 8th day of postsurgery, the patient was noticed to have developed a complete wound dehiscence () and a fecal fistula. He had fluid resuscitation and was taken back to the theatre on the 10th day after the first surgery, for wound exploration and debridement of the anterior abdominal wall gangrene. A new intestinal perforation was seen at about 2 cm from the initial perforation. The intestinal perforation was exteriorized as an ileostomy, and the abdominal wound closed with tension sutures. Postoperatively, the patient's condition remained unstable, with intractable shock. He died six days after the reexploration of septic shock.
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pmc-6196796-1
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A 62-year-old female patient presented after an episode of light-headedness followed by fall, loss of consciousness, and amnesia for several minutes. She gave a history of use of laxatives for the complaints of constipation and lower abdominal discomfort. A week prior, she had an episode of near syncope. She reported gradually progressive back pain of four months for which she left her job at a school cafeteria few months prior. There was no history of fever, cough, night sweats, weight loss, or burning micturation. She smokes half a pack per day for forty years. She denied alcohol intake. On examination in A/E, she was awake, alert, oriented, and afebrile with stable vitals and normal extremities and chest was clear, cardiac exam with a normal sinus rhythm, no murmurs. She had mild paravertebral tenderness, right lower back worse than left.
CT scan brain plain showed small bilateral SAH in posterior Sylvian fissures and small left IVH. CT scan of thoracic spine indicated sclerosis at T8 and T9 with a large paraspinous mass to the right of the midline at T8 and T9, asymmetric T8-T9 disc space widening. The diagnosis was confirmed on MRI thoracic spine as discitis osteomyelitis at T8-T9 with an associated 1.6 × 0.8 × 1.5 cm mature rim-enhancing right anterior paravertebal abscess (Figures and ). There was abnormal enhancement and STIR hyperintense signal in the anterior most portion of the T8-T9 disc space with small adjacent endplate erosions along with diffuse marrow edema and enhancement throughout the T8-T9 vertebral bodies. No posteriorly directed epidural abscess in the spinal canal was identified. At T10-T11, a small right central disc protrusion resulting in mild deformity of ventral thecal sac, but no spinal cord impingement, was observed.
S. pneumoniae was isolated as a direct sample of the spinal tissue biopsy on the primary culture plate, and also she underwent needle aspiration of paravertebral abscess resulting in purulent material from the paraspinous soft tissue mass that cultured streptococcal pneumoniae (). Lab results showed TLC: 18900/µL, Hb: 13.4 g%, Plt: 379000/µL, erythrocyte sedimentation rate (ESR): 78 (normal 0–30), and CRP: 82 (normal 0–5). She had normal serum protein electrophoresis (SPEP), quantitative immunoglobulins, negative blood cultures, and negative transesophageal echocardiogram (TEE). Gallium scan was performed to rule out alternative etiologies for bacteremia, which indicated uptake in the pubic area. MRI pubis symphysis confirmed osteomyelitis and septic arthritis of the pubic symphysis and pubic rami. The rate of penicillin resistance was high with S. pneumoniae, and resistance to ceftriaxone is negligible. She was treated with Unasyn as inpatient transitioned to high-dose ceftriaxone (2g IV 12 hourly) for 6 weeks. She subsequently transitioned to Keflex (500 mg four times a day) for 4 weeks. Repeat CT scan showed resolution of the abscess. She had no neurological sequelae, no apparent adverse effects of medication, and significant decrease in pain.
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pmc-6196887-1
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A 35-year-old male patient reported to the dental clinic with a history of sharp pain in the left lower back region for the last two days. Clinical examination revealed a deep carious lesion in the left mandibular third molar and a missing left mandibular second molar, extracted two years back. The oral findings were confirmed with an intraoral periapical (IOPA) radiograph depicting a deep carious lesion approaching the pulp in the left mandibular third molar. The IOPA radiograph further revealed curved mesial and distal canals, a sickle-shaped curvature extending from the middle half of the root till apex (). Pulp vitality tests (cold and electric pulp test) confirmed the diagnosis of symptomatic irreversible pulpitis. The patient had an intention to restore the missing mandibular second molar; hence, an endodontic treatment was planned for mandibular first and third molar in view of providing a fixed partial denture.
After adequate local anaesthesia and isolation with a rubber dam, the access cavity was prepared using Endo Access kit (Dentsply) in the mandibular left third molar. After gaining an adequate access, initial scouting of all the root canals was done with K-file no. 10, and the patency of root canals was established. Gates Glidden (GG) drills were placed sequentially in a step-back fashion (i.e., nos. 1, 2, and 3) to allow easy placement of instruments and to gain a straight line access to the apex. The working length was confirmed using an apex locator (Root ZX J. Morita) and SS K-file no. 15 through an IOPA radiograph (). Succeeding, path finder files (Dentsply) of intermediate sizes, i.e., no. 13, no. 16, and no. 19, were used in order to closely follow the curvature and maintain the apical spatial orientation. Each filing sequence was accompanied with 17% EDTA (Glyde, Dentsply) followed by copious irrigation with saline and 3.2% NaOCl. The rotary Hero Shaper files were subsequently used in the fashion as instructed by the manufacturer (20-0.6, 20-0.4). Following the biomechanical preparation, the canals were irrigated, flushed with EDTA 17%, and dried prior to obturation. Single cone 4% taper gutta percha cones (Dentsply) were used to obturate all the canals (). The post obturation restoration was done with a composite to maintain a good coronal seal (). Similarly, endodontic treatment was executed for 36. A three-unit bridge was given to the patient finally.
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pmc-6196918-1
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An approximately four-year-old female intact Staffordshire Bull Terrier was presented for evaluation of a large and fast-growing mass on the front of lower jaw, involving several teeth (). The dog was rescued from a pound by an animal rescue shelter about 3 weeks prior to evaluation. Upon evaluation, the dog was underweight, was malnourished, and has a grade II/VI systolic heart murmur on cardiothoracic auscultation. She received a rabies vaccination and was placed on steroids, gabapentin, and antibiotics.
A large ulcerated mass was present on the rostral portion of the mandible. Full mouth dental radiographs revealed a mass that encompassed all of her lower incisors as well as her left canine and premolars. Her abdomen palpated soft and nonpainful. On palpation, her submandibular and prescapular lymph nodes were enlarged. The dog was anesthetized and a computed tomography (CT) scan of the head was performed with contrast. The CT scan revealed a large, interosseous, and expansile soft tissue attenuating mass at the most rostral aspect of the mandible, involving teeth, jaw bone, and oral membranes, and expanding almost to the frenulum of the tongue. There was a bilateral symmetrical enlargement of the mandibular lymph nodes with moderate heterogeneity following contrast. Both retropharyngeal, both prescapular and right superficial cervical lymph nodes were also enlarged. Histopathology of tissues from the mandibular mass was consistent with papillary squamous cell carcinoma. Cytology of the submandibular lymph nodes indicated reactive lymphoid hyperplasia and neoplasia. Because the mass was quite expansive and the cancer had metastasized, surgery intervention with a goal of achieving 12-15 mm margins without involving the frenulum was impossible
Shortly after diagnosis, the dog was accepted into the HylaPlat chemotherapy study sponsored by HylaPharm (Lawrence, Kansas). The dog received four intralesional injections of HylaPlat under anesthesia at three-week intervals on days 1, 22, 43, and 64. The doses ranged from 5 to 10 mg/m2 (mg of chemo per m2 of body surface area). The dog weighed 50.8 ± 1.2 lbs throughout the entire study. All four treatments went smoothly and recovery after sedation was uneventful. One hour after the first treatment, a blood sample was collected to determine the systemic exposure of the medication. Clinical chemistry and complete blood counts were performed approximately biweekly between treatments to evaluate the tolerability of HylaPlat and the results were compared to the prestudy values. Specifically, the blood samples were collected on days 0 (prior to the study), 8, 18, 26, 30, 36, 40, 47, 51, 55, 58, 61, and 68. Prior to each treatment, tumor size was measured using a caliper to monitor patient's response to the chemotherapy.
Clinical chemistry indicated that liver enzymes (AST, ALT, and ALP) were within normal limits during the entire study except on day 58 when AST and ALT were temporarily elevated and over the upper limit (AST was 157 IU/L with a REF range of 15-66 IU/L; ALT was 161 IU/L with a REF range of 12-118 IU/L). All three liver enzymes returned to normal range by day 68 (). Total bilirubin was also monitored and the results were within the normal limits during the entire study. According to the Veterinary Cooperative Oncology Group-Common Terminology Criteria for Adverse Events (VCOG-CTCAE), a Grade I AE may be considered for the asymptomatic, transient elevation of liver enzymes as medical intervention was not indicated.
Because platinum chemotherapy may induce tubular injuries and may cause nephrotoxicity, renal function was monitored by BUN and creatinine tests during the study to assess systemic tolerability []. Test results demonstrated that neither BUN nor creatinine was elevated during the treatment period. Both values were within normal limits (Supplemental ). According to the VCOG-CTCAE, no renal AEs were reported.
A complete blood count with differentials was performed to determine the patient's general health status. Specifically, neutrophil and hematocrit counts are monitored to determine whether chemotherapy significantly affects hematopoietic effects and myelosuppression. Platelet count is also evaluated as myelosuppression is one of the systemic intolerabilities of platinum-based chemotherapies. Results indicated that neutrophil counts at pre- and posttreatments were not statistically different (p>0.05, AVONA, GraphPad Prism), suggesting that HylaPlat chemotherapy did not result in bone marrow suppression for this patient (). Similarly, hematocrit did not alter significantly between pre- and posttreatments (p>0.05, AVONA, GraphPad Prism), indicating that HylaPlat chemotherapy did not significantly compromise hematopoiesis (). Because thrombocytopenia is a potential side effect of platinum chemotherapy, the patient's platelets were monitored during the study. Temporary thrombocytopenia was reported from day 4 to 8 after the third treatment (). The effect was transient and the patient's low platelets resolved without medical intervention on day 12 after the third treatment. According to the VCOG-CTCAE, a Grade I AE may be considered for the asymptomatic, transient thrombocytopenia as medical intervention was not indicated.
The patient received four treatments of HylaPlat at 3-week intervals. The doses were 5, 7.5, 10, and 7.5 mg/m2. The mass measured 5.2 cm by 5.1 cm by 3.3 cm on the day of the first treatment. After the first treatment, owner reported that the dog ate well and acted normally and no side effects were observed. Prior to the treatment the front of the tumor bled easily and after the first treatment the bleeding stopped. Three weeks after the first treatment, the dog returned for the second injection. The attending veterinarian reported that the dog's left submandibular lymph node was not as enlarged as before the first treatment on palpation. The tumor measured wider across (7.1 cm by 5.4 cm by 3.9 cm); however, the appearance of the mass improved visually with less purulent discharge and less observed bleeding. After the second treatment, the dog ate well and acted normally. Necrotic pieces of tumor tissues were observed to slough off days after the treatment. The exposed blood vessels caused by the sloughing tissues resulted in bleeding. The bleeding was managed by administration of a tranquilizer (acepromazine) to lower blood pressure and reduce activity. The dog responded to the medication and the bleeding stopped. On the day of the third treatment, the tumor measured slightly smaller on two dimensions and slightly larger on the third dimension (6.7 cm by 5.6 cm by 3.8 cm) compared to measurements made at the second treatment. After the third treatment, the owner reported that more tumor tissues fell off and the dog had heavy bleeding from the sloughing mass for 3 days. The bleeding complication may be associated with the effect of the chemotherapy (e.g., killing cancer cells, promoting tumor necrosis) as well as the friable nature of the tumor (e.g., tissue sloughing, exposing live blood vessels). Besides these symptoms, the dog acted healthy and ate well. Three weeks later, the dog received the fourth and the last injection. The tumor measured slightly smaller on two dimensions and slightly larger on the third dimension (6.5 cm by 5.5 cm by 4.8 cm) compared to measurements made at the third treatment, which we considered to be stable. Clinically, the patient was doing well; however, the tumor did bleed when it was debrided. The owner reported that the dog ate well and did well after the fourth treatment. On day 7 after the fourth treatment, heavy bleeding began from the posterior of the tumor. The dog had a single, brief seizure, the cause of which was unknown, though considerations may include blood loss, idiopathic epilepsy, liver disease, kidney failure, brain tumor, and other possible seizure-triggering conditions. The dog responded to diazepam and seizure did not recur (Grade II AE per VCOG-CTCAE). Additional diagnoses were not performed. After recovery from the ictal period, the dog was assessed as neurologically healthy.
Approximately one month after the last treatment, radiographs, CT, lymph node biopsy, and a physical exam were performed to determine the status of the tumor and evaluate the feasibility of a mandibulectomy to remove the SCC. Chest radiographs suggested no evidence of metastasis to the lungs. CT of the head demonstrated that the margins of the mass had regressed compared to the CT prior to the chemotherapy and were no longer invading the lingual frenulum (). Histopathology of the enlarged mandible lymph node had no evidence of neoplasia. Based on the findings of the exams, the patient became eligible for mandibulectomy to remove the SCC.
The dog was placed under general anesthesia and underwent surgery to have an enlarged lymph node and the SCC removed. Surgery went as planned and without complication. She was laid on her left side and an incision was made on the right side of her neck to have the enlarged retropharyngeal lymph node removed. Then she was placed on her back and had most of her lower jaw removed in an effort to achieve clean margins around the SCC. The jaw was cut just in front of her first molar on the left side and just behind her third premolar on the right side. She recovered uneventfully following surgery.
Histopathology of the mandibular mass indicated that soft tissue excision was complete with clean margins. Histopathology of the right retropharyngeal lymph node was consistent with the reactive lymph node. In the days following surgery, the dog had moderate-to-severe edema and swelling around her surgical sites, which may be resulted from disruption of the lymphatic system considering the surgical locations, inflammation caused by the rostral bilateral mandibulectomy, or possible infections of the tissues and the mandibular bone at the surgical site. In an attempt to prevent infection at this site, a short course of antibiotics (Cephalexin, 500 mg capsules, one capsule every 12 hours for 7 days) was recommended. The dog was present 4 weeks after the surgery and a physical exam indicated that the healing of the surgical site was satisfactory (). Over one year after the chemotherapy treatments and surgery, the dog is doing well and cancer remains in complete remission.
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pmc-6196920-1
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A 75-year-old male presented to the hospital with a 2-month history of decreased leg strength bilaterally, weakness and urinary retention. He had an extensive workup, including MRI of the head and spine and lumbar puncture. MRI showed multiple, nonspecific, small, scattered supratentorial white matter T2 hyperintensities worrisome for demyelination within the brain and also in the spine.
He was clinically diagnosed with transverse myelitis and started on methylprednisolone. His symptoms improved following this treatment, and he was doing well. While on the rehabilitation ward, he began to have medical issues requiring further assessment. These included a macular rash over his lower extremities and back, anaemia, cognitive decline, lymphadenopathy, and lung infiltrates which were found on chest X-ray. He underwent bronchoscopy with bronchoalveolar lavage, skin biopsy, bone marrow biopsy which were all reported as unremarkable. He had a computed tomography (CT) scan of the chest which showed bilateral ground glass opacities predominantly in the upper lobes with increased nodularity and small-volume mediastinal lymphadenopathy (). Laboratory tests were unremarkable except elevated serum lactate dehydrogenase (LDH) of 1491, C-reactive protein (CRP) of 39.8 and erythrocyte sedimentation rate (ESR) of 23. Eventually, video-assisted thoracoscopic surgery (VATS) resection was performed (wedge resection from right upper, middle, and lower lobes) and submitted for histopathological analysis.
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pmc-6196923-1
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A previously healthy 6-year-old boy presented to a pediatric hospital with a 3-week history of torticollis. He had symptoms of an upper respiratory tract infection four weeks prior and had 2 days of documented fever at home during that time. He had been treated with a 7-day course of amoxicillin by the primary care physician for suspected streptococcal pharyngitis. Four days into the course of antibiotics, he woke up from sleep with pain on the left side of his neck. Despite taking ibuprofen and acetaminophen, he presented to the Emergency Department 3 weeks later due to persisting torticollis. Pain was worse with movement. There was no history of head/neck trauma. At the time of presentation, the infectious symptoms had resolved. Some fatigue was noted but he remained generally active, continuing to play hockey. There was no history of rash, peripheral joint pain, or weight loss. Past medical history and family history were unremarkable.
On examination, the patient was afebrile with normal blood pressure for age and a maximum heart rate of 110 beats per minute. The patient's head was tilted to the right with chin rotation to the left. No lymphadenopathy or masses were noted on palpation of the neck. There was no tenderness to palpation of bilateral sternocleidomastoid muscles. There was a limited range of motion in all planes of rotation of the neck secondary to pain, particularly in lateral flexion. Bilateral injected conjunctivas were present. The oropharynx was normal with no erythema or mucus membrane changes. Cardiovascular exam revealed normal peripheral pulses, a quiet precordium with normal heart sounds, and no murmur. Respiratory exam was normal. The abdomen was soft with no distension, tenderness, or hepatosplenomegaly. There were no bruits heard on auscultation of major vessel regions. There were no rashes or desquamation of the skin. Neurological exam was normal.
At the time of presentation, laboratory investigations revealed an elevated white blood cell count of 17.4 × 109/L with a neutrophil count of 14.1 × 109/L. Hemoglobin was normal for age at 110 g/L. Inflammatory markers were elevated including platelet count of 860 × 109/L and CRP of 38.5 mg/L. Renal function (BUN and creatinine) and liver function (ALP and ALT) were normal for age. Because of the unexplained elevated white blood cell count and evidence of inflammation, a chest X-ray was performed which revealed normal lung fields but an enlarged cardiac silhouette. X-ray of the cervical spine was normal with no atlantoaxial rotary subluxation demonstrated. Ultrasound of the neck revealed mild thickening of the left sternocleidomastoid muscle and no lymphadenopathy. Abdominal ultrasound with Doppler was normal.
Additional investigations included a normal throat swab for group A streptococci and a negative anti-streptolysin O antibody titer. High-sensitivity troponin was elevated to 176 ng/L. Creatinine kinase was normal. ANCA was normal. Electrocardiogram showed normal sinus rhythms without evidence of chamber hypertrophy. The patient underwent an echocardiogram to further characterize the enlarged cardiac silhouette identified on the chest X-ray. This revealed massive ectasia and aneurysmal dilatation of the right coronary artery, left main artery, left anterior descending artery, and circumflex arteries, as seen in . Left ventricular function was normal. The aortic arch was normal as were the proximal neck vessels.
Because of the dilated coronary aneurysms, the patient was diagnosed with KD. Despite lack of fever, given the evidence of ongoing inflammation and initial presence of bilateral nonsuppurative conjunctivitis, in addition to the coronary artery changes, the patient was treated with high-dose IVIG (2 g/kg) and started on daily low-dose aspirin. Low-molecular-weight heparin was started as antithrombotic therapy and once stabilized, daily atenolol was initiated. Activity was restricted as much as possible.
Inflammatory markers were followed. Platelets revealed a peak of 952 × 109/L and CRP a peak of 54.6 mg/L. After treatment, both platelet and CRP levels normalized.
The patient's neck pain and the limited range of movement resolved immediately after treatment, as did the bilateral conjunctivitis. The patient was stable and appeared well at time of discharge. His aspirin, low-molecular-weight heparin, and atenolol were continued. The CT angiogram performed after discharge revealed massively dilated and aneurysmal coronary arteries, as shown in .
In follow-up cardiology and rheumatology clinics, he has been doing well with no further neck pain or stiffness. He did not develop desquamation during follow-up, and the repeat echocardiogram one month after discharge was unchanged. He will continue long-term anticoagulation therapy with low-dose heparin with a target level greater than 0.5 IU/ml. He will also continue low dose aspirin and atenolol. His family was advised to have the annual influenza vaccine.
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pmc-6196924-1
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A 24-year-old man arrived at the emergency department with a four-month history of pain and swelling of the left testicle. A week ago, he developed progressive edema in the lower limbs, which was followed 48 hours before admission by dysesthesia and paraparesis as well as urinary incontinence. He had no previous medical history and did not consume alcohol or use any illicit drug or medication.
Physical examination on admission showed a blood pressure of 100/60 mmHg, a temperature of 36°C, a pulse rate of 90/min, and a respiratory rate of 22/min; his height was 1.75 m, weight 98 kg, and BMI 32 kg/m2. He referred bilateral paresthesia of the lower limbs and pain on mobilization of the lumbar spine. The left scrotal sac was enlarged and indurated, and there was a mass in the left scrotum that was indistinguishable from the right testis and provoked displacement of the penis and right testis. No inguinal lymphadenopathy was identified. When examining both lower extremities, we found edema without fovea. Neurological examination revealed hypoesthesia, areflexia, and decreased muscle strength.
Laboratory tests revealed normal liver function. Hemoglobin was 9.13 g/dL, MCV was 87.9 fL, WBC was 11.9 K/μL, neutrophil count was 9.81 K/μL, lymphocyte count was 1.59 K/μL, and the platelet level was 252 K/μL. Serum glucose was 148 mg/dL, BUN was 38 mg/dL, creatinine was 1.3 mg/dL, and calcium was 8.9 mg/dL. Serum alpha-fetoprotein (AFP) was 11.28 ng/mL, lactate dehydrogenase was 1687 U/L (normal range 91–180 IU/L), and human chorionic gonadotropin level was 10.05 IU/mL.
A scrotal ultrasound showed a hypoechoic oval-shaped infiltrative lesion in the left testicle of 6.1 × 3.2 × 2.7 cm, associated with a large left hydrocele, with an estimated volume of 450 mL. Microcalcifications were seen in both testis.
A contrasted CT scan of the thorax, abdomen, and pelvis showed bilateral supraclavicular adenopathies and multiple round pulmonary nodules with well-defined edges of bilateral and diffuse distribution. In the liver, there were two hyperdense lesions: one larger lesion of 4.8 × 2 cm located in hepatic segment IV and a second lesion of 1.9 cm in segment VIII, with partially defined borders and enhancement to contrast administration. There was also a large retroperitoneal ganglionar conglomerate of 10 × 6 cm, which infiltrated the spinal cord.
An MRI of the dorsal and lumbar spine showed a large and heterogeneous retroperitoneal conglomerate which invaded the left psoas muscle and infiltrated the spinal cord through the intervertebral foramina of T11 with displacement of the L1 vertebral body (). Metastatic infiltration of the vertebral bodies L3–L5 was also seen ().
A diagnosis of clinical stage IIIC testicular cancer was established with a poor prognosis due to nonpulmonary visceral metastases. After initial treatment with high-dose intravenous corticosteroids, the case was evaluated by a multidisciplinary oncological team. Three fractions of external beam radiotherapy were given. He then underwent a left radical orchiectomy by an inguinal approach with a left hemiscrotectomy, without complications. A postoperative biopsy showed a pure embryonal carcinoma with invasion of the spermatic cord and necrosis of more than 50% of its surface. After finishing 10 fractions of radiotherapy for a total of 30 Gy, and improving his renal function with intravenous crystalloids, the patient received his first cycle of chemotherapy (CT). We planned a CT regimen based on bleomycin, etoposide, and cisplatin (BEP), repeated every 21 days for a total of four cycles.
After the eighth day of CT, the patient presented sudden dyspnea at rest that progressed to hypoxemic respiratory failure. Due to this condition, we decided to perform orotracheal intubation and the patient was then transferred to the intensive care unit. An angio-CT revealed the presence of bilateral pulmonary thromboembolism. Despite the treatment received, the patient died three days later.
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pmc-6197025-1
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A 30-year-old man was admitted to a private clinic with a five-day history of pain in the left chest and progressive weakness in the left arm. The pain was typically experienced as a paroxysmal dull ache that worsened after activity. The patient had no history of similar symptoms. He consulted with a general practitioner, and analgesic poultices were applied as symptomatic treatment to alleviate the pains. However, the patient developed dizziness during work the next day. He subsequently developed inspiratory dyspnoea without any other clinical symptoms, and he was ultimately confirmed dead by emergency physicians.
A systemic pathological autopsy was performed for histopathological examination and judicial poisoning analysis. Nebulous and reddish lividity were anomalously distributed on the back. The face, anterior neck and upper chest exhibited diffuse ecchymosis. Petechial haemorrhage was found on the bulbar and palpebral conjunctiva, showing signs of asphyxia. The lips, fingernails and toenails showed cyanosis. No bleeding or fractures were observed throughout the body including in the craniocerebrum; neck, chest and abdominal subcutaneous tissues; hyoid bone; thyroid cartilage; cricoid cartilage and trachea.
A neoplasm measuring 2.5 cm × 1.0 cm × 1.0 cm was located in the left cervical spinal cord (C3–C5) underneath the spinal nerve root, with a clear boundary and integrated surface of the neoplastic capsule (). The compressed spinal cord was visibly sunken. Microscopic examination revealed widespread and recurrent haemorrhage, haemosiderin particle aggregation and scattered infiltration of lymphocytes within the subarachnoid space of the C3–C5 spinal cord. The tumour cells exhibited a long spindle shape, were uniform in size and had a fence-shaped, whirlpool, wavy arrangement. Sporadic Verocay bodies (Antoni type A), haemangiectasis with paralytic congestion, myxoid matrix deposition and vascular wall hyaline degeneration (Antoni type B) were present within the tumour ((A)). The axons of the ventral root of the spinal nerve were unevenly thick and homogeneously solid, and they exhibited hollow degeneration ((B)). Netlike and loose spinal cord parenchymal nerve fibres were irregularly and hyperchromatically enlarged, distorted and fractured, and they extended to the outer space. Hydropic degeneration and lipofuscin pigment deposition were observed in ventricolumnar motor neurons ((C)). The pathological diagnosis was a cervical intramedullary spinal cord schwannoma. The neurons exhibited slight oedema, and no necrocytosis was found in the compressed spinal cord and brain stem. The other organs and tissues exhibited diffuse hydropic degeneration. The peripheral blood, gastric contents, urine and hepatic tissue tested negative by conventional toxicology analysis.
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pmc-6197028-1
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The patient is a 73-year-old female with past medical history of sero-positive ocular Myasthenia Gravis (with anti-acetylcholine receptor antibody) and COPD who presented by helicopter with altered mental status and weakness. On presentation the patient was hypotensive and afebrile with a heart rate in the 20s. She received atropine en route and was started on a dopamine drip along with aggressive fluid resuscitation. Blood pressure improved to 111/54 with HR increasing to the low 30’s. Lab work at outside hospital showed sodium 145 mmol/L, potassium 6.0 mmol/L, chloride 107 mmol/L, with a BUN of 41 mg/dL and creatinine 2.32 mg/dL. POC glucose was 191 and AST 55 U/L with ALT 37 U/L. Her hyperkalemia was treated with insulin and dextrose and had returned to normal limits by time of presentation. Repeat kidney, liver function tests, and serum electrolytes were within normal limits. Troponins were drawn and found to be 0.062 ng/mL with a Brain Natriuretic Peptide of 60 pg/ml. ECG was performed and showed patient had a complete heart block. The patient was admitted to Medical Intensive Care Unit where transcutaneous pacing was attempted but found to be ineffective. The patient was brought to the cardiac cath lab for transvenous pacing. Review of prior to admission medications showed that the patient had taken her PO pyridostigmine prior to developing bradycardia with altered mental status. She denied taking any of her COPD medication prior to presentation including any short or long acting beta agonists. Neurology was consulted for further evaluation of causes of heart block in a patient with MG. Pyridostigmine was held for concern of its affect in leading to complete heart block. With pyridostigmine held, the patient reverted back to sinus rhythm and transvenous pacing was removed. Using the Naranjo scale for adverse drug reaction patient received a score of 6 for a probable adverse drug reaction. Given the patient’s lack of infectious etiology, including negative Lyme serology, alternative pharmacological causes of heart block and reversion to sinus rhythm after removal of the offending agent, pyridostigmine was diagnosed as the causative agent. When the patient was stabilized she was discharged home with pyridostigmine held with close follow-up with her neurologist for further management of her MG.
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pmc-6197108-1
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A 53-year-old man collapsed suddenly at work and was taken to hospital, where he presented respiratory and cardiac arrest. Adrenalin and other rescue medications have been used. No vital sign was revealed through the resuscitation and death was declared soon after. A forensic examination was performed two weeks after death. Since forensic autopsy of the suspected deaths due to illness are not forced by law in China. And such autopsy must be granted by the relatives, which may lead to the very long time interval between the autopsy and death. The victim was 175 cm in height and had a normal body shape. Permission for PMCT, PMCTA and autopsy was granted by the victim's relatives.
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pmc-6197113-1
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A 68-year-old male was admitted to hospital with a history of dyspnea at rest for 2 d, a cough producing mucus, loss of appetite and diarrhoea. Pneumonia was suspected, and the patient received intravenous antibiotic treatment. During the patient's second night in hospital, he was found groggy and walking near his room by the nurses. They took him back to his room and connected a new antibiotic dose to his venous catheter. A few hours later, he was found unconscious, lying on the floor next to his bed. His peripheral venous catheter was connected to a nasal cannula delivering O2 from the wall, with a flow rate of approximately 2 L/min, and the antibiotic dose was found in the garbage can. As post-mortem lividity was present, no resuscitation attempts were performed by the clinicians and the death was pronounced. The body was immediately brought to the University Center of Legal Medicine (Lausanne, Switzerland).
Before any manipulation of the corpse, a native CT scan was carried out at around 10 h post-mortem using an eight-row CT unit (CT LightSpeed 8, GE Healthcare, Milwaukee, WI). All scanning parameters are detailed in . A forensic pathologist immediately viewed the native CT images. To evaluate the distribution of gas because of physiological changes in the body after death, the radiological alteration index (RAI) was used as proposed by Egger et al. []. The RAI was based on the analysis of samples from seven sites (heart cavities, liver parenchyma and vessels, left innominate vein, abdominal aorta, kidney parenchyma, L3 vertebra and the subcutaneous pectoral tissues).
All images were interpreted in a consensus reading by one board-certified radiologist and one forensic pathologist who were trained in forensic imaging. A post-mortem radiological report was prepared and described all findings from the native CT scan. The native CT scan revealed the presence of subcutaneous, intramuscular and intravascular gas, a pneumoperitoneum, a bloated heart with the right cavities filled with gas and a left pneumothorax ((a–d)). The maximum RAI score is 100, and RAI scores greater than 50 are usually seen in cases of severe changes []. Therefore, given the short post-mortem interval (<12 h), the elevated RAI obtained in this case (75) strongly suggests an exogenous source of gas in the tissues.
CT-guided gas samples were immediately taken from multiple sites (carotid artery, jugular vein, right auricle, thoracic aorta, pectoral muscle, thoracic cavity, abdominal cavity, scrotum and gluteal soft tissues, (e,f)) according to the protocol for gas analysis described by Varlet et al. [].
The external examination revealed massive subcutaneous emphysema with audible crepitations on the entire surface of the body. Additionally, there was a bruise on the left part of the forehead, a small contusion near the left eyebrow surrounded by a purplish-blue bruise and some bruises of different ages on the inferior part of the thorax and on the legs. On the left arm, a venous catheter was still in place.
Samples for toxicological and biochemical investigations (blood and urine) were collected in S-Monovette® tubes with sodium fluoride or ethylenediaminetetraacetic acid as a preservative (Sarstedt, Nümbrecht, Germany). Biological samples were collected as soon as possible on arrival of the body at the morgue (vitreous humor) and during autopsy (femoral blood, pericardial fluid and urine). During sampling of femoral blood by incision with a scalpel, multiple gas bubbles were visible in the blood ((a)).
Toxicological analysis of the collected samples revealed acetone at a physiological level in the blood, as well as caffeine and paracetamol in both blood and urine. Post-mortem chemistry analysis of the serum samples collected during the autopsy revealed elevated values of C-reactive protein (175 mg/L), consistent with an inflammatory state and a procalcitonine level compatible with a bacterial infection (0.69 μg/L). The results also revealed signs of cardiac dysfunction with a very high level of N-terminal prohormone of brain natriuretic peptide (>35 000 ng/L) and cardiac necrosis with a very high troponin-T level (>56 000 ng/L).
The autopsy was performed by one board-certified forensic pathologist and one forensic pathologist in training. Opening of the thoracic cavity revealed numerous gas bubbles in the fatty tissue covering the heart. The pericardium was opened and filled with water, and the heart floated. An incision was made in the right ventricle with a scalpel, and blood with gas bubbles escaped. This technique was performed mainly for training purposes and not for gas sampling, as the gas from the cardiac cavities had already been sampled during CT imaging. The autopsy revealed the presence of numerous gas bubbles throughout the vascular system, even in the small vessels of the brain ((b)). Additionally, changes to the pulmonary parenchyma were consistent with pneumonia. No other major findings were made.
An Agilent 6890N GC (Agilent Technologies, Palo Alto, CA) combined with a headspace gas autosampler and equipped with an Agilent Select Permanent Gases column arrangement was used. This column arrangement is specially designed for gas analysis and contains a molecular sieve 5 Å PLOT capillary column (10 m × 0.32 mm i.d.) and a Porabond Q column (50 m × 0.53 mm i.d.) in parallel, which allows for separation of carbon dioxide (CO2). The column temperature was maintained at 45 °C for 13 min. The injector temperature was 100 °C, and the injection was conducted in splitless mode. Helium was used as the carrier gas at a constant flow rate of 8 mL/min. The gas detection and quantification were performed with a thermal conductivity detector set at 150 °C. The system was calibrated for each gas with standard gases of H2S (Multigas, Domdidier, Switzerland), O2 and N2 (from laboratory air), and CH4 and CO2 (Carbagas, Lausanne, Switzerland). With this system, all the gases could be detected in the same run. The gas compositions for the different intracadaver sampling sites are displayed in .
Samples of the brain, heart, lungs, liver and kidneys were taken during autopsy and stained following a standard haematoxylin and eosin protocol. The heart samples were taken from both ventricles, and the interventricular septum exhibited epicardial vessels deprived of red cells and small intramyocardial haemorrhages. The lung samples taken from each lobe contained numerous clusters of leucocytes, mainly neutrophils, and, to a lesser extent, some macrophages, predominantly in the left lower and right upper lobes. These findings are consistent with the pneumonia suspected on admission to the hospital. There were no significant findings from the other organ samples. Samples of the heart were prepared and analysed for deposition of the plasma antigen fibronectin and the terminal complement complex C5b-9 to look for early cardiac damage, especially the right ventricular ischemia. None of the samples showed reaction for C5b-9, and only small groups of cells stained for fibronectin. Based on these results, cardiac ischemia was not likely in this case.
In light of the different results, the cause of death was attributed to a fatal O2 embolism. This was caused by the infusion of pure O2 from the wall into the vascular system through a venous catheter connected to a nasal cannula. Although from a medico–legal point of view the circumstances of the death (accident, suicide and homicide) remained unclear, police investigations led to the suspicion that the patient had connected the nasal cannula to the venous catheter. The wound on the forehead and the bruises observed during the external examination could be explained by a fall from or next to the hospital bed, as suggested by the police.
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pmc-6197134-1
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In August 2006, the remains of a 38-year-old female were discovered in Pachangnoi Subdistrict (N 19°19′24.24″; E 100°27′17.28″), Pong District, Phayao Province, northern Thailand (), and transferred to the Department of Forensic Medicine, Faculty of Medicine, Chiang Mai University. Forensic autopsy revealed the impact of blunt and sharp forces on the head and the abdomen. The bloated body was infested by fly maggots. Two blow flies, C. megacephala and C. villeneuvi, and one muscid species belonging to the genera Hydrotaea were identified. The minimum post-mortem interval (PMImin) was estimated to be six days, based on the developmental rate of C. megacephala. However, identification of one fly species could not be achieved due to the limited information on fly larvae morphology of species of forensic importance in Thailand at that time.
Initial macroscopic examination of the fly larvae sampled showed two groups of maggots: third instars of non-hairy maggots and third instars hairy maggots. The non-hairy maggots were identified as the blow fly, C. megacephala and the muscid Hydrotaea sp. The hairy maggots were mainly identified as third instars of the blow fly, C. villeneuvi []. Interestingly, we sampled about 20 third instars specimens that were unfamiliar to us. All larvae sampled were preserved in 70% alcohol. The largest larva was measured using Vernier calipers. In 2017, we decided to reanalyse the unidentified larvae of this case. Some specimens were cut using a sharp blade at two sites, across the third thoracic segment and across the middle of the eighth abdominal segment, to examine the cephaloskeleton and posterior spiracle, respectively. The anterior and posterior ends were transferred into a small eppendorf tube consisting 10% KOH. These tubes were then put into a beaker (half-filled with boiling water) which was placed on a hotplate (Barnstead/Thermolyne, Model: SP46920-26, USA) for 10 min. Specimens were washed twice with distilled water. To remove the alcohol, specimens were placed in Cellosolve (ethylene glycol monoethyl ether) and left for 5 min. The specimens were then transferred onto a glass slide and one drop of Euparal® was added. Specimens were arranged in their appropriate positions and covered with a cover slip. Prepared anterior and posterior ends were examined and photographed under a light microscope (Olympus CX41, Tokyo, Japan, with Olympus DP22 digital camera). For photographing, the focus stacking was shot by taking a series of images with the same composition and gradually changing the area of sharp focus. The number of pictures taken depended on the thickness of the specimen. For preserved larvae, stacking pictures were taken using a Nikon D7100 digital camera with a Nikkor lens Af-s macro 60 mm f2.8G. Each picture was merged in the program Helicon Focus 6.6.0 using method C (pyramid stacking). Terminology for general larval morphology followed Courtney et al. [], for peripheral tubercles of larvae followed Liu and Greenberg [] and for modifications of larval cephaloskeleton followed Szpila et al. [].
The largest specimen was 12.8 mm long. The prominent features are the protuberant tubercles encircling body segments, of which the prominent tubercles originate from the first abdominal segment and are present until the eighth abdominal segment ((A–C) and (A)). Spine bands between segments are obvious in the thoracic segments, with the most prominent between the first and second thoracic segments ((A)). These spine bands between thoracic segments are relatively sclerotized ((A,B). However, spine bands between the abdominal segments are unnoticeable ((C)).
A very distinct ultrastructure of the surface integument is seen on the body surface, which is covered with dense variable size of denticles ((A–C) and (B)). The anterior spiracle consisted of 9–11 lobes (n = 13) arranged in a single row ((B,C)). The cephaloskeleton ((C)) has large and heavily sclerotized mouthhooks, curved downwards. The posterior base of the mouthhooks is large and broad. An accessory sclerite is moderately sclerotized, adjacent to the base of the mouthhooks. The dental sclerite is apparent, curved backward apically and connected to the base of the mouthhooks. An intermediate sclerite is present. The parastomal bar is slender and slightly curved upward apically. The dorsal bridge is slender apically and bent downward, with the same length as the anterior margin of parastomal bar. The dorsal cornua, vertical plate and ventral cornua are heavily sclerotized. The dorsal cornua are much longer than the ventral cornua. The ventral cornua have an opening or window ((C), arrow).
The posterior end of the third instar shows six prominent pairs of tubercles along the peripheral rim of the eighth abdominal segment ((A,C) and (E)), of which all six pairs (inner dorsal, median dorsal, outer dorsal, outer ventral, median ventral and inner ventral tubercles) are almost equally in their protuberance. Viewed posteriorly, there is a remarkably sculpture encircling the posterior spiracles and adjacent to these tubercles ((E)). Higher magnification of the posterior spiracles revealed thick, heavily sclerotized complete posterior spiracular peritremes enclosing three spiracular slits ((F)). The button (or ecdysial scar) is indistinct ((F), arrow).
A key for identification of the third instar of blow flies of forensic importance in Thailand is provided as follows:
Abdominal segments with large, elongate tubercles ((A) and (A))…………2 Abdominal segments lacking large, elongate tubercles ((B))…………3 Tubercles bear numerous small spines at tip ((B)); anterior spiracle with 9–12 lobes; posterior spiracle large, with heavily sclerotized incomplete peritreme ((C))………C.rufifacies (Macquart) Tubercles bear numerous small spines throughout ((D)); anterior spiracle with 13–15 lobes; posterior spiracle large, with heavily sclerotized incomplete peritreme ((E))…………C.villeneuvi Patton Abdominal segments bear protuberant tubercles ((A–C)); anterior spiracle with 9–11 lobes; six prominent pairs of tubercles along the peripheral rim of the eighth abdominal segment ((D,E)); posterior spiracle large, with moderately sclerotized complete peritreme ((F))………C.chani Kurahashi Abdominal segments without protuberant tubercles…………4 Peritreme incomplete………5 Peritreme complete…………7 End of upper peritreme gradually enlarged ((A)); spines between the first and second thoracic segment large, multipointed ((B)); some specimens with brown patch on dorsal integument ((C)), but some not ((D))……………C.nigripes Aubertin End of upper peritreme normal; never with brown patch on dorsal integument………6 Anterior spiracle with 4–6 lobes; posterior spiracle large, with moderately sclerotized incomplete peritreme ((A)); spines between the first and second thoracic segment large, single point ((B))…………C.bezziana Villeneuve Anterior spiracle with 9–12 lobes; posterior spiracle large, with moderately sclerotized incomplete peritreme ((C)); spines between the first and second thoracic segment moderate, single or multipointed ((D))…………C.megacephala (F.) With prominent outer ventral tubercle at the rim of the eighth abdominal segment; anterior spiracle with 9–12 lobes; posterior spiracle lightly sclerotized with incomplete peritreme ((A)); spines between the first and second thoracic segment large, arrange singly or rows ((B)); accessory sclerite heavily sclerotized ((C))…………Lucilia sinensis Aubertin Without prominent outer ventral tubercle at the rim of the eighth abdominal segment…………8 Posterior spiracle small, lightly sclerotized ((D)); anterior spiracle with 3–6 lobes; spines between the first and second thoracic segment small, arranged mostly in group or row ((E)); accessory sclerite unsclerotized ((F))…………L.cuprina (Wiedemann) Posterior spiracle large, moderately sclerotized ((G)); anterior spiracle with 5–9 lobes; spines between the first and second thoracic segment small, arranged mostly in rows ((H)); accessory sclerite unsclerotized ((I))………L.porphyrina (Walker) Specimens of larvae used in the key were from laboratory colony and/or forensic death scenes, by which adults were confirmed by morphology.
Some samples of these unknown species have been preserved in 70% ethanol since 2006 with the label of date and time of collection. To confirm the state of the species, molecular identification was performed in 2017 using these samples kept for more than 10 years.
For DNA extraction, polymerase chain reaction (PCR) amplification and DNA sequencing, the genomic DNA was extracted from one larva according to the dilution protocol of the Phire Animal Tissue Direct PCR Kit (Thermo Scientific). After measuring the DNA concentration, 100 ng/mL of the extracted DNA was subsequently used for PCR reaction.
DNA amplification was performed according to the PCR protocol of the kit. Partial COI sequences were amplified using the primers TY-J-1460 (5′-TACAATTTATCGCCTAAACTTCAGCC-3′) and C1-N-2800 (5′-CATTTCAAGCTGTGTAAGCATC-3′) []. Cycling condition was initially denaturation at 98 °C for 5 min, followed by 40 cycles of denaturation at 98 °C for 5 s, annealing at 61.7 °C for 5 s, extension at 72 °C for 30 s, followed by extension at 72 °C for 1 min. PCR products were electrophoretically separated in an 1% agarose gel, stained with RedSafe™ (Intron Biotechnology). For sequencing, the unpurified PCR products were sent to the First BASE Laboratories Sdn Bhd (Selangor, Malaysia).
To perform the sequence alignment and phylogenetic analysis, the obtained DNA sequences from both directions were edited and assembled using BioEdit software version 7.0.9.0. []. For the highest similarity search, the sequence was compared with the available sequence database via a Basic Local Alignment Search Tool (BLAST) search at the National Center for Biotechnology Information (). Using MEGA6 software [], neighbour-joining tree [] was constructed using Kimura 2-parameter (K2P) model [] with 1 000 bootstrap replications. Additionally, reference sequences retrieved from GenBank covering a fragment length equal or longer than ours were aligned, trimmed and added to the analyses.
Based on the BLAST search, our larval sample collected from the human corpse was 100% identical to C. chani (GenBank accession no: KR921606), confirming to be C. chani, based on 1 205 bp of COI (). Additionally, analysis of phylogenetic analysis revealed that C. chani was grouped within C. megacephala, C. pinguis, C. thanomthini, C. bezziana and C. nigripes [].
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pmc-6197143-1
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A 29-year-old woman, gravida 2, para 0, was hospitalized for abdominal pain in the lower part of the abdomen with no apparent cause at 32+2 weeks of gestation. Ultrasound showed MC triplet gestation with a difference in fetal weight estimates (two fetuses at 32 weeks and the other at 31 weeks). Placental function ranged between grades I and II. The umbilical cord of one fetus was once around the neck. Umbilical artery Doppler and a non-stress test showed no unusual symptoms. No distinct evidence of FFTS was observed during the entire pregnancy. After the woman was diagnosed with threatened premature delivery, she was treated with phloroglucinol for tocolysis and dexamethasone for promoting lung maturation of the three fetuses. The next day, the woman complained of continuous abdominal distension, while her previous abdominal pain was relieved. An ultrasound examination on the fourth day showed two fetuses with a weight estimated for 33 weeks and the other for 32 weeks of gestation. The non-stress test indicated slight abnormality. Therefore, conservative medical care was continuously implemented. At 4:30 am on the fifth day, the three fetal heartbeats stopped and fetal movement ceased. Thereafter, the three deceased fetuses weighted 1700, 2100 and 1800 g when they were delivered by caesarean section.
An external examination showed that fetuses A and C were thin and pale, while fetus B was heavy and red (). Observation of the internal organs showed that fetuses A and C suffered anaemic changes ((A,C)). However, fetus B presented congestion and haemorrhage of the organs ((B)).
Examination of the MC placenta indicated that the umbilical cord of the recipient performed differently with the donor ((A)). All three fetuses shared relatively symmetrical triple placental portions of the single placental disc. Two types of vascular anastomoses, including arterio-arterial anastomosis (AAA) and arterio-venous anastomosis (AVA), were observed in the placenta ((B)).
Microscopic examination of placental villi showed differences in anaemia and hyperaemia. We observed relatively large and immature villi with interstitial oedema in the anaemic area of the placenta ((A,C)). In the hyperaemic area of the placenta, villi were mature with congestive interstitial telangiectasia. Furthermore, syncytiotrophoblast nuclei were degenerated, concentrated and gathered into multi-core nodules ((B)).
Laboratory studies showed that haemoglobin values of the fetuses were 58, 135 and 65 g/L for fetuses A, B and C, respectively.
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pmc-6197332-1
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An asymptomatic 23-month-old boy, weighed 10.5 kg, was incidentally diagnosed with congenital heart disease while presenting to the hospital for another illness. Transthoracic echocardiography (TTE) revealed one 18-mm ASD located in the inferior portion of the atrial septum that resulted in an overriding inferior vena cava (IVC), and the right inferior pulmonary vein (RIPV) partially returned to the right atrium (RA) near the orifice of the IVC. TTE also showed a complete left-to-right atrial shunt, no tricuspid regurgitation, and right ventricular dilation (with a diameter of 15 mm). Cardiac catheterization confirmed a normal anatomy of coronary arteries and a pulmonary to systemic flow ratio (Qp/Qs) of 3.2:1.
The patient was placed in a supine position with the right side of the body elevated to 30°. Two arms were placed along the body and the patient was under general anesthesia with a single-lumen endotracheal tube. One 14F-arterial cannula (Medtronic, Inc., Minneapolis, Minn, USA) used as a superior vena cava (SVC) cannula was inserted through the right internal jugular vein with Seldinger technique. Four trocars were set up on the right chest wall, included the following: one 12 mm trocar in the 5th intercostal space (ICS) at the anterior axillary line as the main working port, one 5 mm trocar in the 4th ICS at the mid-axillary line as the secondary working port, one 5 mm trocar in the 5th ICS at the mid-axillary line as the camera port and one 5 mm trocar in the 6th ICS at the mid-axillary line for right heart sucker.
The ventilation volume was reduced to 50%–75% compared with conventional practice. The anesthetist continuously monitored the oxygen saturation with a finger pulse oximeter and maintained it ≥95% throughout the operation. With this ventilation technique, the lungs were collapsed enough for the surgeon to open and hang up the pericardium. The large right lobe of the thymus covered the majority of the pericardium surrounding the aorta and the SVC. Therefore, we dissected this lobe from the pericardium (while preserving the tissue and supplying vessels) and hung it on to the anterior chest wall with a suture. The pericardium was opened parallel to and at 1.5 cm away from the anterior chest wall. The inferior edge of the pericardium was hung up to the diaphragm (the caudal end) and through the trocar (the cephalic end) by some sutures to expose the surgical field (Video 1). At this stage, respiratory ventilation was continued as usual.
To expose the ascending aorta, the top of the right atrial appendage was sutured and pulled down through a trocar. A 2–0, 17 mm braided suture (ETHIBOND EXCEL® Polyester Suture, ETHICON, JOHNSON & JOHNSON, Shanghai, China) was used to make a purse-string suture on the anterior wall of the ascending aorta, right beneath the semicircular fat plica (A) (Video 2). A 12F-arterial cannula (Medtronic, Inc., Minneapolis, Minn, USA) was placed superiorly through right anterior chest wall in the 4th ICS, 1 cm away from the right border of the sternum. This process was performed from the outside combined with endoscopic visualization from inside to avoid injury to the internal thoracic artery and ensure that the cannula was best directed to the purse-string suture (). We placed a piece of a 10 F rubber catheter (Red Rubber Latex All-Purpose Intermittent Catheters, Medline, USA) about 1.3 to 1.5 cm away from the tip of the arterial cannula to work as a brake. Subsequently, a surgical scalpel blade No.11 (Aesculap, Inc.) was used to open the ascending aorta inside the purse-string suture. The arterial cannula was then introduced via this ostium into the ascending aorta until the brake on the cannula reached the aortic wall (B, C) (Video 3). The arterial cannula was fixed and the cardiopulmonary bypass (CPB) was started.
A CO2-pump line connecting to the camera port () was used to fill the pericardial and pleural spaces with CO2. Initially, CO2 was pumped with a rate of 0.5 l/min, and then the pump rate was adjusted to maintain the partial pressure of CO2 in arterial blood ranging from 35 to 40 mmHg. Arterial line pressure was maintained >50 mmHg during the operation.
A loop was placed around the SVC to act as a tourniquet but not snaring. The patient was placed in the Trendelenburg position. The tourniquet on the SVC was tightened after opening the RA (Video 4). The blood returning to the RA from the IVC was drained by a stiff sucker, which also acted as an atrial retractor to expose the lesion. The edges of RA were hung to the pericardium by stitches to expose structures inside the RA. After determining the location, size of the ASD, as well as the anatomical correlation between the IVC and the RIPV, an artificial patch was used to close the ASD and form a canal to drain blood from the RIPV to the LA through the ASD (A, B) (Video 5). Right before completing the ASD closure, the lung was inflated to remove air from the left atrium. The RA was closed in a two-layer fashion using continuous stitches. The extracorporeal circulation was stopped and the surgery was finished uneventfully. The operative and cardiopulmonary bypass times were 259 and 133 min, respectively. The patient stayed in the intensive care unit for 18 h and was discharged on postoperative-day 7 without neurological complication or blood transfusion. TTE prior to discharge revealed a completely closed ASD, patent IVC, and RIPV ostia. Both the patient and his family were extremely satisfied with the cosmetic results of surgical scars (C).
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pmc-6197384-1
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A 69-year-old man had epigastric pain after eating fried shrimp without peeling shell 4 months ago and the symptom subsided 3 days later from that date. However, he has had intermittent epigastric pain from then on. He called at our emergent department because of epigastric pain with fever up to 38.2 ℃ since 2 days ago. He denied constipation, diarrhea, but has nausea. He denied peptic ulcer history nor any systemic diseases. The epigastric pain will be alleviated by bending abdomen and aggravated by laying down. Physical examination showed epigastric tenderness without muscle guarding nor rebounding tenderness. Laboratory tests documented elevated inflammatory markers with C-reactive protein 7.6 mg/dL. Other laboratory data were within normal ranges.
Computed tomography revealed a hypodense region 52 mm in diameter with a 21-mm hyperdense linear object beside the transverse colon. (, ).
The patient was diagnosed as foreign body perforation of the transverse colon and intra-abdominal abscess. Although the patient described epigastric pain, he has remained able to eat for 4 months; he was treated with antibiotics (Sulbactam /Ampicillin, 6 g/d) at first. Persisted epigastric pain of the patient was still noted, so he asked for surgical intervention. Therefore, laparoscopic removal of the abscess and the foreign body with drainage was performed. During the operation, severe adhesion between abscess and diverticulum of T-colon was found. The foreign body was a 26-mm shrimp leg found in the abscess (, ). The patient was discharged 3 days postoperatively with no complications.
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pmc-6197500-1
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A 25-year-old woman reported to the outpatient department of our tertiary care hospital with lower abdominal pain, vaginal spotting, and severe nausea lasting 10 days. At the time of presentation, she was primigravid at 18 weeks and six days of gestation according to her last menstrual period. The pregnancy was a planned conception occurring within three months of marriage. The pregnancy was confirmed with a positive urine pregnancy test. Further questioning about menstrual history revealed cycles of four to five days every 28 to 30 days. Past medical, surgical or family history was unremarkable. On physical examination, she was alert and pale with a blood pressure of 150/90 mmHg and a pulse of 80 beats/minute. Her abdomen was soft, non-tender with a 32-week fundal height. A speculum examination revealed no active bleeding or discharge; the os was closed. Apart from the elicited anemic signs, the remainder of the physical examination findings were within normal limits. An ultrasound examination revealed a single live fetus of 18 weeks and two days in duration (Figures -). We also noted a partial mole indicated by mass resembling a bunch of grapes and measuring 19 cm by 8 cm. The molar vascularity was insignificant on the Doppler evaluation. We ordered additional investigations through our hospital’s designated laboratory.
At 20 weeks of gestation, a repeat ultrasound revealed a single live fetus of 20 weeks duration with no gross fetal anomaly. A large mass with multiple short cysts was noted in the fundal region at the site of the placenta. We also noted evidence of a partial mole in the anterior fundal region of the uterus measuring 19.7 cm by 10.9 cm, bulging into the amniotic cavity and compressing the fetus. Repeat serial measurement of β-hCG at this point of pregnancy was 561,771 mIU/mL. At 20 weeks and two days, she reported concerns of a passage of grape-like vesicles, and spontaneous abortion followed. A live male fetus was expulsed with no grossly detectable anomalies, weighing 200 g, and placenta was expulsed weighing 100 g. After this, she expelled a large amount of molar tissue weighing approximately 500 g (Figure ). The molar tissue was expelled intermittently via uterine contraction. After the expulsion of molar tissue, short-term general anesthesia was administered to the patient followed by suction curettage. An ultrasound after the procedure confirmed an empty uterine cavity. β-hCG levels were monitored post-termination. Approximately in one day the patient was discharged home, we noted a β-hCG level of 210,310 mIU/mL. Upon discharge, we instructed the patient to follow up with repeat serial measurements of her β-hCG levels. Four weeks following the abortion, her β-hCG levels were 320 mIU/mL, and six weeks following the abortion, β-hCG was undetectable. A plot of the change in the patient’s β-hCG levels over the course of her treatment is presented in Figure .
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pmc-6197502-1
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A 79-year-old man with a history of prior basal cell carcinoma presented with a new lesion on the right chin. His other medical conditions included acid reflux, osteoarthritis, seasonal allergies, and spinal stenosis; his medications included fexofenadine 60 milligrams twice daily and lansoprazole 15 milligrams daily.
The physical examination revealed a tender, pearly, skin-colored 3-millimeter papule on the right chin (Figure ). A provisional diagnosis of basal cell carcinoma was rendered and a punch biopsy was performed. A histologic examination of hematoxylin and eosin stained sections revealed an atypical basaloid keratinocyte proliferation in a nodular configuration with associated inflamed fibromyxoid stroma, and a subjacent aggregate of osteoid in concentric whorls (Figure ). The patient had normal calcium levels and kidney function and did not have a history of acne with scarring.
The correlation of clinical presentation and histopathologic findings established a diagnosis of nodular basal cell carcinoma with osteoma cutis. The tumor and associated bone were excised using Mohs micrographic surgery, which is a surgical technique that incorporates the microscopic examination of excised margins to ensure the complete removal of cancerous cells while sparing as much normal skin as possible. It is the most effective technique for the removal of squamous and basal cell cancers. In our patient, there has been no recurrence to date.
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pmc-6197508-1
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A 27-year-old female with no past medical or surgical history was admitted with complaints of fever, altered consciousness and hypotension. She reported a rash on the face that has been there for the last one year which did not respond to topical treatment with steroids. She did not have any history of contact with sick people and had not traveled recently. Physical examination revealed typical physical signs of dermatomyositis i.e. heliotrope rash as shown in Figure .
Lab work up showed hemoglobin concentration 8.5 g/dl (normal = 12.3-15.5 g/dl), hematocrit 0.28 (normal 0.35-0.44), red blood cells 3.30x1012/L (normal 4.2-5.2x1012/L, platelet count 40,000/ul (normal = 150,000-450,000/ul), lactate dehydrogenase (LDH) 814 IU/L(normal < 200 IU/L), total bilirubin was 2.2mg/dl (normal = 0.1-1.2mg/dl), prothrombin time (PT) 16 sec (normal = 12-14 sec), activated partial thromboplastin time (aPTT) 38 sec (normal < 35 sec), blood urea nitrogen (BUN) 42 mg/dL (normal 7-20 mg/dl), creatinine 3.5 mg/dL (normal 0.5-1.1 mg/dl). Spinal tap, urinalysis and blood cultures were negative, ruling out any infectious etiology of presentation. A diagnosis of TTP was made owing to presence of fever, altered state of consciousness, renal failure, anemia and thrombocytopenia. Antinuclear antibody (ANA) and anti Jo were positive consistent with autoimmune etiology of dermatomyositis. Patient was treated with plasmapheresis and her condition improved.
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pmc-6197510-1
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A 41-year-old obese female with a medical history of depression, post-traumatic stress disorder and premature ventricular contractions (PVC) on propranolol, sertraline, alprazolam and phenazopyridine, was admitted for elective bladder sling procedure. The patient had a normal preoperative evaluation and pre-anesthesia consultation. Laboratory values were unremarkable. A recent echocardiogram revealed an ejection fraction of 55%-60%. General anesthesia was induced uneventfully with midazolam, propofol, rocuronium, lidocaine and fentanyl. Prior to starting the surgical procedure and thirty minutes after induction, an intravaginal injection of 10 mL of lidocaine 2% with epinephrine 1:100,000 for local anesthesia was administered. Within a minute after local anesthetic administration, the patient developed severe bradycardia. Despite administration of epinephrine and atropine, the patient’s condition worsened to pulseless electrical activity. Chest compressions were started along with supportive therapy using vasopressors and inotropic agents and the patient subsequently developed pulmonary edema. A dose of 100 mL of intralipid (a chelating agent for lidocaine) for suspected local anesthetic toxicity was given to the patient. Supportive therapy with milrinone, epinephrine and norepinephrine were used which eventually stabilized the patient. An intraoperative transesophageal echocardiography (TEE) showed global impairment in cardiac motion—septal dyskinesia; left ventricular hypokinesia, severe dilation with systolic dysfunction; anterior wall abnormalities without right ventricular strain and an ejection fraction of 20% as shown in the figure below (Figure ). An electrocardiogram revealed prolonged QT and non-specific t-wave abnormalities.
Pulmonary embolism was ruled out. Due to the suspected acute coronary syndrome, the patient was taken to the cardiac catheterization lab, where an intra-aortic balloon pump was placed to increase cardiac output and decrease afterload. Troponin T values were found to be elevated (1.03 ng/mL) and coronary angiography showed no evidence of coronary artery obstruction/plaque rupture leading to the diagnosis of TC. The patient was transferred to the intensive care unit where supportive therapy was continued, and the next day, a follow-up echocardiography showed an improvement of ejection fraction to 58% (Figure ).
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pmc-6197512-1
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The police brought a 30-year-old man to the emergency department at Frederick Memorial Hospital in Frederick, Maryland, at the request of his mother. She reported that he had been unusually volatile for the past three weeks with multiple violent outbursts. In addition, there were episodes where she witnessed the patient shouting at the sky. She made the decision to call emergency services in response to her son’s homicidal threats toward his ex-girlfriend. According to her, the patient had no history of manic episodes or any family history of mood disorder. She said his current symptoms began when he started attending a new gym three weeks prior to his hospital admission. She suspected that he had begun injecting anabolic steroids.
In the emergency department, the patient’s complete blood count and basic metabolic panel were within normal limits. His urine toxicology screen tested positive for cannabis. The patient’s past medical history was significant for anxiety, depression, post-traumatic stress disorder (PTSD), opioid addiction with methadone maintenance therapy, and hepatitis C. His time in the emergency department was marked with extreme agitation that culminated in attempts to fight with the staff. He was treated with haloperidol 5 mg IM and lorazepam 2 mg IM. He was admitted to the behavioral health unit.
The patient was initially started on olanzapine 10 mg PO QD. The patient was unable to give a history for the first week of his hospitalization. On examination, he presented with expansive mood, pressured speech, psychomotor agitation, racing thoughts, inflated self-esteem, and decreased need for sleep. He was often seen pacing the hallways while talking loudly to himself and singing. His actions toward female staff were inappropriate and signaled that he was not cognizant of personal boundaries. He made verbal threats to staff and other patients and was not verbally redirectable. On two occasions where the patient became violent, ziprasidone 20 mg IM and lorazepam 2 mg IM were needed. He showed little improvement in his symptoms going into the second week of his hospitalization. His olanzapine was titrated to 15 mg QD, and chlorpromazine 100 mg PO QID was added.
At each attempt to ascertain the patient history, a similar pattern was observed. The patient quickly went off on tangents relating to marijuana and bodybuilding, which seemed to be a source of pride for him. He said he has used cannabis for recreation and for the treatment of PTSD from time spent in prison. He is the owner of a medical marijuana card and has used the substance multiple times a day since he was a teen in a variety of methods, including edibles, vaporization, smoking, and ingestible oils. He also grows and sells marijuana and claims he has a reputation for being the best. His agitation level rose greatly when he spoke of people who doubted his abilities. He would then tense up and begin to speak of his training in martial arts and all the time he had spent building muscle in the gym. When asked directly about anabolic steroid use, he would change the subject, deny it altogether, or say he only took supplements. Staff reported, however, that the patient had spoken of “Deca” (a colloquial name for Deca Durabolin, an anabolic-androgenic steroid) multiple times in his pressured, tangential conversations with them. The patient’s mother had since spoken with friends of the patient who said he had been injecting anabolic steroids once weekly at an unknown dose. Although the mother requested a laboratory test for anabolic steroids, it was not available at the hospital. She had contacted poison control about the availability of the test and planned to pursue it further when the patient moved to the outpatient setting.
The patient’s mania and psychosis improved gradually in the final week. He was discharged on chlorpromazine 100 mg PO TID and olanzapine 15 mg PO QD and scheduled with follow-up appointments with a psychiatrist and a therapist. He was not readmitted to any psychiatric unit since he was discharged.
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pmc-6197529-1
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A 27-year-old otherwise healthy male weighing 112 kilograms was brought to the neurosciences intensive care unit (NSICU) for the management of a diffuse subarachnoid hemorrhage (SAH) secondary to aneurysm rupture with a Glasgow Coma Scale (GCS) of three, Hunt and Hess Grade 4 and Fischer Grade 5. The non-contrast computed tomography (CT) scan showed diffuse cerebral edema, SAH, intraparenchymal hemorrhage with interventricular extension along with 6 mm of leftward midline shift (Figure ) with obstructive hydrocephalus and brainstem compression (Figure ). The computed tomography angiography (CTA) of the head and neck showed 4.0 x 2.3 x 2.8 mm saccular anterior communicating artery aneurysm which was managed by coil embolization. Ventriculostomy was done for impeding obstructive hydrocephalus with the placement of an extraventricular drain (EVD).
The hospital course was complicated by intracranial hypertension which was managed with additional agents including fentanyl, cisatracurium and propofol infusions. On day one, propofol infusion was started on 10 mcg/kg/min (microgram/kilogram/minute) and titrated by 5 mcg/kg/min every five minutes to a maximum of 80 mcg/kg/min. The cerebral edema was managed with the use of mannitol and 3% hypertonic saline; the monitoring goals for this patient were sodium of 150 to 155 mEq/L, intracranial pressure (ICP) of less than 20 mmH20 and cerebral perfusion pressure (CPP) of 60 to 70 mmHg. The first triglyceride level was drawn on day two, with a result of 330 mg/dL.
In the due course, the patient developed poor renal function secondary to contrast nephropathy or mannitol-induced acute kidney injury. His creatinine increased to 1.84 mg/dL and creatinine kinase increased to 703 U/L. On day three, clevidipine was started as the antihypertensive agent over other intravenous antihypertensive class due to acute kidney injury. On day four, a repeat triglyceride level was noted of 527 mg/dL. In concern of the rising triglycerides, clevidipine was changed to labetalol 200 mg three times a day for blood pressure management and the enteral nutrition infusion was reduced to 20 mL/hr to compensate for additional calories from the propofol infusion.
On day six, the patient’s triglycerides had reduced to 240 mg/dL after discontinuing the clevidipine on day four (Table ). On days five and six the propofol infusion was minimized but then titrated up for increased ICP on day eight and continued at similar rates on day nine. On day ten, his amylase increased to 204 U/L, and lipase increased to 493 U/L. Propofol was discontinued for the next two days (day eleven and twelve), and a fall in the subsequent amylase and lipase levels was noted. Propofol was restarted for ICP management on day thirteen and continued through day sixteen. The average infusion rate of propofol each day is noted in Table . Further improvement in pancreatic enzymes was seen after stopping the propofol infusion on day sixteen. Also, the liver enzymes (aspartate aminotransferase (AST)/alanine transaminase (ALT)) was noted to be on the higher side of the normal range, throughout the stay in the hospital (Table ).
The patient was started on enteral nutrition with Fibersource. The enteral nutrition was titrated up to 80 mL/hr which accounted for a total daily calorie intake of 2,304 kcal and total fat of 76.8 grams each day. The patient received a total propofol infusion of 44,391.2 mg over 16 days which accounts for 4,882.99 kcal and 443.91 grams of fat. Total clevidipine infusion of 297 mg over the 48-hour period contributed 594 kcal and 59.4 grams of fat (Table ).
On subsequent physical exam, he started following commands including opening eyes, showing two fingers, and wriggling toes. The patient also had an intact cough, gag, and oculocephalic reflex and additionally withdrew to noxious stimuli. His further management included placement of tracheostomy and percutaneous endoscopic gastrostomy with transfer to a long-term acute care hospital after discharge.
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pmc-6197534-1
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A 36-year-old multiparous Afghan woman presented to the emergency department with a history of abdominal pain and vomiting for one week with sudden increase in intensity of pain for the last three hours. There was no history of fever, prior surgeries or trauma. On physical examination, abdominal distension was noted. There was diffuse abdominal tenderness more pronounced in the lower abdomen, but no definite palpable mass was felt.
Computed tomography (CT) was performed which revealed absence of the spleen in the left upper quadrant. An abnormally placed, enlarged spleen was noted in the lower abdomen reaching up to the pelvis. It had an elongated and twisted vascular pedicle. On contrast-enhanced images there was a lack of parenchymal enhancement in the spleen with homogenous low attenuation and peripheral enhancement. The pancreatic tail was also involved in the torted pedicle (Figures , ).
Non-contrast images confirmed the typical findings of an abnormally located spleen, with a hyperdense splenic pedicle and whorling of the vessels and fat, which were characteristic of torsion (Figure ).
The findings were concluded as wandering spleen with hilar torsion and liquefactive infarction. The patient underwent an exploratory laparotomy. On surgery, the spleen appeared congested and infarcted, and the splenic vessels were thrombosed. Therefore, total splenectomy was performed because of non-viability. The post-operative recovery of the patient was uneventful and she was discharged after two weeks.
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pmc-6197535-1
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A 45-year-old female presented to Endocrinology clinic for evaluation of weight gain. During the interview, she reported polyphagia and polydipsia. Her past medical history was significant for hypertension, hyperlipidemia, obesity, sleep apnea, peripheral vascular disease and mood disorder. She was also diagnosed with mild mental retardation as a child. She experienced her menarche at the age of 13 and had regular periods. She was living in a group home and was not married or had kids. The patient’s height was 4 feet 11 inches and she had a body mass index (BMI) of 40. Physical examination was significant for short stature, facial dysmorphism with prominent forehead, upslanted eyes, flat nasal bridge and a thin upper lip. Extremity examination revealed short 4th and 5th metacarpal and metatarsal bones bilaterally.
Secondary causes of obesity and AHO were considered in differential diagnosis. On blood testing the patient’s calcium, phosphate and parathyroid hormone (PTH) levels were normal. Cortisol, thyroid stimulating hormone (TSH), and free thyroxine (FT4), follicle stimulating hormone (FSH) and luteinizing hormone (LH) levels were all normal. The patient was sent for genetic testing with a presumed diagnosis of PPHP. Karyotype test showed terminal deletion of the long q-arm of one chromosome 2 in all analyzed cells-46, XX, del (2)(q37.1), consistent with BDMR.
Figure shows round face of our patient and Figure shows the brachydactyly of third and fourth fingers.
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pmc-6197536-1
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A 68-year-old male was admitted to Queens Hospital Center with a complaint of shortness of breath for two days duration. The patient had been noticing a decrease in exercise tolerance for a few weeks. Two weeks earlier he was admitted to Queens Hospital with a complaint of chest pain, and acute coronary syndrome was ruled out. On evaluation, the patient had tachycardia with a heart rate of 120–124/min and blood pressure (BP) of 110/80 mm Hg. Lungs were clear to auscultation, and there was no evidence of infection or blood loss. The patient was empirically started on anticoagulation for pulmonary embolism. Computed tomography (CT) chest was not done initially due to elevated creatinine of 1.6, ventilation/perfusion scan (V/Q scan) showed the intermediate probability for pulmonary thromboembolism. The patient was continued on anticoagulation. However, patient tachycardia was persistent, and a blood test showed persistent hypocalcemia 6.8–7.9 mg/dL and hypokalemia 3.2–3.4 mEQ/L with normal thyroid function test. 25-hydroxyvitamin D test was low (6 ng/ml) and intact parathyroid hormone (PTH) was elevated (85.1 pg/ml). The patient was put on metoprolol 50 mg twice daily. The patient was also continued on intravenous (IV) hydration; electrolytes were supplemented. CT chest was done which confirmed the presence of old embolism and showed a 1.1 cm nodule in the left upper lobe (Figure ).
Endocrinology was consulted for persistent tachycardia with relatively stable BP. The patient had spikes of temperature for two days and was empirically started on Tamiflu and Rocephin while waiting for blood culture. The patient was insisting on being discharged home when he suddenly collapsed and went into cardiorespiratory failure, then he was intubated and transferred to the intensive care unit (ICU) where attempts to resuscitate were futile. The family refused an autopsy. Result for pheochromocytoma workup received after the death of the patient and it showed elevated levels of 24-hour urine metanephrine at 2001 mcg and norepinephrine at 1499 mcg and Vanillylmandelic acid (VMA) at 6.6 mg/24 h.
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pmc-6197568-1
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An 83 year old female presented to the emergency department complaining of vomiting and constipation that started a few days ago. She did not report abdominal pain or distention. The patient was otherwise healthy and has not undergone any previous abdominal or pelvic surgeries. On arrival the patient was malnourished, well oriented, and afebrile. Her blood pressure was 100/80 mm hg and her pulse was 80 beats per minute. On physical examination the abdomen was soft, not tender, and not distended, and no hernias were palpable. The rectum was found to be empty on rectal examination. Laboratory findings showed a hemoglobin level of (17.6 gr/dL), mild leukocytosis (11400/μL) with a left shift (81%), and a platelets level of (279000/μL). Urea (285 mg/dL) and creatinine (3.9 mg/dL) levels were elevated. The patient also had low serum sodium (131 mEq/L) and a normal potassium level (3.5 mEq/L). The initial plain abdominal radiography showed air fluid levels with air present in the rectum suggesting small intestinal obstruction (). Abdominal ultrasonography revealed dilated small bowel loops. The patient was admitted to the hospital and was initially managed conservatively with nasogastric suction and intravenous fluids. The nephrology unit was consulted for the diagnosis of renal failure. Two days later the patient did not improve significantly. Her white blood cells rose to (12000/μl) but her kidney function improved. Plain abdominal radiography was repeated and it showed increased air fluid levels without air in the rectum which is also consistent with small intestinal obstruction (). Because the cause of small intestinal obstruction was unclear, the patient underwent an exploratory laparotomy via a midline incision. As the abdominal cavity was reached, it was possible to visualize a Richter type hernia protruding through the left obturator canal (). We reduced the hernia successfully and examined the bowel for signs of necrosis. A small necrotic area was found and was resected with end to end anastomosis (). The defect at the hernia site was closed by interrupted sutures. The patient had an uneventful recovery and no recurrence of the hernia was noted during follow up.
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pmc-6197605-1
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A 35-year-old Greek female patient, gravida four and para two, presented to our breast unit department due to a non-palpable breast lesion which was revealed via ultrasonic examination, with malignant ultrasonographical features. Her personal medical history and her psychosocial history were uneventful and Pap Smear tests were up to date and all negative. She was non-smoker and consumed alcohol only in social occasions. Her body mass index (BMI) was 30, 48 Kg/m []. Because of her mother’s breast cancer history (diagnosed with breast cancer at the age of 50, but never tested for BRCA), the patient was followed up via transvaginal ultrasonography and breast ultrasonic examination every six months since 2004.
Upon arrival, physical examination of her breasts did not reveal any palpable mass. Breast ultrasonography showed a hypoechoic lesion of 0,9 × 0,8 cm located in the lower inner quadrant, while breast magnetic resonance imaging confirmed the suspicious and possible malignant finding on her right breast (MRM BIRADS IV) (). The chest x-ray test was normal. Her laboratory workup was all normal.
Under general anesthesia, a total excision of the lesion (which had been marked via a hook wire) was performed by a gynecologist specialized in breast surgery with 6 years’ experience in breast surgical procedures. The normal tissue of the breast, the nipple and the areola were conserved. The excised mass was almost 1 cm in diameter. The contralateral breast was normal. Ultrasound guided biopsy was not performed because BIRADS IV was overestimated (not necessarily malignancy). There were no peri-operative complications and the breast healed well. Post-operatively, the patient was followed up in the breast ward. She was administered antibiotics, fluids and painkillers intravenously. Histology confirmed the ultrasonic diagnosis, revealing a central low grade invasive ductal carcinoma and a peripheral in situ ductal breast carcinoma grade III (). The margins of the resected surgical specimen were negative for cancer cells.
After almost a month from the first operation, the patient underwent a new surgical excision of unilateral right axillary lymphadenectomy of the first and second lymph nodes level. Twenty-two excised axillary lymph nodes were negative for metastasizing breast disease (0/22). Immunohistochemical analysis in cellular level showed ER (clone 6F11) stain positive in 100% of tumor cells, PR (clone 636) stain positive in 2% of tumor cells, Ki-67 (clone M1B1) stain positive in 60% of tumor cells and c-erb-2 (clone CB11)/HER 2 showing 3+ positive for cancer. Subsequently, the patient underwent treatment via chemotherapy, radiotherapy, herceptin and hormone therapy for the invasive ductal carcinoma Grade III (TNM staging: T1N0MO).
Gene evaluation for genetic mutations showed a BRCA 1 mutation; gene BRCA1 analysis was positive for mutations predisposing for breast or ovarian malignancy. More specifically the mutation p.Gly1738Arg (HGVS nomenclature)/ G1738R (BIC nomenclature) was detected. Thus, a prophylactic bilateral mastectomy was performed and followed by a successful plastic reconstructive surgery done by an experienced plastic surgeon, allowing for optimal aesthetic results.
A year after the first operation, the patient underwent a prophylactic laparoscopic bilateral salpingoophorectomy and uterine diagnostic curettage at the age of 37. Before surgery, blood tests including tumor markers were all in normal levels (CEA: 1,3 ng/ml, CA15-3: 6U/ml, CA125: 8U/ml, CA19-9: 30 U/ml, AFP: 1,2 IU/ml). The chest x-ray test was normal and the preoperative magnetic resonance imaging of the upper and lower abdomen did not detect any pathological finding. The histological diagnosis of the surgical specimens was suggestive of a unilateral invasive high grade salpingeal cancer, mostly intraepithelial and minimal invasive (0, 1 cm) serous carcinoma of one salpinx. Immunochemical analysis was positive for P53 and Ki67 (). No other pathologic finding was detected.
A further surgical evaluation was decided by the oncology council. Thus, the patient underwent an open total abdominal hysterectomy, omentectomy and bilateral pelvic lymphadenectomy by a gynecologist specialized in gynecologic oncology under general anesthesia. Postoperatively, the patient was followed up in a gynecology ward. She was administered antibiotics, fluids and painkillers intravenously. After a hospitalization of 5 days, she was discharged on the 6th postoperative day, in good condition. She was prescribed tinzaparin (u) for 8 days and cefuroxime (u) peros for 7 days. The patient was compliant with the therapeutic program which was well tolerated with no significant side effects. Histology was totally normal without any other pathological findings. Bone scanning was normal and did not reveal areas of increased radionucleide uptake. Similarly, the hip and pelvis x-ray were normal.
The patient is in excellent clinical condition and she is followed up via ultrasonography of upper and lower abdomen and breast magnetic resonance imaging every 6 months, without any pathological finding after the last surgery.
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pmc-6197703-1
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A 42-year-old woman presented to the clinic with a palpable mass in her left inguinal region which was noticed 1 month prior. The mass had not been present in infancy oradolescence. History of trauma and operations were not found in the patient's history. There was a cyst aspiration story from 2 months ago. On physical examination, a soft-consistency, mobile mass of about 4 cm in size was seen in the left inguinal region. During the Valsalva maneuver, the mass did not change in size and shape. The patient's laboratory findings (complete blood count, urinalysis, blood biochemistry) were within the normal range. Ultrasonography revealed a hypoechoic cystic mass with a size of 40 × 50 mm in the left inguinal area without any vascular flow and no peristalsis ().
Abdominal magnetic resonance imaging (MRI) was performed to examine the communication between the cystic mass and peritoneal cavity, and the precise anatomy around the cystic mass.
It was found that the cystic mass in the inguinal canal included thin septa, and hydrocele of the canal of Nuck was suspected because of the low and high signal intensities observed on the T1- and T2-weighted images, respectively. Only the wall and septa were contrast-enhanced. The cystic lesion which was seen to be originated from the inguinal canal was excised in the exploration made by suspending the round ligament by passing through the anatomical folds with the incision made from the left inguinal region (, ).The defect was repaired with prolene mesh after high ligation. Histopathologic examination was evaluated as Simple cystic structure with cubic epithelium (). Patient was discharged on the 1 st postoperative day. The patient provided written consent to utilize her medical record with no patient identifiers.
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pmc-6197709-1
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A 61 year old male with history significant for right lower extremity deep venous thrombosis (DVT); on warfarin 7.5 mg, hypertension, diabetes mellitus, asthma, and chronic kidney disease presented to the emergency department with 3 day history of generalized abdominal pain associated with multiple episodes of nausea, vomiting and obstipation. Patient was awake, alert and oriented. Blood pressure was elevated, rest of vitals were benign. On physical examination, patient had distended abdomen with mild generalized tenderness, no signs of peritonitis. Remainder of his exam was un-remarkable. Labs were significant for INR; >6 and PTT; 91.9. CT abdomen was obtained that showed high grade small bowel obstruction (, ). Patient was admitted under surgical team and initially managed by bowel rest with nil per os(NPO), nasogastric tube to suction showed 1.3 L of bilious fluid, and foley catheter was inserted. Patient was given 6 FFPs to resuscitate in the emergency room. Repeated INR obtained and was found to have 2.16. At this time, patient was taken to the operating room for diagnostic laparoscopy. Intra- operatively, patient was found to have dilated small bowel along with a segment of bowel with intramural and mesenteric hematoma involving 30 cm of the jejunum (, ). Ischemic small bowel with intramural hematoma was resected and anastomosed primarily. Post-operative management in intensive care unit (ICU) was uneventful and patient was discharged home on post op day 5 without any further complications.
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pmc-6197733-1
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An 87-year-old woman presented to the Emergency Room (ER) complaining of an acute (few hours) and intense thoracic pain radiating to the dorsum and upper abdomen, associated with nausea, dyspnea, sudoresis and hypertensive peak. She had a medical history of hypertension. Eight years ago, she underwent a hip arthroplasty, complicated by pulmonary thromboembolism (PTE), when she was started on anticoagulants for the first six months and then on aspirin associated with cilostazol daily.
On the ER's initial evaluation, chest radiograph revealed an enlarged mediastinum. The patient had persisting pain, despite the administration of morphine and nytroglicerin, without hemodynamic instability. She had normal cardiac enzymes and electrocardiogram with elevated D-dimer test (8.02μg/mL). The emergency thoracic computed tomography angiography (CTA) showed an ectatic ascending aorta (4.0cm), descending aorta aneurysm with mural thrombus and laminar mediastinal fluid, excluding PTE. The echocardiogram identified in addition to the aortic ectasia, a mild aortic insufficiency. Subsequently, the patient was admitted to the hospital and transferred to the Intensive Care Unit (ICU).
As the pain subsided, she was managed conservatively. After three days, she had another burst of severe epigastric pain, associated with hematemesis, hemoglobin drop to 7.9g/dL (previously 11.9g/dL), hypotension (80 × 40 mmHg) and tachycardia (113 bpm).
The standard protocol of care of patients with acute upper gastrointestinal bleeding was executed: NPO diet, intravenous fluids, blood transfusion (which was necessary in this case) and proton pump inhibitors (PPI), followed by esophagogastroduodenoscopy (EGD). The EGD diagnosed an enormous bright red subepithelial mass, occupying the fundus and corpus in the lesser curvature, with luminal bulging, no mucosal bleeding, nor ulcerations/erosions, as shown in , . No therapy nor biopsies were performed. The remainder of the stomach, esophagus and duodenum were normal.
The patient denied any recent trauma, surgery or endoscopic intervention. Hence, she underwent further investigation in order to determine the nature of the hematoma, with the working differential diagnoses of intramural neoplasia and dissecting visceral aneurysm.
After the EGD, she was submitted to a new CTA, with evidence of a slightly hyperdense wall thickening of the corpus (4.0cm) and fundus (2.5cm), a small amount of blood compatible material in the lumen and perigastric adipose tissue hyperdensity, as shown in , . It also showed a descending aorta aneurysm with an extensive (from the subclavian origin to the thoraco-abdominal transition) crescent-like mural thrombus, which was hyperdense in the non-enhanced scan, with intimal flap and false lumen, suggesting dissecting acute intramural aortic hematoma, as demonstrated in . There were signs of rupture, characterized by the presence of mediastinal hematoma measuring 6.4 × 5.4 × 3.3cm at the level of the pulmonary trunk and left pleural effusion. The conjoint findings made the final diagnosis of aortic dissection with contained rupture.
Hematologic examination found no other coagulopathies and the only predisposing factor was the previous use of two antithrombotic antiplatelet drugs, which were suspended since the admission. Thus, the aortic dissection with contained rupture was considered the cause for the GIH.
Endovascular repair was performed, with implant of an aortic graft. The angiography presented no contrast extravasation of the gastroparietal branch, nor a visceral aneurysm. In the next day, control EGD revealed the GIH was stable, with slight regression. The oral diet was resumed on the second post-operative day. The GIH was managed conservatively and the patient had favorable outcome.
Approximately three weeks after the diagnosis, another EGD showed GIH involution. She was discharged after a few days and the prescription of aspirin was restarted. On routine follow-up, on the 30th postoperative day, she was submitted to a control Endoscopic Ultrasound (EUS), which was normal, the GIH had disappeared. The patient remains on follow-up and regular appointments with her vascular surgeon and geriatrician. The patient gave consent for publication of her case in the medical literature.
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pmc-6197945-1
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A 67-year-old African-American woman with type 2 diabetes mellitus, hypertension, chronic obstructive pulmonary disease, and coronary artery disease presented with an exquisitely painful bullous eruption.
Three days after administration of the quadrivalent influenza vaccine, she presented with multiple 2.5- to 5-cm hyperpigmented patches and plaques with a peripheral rim of erythema (, A), some with overlying flaccid bullae, located on her bilateral hips and lower back (, B). She had a background of numerous hyperpigmented patches coalescing on the back, flanks, and buttocks consistent with postinflammatory hyperpigmentation as a result of a similar bullous eruption after the influenza vaccine 1 year prior.
A thorough medication review failed to find any other recent medication changes. New bullae continued to appear across her groin, lower back, buttocks, axillae, and abdomen over the subsequent days. Although a few oral erosions involving the hard palate and vermilion lips were present, there was no evidence of ocular or genital mucosal involvement. There was complete sparing of the distal extremities including the palms and soles. She remained afebrile with a normal complete blood count and comprehensive metabolic panel.
Biopsy found vacuolar interface dermatitis with subepidermal vesicle formation, epidermal necrosis, marked pigment incontinence, and mild lympho-eosinophilic infiltrate (, C). These findings were consistent with the biopsy results from 1 year prior. The episode at that time was of a similar clinical presentation and resolved after 7 days of topical clobetasol 0.05% ointment.
During the second bullous eruption, the patient required use of both topical clobetasol ointment and an oral prednisone taper. Within 2 days of hospitalization, new lesions continued to appear, now covering 15% of her total body surface area. The patient was transferred to the burn intensive care unit for aggressive wound care. She was discharged after 14 days in the burn intensive care unit, with complete resolution at 30 days.
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pmc-6197948-1
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21-year-old man presented to the Emergency Department complaining of a lower abdominal pain and constipation for 5 days. He had neither a history of chronic medical illness nor abdominal operations. His vital signs were normal, and the abdomen was mildly distended. X-ray of the abdomen was normal. The patient was diagnosed to have a constipation and was discharged home on Psyllium (ispaghula husk) sachets 7 g twice daily as a laxative. Two days later, the condition of the patient became worse and he returned to the Emergency Department complaining of increased abdominal distension without passing any stools, yet, he was passing flatus. On examination, the vital signs were normal. His abdomen was markedly distended but soft and lax. The intestinal sounds were audible. P/R examination was normal with soft fecal matter. Repeated abdominal X-ray showed a huge fecal loading filling the whole colon with no gas fluid levels ().
Computed tomography (CT) scan of the abdomen with oral contrast has shown marked dilatation of the whole colon () especially the sigmoid colon which was markedly distended and filled with gas and fecal matter (). The patient was diagnosed to have an incomplete intestinal obstruction and was admitted to the surgical department for further management. During his stay in the hospital, the patient admitted that prior to the onset of the constipation he had ingested psyllium husks as herbal medicine for the purpose of weight control and health promotion. He ingested the husks without adequate amount of fluids because he was fasting in Ramadan (during fasting hours, no eating or drinking fluids are allowed). While in the hospital, the patient received repeated enemas. He passed a huge amount of fecal matter and the intestinal obstruction was relieved. The patient was discharged home two days later without any operative intervention.
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pmc-6197951-1
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A 40-year-old man was taken to the local hospital by his office supervisor by foot due to concerns over moving a foreign object impaling his chest (A). As soon as the emergency physician noted that an ice pick was penetrating the man’s left chest, we established an intravenous line while preventing the object from moving from its original position, and an ambulance took the patient to our emergency department.
On examination, the patient’s general condition was not critical (Glasgow Coma Scale score 15, respiratory rate 24 breaths/min, blood pressure 123/79 mmHg, heart rate 76 beats/min, oxygen saturation 100% on 2 L nasal cannula, and body temperature 37.2 °C). The patient would not reveal the actual cause of the injury. An interview with his family disclosed no history of depressive disorder, pharmacological treatment, substance use, or alcohol abuse, but the patient had a history of several pneumothorax injuries, one 10 years prior and two a year prior to this episode. In all episodes, he had been hospitalized for several days for chest drainage and discharged without any complications. Considering his condition, we strongly suspected a self-inflicted injury. However, our patient denied any suicidal ideation, depressive mood, or hopelessness. His family members described him as a quiet and gentle person who did not behave impulsively.
Cardiovascular auscultation was unremarkable without murmurs or gallops. Chest X ray demonstrated a clear lung field without pneumothorax or hemothorax (B). Emergency echocardiography disclosed a small amount of pericardial effusion without cardiac tamponade. Computed tomography (CT) of the chest showed linear metallic density in the pulmonary trunk and a small amount of pericardial fluid (A–D). Pneumothorax or bulla was not seen on chest CT. Based on the diagnosis of penetrating cardiac injury, we transferred the patient to the operating theater after cardiac surgery consultation.
We placed the patient in the supine position. After performing a full median sternotomy, the pericardium was opened. There was a small amount of pericardial effusion that seemed to be mixed with blood. The ice pick had been stuck in the main pulmonary artery (MPA) through the pericardium without any injury to the left lung or internal thoracic artery. Heparin was given and cardiopulmonary bypass (CPB) was commenced with ascending aortic and bicaval cannulation. The patient was cooled down to 32 ° Celsius. After aortic cross clamping, the cardioplegia was infused into the aortic root to obtain cardiac arrest. A longitudinal incision was made in the MPA, and we carefully removed the foreign body, which was lodged through the MPA from the anterior to posterior wall near the annulus of the pulmonary valve without injury to the left main coronary trunk. The holes made by the ice pick were closed using 5-0 polypropylene suture. The MPA was closed using 4-0 polypropylene over and over running suture. After rewarming and deairing, the aortic clamp was removed. The patient’s sinus rhythm came back spontaneously. CPB weaning was smooth, and protamine was given. The chest was closed in a normal fashion. Postoperative recovery was uneventful.
A police investigation showed no criminal events associated with the injury. Although the patient denied stabbing himself with the ice pick, we strongly suspected the penetrating cardiac injury was self-inflicted and consulted the psychiatric department on day 7 to closely monitor the patient during hospitalization. During the psychiatric counseling sessions after the surgery, the patient continued to stubbornly deny a suicide attempt. At 16 days POD, the patient was discharged to home without psychiatric follow-up.
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pmc-6197958-1
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A 16-year-old male presented at our hospital with postprandial abdominal pain that had been occurring for about 3 years. The patient was refusing to attend high school because of the abdominal pain, and he had symptoms of depression. Enhanced three-dimensional computed tomographic angiography of the abdomen showed stenosis of the celiac trunk (). An abdominal echogram showed translocation and deformity of the celiac artery between inspiration and expiration (A), and an abdominal Doppler ultrasonic echogram showed that the blood flow of the celiac trunk varied between inspiration and expiration (b). Hence, the patient was diagnosed with MALS. Informed consent was obtained from the patient and his parents, and laparoscopic ligament dissection was performed.
General anesthesia was induced, and the patient was placed in the supine position with a 30° degree both legs opened. An open method was used to insert a 12-mm umbilical camera port, two 5-mm ports in the left upper abdomen, and a 10-mm and a 5-mm port in the right upper abdomen. Laparoscopy was performed with a 30° laparoscope (KARL STORZ Endoskope, Tokyo, Japan). After liver retraction, a Harmonic Scalpel® (ETHICON, Tokyo, Japan) was used to open the minor omentum and dissect between the right diaphragmatic crus and the gastric ligament. The left gastric vein and artery were identified and taped (). The median arcuate ligament (MAL) and nervous plexus around the celiac artery were identified, and the thickened diaphragmatic crura were exposed (). The MAL and the nervous plexus were then dissected, and the dissection line was continued to the front of the abdominal aorta. The diaphragmatic fibers anterior to the aorta were dissected for approximately 5 cm in the cephalad direction, exposing about 4 cm of the abdominal aorta (, ). Intraoperative Doppler ultrasonography confirmed that the celiac arterial blood flow no longer varied between expiration and inspiration (). After surgery, the patient’s symptoms were relieved, and decompression of the celiac artery was confirmed using three-dimensional computed tomographic angiography (). The patient was discharged from hospital on postoperative day 7, and has no recurrent symptoms at 12 months postoperatively.
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pmc-6198069-1
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A 28-year old female presented with a history of progressive left-sided temporal vision loss over a year. She noticed that she was running into objects and people on the left side of her field of vision. The patient also complained of a dull ache in her left eye but denied any other focal neurological symptoms.
On physical examination, the only pertinent finding was left temporal hemianopia.
MRI of the orbits with contrast revealed a heterogeneously enhancing large mass lesion occupying the mid- and posterior thirds of the optic nerve pathway. The typical “tram-track” appearance of sheath enhancement around the central optic nerve expected for an optic nerve meningioma was absent. Rather, the lesion essentially replaced the optic nerve and appeared to demonstrate infiltration into the nerve, which raised the suspicion for an optic nerve glioma (Figure ). The lesion extended through the optic canal with a component extending superiorly onto the left side of the planum sphenoidale rather than remaining intrinsic to the nerve, as it would be expected for an optic nerve glioma. By virtue of dural involvement of the planum sphenoidale, this mass was suggestive of an atypical left ONSM, which had on some images apparently replaced the nerve (Figure ). Given this unusual clinical presentation and the lack of typical findings on MRI, decision was taken in a multidisciplinary tumor board to proceed with a 68Ga-DOTATATE PET/CT scan, particularly to rule out an optic nerve glioma. Biopsy was deemed too morbid in this context.
68Ga-DOTATATE PET/CT scan revealed an asymmetric fusiform enlargement of the left optic nerve with associated conspicuous diffuse radiotracer uptake and maximum standardized uptake value (SUVmax) of 10.8. Portions of the lesion showed increased attenuation on non-contrast CT, suggesting calcification. There was a nearby but separate focus of activity more superoposteriorly, which localized to the left aspect of the planum sphenoidale (Figure ). The combination of anatomic and metabolic findings was compatible with an optic sheath meningioma, as an optic nerve glioma, similarly to a pilocytic astrocytoma, would not be expected to demonstrate significant uptake on 68Ga-DOTATATE PET ().
Based on this non-invasive diagnosis, volumetric-modulated arc therapy (VMAT) to a total dose of 50.4 Gray (Gy) in 28 fractions was delivered. A VMAT radiation plan (Figure ) was chosen over a proton therapy plan (Figure ) because of improved dose conformity and target coverage in the former. The patient had no major complications. She developed Common Terminology for Adverse events (CTCAE) Grade 1 fatigue and Grade 1 headaches during treatment. Five months after treatment completion, the patient had significant improvement in her left temporal hemianopia based on subjective report and on objective assessment through a formal visual field examination performed by her ophthalmologist. The meningioma on the 2- and 5-month follow-up MRIs was found to be less enhancing and its size was slightly decreased to stable (Figure ), indicating good local control.
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pmc-6198101-1
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A 41-year-old woman underwent breast conserving surgery and axillary dissection in February 2002. The Pathological results revealed that she had pT2N2M0 disease (stage IIIA, luminal-HER2 type breast cancer). The patient underwent postoperative chemotherapy using 4 cycles of 5-fulorouracil plus epirubicin plus cyclophosphamide. As trastuzumab had not been approved as an adjuvant therapy in Japan at that time, the patient also received luteinizing hormone-releasing hormone agonist (LHRH-a) with tamoxifen and tegafur plus uracil after the chemotherapy and whole-breast radiotherapy. At 4 years after surgery, and during adjuvant systemic therapy, she experienced recurrence in multiple supraclavicular lymph nodes. Thus, first-line treatment for MBC was started using paclitaxel (PTX; 80 mg/m2 on days 1, 8, and 15) and trastuzumab (4 mg/kg as a loading dose followed by 2 mg/kg as a weekly maintenance dose). After 4 cycles of the first-line treatment, the patient achieved a complete radiological response and a non-pathological values for CEA and CA15-3. The patient remained in cCR during 5 years of maintenance therapy using trastuzumab, and subsequently terminated systemic therapy. The last follow up was August 2018 and she has survived for 11.5 year after termination of anti-HER2 therapy ().
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pmc-6198101-2
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A 41-year-old woman with cT3N2M0 disease (stage IIIA, luminal-HER2 type cancer) underwent preoperative chemotherapy using 2 cycles of epirubicin plus cyclophosphamide followed by 2 cycles of weekly PTX in 2013. Mastectomy and axillary lymph node dissection revealed a Grade 1b therapeutic effect. The association between pathological complete response and long-term outcomes was strongest in patients with triple-negative breast cancer and in those with HER2-positive, hormone-receptor-negative tumors who received trastuzumab []. However, the impact of pathological CR on luminal-HER2 type breast cancer patients is currently unknown.
The patient subsequently received trastuzumab and LHRH-a with tamoxifen, but did not undergo post-mastectomy radiotherapy. At 2 years after surgery, and during adjuvant endocrine therapy, pathology results revealed lung and internal mammary lymph nodes metastases. Thus, first-line treatment for MBC was started using docetaxel (75 mg/m2 on day 1) with pertuzumab (840 mg as a loading dose followed by 420 mg on day 1 of each subsequent cycle) and trastuzumab (8 mg/kg followed by 6 mg/kg on day 1). After 4 cycles of the first-line therapy, the patient achieved a complete radiological response and a non-pathological values for CA15-3 and NCC-ST-439. She subsequently underwent irradiation to the chest wall and internal mammary lymph node region, and received maintenance therapy using pertuzumab plus trastuzumab for approximately 18 months. She stopped maintenance therapy at October, 2017. The last follow up was August, 2018 and she has survived for 10 months after termination of anti-HER2 therapy ().
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pmc-6198101-3
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A 32-year-old woman was diagnosed with cT3N3M1 disease (HER2-enriched breast cancer), and multiple lung metastases were detected in CT in 2014. Docetaxel with pertuzumab and trastuzumab was not approved as a first-line treatment in Japan at that time. Radiological evaluations revealed no therapeutic effect from 2 cycles of first-line treatment using epirubicin (90 mg/m2) plus cyclophosphamide (600 mg/m2). Thus, weekly PTX and trastuzumab were administered as second-line therapy, and the patient achieved cCR after 4 cycles. She continued maintenance therapy using trastuzumab for 1 year and subsequently terminated her therapy at December, 2015. The last follow up was June 2018 and she has survived for 2 years and a half month after termination of anti-HER2 therapy ().
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pmc-6198101-4
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A 56-year-old woman with cT4bN2M1 disease (HER2-enriched breast cancer) had contralateral lymph node metastasis that was pathologically detected in 2016. The patient started first-line treatment using docetaxel with pertuzumab and trastuzumab, achieved cCR after 4 cycles. Although the tumor disappeared from her left chest, an abscess-like secretion persisted from a skin ulcer. Mastectomy and sentinel lymph node biopsy were performed, and confirmed a pathological complete response. The patient continued maintenance therapy using pertuzumab and trastuzumab, but subsequently terminated systemic therapy after approximately 18 months at November, 2017. The last follow up was July, 2018 and she has survived for 8 months after termination of anti-HER2 therapy ().
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pmc-6198127-1
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A 55 years old female obese patient with Body Mass Index (BMI) of 42 kg/m [] (120 kg, 1.70 m) with past medical history of hypertension, type 2 diabetes mellitus, dyslipidemia, arthritis. She had also a past surgical history of open appendectomy, incisional hernia treated with intraperitoneal mesh, complicated with right colic erosion and fistula requiring right colectomy. She arrived from another hospital with multiple median and transverse abdominal scar incisions and recurrence of an enormous incisional hernia in her right iliac fossa (). No tobacco or alcohol intoxications were noted. Preoperative upper fibroscopy revealed BE along 2 cm in height without dysplasia on biopsy. A multidisciplinary team decided to perform SG initially followed by incisional hernia repair. Open SG was performed. No intraoperative complication occurred. Two weeks later a gastric leak appeared requiring surgical reoperation and drainage by Kehr tube. Fistula was dried up six months later.
Seventeen months after SG and a 40 kgs weight loss, the median and right iliac hernias were repaired with pre-aponeurotic polypropylene mesh along with abdominal dermolipectomy. The latter operation was complicated with cutaneous dehiscence, which required treatment by vacuum therapy. One year later, a supraombilical incisional hernia was repaired successfully using a preaponeurotic subcutaneous polypropylene mesh ().
Three years following SG, the patient presented with complaining of persistent vomiting over several months. Upper digestive tract opacification showed lacunar image on the lower esophagus (). Endoscopy revealed 5 cm pseudo-polyp neoformation located 30–35 cm from the dental arches. Upper endoscopic ultrasound described a supra-cardial pediculate hypoechoic lesion of 2 cm in diameter without peritumoral lymph nodes. After multidisciplinary discussion, endoscopic mucosectomy was done (). Biopsy showed well-differentiated intra-epithelial adenocarcinoma arisen in BE with safe surgical margins and without lymphatic embolus or perineural sheathing; classified as pT1 with negative HER2 expression. Work up being negative for hepatic or extrahepatic disease, close supervision and follow up of the patient with serial gastroscopy were suggested later on.
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pmc-6198218-1
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A 52-year-old man was first diagnosed with melanoma in 2008 (Clark level IV, Breslow 0.61). In February 2015, he was diagnosed with multiple MBM (one frontal left, two frontal right, one occipital right), metastases of the cervical spine (C2, C5, C6), and a lymph node metastasis in the neck. A biopsy of the lymph node metastasis showed the absence of a BRAF mutation. A treatment with ipilimumab was initiated (3 mg/kg every 4 for weeks. The MBM were each treated with SRT (20 Gy in one fraction). After three cycles of ipilimumab, the patient had a progressive intracranial and extracranial disease. A treatment with pembrolizumab was initiated at a dose of 2 mg/kg.
A cerebral MRI in December 2015 showed an increase in volume of the left frontal lesion (Figure A). As the patient had a complete remission of the extracranial disease and the other MBM remained stable, there was a suspicion of RNB. Further investigations with an MR spectroscopy were inconclusive in differentiating between RNB and disease progression.
In January 2016, the patient developed complex partial epileptic seizures. A treatment with corticosteroids was started. Due to a worsening neurological symptoms and increasing size of the lesion, a surgical resection was indicated. The histology confirmed the presence of RNB. In July 2016, the patient developed a paresis of the right hand due to increasing edema surrounding the resection cavity. The patient had a good clinical response after initiation of corticosteroids. However, after tapering he had a recurrence of neurological symptoms in October 2016. A treatment with bevacizumab at 5 mg/kg was initiated during 4 cycles. The corticosteroids could be stopped with a neurological stabilization; however, 5 months later he had an increased neurological deficit. Thereupon bevacizumab treatment was resumed with symptomatic improvement.
Until the last follow-up in December 2017, the patient remained in complete intracranial (Figure A) and extracranial remission after 14 cycles of pembrolizumab.
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pmc-6198218-2
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In March 2012, a 63-year-old woman was diagnosed with a melanoma on the right arm (Clark IV, Breslow 2.5 mm). In August 2013, a treatment with DTIC/carboplatinum was initiated for the diagnosis of a metastatic melanoma (stVIM1c). After two cycles, the patient had a progressive disease with the diagnosis of two MBM. In November 2014, these were treated with SRT (20 Gy, 1 fraction). At that time, treatment with ipilimumab (3 mg/kg) was initiated. In March 2014, two new asymptomatic MBM were diagnosed and treated with SRT (20 Gy, 1 fraction). For progressive disease, a systemic treatment with fotemustine was initiated in June 2014. After for a total of three cycles, a treatment with pembrolizumab (2 mg/kg) was started. Over several months, a right frontal MBM increased in size with an increase in perilesional edema (Figure B). In January 2015, there was a further increase in size and edema and a surgical resection was indicated (Figure B). The histological analysis confirmed the presence of RNB (Figure ). After the surgery, the corticosteroids could be stopped. Currently, the patient is still treated with pembrolizumab and has no active intracranial or extracranial disease.
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pmc-6198218-3
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A 43-year-old woman was diagnosed with a BRAF wild-type metastatic melanoma in 2014. After surgical resection of a single lymph node metastasis, the patient was recruited for the DC-MEL study examining the effect of an autologous dendritic cell vaccination in patients with stage III/IV melanoma without measurable disease (EudraCT number: 2011-001410-33). In June 2014, a treatment with ipilimumab (3 mg/kg) was initiated for progressive disease. In September 2015, she developed liver and brain metastases. A treatment with pembrolizumab at 2 mg/kg was immediately initiated en the solitary MBM was treated with SRT in October 2015 (20 Gy). In April 2016, a complete extracranial remission was obtained. In June 2016, the patient experienced complex partial epileptic seizures followed by a paresis of the left foot. The cerebral MRI showed an increased volume of the lesion with important perilesional edema, which was suggestive for RNB. An MRI spectroscopy was performed that confirmed the diagnosis of RNB. After a treatment with corticosteroids, the patient had a good clinical response; however, in August 2016 she had increasing epileptic seizures. A treatment with bevacizumab (7.5 mg/kg, every 3 weeks, 4 cycles) was initiated with a good clinical and radiological response. Until latest follow-up, the patient has no clinical or radiological signs of disease recurrence.
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pmc-6198218-4
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A 53-year-old man was randomized in the DC-MEL trial (EudraCT number: 2011-001410-33) in May 2014 after the resection and radiation of lymph node metastases of a BRAF wild-type melanoma. In December 2014, he developed recurrent lymph node metastases and a left occipital MBM. A treatment with Trimix DC vaccination and ipilimumab (3 mg/kg) was initiated. After four cycles, he was progressive and a treatment with pembrolizumab (2 mg/kg) was started. Due to the development of an autoimmune colitis and orchitis, the treatment with pembrolizumab had to be stopped after 4 cycles. In June 2015, he developed a right parietal MBM that was treated with SRT (1 × 20 Gy). One month later, the right parietal lesion disappeared; however, there were two new cerebral lesions. These were also treated with SRT (1 × 20 Gy). One month later, a treatment with temozolomide (autoimmune side effects) was initiated for a progressive extracranial disease. In June 2016, there was an increase in volume of the right parietal brain lesion. The cerebral MRI was suggestive for RNB. The FDG-PET CT showed the presence of hypometabolic lesion, which strongly suggests RNB (Figure C). The patient remained asymptomatic, and no specific treatment was necessary. The patient remains in complete remission.
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pmc-6198218-5
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In July 2011, a 48-year-old woman had a bilateral mastectomy with axillary lymph node resection for metastases of a BRAFV600E-positive melanoma followed by a treatment with vemurafenib. After 11 months of treatment, the patient had progressive disease. A treatment with ipilimumab (10 mg/kg) and DC vaccination was initiated (TriMixDC-MEL plus ipilimumab). In November 2012, the patient developed a MBM frontal right that was treated with SRT (1 × 20 Gy). After 18 months of maintenance therapy with ipilimumab, the treatment a rechallenge with vemurafenib/trametinib was initiated for progressive disease. In October 2016, pembrolizumab was initiated for a progressive disease. The cerebral MRI at that time showed an increase in volume of the right frontal metastasis with significant perilesional edema. The appearance was suggestive for RNB. In absence of neurological symptoms, no specific treatment was initiated. A stable intra- and extracranial disease was obtained. In June 2017, the right frontal lesion further increased. The 18-FET-PET imaging showed a hypometabolic activity frontal right, which confirmed the presence of RNB. In July 2017, the patient developed headaches and a transient neurological deficit. A treatment with corticosteroids was initiated with an improvement of the neurological condition. Because a tapering of corticosteroids was impossible, the treatment with pembrolizumab was interrupted and a treatment with dabrafenib/trametinib was initiated.
Despite the treatment with corticosteroids, there was a further neurological deterioration in September 2017. Hence, a neurosurgical resection was performed in October 2017. The histological analysis confirmed the presence of radiation necrosis without signs of residual disease (Figure ). Postoperatively, the corticosteroids could be tapered and stopped; however, the patient developed increasing headaches and a left hemiparesis. The cerebral MRI showed an increased contrast enhancement in the resection cavity and increasing perilesional edema. As there was only a minor improvement after reinitiating corticosteroid therapy, a treatment with bevacizumab was initiated in November 2017. Under this treatment, the patient had a remarkable neurological improvement with the recuperation of the left hemiparesis. Until latest follow-up, the patient had a systemic and radiological stable disease.
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pmc-6198281-1
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A 5-year-old boy presented with an unsightly and protuberant swelling over the anterior third of tongue that had started as a small nodule soon after birth and gradually progressed in size after 9 months of age. It was associated with difficulty in swallowing, speech, recurrent blistering with pain and bleeding with minor trauma. He was unable to contain it within his mouth necessitating to sleep open-mouthed. He had also developed multiple dental caries with it.
He had been managed conservatively with beta-blockers and steroids elsewhere for years with no appreciable benefit. General examination was normal. Oral examination revealed a well demarcated swelling involving the anterior third of tongue extending dorso-ventrally (occupying mostly the ventral surface) and measuring 6×5×3 cm3, red in color with a purplish hue. It had a smooth surface, with fine granularity. It was partially compressible and soft in consistency as shown in Fig. . Also noted were multiple dental caries. Examination of the neck and rest of the systems was unremarkable.
A working clinical diagnosis of macroglossia secondary to lingual hemangioma was made and partial glossectomy using an inverted V incision was performed as shown in Fig. .
The limbs of V were approximated to reconstruct the residual tongue as shown in Fig.
At 10 day outpatient follow up, improved swallowing, speech clarity and good healing was observed as shown in Fig. .
The histopathology report showed mucosa lined by stratified squamous epithelium, with underlying stroma showing dilated vascular channels lined by endothelial cells and containing red blood cells in their lumen confirming the diagnosis of lingual cavernous hemangioma as shown in Figs and .
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pmc-6198365-1
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A 49-year-old female presented to our hospital in September 2016 due to numbness and weakness in both lower extremities for 2 weeks. Because of progressive weakness involving both lower limbs, the patient became disabled with unstable gait, unable to walk independently. Physical examination showed 4-grade muscle strength of both lower legs. After admission, detailed examinations were performed. Electromyogram revealed findings consistent with an acquired demyelinating polyneuropathy with increased distal latencies, conduction blocks, prolonged F wave latencies, and reduced conduction velocity. The cerebrospinal fluid examination was normal. Ultrasonography detected splenomegaly, ascites, pericardial effusion, and pleural effusion. No signs of sclerotic or lytic lesions were noted in X-ray or CT scans. Blood cell count and clinical biochemistry examinations were normal. Serum monoclonal protein was detected by serum protein electrophoresis. A largely elevated serum vascular endothelial growth factor (VEGF) level (2350.0 pg/mL) was detected by enzyme-linked immunosorbent assay (ELISA). Decreased cortisol and thyroxine level was recorded. Bone marrow aspiration and biopsy identified monoclonal plasma cells. According to immunohistochemistry (IHC) analysis, plasma cells (CD138+) accounted for 3%, and BCMA and CD138 expression was highly concordant (Fig. ). 1.1% of cells were considered monoclonal plasma cells in flow cytometry (Fig. ). According to the latest diagnostic criteria for POEMS [, ], the diagnosis of POEMS syndrome, in this case, was established based on the following evidence: major items including polyneuropathy, monoclonal plasma cell neoplasm, and elevated serum VEGF and minor items including splenomegaly, endocrine abnormality, and extravascular volume overload.
We started treatment with lenalidomide (25 mg/day for 21 days of a 28-day cycle) and once-weekly dexamethasone (10 mg). Serum monoclonal protein drastically decreased and neuropathy relieved after treatment for two cycles; however, frequent episodes of treatment-related orthostatic hypotension resulted in dose reduction of lenalidomide (10 mg/day for 21 days of a 28-day cycle) for the next 3 months. The serum monoclonal protein was largely reduced, and walking difficulty alleviated. However, the serum monoclonal protein quickly re-augmented, and symptoms worsened as low-dose treatment continued (Fig. ). The patient refused recommended salvage treatment with the bortezomib-based regimen or autologous stem cell transplantation (ASCT). Considering the reported anti-myeloma effects of anti-BCMA CAR-T cells [, ] and abundant expression of BCMA on monoclonal plasma cells (98.6% as revealed by flow cytometry) (Fig. ) and elevated serum BCMA (Fig. ) in this patient, we encouraged her to participate in a clinical trial involving anti-BCMA CAR-T treatment, to which she agreed. Chemotherapy (fludarabine 25 mg/m2 and cyclophosphamide 20 mg/kg) was administered 4, 3, and 2 days before the first infusion of CAR-T cells (June 25, 2017) which was followed by two infusions administered once daily for the next 2 days. The effective anti-BCMA CAR-T cells totaled /1 × 107/kg (Fig. ).
The patient developed a fever (37.4 °C, peaked 39.2 °C on the next day, and lasted for 7 days) and tachycardia about 24 h after the first infusion. The peak of serum ferritin and IL-6 (17.5 times higher than baseline) was detected on the third-day post-infusion, which was just the time where toxicity culminated (Fig. ). Cytopenia was observed but not central nervous system (CNS) toxicities. White blood cells and neutrophils returned to normal on the tenth day after infusion. No infusion of red blood cells or platelets was required during the entire treatment period (Fig. ). All coagulation and biochemistry parameters remained normal over the treatment period. She experienced grade 1 cytokine release syndrome (CRS), which was graded as described [, ]. The CAR-T cells remained detectable, and B cells gradually returned to normal levels in the blood 10 months after infusion (Fig. ).
Her serum monoclonal protein started to decrease 15 days after CAR-T cells infusion and became undetectable 90 days after infusion (Fig. ). The soluble BCMA and VEGF levels also decreased in a short time after treatment (Fig. ). Sixty days post-infusion, no abnormal plasma cells were found by bone marrow aspiration or flow cytometry (Fig. , ), and she was able to do some cooking and walk without difficulty. An electromyogram showed that the conduction velocity of bilateral tibial nerves, peroneal nerves, sural nerves, and superficial peroneal nerves recovered, and the sensory nerve action potential (SNAP) amplitude was in normal range. F waves from bilateral tibial nerves showed a normal latency of 26.7 ms.
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pmc-6198366-1
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A.M was a 3 year old female child of African ethnicity who presented in July 2012 with a 8 day history of high grade fever and a 1 day history of swelling of the hands and feet. (Table – timeline of case 1). She had received oral antibiotics, anti malarials and antihistamines with no improvement in symptoms. Her past medical history had been uneventful and her vaccinations were uptodate.
On examination, she was alert, had dry, red lips and non pitting edema on her hands and feet. She had cervical nodes measuring about 0.5 cm. Her cardiovascular exam was normal. She was admitted for further work up for the cause of her fever.
Her results showed an elevated WBC count of 36,000/μl, Hb of 9.1 g/dl and platelets of 380,000/μl. Her CRP was 173 mg/l. Malaria antigen and slide were both negative. Her urinalysis was normal. She was started empirically on ceftriaxone for presumed bacteremia and blood and urine cultures were sent. A differential diagnosis of sickle cell anaemia with dactylitis was also made and a peripheral smear, reticulocyte count and sickling test were requested. The reticulocyte count was low with a percentage of 0.38. Sickling test was negative and the peripheral smear was normal.
She continued to have fever spikes despite the antibiotics and then developed a maculopapular hyperemic rash on her chest. A diagnosis of incomplete kawasaki disease was now made which met 3 out of the 5 required criteria. An echocardiogram was done which showed normal coronary arteries. High dose aspirin was started at 80 mg/kg/day. IVIG was not available at the institution at that time. A repeat complete blood count showed some improvement in white blood cells (28000/μl) but elevated platelets of 644000/μl. The blood and urine culture were both reported as negative after 48 h.
The child was then flown out to Nairobi, Kenya for IVIG treatment which she received uneventfully. She was discharged on low dose aspirin and subsequent echocardiograms remained normal.
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pmc-6198376-1
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We present a case of an 18-year-old man with acute renal failure that was admitted to the hospital. The patient, who worked as a chef and reported himself as a smoker,had no known past medical history. He presented with abdominal pain, vomiting and diarrhoea. At presentation he had a tachycardia of 98 beats/min, with a blood pressure of 139/72 mmHg, a body temperature of 36.9 °C and normal urine output. Routine blood analysis showed leucocytosis (40,5 × 109/l (87% neutrophils)) and a serum creatinine of 233 μmol/l, resulting in an estimated glomerular filtration rate of 32 ml/min/1,73 m2, C-reactive protein was 44 mg/l, ALT 32 IU/l, AST 78 IU/l, GGT 25 IU/l, Alkaline Phosphatase 73 IU/l, bilirubin < 17 μmol/l and albumin 24 g/l. Post renal obstruction was excluded using ultrasound sonography. Urine analysis showed erythrocyturia, without casts, and massive proteinuria (protein/creatinine ratio 842.7 mg/mmol creatinine with urine creatinine of 15.7 mmol/l). It was considered as an acute glomerulonephritis, most likely caused by an infectious cause, e.g., leptospirosis or hantavirus infection, or an autoimmune disease, e.g., AAV or anti-GBM glomerulonephritis, considering age, history and physical examination. No specific history indicating hantavirus infection was recorded, e.g., cleaning up dusty sheds, contact with rodents.
ANCA was negative. Anti-GBM was 9.7 kIU/l, which is within equivocal range (7–10 kIU/l).
The detectable anti-GBM antibodies, with hantavirus serology still in progress, lead to a diagnostic and therapeutic dilemma, even though it was still in equivocal range. Treatment of anti-GBM glomerulonephritis consists of plasmapheresis in combination with immunosuppressants, which may be disadvantageous in case of a viral infection and has possible adverse effects. Late initiation of therapy in case of an anti-GBM glomerulonephritis, however, would increase the risk of developing end-stage renal failure [].
Because of the equivocal anti-GBM antibodies, a slight improvement of renal function on day 2 and no other signs or symptoms of anti-GBM glomerulonephritis, like elevated C-reactive protein or pulmonary involvement, there was a considerable doubt about anti-GBM glomerulonephritis being the correct diagnosis. Therefore no renal biopsy was done and plasmapheresis and immunosuppressants were not administered.
Four days after admission a second anti-GBM measurement showed an increase in anti-GBM antibodies and was undeniably positive (16.0 kIU/l), with further renal and clinical improvement.
Only supportive care was initiated. Dialysis was not needed. The patient’s kidney function improved without further intervention and after 5 days of admission he was discharged from the hospital.
On day seven serum was found positive for anti-Puumala hantavirus IgG as well as IgM antibodies, suggesting acute viral infection.
The spontaneous improvement of renal function is consisting with the natural course of NE and hantavirus infection became the most likely diagnosis. No renal biopsy was done to exclude other causes.
Twelve weeks after admission, renal functions were completely restored and anti-GBM antibodies were no longer detectable. A second serum sample showed a persisting highly.
positive result for anti-Puumala IgG (Ratio 5.2), and a disappearance 118 of IgM antibodies.
119 (Ratio 0.03; < 1.0 is regarded negative) consistent with a completed seroconversion of anti-Puumala hantavirus antibodies, confirming the diagnosis of acute hantavirus infection (Table ).
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pmc-6198377-1
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A 63 year-old male farmer with a history of a tick bite presented with a sudden onset of fever on May 28, 2014, with a temperature of 38.6 °C, accompanied by rash, nausea, anorexia, fatigue, enlarged lymph node, and general body aches. He presented to the emergency department of The First Affiliated Hospital of China Medical University (CMU) on June 4. Routine blood tests showed leukocytosis with peripheral blood plasmacytosis (white blood cell [WBC] count, 24.46 × 109/L; proplasmacytes, 5%; mature plasma cells, 18%) and thrombocytopenia (platelets[PLT], 75 × 109/L). Cytological examination of the bone marrow demonstrated plasmacytosis. With a preliminary diagnosis of MM, he was admitted to the Department of Hematology of CMU on June 6. Physical examination revealed palpable swollen lymph nodes in the submandibular and bilateral axillary regions, accompanied with dispersed red papules on chest and abdomen. Laboratory tests upon admission showed thrombocytopenia, increased alkaline phosphatase (ALP) and lactate dehydrogenase (LDH) levels, prolonged prothrombin time (PT) and activated partial thromboplastin time (APTT). Immunofixation by electrophoresis revealed a polyclonal pattern with increased amounts of immunoglobulin (IgA, IgG, and IgM) and immunoglobulin light chains. Proteinuria was observed but Bence Jones proteinuria was negative. Because of abnormal bone marrow cytology, X-ray of skull, thoracic and lumbar vertebrae, and pelvis was performed, with normal findings. ELISA and RT-PCR were performed to detect SFTSV-specific IgM/IgG and SFTSV RNA as previously described [, ]. And the IgM antibody and RNA to SFTSV were positive. Bone marrow cytology showed plasmacytosis, with plasma cells accounting for 29.2% of all nucleated cells, including proplasmacytes (6.8%) and mature plasma cells (22.4%) (Fig. ). However, flow cytometric immunophenotyping (FCI) of bone marrow revealed that the increased plasma cells were not monoclonal (Fig. ). The patient was administered doxycycline, human immunoglobulins, plasma transfusion and other supporting treatments, and was improved. Finally, a diagnosis of reactive plasmacytosis associated with SFTS was reached. The clinical and laboratory findings are summarized in Table .
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pmc-6198377-2
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A 42 year-old female farmer who had a sudden onset of fever on July 7, 2014, with a temperature of 39.2 °C, accompanied by rash, nausea, and multiple skin petechiae or ecchymosis. She visited a local hospital on July 14, 2014. Laboratory examination indicated pancytopenia, increased aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels, and normal coagulation index. Three days later, no clinical improvement was observed, and she was admitted to the Hematology Department of The First Affiliated Hospital of CMU. Laboratory tests found prolonged APTT and decreased fibrinogen (Fg). Serum enzymes, including creatine kinase (CK), LDH, and AST, were also assessed. Serum IgM and IgG antibodies and RNA to SFTSV in patient samples were detected by ELISA and RT-PCR respectively which were performed by Liaoning CDC as previously described [, ]. The patient was suspected with SFTSV infection, and sent to the Department of Infectious Disease of our hospital on July 22. Meanwhile, serum amylase and lipase levels were obviously elevated, with no abdomen pain; however, the patient showed apathy, with suspicious neck stiffness. Certain amounts of abnormal plasma cells were found in both BM (proplasmacytes, 39.6%; mature plasma cells, 10.8%) (Fig. ) and peripheral blood (proplasmacytes, 10%; mature plasma cells 36%). In addition, about 44.7% suspicious abnormal monoclonal plasma cells were determined by FCI on July 23 (Fig. ). Thus, malignant plasma cell disease could not be excluded. The immunoglobulins (IgA, IgG, and IgM) and their light chains were polyclonal as tested by Immunofixation. Here also, proteinuria was positive but Bence Jones proteinuria was negative. On July 26, gastrointestinal symptoms such as nausea, vomiting and abdominal discomfort began to appear; abdominal enhanced computed tomography was performed, and indicated pancreatic edema and multiple swollen lymph nodes in the left inguinal and inner thigh regions. Doxycycline, human immunoglobulins, somatostain, plasma transfusion, and the other supportive medications were administered to the patient. On July 31, the IgM antibodies and nucleic acid of SFTSV were detected, with her clinical symptoms improved. In the meantime, abnormal plasma cells in the bone marrow and blood were absent at reexamination of bone marrow cell morphology (Fig. ) and FCI (Fig. ). Eventually, a diagnosis of reactive plasmacytosis in association with SFTS was reached. These clinical findings are summarized in Table .
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pmc-6198380-1
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A 14-month old boy presented with respiratory distress, wheeze and hypoxia. This was preceded by 24 h of coryza, fever and reduced feeding. He was admitted directly to intensive care, where he was diagnosed with pneumonia. Treatment was started with empirical antibiotics. He was intubated and ventilated for 4 days, after which he was extubated and stepped down to high dependency unit (HDU) on continuous positive airways pressure (CPAP) ventilation. He continued to have persistently increased work of breathing, persistent expiratory wheeze and symptoms and signs of air trapping.
Though he did not progress to respiratory failure, weight gain and oxygenation was achieved only by initiation of Heated Humidified High Flow Nasal cannula therapy (HHHFNC).
Polymerase chain reaction (PCR) testing of nasopharyngeal aspirates (NPAs) revealed the persistence of rhinovirus and adenovirus for 10 weeks in both NPAs and bronchoalveolar lavages (BALs), with parainfluenza type 3 found in just one NPA sample. Adenovirus was also found in blood by PCR testing. Rhinovirus is not normally tested for in blood samples and there was no validated assay available for this. One BAL and one urine sample were screened for cytomegalovirus (CMV) by PCR also, and found to be negative. One stool sample was tested and found to be negative for rotavirus and adenovirus (despite the persistence of adenovirus in the respiratory samples at this time).
There was no significant family history of any genetic diseases and the patient was born at term. Mum is a smoker but states that she did not smoke during her pregnancy. During pregnancy, intrauterine growth restriction was identified. At the time of admission his weight was < 0.4th centile, but this was increasing. His parents report poor feeding since birth and an increased work of breathing from 5 months old. He is known to the allergy services for severe eczema and faltering weight, and has been diagnosed with cow’s milk protein allergy. His eczema medications have included topical tacrolimus, moderately potent topical steroids and emollient therapy.
His other medical history includes two previous episodes of bronchiolitis, three and 1 month prior to this admission (including one overnight stay during a trip to Australia). Prior to admission, he had only received two courses of antibiotics in his life – once for an infected BCG vaccine site and once for an ear infection.
During his inpatient stay, he had 2 further intercurrent bacterial respiratory infections, treated by broad spectrum antibiotics. Immunological investigations revealed an IgG and IgM hypogammaglobunemia but a normal IgA. He was up-to-date with his childhood immunisations prior to admission, but has had a poor response to tetanus and twice to haemophilus B, demonstrated by low IgG levels to both. He was also found to have near absent B-cells and a reduced number of T-cells, and prophylaxis for Pneumocystis jiroveci was commenced. HIV testing was negative on two occasions. We withheld any further live virus vaccinations, in view of his immunodeficiency (Table , Fig. ).
At this stage, a CT chest was performed (Fig. ). The findings (mosaic pattern attenuation of both lung fields with a combination of air trapping and oligaemia, more pronounced in expiratory images) were consistent with BO which was believed to be secondary to the persistent adenovirus rather than the rhinovirus infection. Reasons for this were mainly that rhinoviruses are predominantly upper respiratory tract infections, whereas AdVs can cause more systemic infections and AdV DNA was detected in the blood. No biopsy or histopathological investigation was performed in order to confirm BO.
A flexible bronchoscopy showed structurally normal airways. Screening for tuberculosis was negative.
After a multidisciplinary team discussion, it was decided to commence pulse methyl-prednisolone therapy (3 daily doses of 10 mg/kg given every month) and long-term azithromycin (10 mg/kg 3 doses every week) for management of BO. Prior to commencing the steroids, cidofovir was administered to control the adenovirus replication and reduce the dissemination of the adenovirus.
Cidofovir (5 mg/kg, intravenously, with probenecid) was given on fortnightly basis for 4 months, during which he his renal function was monitored weekly. All the patient’s renal function test results remained within our institutional laboratory’s normal ranges (sodium 133–146 mmol/L; potassium 3.5–5.0 mmol/L; urea 1.5–5.0 mmol/L; creatinine 15–31 μmol/L) throughout the treatment period with cidofovir, indicating good tolerance to the drug. Since commencing steroid therapy, it was possible to gradually wean him off his HHHFNC, and he has since been thriving well. He was discharged on supplemental oxygen, which was weaned off over the next 12 months.
An array comparative genomic hybridisation (CGH) revealed mosaic tetrasomy of short arm of chromosome 9. There are no reports yet of immunodeficiency in association with mosaic tetrasomy 9p, nor any specific vulnerability to any particular viral infections [], but has previously been associated with auto-immune conditions associated with IFN-1 dysregulation (predisposing to inflammatory myositis and lupus-like syndrome) [].
A conclusive diagnosis of a specific immunodeficiency/immunomodulation is yet to be made in this case, as this profile does not fit any primary immune deficiency. Molecular genetic testing for mutations in 82 genes associated with primary immunodeficiency and gastrointestinal disorders (TIGER panel, Great Ormond Street Hospital, London) did not identify any clearly pathogenic variants. The patient is currently being given weekly supplemental immunoglobulins.
Most recently, the child has now cleared the long-term rhinovirus and adenovirus infections, and has been weaned off steroids and long term oxygen therapy. His weight gain has been adequate on air, and he is still too young for spirometry testing. Ongoing problems include recent Pseudomonas aeruginosa infection, with a wet cough, which is likely due to the bronchiectasis component of the BO disease.
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pmc-6198422-1
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A 53-year-old male denied past medical and trauma history presented to our hospital with blurred vision of his right eye for one week with floaters and obscured sensation over nasal visual field. Ocular examination showed a 120 degree retinal tear (from 7 to 11 o’clock) with large inverted rigid flap and retinal detachment from 6 to 12 o’clock with macula-off in his right eye (Fig. ). The grading of proliferative vitreoretinopathy (PVR) was “Grade C”. The BCVA of his right eye was only naming digit at his first presentation. Under the impression of GRT with retinal detachment, 23-gauge pars plana vitrectomy were performed using Constellation high speed vitrectomy system (Alcon Surgical, Fort Worth, TX, USA) and Topcon non-contact wide angle viewing system (Topcon Medical Inc. Livermore, CA, USA). During surgery, the temporal retina was noted to have a rolled and stiffened edge at the posterior aspect of the giant retinal tear (Fig. ). The vitreous was removed and tractions were relieved as much as possible, then perfluorocarbon liquids (PFCL) was injected to help unfolding the large inverted retinal flap (Fig. ). Three stainless steel retinal tacks were inserted along the posterior edge of the giant retinal tear using a modified Southerland intraocular forceps [] to help fixating the large inverted retinal flap (Fig. and ). Then, fluid-gas exchange, endolaser photocoagulation and intraocular silicone oil tamponade were performed as well (Fig. ). Initial reattachment of his right retina was achieved and his best corrected visual acuity improved to 0.3 of his right eye postoperatively. There was no recurrent retinal detachment during follow up period of 19 months (Figs. and ).
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pmc-6198426-1
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A previously healthy 34-year-old Korean female was admitted to a regional hospital for fever, myalgia and severe headache that had started 2 weeks ago. Brain MRI showed a high T2 signal intensity change and diffuse swelling of the right temporal lobe, insula and hippocampus (Fig. ). Lumbar puncture showed lympho-dominant pleocytosis. The patient was started on intravenous (iv) dexamethasone and acyclovir under a high suspicion of viral encephalitis.
She was transferred to our institution 4 days later due to persistent headache despite treatment. A noncontrast computed tomography (CT) brain scan taken at our institution showed a hemorrhagic transformation of the right temporal lobe, which was not observed on the initial MRI (Fig. ). Follow-up lumbar puncture showed 510 white cells per mm3 (82% lymphocytes), 144 mg/dL protein and 61 mg/dL glucose. CSF culture studies were negative for bacteria, fungi and tuberculosis. PCR of the CSF confirmed the presence of HSV1. The patient was free of neurological symptoms, with a Glasgow Coma Scale of E4M6V5, and was admitted for close observation and continuation of iv acyclovir. Corticosteroid treatment was discontinued upon her admission. On day 3 of hospitalization, the patient presented with a sudden onset of vomiting and severe headache. Brain CT showed an increased amount of temporal lobe hemorrhage and a leftward shift in the midline (Fig. ). Mannitol was administered but did not seem to have a significant effect. The patient became increasingly drowsy, and her right pupil became dilated. She underwent emergency right decompressive craniectomy, expansile duraplasty and ICP monitor insertion. Postoperative brain CT showed alleviation of midline shifting (Fig. ). The patient recovered fully 5 days after the surgery. Apart from mild intermittent headache and dizziness, she did not show any other significant clinical symptoms, including neuropsychological problems. There were no significant neurologic deficits upon neurological examinations performed by the attending neurosurgeon and neurologist. The patient was discharged after completion of 2 weeks of acyclovir and returned 2 months later for cranioplasty. She was followed up 3 more times after cranioplasty. She was stable, without any neuropsychological problems or neurologic deficits, and was able to successfully return to work as a public official.
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pmc-6198447-1
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A 52-year-old male with a five-year history of type 2 diabetes mellitus presented with loose stools, abdominal pain and reduced urine output for two days. He had ingested of 200 ml of homemade star fruit juice made from four whole star fruits a few hours prior to the onset of symptoms. Notably he complained of intractable hiccoughs. His serum creatinine three months prior to the presentation had been 0.7 mg/dl. On admission he was mildly dehydrated and had a blood pressure of 140/90 mmHg. There was no evidence of diabetic retinopathy. Investigations revealed the following: haemoglobin- 13.5 g/dl, white cell count – 17, 840/ cumm (Neutrophils 79%, Lymphocytes- 10%, Eosinophils-0%), platelets 345,000/ cumm, serum creatinine 4.5 mg/dl, serum potassium 5.3 mmol/l, serum sodium 138 mmol/l, C – reactive protein- 164 mg/l. The urine sediment was bland with no proteinuria. His Anti-nuclear antibody (ANA) titre, Anti-streptolysin O titre (ASOT), Hepatitis B, C serology, Antineutrophil cytoplasm antibody (ANCA) titre, and Complement 3 (C3) and Complement 4 (C4) levels were normal. Renal ultrasound showed normal kidneys with preserved cortico-medullary demarcation. Urine and blood cultures were sterile. He was commenced on intravenous cefotaxime for suspected sepsis. By day 5 of illness serum creatinine rose to 9 mg/dl leading to the initiation of haemodialysis.
Renal biopsy was done on the sixth day. This showed ten glomeruli, one of which was sclerosed, the others being normal. Some of the tubules showed oxalate crystals associated with acute tubular epithelial injury and evidence of regeneration. Patchy tubular atrophy was seen. The interstitium was oedematous and infiltrated by a moderate inflammatory infiltrate comprising lymphocytes, plasma cells, eosinophils and neutrophils. Mild interstitial fibrosis was seen. A diagnosis of star fruit induced oxalate nephropathy was made.
He required haemodialysis only once and was discharged on the seventh day with a falling serum creatinine. Serum creatinine three months later had stabilized at baseline levels.
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pmc-6198447-2
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A 65-year-old male with a history of type 2 diabetes mellitus and hypertension presented with poor appetite, poor sleep, nausea and dyspeptic symptoms for five days. His urine output had been normal. On examination he was afebrile. His blood pressure was 120/70 mmHg. His laboratory investigations on admission revealed a serum creatinine of 7.3 mg/dl and serum K of 5.9 mmol/l. His serum creatinine done seven months before had been 1.2 mg/dl. Urinalysis was bland with no proteinuria. Serum ANA, ANCA, C3/C4, and Hepatitis B and C serology were normal.
He had not taken any prescription or over the counter medications in the recent past except for his usual anti diabetic medications. On direct questioning he admitted eating three fruits of star fruit immediately prior to the onset of symptoms. A clinical diagnosis of acute star fruit nephrotoxicity was made.
His serum creatinine remained static despite good urine output. A renal biopsy was performed due to the delay in recovery. It revealed acute tubulo-interstitial nephritis without oxalate deposition. Prednisolone was started at 30 mg daily. His serum creatinine subsequently improved and 10 months later had reduced to 1.4 mg/dl.
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pmc-6198447-3
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A 57-year-old male with a history of hypertension and type 2 diabetes mellitus was admitted with loss of appetite, nausea and diarrhoea. On examination he was afebrile with a blood pressure of 140/90 mmHg and background diabetic retinopathy. His serum creatinine was 13.16 mg/dl on admission with serum potassium of 4. 8 mmol/l. His urinalysis revealed 8–10 pus cells and 35–40 red cells per high power field without any casts or proteinuria. His ANA, ASOT, Hepatitis B, C serology, ANCA and C3/C4 were normal. His renal ultrasonography showed normal sized kidneys with multiple calculi in the upper and middle calyces of the right kidney with cortical thinning and slightly increased cortical echogenicity of the left kidney.
On direct questioning he revealed having consumed on average one star fruit daily over the preceding one year with increased consumption to three fruits per day over the preceding one month.
He underwent several sessions of haemodialysis. A renal biopsy was performed which revealed tubular injury in the presence of oxalate crystals within the tubular lumina (Fig. ). There was mild tubular atrophy and mild focal interstitial fibrosis. Some glomeruli were enlarged. Three glomeruli showed increase in mesangial matrix. Few glomerular capillaries showed thickened basement membranes Appearances were compatible with oxalate nephropathy occurring in the background of early diabetic nephropathy.
He was started on prednisolone 30 mg daily. His serum creatinine subsequently improved and had reduced to 2.98 mg/dl two months after the initial admission.
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pmc-6198491-1
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We report the case of an 82-year-old man, who underwent a total body computed tomography (CT) on February 2017, due to the occurrence of cough. CT showed an extensive mass in the left upper lobe of the lung. Thus, a bronchoscopy with transbronchial needle aspiration (TBNA) was performed. The cytological examination was compatible with lung adenocarcinoma. Epidermal growth factor receptor (EGFR) mutations and anaplastic lymphoma kinase (ALK) translocation were tested to determine the most appropriate treatment but no mutation was detected. It was not possible to test PD-L1 expression because only cytological samples were available.
To complete the staging of the disease, the patient underwent a positron emission tomography (PET) examination. PET showed a massive tracer uptake at the pulmonary mass and showed an extensive involvement of the hilar and mediastinal lymph nodes. Before starting the treatment, a further TC scan was performed in May. TC showed an increased pulmonary mass involving approximately the entire left lung.
The patient referred a smoking history and as comorbidities: arterial hypertension, osteoporotic and traumatic vertebral fractures, iatrogenic bone marrow lesion resulting from surgery for discopathy, benign prostatic hypertrophy treated with transurethral resection, pulmonary emphysema, carotid vasculopathy and abdominal aneurysm. He had an ECOG performance status of 2.
In relation to clinical conditions, age and comorbidities, the patient underwent two chemotherapy cycles with oral vinorelbine (day 1,8 every 21), the latter of which was administered in July. During the treatment, the patient experienced fatigue G1, diarrhea G1, constipation G1, anorexia G1 and hyperkinetic supraventricular arrhythmia treated with amiodarone.
The restaging CT was performed in July and showed lung disease progression. Thus, from July to December, the patient received 3 mg/kg nivolumab (day 1 every 14) as second-line treatment for a total of seven cycles. Based on body weight, nivolumab was administered at a dose of 195 mg for the first two cycles and 205 mg for the subsequent five cycles. After the first two doses, the patient was hospitalized at the emergency department with a diagnosis of pneumonia. Therefore, antibiotics and corticosteroids were administered and a clinical improvement was obtained. In September, the patient recovered a good respiratory performance, so he was able to restart nivolumab. The subsequent five cycles were well tolerated except for grade 1 hypothyroidism and grade 2 fatigue.
Collaterally, in September, the patient showed a small nodular dome shaped lesion on the upper eyelid. He consulted a private ophthalmologist who initially suspected a diagnosis of chalazion. However, the lesion increased until about 3 cm within a few weeks. Thus, the patient came back to the Ophthalmology Department of Azienda Ospedaliera-Universitaria Careggi of Florence where a biopsy was performed, with the suspect of a malignant lesion. The histological examination indicated an MCC (immunohistochemical study: cytokeratin 20+, synaptophysin+, chromogranin+, CD20-, CD3-, Ki67 60–70%) (Fig. ).
A nuclear magnetic resonance imaging of the orbit and neck did not show lymph node involvement.
The case was collegially discussed. Since the patient was at high risk for anesthesia due to his clinical conditions and given the prognosis of lung cancer, surgery was contraindicated. Thus, a palliative radiotherapy was proposed and was started in December when the lesion had expanded to about 5 cm in the anteroposterior diameter. Following radiotherapy, the size of the eyelid lesion was reduced to a few millimeters.
In December 2017, after a total of seven cycles of nivolumab, a CT scan was also performed. It showed a lung and cerebral disease progression. In addition, there was a worsening of clinical conditions with increased cough and fatigue. Therefore, the best supportive care was provided until the patient died in March 2018.
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pmc-6198507-1
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A 56-year-old Japanese woman presented with right-sided Bell’s palsy due to dysfunction of the facial nerve (VII) in October 2013. She had been diagnosed as having rheumatoid arthritis in August 2013 and treated with methotrexate orally. She had no relevant medical family history. Methotrexate was discontinued when she suffered from Bell’s palsy. Her condition improved following oral administration of 30 mg of prednisolone/day. Soon after prednisolone was discontinued in November 2013, methotrexate was resumed. In March 2014, she was admitted to a general hospital due to the appearance of bilateral floaters. Her best corrected visual acuity was 20/20 in her right eye and 20/25 in her left eye; an ophthalmological examination revealed bilateral granulomatous uveitis. Chest computed tomography (CT) revealed bilateral hilar lymphadenopathy and mediastinal lymph node swelling. Laboratory tests revealed elevated levels of serum angiotensin-converting enzyme (ACE) (35.0 IU/L; normal, 8.3–21.4 IU/L). She was histologically diagnosed as having sarcoidosis following skin and transbronchial lung biopsies. In February 2015, she developed dysphagia due to dysfunction of the vagus nerve (X), following which she was treated with 30 mg of prednisolone/day. Following an improvement in her symptoms, prednisolone dosage was tapered to 4 mg/day by August 2015. However, she experienced recurrence of nerve VII palsy in November 2015, despite continued treatment with 4 mg of prednisolone/day. In December 2015, magnetic resonance imaging (MRI) revealed brain parenchyma lesions (Fig. ), although no neurological symptoms, such as motor/sensory paralysis or paresthesia, were observed. In January 2016, she experienced recurrence of nerve X palsy, following which she was treated again with 30 mg of prednisolone/day. In May 2016 (prednisolone, 20 mg/day), she exhibited increased intraocular pressure (IOP) in her right eye and was referred to our hospital for treatment.
At the initial visit, her best corrected visual acuity was 20/20 in her right eye and 20/28 in her left eye. The IOP was 40 mmHg in her right and 14 mmHg in her left eye. There was no inflammation in the anterior chamber, although massive, diffuse vitreous opacities were observed in both eyes (Fig. ). Retinal periphlebitis and macular edema were detected using fluorescence angiography. In July 2016, she underwent trabeculectomy of her right eye, following which the right eye IOP decreased to 10 mmHg. No postoperative inflammation was observed. As a result, 20 mg of prednisolone/day was continued, and she experienced no worsening of brain parenchyma lesions or recurrence of cranial nerve palsy to date. The clinical course is presented in Fig. .
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pmc-6198527-1
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A 56-year-old male patient with a history of paroxysmal atrial fibrillation was diagnosed with hypopharyngeal squamous cell carcinoma in June 2011. The disease progressed with pathology-proven metastatic liver tumor, for which he first received RFA in October 2012. In June 2014, abdominal sonography disclosed a mixed echogenic tumor of about 5 × 4 × 4 cm located in the left lobe just below the heart. A computed tomography scan showed a recurrent liver tumor of around 3.6 cm in the S2 segment (Fig. ). He was admitted for repeat RFA on August 5th, 2014. Three Covidien 15 × 3 cm cool-tip needles were inserted under echo guidance, and RFA was smoothly applied for a total of 16 min. No immediate complications were noted during the procedure.
Upper abdominal fullness was noted after the RFA. Abdominal sonography showed no new ascites and his hemogram remained stable. Hemorrhagic complications were therefore unlikely. The abdominal pain and fullness gradually subsided on the next day, however new-onset chest pain and paroxysmal atrial fibrillation with a rapid ventricular response developed. Fever and chills were also noted, and a physical examination revealed friction rub. An electrocardiogram (ECG) showed diffuse ST elevation except for leads AVR and V1 (Fig. ), and the baseline ECG was normal (Fig. ). A chest X-ray showed a water-drop appearance and left pleural effusion (Fig. ). Inflammatory markers including leukocyte count and the concentration of serum C-reactive protein (CRP) were elevated (Hb 9.6 g/dL, WBC 10300/L, CRP 19.8 mg/dL), however levels of cardiac enzymes were not elevated (CK 202 U/L, CK-MB 0.6 ng/mL, troponin-I 0.013 ng/mL). Echocardiography showed a small amount of pericardial effusion with a maximal depth of about 1.12 cm, without signs of tamponade (Fig. ). Acute pericarditis was the most likely diagnosis, and he was treated with aspirin 100 mg and diclofenac 100 mg per day. Three days later, the ST segment (elevations) had mostly returned to baseline (Fig. ) and the size of his heart and left pleural effusion had also decreased (Fig. ). His symptoms improved and the levels of inflammatory markers declined (Hb 9.1 g/dL, WBC 7720/L, CRP 8.357 mg/dL). He was discharged uneventfully five days later. There was no pericardial effusion (Fig. ) and the ST segments had totally returned to baseline (Fig. ) 1 month later.
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pmc-6198544-1
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A seventy-three-year-old gentleman presented with progressive shortness of breath over a two-month period. His medical history was significant for Klinefelter syndrome, heart failure with reduced ejection fraction, obstructive sleep apnoea, hypogonadism, haemochromatosis, and secondary polycythaemia requiring 6–12 monthly venesections. Clinical examination was unremarkable. On presentation, he had a normochromic normocytic anaemia with a haemoglobin of 82 g/L, a reticulocyte count of 2 × 109/L, and an elevated haptoglobin (). Vitamin B12, folate, and thyroid-stimulating hormone studies were normal, and serum ferritin was increased (). These laboratory results, notably the markedly reduced reticulocyte count, were consistent with reduced production of red cells in the bone.
Bone marrow biopsy demonstrated a marked reduction in erythroid precursors (two percent of the differential) consistent with pure red cell aplasia (). Normal granulopoiesis and megakaryopoiesis were evident. Autoimmune screening (ANA, ENA, dsDNA, RF, and anti-cardiolipin antibodies) and viral screening were negative (hepatitis B, hepatitis C, human immunodeficiency virus, and parvovirus B19). Serum protein electrophoresis and immunosubtraction were negative for monoclonal bands, and flow cytometry was normal. There were no recent medication changes. A chest computed tomography was performed revealing an anterior mediastinal mass consistent with thymoma (). An elective thymectomy was arranged with a cardiothoracic surgeon, and intermittent blood transfusions were provided while awaiting surgery.
Thymectomy was performed via a median sternotomy. Histopathology demonstrated normal thymic tissue mixed with mature adipose tissue, diagnostic of thymolipoma. No inflammation, granulomata, or neoplasia was identified. Three weeks following thymectomy, the patients' haemoglobin normalised with a complete resolution of his symptoms. After a year of follow-up, the patients' haemoglobin remains normal, without immunosuppressive therapy or ongoing transfusions.
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pmc-6198545-1
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A 45-year-old BRCA-negative Caucasian female presented with a self-detected breast mass and signs of breast dimpling. Imaging demonstrated a 9.5 cm breast mass with biopsy returning a finding of ER/PR positive invasive lobular carcinoma of intermediate grade. She underwent uneventful bilateral mastectomy revealing invasive lobular BC involving one out of 12 axillary lymph nodes; stage IIIA T3 N1. Bone scan prior to adjuvant chemotherapy was negative. She had no acute abdominal pain but reported some recurrent abdominal discomfort during the past year. Therefore, a CT scan was done, which demonstrated a significantly enlarged appendix without stranding or inflammation indicating AMC (). Appendiceal carcinoma or BC metastasis could not be ruled out. The patient had a normal WBC. Possible appendix malignancy and risk to develop AA during chemotherapy were discussed, and consent for LA was obtained.
5 mm trocars were placed in the left upper and lower quadrant and a 10–12 mm trocar into the umbilicus. The AMC was gently lifted up (); at no point, the appendix was grasped. A window was created behind the AMC, and the mesoappendix was stapled. The cecal pole was mobilized, and the AMC was stapled off with a 1 cm rim of cecal wall () avoiding stenosis of the terminal ileum. The specimen was immediately placed into an endobag and removed from the abdomen via the dilated umbilical port. The postoperative course was uneventful. Histopathology revealed a benign cystadenoma with clean margins and no rupture. Chemotherapy for her BC was started four weeks later. Bilateral salpingo-oophorectomy was done a year later. The patient is alive and well without tumor recurrence and without intraperitoneal symptoms after 3 years.
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pmc-6198549-1
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A 34-year-old male presented to our Emergency Department via emergency medical services complaining of palpitations, shortness of breath, and cough with one episode of hemoptysis that started earlier that day. The patient reported that his symptoms rapidly progressed a few hours prior to arrival shortly after smoking crystal methamphetamine. The patient reported chronic methamphetamine use for 10 years, becoming an everyday user 3 years ago. He admitted to occasional polysubstance use that included ketamine, ecstasy, and alcohol, although those were limited to a few times a month. Past medical history was significant for two remote spontaneous pneumothoraces, but the patient stated that his current symptoms felt unrelated. Past surgical history was noncontributory and he denied allergies or taking any prescribed medications. Reviews of systems were positive for myalgias and fatigue. The patient denied any chest pain, chest tightness or abdominal pain.
Initial vital signs include the following: HR: 153 bpm, RR: 20, BP: 115/56 mmHg, Temp (O): 102.1 °F, and SpO2 96% (RA).
The physical exam demonstrated a well-nourished, well-kept male appearing in his stated age in good physical shape. The patient appeared anxious and diaphoretic with dry mucus membranes and labored breathing. The patient was alert and oriented but found to have mild confusion upon detailed questioning. The rest of the neurologic exam was normal and the patient did not display any meningeal signs. Auscultation of the chest revealed rales at the lung bases. Cardiac exam demonstrated a regular, tachycardic rhythm without the presence of obvious murmurs, rubs, or gallops. There was no lower extremity edema present and patient displayed normal muscle tone. The rest of the physical exam was unremarkable.
Upon the patient's initial presentation, orders for initial management were with a normal saline bolus of 2 liters, supplemental oxygen with 2 liters on nasal cannula, broad spectrum antibiotics (piperacillin/tazobactam and vancomycin), antipyretics, and multiple doses of IV lorazepam with the aim of treating his tachycardia with a mixed picture of sepsis, dehydration, and methamphetamine intoxication.
Point-of-care ultrasound (POCUS) of the inferior vena cava (IVC), heart, and lungs was obtained. The IVC diameter measured 1.85 cm (max) in inspiration and 1.71 cm (min) in expiration (Images -, ). Cardiac views demonstrated gross biatrial and biventricular dilation on visual inspection with a severely reduced left ventricular ejection fraction (LVEF) and a small pericardial effusion (Images -, Videos -). Lung ultrasound demonstrated bilateral lung sliding anteriorly and B-lines in bilateral inferolateral lung fields (Images -, Videos -). With the information obtained via US, the decision was made to consult cardiology and to restrict IVF administration by giving the patient 1 liter of normal saline over the course of two hours instead of a 2-liter bolus.
Electrocardiogram (ECG) revealed sinus tachycardia with a normal axis and without ST elevations, PR depressions, or significant T-wave inversions (). Chest X-ray demonstrated a right lower lobe consolidation with evidence of central pulmonary venous congestion and cephalization (). Significant laboratory findings included a WBC count of 21,000 with a neutrophilic predominance of 81%, lactic acid of 1.8 mmol/L, Pro-BNP of 1,741 pg/ml, and Troponin I < 0.04 ng/ml. The rest of the basic blood works including renal function and electrolytes were within normal limits for our institution. Computed tomography (CT) pulmonary angiogram was performed and demonstrated a right lower lobe consolidation and no evidence of pulmonary embolism. Also evident on CT were atrial and ventricular enlargement, central pulmonary venous congestion, and signs of early pulmonary edema (Images -).
The patient was admitted to the ICU and was continued on broad-spectrum antibiotics and a normal saline infusion of 75 ml/hr. On day two, all IV fluids were discontinued and diuresis was employed for decompensated heart failure with developing hypoxia. The patient grew 2/2 positive blood cultures for Streptococcus pneumoniae and was found to be Influenza B positive. Comprehensive 2D Echocardiogram revealed dilated cardiomyopathy with LVEF of 16%, restrictive left ventricular filling pattern, elevated filling pressures with E/e'=30, moderate/severe mitral regurgitation, and severe tricuspid regurgitation.
The patient improved during hospitalization and was discharged on day 9 with a diagnosis of severe sepsis, right lower lobe pneumonia, Streptococcus pneumoniae bacteremia, Influenza B, and Methamphetamine-Associated Cardiomyopathy.
He was discharged on antibiotic therapy and a heart failure regimen consisting of lisinopril and metoprolol.
His cardiac dysfunction improved with drug abstinence and medical therapy. A four-month echocardiogram demonstrated resolution of dilated atrial and ventricular chambers, with improvement of LVEF to 45% and improvement in valvular dysfunction to only trace mitral and tricuspid regurgitation.
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pmc-6198557-1
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A healthy 27-year old woman without any history of thrombosis or thrombophlebitis, gravida 1 para 0, conceived spontaneously. There were no problems with the course of her pregnancy. At 40 weeks of gestation, she vaginally delivered a healthy 3156-g baby, and vacuum extraction was needed because of a nonreassuring fetal status. On the fourth day after delivery, she complained of a 38.5 degree fever and lower abdominal pain. The right side of her uterine fundus was tender to palpation. There was no rebound pain or muscular defense. There was no evidence of infection in her perineal laceration. Transabdominal ultrasonography showed no abnormal findings. Her blood profile was as follows: white blood cells, 12600/mm3 (normal range: 4000-9000/mm3); C-reactive protein, 2.2 mg/L (normal range: <0.3mg/L). Her urine profile was unremarkable. She was treated with antibiotics (1.5 g ampicillin/sulbactam 4 times per day) according to a diagnosis of postpartum endometritis. Her symptoms persisted despite the antibiotic treatment. On the seventh day after delivery, her right lower abdominal pain worsened, and rebound pain appeared as well. In addition, right costovertebral angle tenderness appeared. There was no tenderness in her uterine fundus. Her blood and urine profiles showed no remarkable changes. On the eighth day, an abdominal dynamic CT scan showed a sausage-like, ring-enhanced right retroperitoneal mass with a diameter of 27 mm, which was distinct from the right ovarian vein (). Retroperitoneal appendicitis was suspected, and exploratory laparoscopy was performed on the ninth day. During the surgery, a normal appendix and right ovarian vein were identified. The peritoneum located on the dorsal side of the appendix was bulging. When the peritoneum was incised, blood clots appeared on the right side of the right ovarian vein. No additional pathologic processes were identified. After the surgery, we reconstructed the CT images for further investigation. The coronal reconstructed CT images revealed that the retroperitoneal mass was connected to the right renal capsule vein (). Based on a diagnosis of thrombophlebitis of the right renal capsular vein, anticoagulant treatment (2000 IU subcutaneous enoxaparin twice per day) was started on the tenth day. Antibiotics were stopped because no signs of infection were observed during the surgery. Her temperature returned to normal on the fourth day of anticoagulation, and her abdominal pain disappeared on the sixth day. On the seventh day, anticoagulation was stopped, and she was discharged from our hospital. After a month of follow-up, she was still in good condition, and the CT showed that the mass had disappeared ().
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pmc-6198566-1
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A 75-year-old woman (weight: 72 kg) was admitted to the Emergency Department for agitation with an altered consciousness. Patient's medical history was unremarkable, except for arterial hypertension treated by atenolol. Symptoms started acutely a few hours earlier, with a progressive loss of verbal contact. On admission, the Glasgow Coma Score (GCS) score was 9/15 (E4, V1, M4), with moderate neck stiffness. There was no lateralized deficit and pupils were mid-size, reactive, and symmetric. Vital signs were as follows: body temperature of 36.6°C, arterial blood pressure of 180/95 mm Hg, heart rate of 120/min, and respiratory rate of 60/min. A brain computed tomography (CT) without iodinated contrast agent (CA) perfusion before lumbar puncture did not reveal any significant abnormality. Intubation was required because of progressive respiratory distress. The cerebrospinal fluid (CSF) analysis revealed white blood cells (WBC) count at 560/μl, with 99% granulocytes, proteins at 1264 mg/dl, glucose at 3 mg/dl, and lactate at 27 mmol/l.
The CSF and blood cultures grew positive for Streptococcus pneumoniae sensitive to penicillin G and ceftriaxone. The minimal inhibitory concentration (MIC) was 0.016 mcg/ml for penicillin G and ceftriaxone in the CSF and 0.012 and 0.008 mcg/ml in blood for penicillin G and ceftriaxone, respectively. Treatment combining dexamethasone (10 mg q6h for 4 days) and ceftriaxone (2 g q12h for 14 days) was initiated. CSF analysis was repeated after 10 days and confirmed both a drop in WBC count and eradication of the causative microorganism.
The patient was subsequently referred to the Intensive Care Unit (ICU) because of worsening of GCS score at 6/15 (E1, V1, M4). Despite the lack of evidence of acute hydrocephalus, intracranial pressure (ICP) was monitored by intraventricular catheter and remained within the normal range during the whole ICU stay. The mean arterial blood pressure was around 80 mm Hg. No sedative drugs were required for mechanical ventilation and GCS score remained stable at 6/15. A control brain CA-enhanced CT after 72 hours of therapy failed to reveal brain abscesses, thrombosis, or ischemic lesions. The patient was repeatedly examined by electroencephalogram (EEG) in order to exclude nonconvulsive status epilepticus. There was only diffuse slowing with predominance of delta and theta waves, together with some triphasic activity. A brain positron emission tomography (PET) using 18fluorodeoxyglucose (FDG) as tracer was performed on day 13 and was consistent with a diffuse cortical hypometabolism and relatively preserved uptake within grey nuclei (). A transcranial Doppler (TCD) examination performed at day 3 suggested that cerebral autoregulation at different levels of mean arterial pressure was abolished. There was no increase in cerebral blood flow velocity (CBFV).
Brain magnetic resonance imaging (MRI) was performed on days 8, 11, and 30. On day 8, while the diffusion-weighted imaging (DWI) and T2/fluid attenuated inversion recovery (FLAIR) sequences were not significantly modified (not shown), there was a marked decrease (300-500 instead of 700.10−6 mm2.sec−1) in the apparent diffusion coefficient (ADC) diffusely in the subcortical areas, at both the supratentorial and infratentorial levels. This finding was suggestive of cytotoxic edema of U-fibers and immediately adjacent superficial white matter. The picture was relatively unchanged on day 11; magnetic resonance spectroscopy in the areas with low ADC values failed to retrieve any peak of lactate. On day 30, while the patient was still comatose, ADC values in the subcortical territories had returned to normal range and no ischemic damage within overlying cortex had appeared.
The patient remained in deep coma (GCS from 4 to 6/15) for more than four weeks but then started progressively to wake up, with eye opening, and became able to understand verbal command.
She presented two episodes of pneumonia during the ICU stay: a first episode with methicillin-resistant Staphylococcus aureus (present at admission screening in the nose and throat sampling) and thereafter a relapse with Pseudomonas aeruginosa which had also been initially detected in the throat. Blood cultures remained negative and the patient did not develop septic shock or acute renal failure. However, due to the extension of nosocomial pneumonia, it was necessary to ventilate the patient with 0.5 FiO2 for a long period. Hypoxemia was never observed. Neuromuscular blocking agents were not used during mechanical ventilation. Thus far, in our opinion, the delayed neurological recovery was independent from these infectious complications. The patient stayed in the ICU for a total of 64 days, mainly because of a difficult weaning from the ventilator due to nosocomial pneumonia and critical illness polyneuropathy. At 6-month follow-up, the neuropsychological testing confirmed excellent recovery.
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pmc-6198577-1
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86-year-old man with a history of hypertension and type 2 diabetes mellitus had been treated for end stage kidney disease with continuous cycling peritoneal dialysis since February 2017. He presented to the home dialysis unit complaining of difficulties with initial drain alarms on his cycler for the last 2 nights and “whitish” dialysate. He denied abdominal pain or constitutional symptoms aside from weight loss associated with resolution of peripheral edema. He did not have any previous episodes of peritonitis or history of TB contact. His examination was unremarkable including normal vital signs and lack of abdominal tenderness.
As per out unit peritonitis protocol, 1L of 2.5% Dianeal was allowed to dwell for 2 hours and the effluent was sent for analysis including cell count, differential, bacterial, and mycobacterial cultures. Given the “milky” appearance of the fluid, triglycerides were also ordered. He received empiric intraperitoneal antibiotics including ceftazidime and vancomycin. Total nucleated cell count was 354 ∗ 106/L with 87% lymphocytes, 8% monocytes, and 3% neutrophils. Cultures were negative. Triglyceride (TG) concentration was 6.3 mmol/L (557 mg/dl). Based on the elevated TG concentration he underwent a CT scan with contrast of the abdomen and a second dialysate sample was sent for cell count, TG, cytology, and flow cytometry (the dialysate was no longer cloudy). He was found to have a mildly enlarged spleen and multiple enlarged lymph nodes in the mesentery, retroperitoneum, and inguinal regions including a cluster of enlarged nodes forming a conglomerate retroperitoneal mass suggestive of lymphoma. There was a moderate increase in density of the mesentery, possibly on the basis of lymphatic obstruction. His total nucleated cell count remained elevated at 420 with 96% lymphocytes; TG concentration was only 0.21 mmol/L. Cytology was negative for malignant cells. Flow cytometry of the dialysate showed predominately monotypic B cells with lambda light chain restriction that coexpressed CD20 and CD19 but lacked CD5 and CD10 suggestive of a monoclonal lymphoid process. An inguinal lymph node biopsy revealed predominant diffuse to nodular pattern of small monotonous lymphocytes, suggestive of B cell lymphoma. Immunohistochemical stain was positive for CD20 (diffuse), BCL2 (diffuse), and few remaining CD21 (FDC). It was negative for CD3, CD5, CD10, CD23, and C43. Kappa and lambda stains were nonspecific. Ki67 proliferation index was less than 5%. Final diagnosis was monoclonal B cell lymphoproliferative disorder, likely of low grade. He is being followed by the malignant hematology team, with no active treatment. From a PD perspective, the patient had no further episodes of chylous ascites and remains on CCPD.
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pmc-6198579-1
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An eight-year-old male presented with loud snoring, witnessed apneas, restless sleep, mouth breathing, nocturnal enuresis, excessive daytime sleepiness, behavioral problems, and academic difficulties due to frequently falling asleep at school. Past medical history included 36-week gestation, hypertension, high-functioning autism spectrum disorder, and attention-deficit/hyperactivity disorder. Physical exam was significant for a body mass index (BMI) of 51.8 kg/m2 (BMI z-score 2.9), a blood pressure value of 105/80 mm Hg (systolic 53.1th percentile-for-age/height, diastolic 93.7th percentile), obese neck, 3+ tonsils, oropharyngeal crowding, hyperactivity, and blunted effect.
In-laboratory polysomnography revealed severe OSA, with AHI 138.2, sleep hypoventilation, and hypoxemia ( and ). Given the severity of his sleep-disordered breathing and morbid obesity, the multidisciplinary pediatric sleep team concluded that the patient was a high-risk candidate for surgery with a very low probability of significant reduction in AHI after adenotonsillectomy. A trial of CPAP was recommended along with weight loss. Both tracheostomy and adenotonsillectomy were planned if these measures failed. During his titration polysomnogram, CPAP via nasal mask was titrated up to 19 cm H2O with only partial reduction of the AHI. Further increases in CPAP pressure were not tolerated.
Despite the pressure intolerance, the patient tolerated the mask well and felt better the following day. Alternative NIV treatment options were considered prior to proceeding with surgery, including VAPS mode with autotitrating expiratory positive airway pressure (EPAP), which has been used successfully in adults with coexisting OSA and hypoventilation syndromes []. NIV mode average VAPS with autotitrating EPAP (AVAPS-AE) with nasal mask was initiated in the clinic and continued for a one-week trial at home. The patient tolerated this well and reported feeling better rested during the home trial. A second titration study was performed one week later to optimize NIV settings. Immediate, substantial reduction of the AHI was achieved, with significant improvement in oxygenation, ventilation, and sleep quality ( and ). A Trilogy® ventilator (Philips Respironics, Murrysville, PA, USA) was prescribed with AVAPS-AE mode and a nasal mask with the following settings: EPAP 5–16 cm H2O, pressure support 4–17 cm H2O, maximum pressure 25 cm H2O, inspiratory time 1.5 sec, rise time 3, tidal volume 390 ml (8 ml/kg), and breath rate 20/min.
In follow-up, data download showed good compliance with therapy and control of OSA, with 90% of days used, and average nightly use of 6.4 h without apneas. The father reported that the child was more active and was no longer falling asleep at school. In the clinic, he demonstrated normal mood and was fully awake, calmer, and more interactive. Further interventions focused on weight management, including an admission to an inpatient multidisciplinary weight reduction program, with plans for adenotonsillectomy if significant weight loss was achieved.
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pmc-6198716-1
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A 75-year-old Caucasian woman was admitted in our department in early fall for altered mental status, opsoclonus, multifocal myoclonus, truncal titubation, and generalized tremor. She had been in usual health until approximately 5 days before, when she was admitted to an internal medicine ward for malaise, tachypnea, nausea, vomiting, and fever. At that moment the symptoms were considered to be suggestive for an upper respiratory tract infection and she was treated with antipyretics. During the fourth day of hospitalization the patient gradually became confused and developed involuntary muscle twitches of all limbs, tremor and “jerky eye movements” which prompted evaluation by a neurologist. Her past medical history consisted of arterial hypertension, diabetes mellitus and chronic autoimmune thyroiditis.
At admission in our department, the patient was somnolent, afebrile, not oriented to person, place and time and was only able to answer “yes” or “no” to simple questions. The pulse was 80 beats per minute, the blood pressure 150/90 mmHg, the respiratory rate 21 breaths per minute and the oxygen saturation 96% while breathing ambient air. Neurological examination revealed mild nuchal rigidity, opsoclonus, bilateral and multifocal myoclonus, more severely affecting the arms than the legs, truncal titubation amplified during active movements, postural and intention tremor, bilateral palmomental reflex, and mild weakness of the right upper limb. She could not maintain a sitting position due to truncal titubation and severe myoclonus.
Initial work-up done in the Department of Internal Medicine was unremarkable with the exception of a mild inflammatory syndrome and mildly increased creatinine kinase levels. Brain computed tomography (CT) scan performed at admission in our department showed leukoaraiosis, without other significant changes. Lumbar puncture revealed normal opening pressure and the cerebrospinal fluid analysis identified 17 leukocytes/mm3, mildly elevated albumin levels (30.6mg/l, local reference range 5–20 mg/l) and normal glucose levels. The electroencephalogram showed slow bilateral frontal waves. At this point we established a presumptive diagnosis of rhombencephalitis with opsoclonus-myoclonus syndrome (OMS) and started work-up for likely causes. Contrast enhanced brain MRI performed 10 days after symptom onset identified bilateral relatively symmetrical scattered white matter hyperintensities, predominantly in the periventricular and subcortical regions, consistent with small vessel disease related changes and no other significant abnormalities. A CT scan of the chest, abdomen and pelvis was performed in order to search for a possible tumor. Results were normal with the exception of a small non-enhancing hypodense lesion in the right ovary which was not correlated with raised CA-125 levels and was considered by the gynecologist an incidental finding. Further work-up for infectious, paraneoplastic, and autoimmune causes of encephalitis (see Table ) was unremarkable, except for positive IgM and IgG antibodies against WNV.
Upon transfer in our department the patient was immediately given empiric therapy with Ampicillin, Ceftriaxone, Vancomycin, and Dexamethasone to cover potential causes of encephalitis and Clonazepam as a symptomatic therapy for myoclonus. The antibiotics were discontinued after the results of the lumbar puncture. Within a couple of days, myoclonus showed visible improvement but the opsoclonus and the cognitive status remained unchanged. Two weeks later, the patient started to improve from the cognitive point of view, as she was more aware of her surroundings and became oriented in time and partly in space. A second electroencephalogram performed at this point showed normal cerebral activity. She was discharged 3 weeks after admission, being able to walk with unilateral support. Neurocognitive evaluation performed at discharge showed severe visuospatial deficit and cognitive impairment (Mini Mental Status score—MMSE—of 12 points, Clock drawing test 2/10 points).
During the following weeks her condition gradually improved and she began to perform more and more activities of daily living by herself. At the follow-up visit, performed 1 month after discharge, neurological examination was normal except for very mild action tremor and mild opsoclonus which was only evident at close examination. Her neurocognitive status greatly improved, as she had a MMSE score of 25 points. A second follow-up visit, performed 6 months after discharge, showed normal neurological examination with complete resolution of previous signs and symptoms. Neurocognitive evaluation was within accepted ranges for her age and intellectual background (MMSE 29 points, Clock drawing test 10/10 points).
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pmc-6199138-1
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We presented a case of a 67-year-old female nursing home resident with a history of diabetes mellitus type 2, hypertension, hyperlipidemia, old ischemic stroke, bronchial asthma, and pernicious anemia on monthly vitamin B12 injection. She was admitted to our hospital with complaints of persistent diffuse joint pain and several tongue ulcerations secondary to traumatic pressure from dentation and associated difficulty swallowing. Otorhinolaryngology and rheumatology services evaluated her and treated her with a course of amoxicillin-clavulanate, acyclovir for 14 days as well as with a short course of oral steroids showing improvement. The serology for herpes simplex was negative. Rheumatological workup was unremarkable apart from elevated C reactive protein (CRP) and low complement 3 (C3) level. She was discharged to a nursing home with a plan to follow-up as an outpatient, but no follow-up was recorded. Two months later she returned with complaints of generalized joint pain and poor intake due to difficulty swallowing. Review of her medical record revealed dysplastic changes during endoscopy in March 2009 showing gastritis and peptic duodenitis. Computed tomography (CT) chest in April 2009 showed incidental left lung nodule and was unchanged on repeat CT chest in October 2009. On physical examination, her blood pressure was 133/70 mmHg, heart rate was 89/min, and oxygen saturation on room air was 99%. She had an enlarged tongue, swollen and tender with several ulcerations between 0.1 and 1 cm in size and with a white discharge. She also had submandibular nontender lymphadenopathy and bilateral joint swelling of shoulder, knee wrist, and elbow. She had normocytic anemia at baseline, mildly elevated white blood cell count 11,000/ml (reference range: 4000-10,000/ml), and elevated blood urea nitrogen/creatinine from a normal baseline two months ago. The urinalysis was negative for protein and positive for trace blood. On the second day of admission, she had gastrointestinal bleed with a drop of her hemoglobin from 8 to 6.4 mg/dl. The CT abdomen with an oral contrast was performed which revealed nodular thickening of the stomach suspicious for malignancy and pelvic ascites with high-density fluid. Endoscopy was postponed as there was concern that macroglossia could comprise the airway.
Further workup did not show any spikes on serum protein electrophoresis. Antinuclear antibody (ANA) and rheumatoid factor were negative. Complement 4 (C4) was normal and repeat complement 3 (C3) was low. Thyroid function test, C1 esterase inhibitor, and insulin-like growth factor 1 (IGF-1) were normal. Renal function test improved subsequently but did not return to baseline. Ultrasound of kidney showed changes suggestive of the renal parenchymal disease. We started prednisone 15 mg twice daily as per rheumatology consultation and observed marked improvement in her joint pain. The tongue remains unchanged without further discharge. A biopsy of the tongue was performed and histological examination was suggestive of amyloidosis.
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pmc-6199139-1
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We illustrate this method by presenting a 54-year-old male with alcoholic and hepatitis C cirrhosis complicated by portal hypertension, refractory ascites, and hepatic hydrothorax. His recent medical history was significant for ST-elevation myocardial infarction treated by percutaneous angioplasty, pericarditis, hemopericardium treated by the pericardial window, and hepatic encephalopathy. With medical management, the patient’s ammonia level was successfully decreased and his encephalopathy had resolved, however, his ascites and hydrothorax were refractory to aggressive medical management. Interventional radiology was consulted by cardiothoracic surgery to be evaluated for TIPS creation for definitively treating his refractory ascites and hepatic hydrothorax. At the time of evaluation, there was no encephalopathy (ammonia 40 ug/dl), his Model for End-Stage Liver Disease (MELD) score was 12, and his left ventricular ejection fraction was greater than 75% by echocardiography. Therefore, he was considered an acceptable risk for TIPS creation.
A TIPS was successfully created from a right hepatic vein to a right portal vein with a 10 centimeter (cm) x 6/2 cm Viatorr stent graft (WL Gore and Associates, Flagstaff, AZ, USA). This reduced the portosystemic gradient from 11 mmHg to the desired 5 mmHg. Following this, the patient’s condition improved and he was discharged home in stable condition.
One month later, the patient presented to the emergency department with decompensated congestive heart failure (CHF). The patient was admitted to the medical intensive care unit (MICU) and was aggressively diuresed. A right heart catheterization by the MICU team revealed elevated pressures (pulmonary artery mean 36 mmHg, pulmonary wedge pressure 28 mmHg) suggesting high output right heart failure likely aggravated by the TIPS. Therefore, the patient was referred to vascular and interventional radiology for TIPS revision.
TIPS revision was performed using a right internal jugular vein (IJV) approach. The percutaneous access of the right IJV was performed using a micropuncture kit and upgraded to an 11 French 10 cm sheath (Terumo, Somerset, NJ, USA). Portography was performed with a 5 French pigtail catheter, which showed a widely patent portal vein with hepatopedal flow through the existing TIPS (Figure ).
Following this, pressures within the portal vein and right atrium measured 25 mmHg and 20 mmHg, respectively, for a portosystemic gradient of 5 mmHg. At this point, the decision was made to revise the TIPS with precision using a piecemeal placement of the stent grafts, thus restricting the overloaded cardiac flow and increasing the portosystemic gradient while minimizing the risk of causing variceal hemorrhage. The goal was to precisely bring the portosystemic gradient to 9 mmHg. At the time of revision, his ammonia was 63 and MELD score was 14. Three Viabahn (Viabahn; W L Gore and Associates, Flagstaff, AZ, USA) stents (two 10 mm x 2.5 cm stents followed by one 9 mm x 5 cm stent) were deployed in a sequential “onion skin” fashion to create a customized hourglass stenosis within the hepatic venous segment of the TIPS. Back table images are shown, demonstrating the relative sizes and configuration of the stent grafts (Figure ).
Computer tomography (CT) acquisitions of the stent grafts were captured with three-dimension and multiplanar reformatting for a further illustration of the stent grafts in an “onion skin” configuration (Figure ).
Portosystemic gradients after each stent layer measured 6 mmHg, 7 mmHg, and 9 mmHg, respectively. The right atrial pressure was decreased from 25 mmHg to 10 mmHg.
A chart was constructed correlating the portal and right atrial pressures after each layer of stent graft material used to create the narrowing of the lumen (Figure ).
The patient was discharged home in a stable condition without clinical evidence of CHF.
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pmc-6199142-1
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Case 1: Ms. K, a 70-year-old woman who immigrated to the US at the age of 53, began complaining of watery eyes, chest pain, lower back and joint pain, leg cramps, and weakness. She harbored delusions of being afflicted with high blood pressure, uterine cancer, blood cancer with bone metastasis, brain cancer with extensive metastasis, and believed that her brain was "shrinking."
She first visited a cardiologist in 2013, complaining of intermittent episodes of chest pain over six months. An electrocardiogram (EKG) at the time showed bradycardia, a first-degree atrioventricular (AV) block and a left bundle branch block. At her sixth visit with the cardiologist, she mentioned non-specific somatic complaints, which she said were because of a "hematological problem." Five months later, she was evaluated for “renal hypertension” and imaging studies showed a renal cyst. While she did not follow up with the nephrologist, she continued to make hospital visits for persistent chest pain. A full medical workup was completed and found to be normal at every ER visit. Medical records from a prior ER visit revealed that she had made claims that the Russian military entered her residence and stole her urine, resulting in the disappearance of her kidneys.
Ms. K was brought to the ER by the police after she showed up with a can of gasoline and matches at her primary doctor's office and threatened to burn it down. She was irate and claimed that all of her doctors, in the US and in her home country, were concealing the fact that she had oncological issues. She vehemently denied any psychiatric illness, stating that these diagnoses appeared on her records as a result of a rumor started by an envious former colleague. She explained that because she had been a former practicing neurologist in her home country, she was confident that she had cancer. Upon repeated questioning, she admitted that in a final bid to receive the medical attention that she was rightfully due, she had devised the plan to burn down the doctor’s office.
While in the psychiatric inpatient unit, she remained somatically preoccupied and reported abdominal pain, lower back pain, and weakness, which she attributed to the metastatic spread of uterine cancer to her spine. Radiological imaging confirmed no evidence of uterine cancer, though a thickened endometrium was reported with recommendations for further testing by tissue sampling. Because Ms. K’s ability to make rational and reasonable decisions about her psychiatric and medical treatment was compromised by her delusions, the team sought and was granted a court order allowing them to treat her over her objection.
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pmc-6199142-2
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Case 2: Ms. M was a 64-year-old woman whose medical history was significant for several visits to different doctors seeking treatment for somatic complaints, including progressive blindness, headaches, hypertension, pelvic pain, vaginal swelling, lower extremity pain, and swelling. She was brought into the ER from her primary care doctor’s office for complaints of pelvic and leg swelling, which she attributed to poor diet. She expressed great concern over her problems being persistently ignored at all ERs she had been to.
Ms. M was found hiding in the closet of a primary care doctor’s office early in the morning before it was open for business. Apparently, she saw the janitor approaching and got scared. When the police were able to get into the closet, they discovered a knife, a pair of scissors, and a child's toy phone (which she had been using to call her doctor) in her possession. She revealed that she had gone to the office out of exasperation because she believed that no doctors were listening to her concerns.
She informed the team of the inpatient psychiatric unit about her history of hypertension and migraine headaches. All radiological imaging was negative for structural problems. She became increasingly irritable and accused the treatment team of not having her best interests in mind. She refused meals because she was convinced that they were poisoned and laced with psychiatric medication. Her persistent complaint of pelvic swelling and subsequent physical examination prompted a gynecology consult, which revealed a vaginal prolapse. She became fixated on the problem and demanded an immediate resolution of the issue. She continually refused psychiatric medications, interviews, blood pressure checks, and blood draws. The treatment team sought a court order and the judge ruled in favor of administering medications against her will, and she conceded and started taking them. In the days ensuing, her insight gradually improved, she became much calmer and less argumentative, and even accepted increased dosages, affirming that she understood the indication. While her somatic complaints did not dissipate, she improved to a point where she was deemed to no longer be a danger to herself or others and she was discharged.
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pmc-6199143-1
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A 41-year-old male was referred to our department for possible neurosurgical intervention following magnetic resonance imaging (MRI) that showed a lesion suspected to be an esthesioneuroblastoma (Figure ). He presented with a several-month history of intermittent headaches and loss of smell. He also reported near fainting episodes and slight unintentional weight loss over the same time period. The review of symptoms was negative for epistaxis and visual deficits, and neurological examination showed no abnormalities other than his aforementioned anosmia.
His MRI showed a large mass with both cystic and heterogeneously enhancing solid components extending from the region of the mid-left ethmoid air cells superiorly into the anterior cranial fossa, left frontal lobe, and left basal ganglia, causing mass effect on the left lateral ventricle and a midline shift.
The tumor was resected through a bifrontal craniotomy; the original surgical plan involved a formal craniofacial resection via a combined neurosurgical-otolaryngological approach. Intraoperatively, the tumor was found to have a clear arachnoid plane with no gross attachment to the surrounding dura or brain, and frozen section did not reveal any atypia, increased mitoses, or other aggressive features. Therefore, we elected to forgo a full craniofacial resection. Gross total resection was achieved and the skull-based repair was completed with a harvested pericranial flap and watertight dural closure. The patient awoke at his neurologic baseline and was discharged home on postoperative day one.
On permanent section, the lesion was determined to be a World Health Organization (WHO) Grade I schwannoma (Figures -). Surgical pathology demonstrated S-100 protein immunohistochemical stain positivity and was negative for meningioma markers (progesterone receptor (PR) and anti-epithelial membrane antigen (EMA)). The patient had an uneventful postoperative course and experienced no significant headaches, neurologic symptoms, cerebrospinal fluid (CSF) rhinorrhea, or other notable complications.
Literature review
We identified 10 cases of nasoethmoid schwannoma with intracranial extension in the medical literature (Table ). These lesions occurred in patients from age 24 to 50, with the vast majority presenting by age 40. There appears to be no association with gender. Although specific symptoms depend on the location and size of each tumor, most patients displayed a gradual onset of anosmia with increasing tumor growth and extension. Headache and visual deficits were also commonly seen among symptomatic patients. Bifrontal craniotomy was the most frequently utilized surgical approach for tumor resection, although several cases required a combined intracranial and transfacial approach. Gross total resection was achieved in all cases except one; the exception was a malignant tumor, which was neither encapsulated nor easily separable from the surrounding anatomical structures like the other benign tumors. Among articles reporting postoperative complications, CSF rhinorrhea was seen in three patients, as well as a variety of other complications associated with the unique locations and operative courses of individual lesions (Table ). Two patients, including the case presented in this paper, had no postoperative complications.
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pmc-6199144-1
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A 45-year-old man presented in the outpatient department of a tertiary care hospital with complaints of exertional dyspnea and occasional heaviness in the chest for the past two months. The chest pain described by the patient was unrelated to a specific time of the day. He was a known case of hypertension and well-controlled type II diabetes mellitus with no history of previous hospitalizations. The vitals were well-defined in the accepted range, with blood pressure measuring 130/85, body temperature of 98.6 F, a respiratory rate of 12 breaths per minute, and a pulse of 90 beats per minute. There were no positive signs of pallor, clubbing, or nail bed fluctuations on the general physical examination. The S1 and S2 systolic sounds were normal, without the presence of any added sounds. There was no abnormality in the routine biochemical tests. For further evaluation, an EKG was performed, which exhibited a sinus rhythm with nonspecific ST changes. Due to this, an exercise tolerance test (ETT) was performed after consent from the patient. ETT was inconclusive, as the target heart rate was not achieved and the patient was unable to walk more than 4 metabolic equivalents (METS). Subsequently, an angiography was carried out via the trans-radial route using a TIG 5 French catheter.
Coronary angiography indicated co-dominance, along with the presence of a rudimentary LAD, which abruptly tapered at the mid-level before reaching the left ventricular apex (Figure ) (Video ). The LCX was enormous and coursed toward the apex, in the coronary sulcus, onto the diaphragmatic cardiac surface and distally rather than ending before reaching the posterior interventricular sulcus, thus behaving like the LAD (Figure ) (Video ). The right coronary artery (RCA) was also dominant and supplied the PDA (Figure ) (Video ). Thus, it was apparent that the LCX was indeed supradominant in its course of supply. There was no hemodynamically significant stenosis and atherosclerosis in the coronary arteries; therefore, the symptoms were treated pharmacologically. The patient was discharged in a medically stable condition with a routine follow-up planned after one month.
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