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Name stringlengths 8 21	Age int64 18 45	Date_of_Birth stringlengths 8 10	Phone_Number stringlengths 4 22	Email stringlengths 16 30	Address stringlengths 13 36	Patient Progress Summary stringlengths 311 19.4k
Opal Booker	24	1998/4/8	620.445.2697x13191	[email protected]	8533 Edward Turnpike	Our patient was a 77-year-old Caucasian man who, 3 years and 11 months before his death, exhibited subacute polyneuropathy that included large fibers with autonomic changes. The clinical presentation of the patient? polyneuropathy did not allow us to differentiate a paraneoplastic pathology from an atypical presentation of diabetic neuropathy. A first episode of focal epileptic crises occurred 13 months after the polyneuropathy event. Two years before the patient? death, an electroencephalogram showed epileptic discharges in the right temporal cortex (Fig. ). After that, his autonomic seizures became more frequent, and ictal pilomotor erection was a common observation. Two years before the patient? death, a diagnosis of probable dementia was made. The presence of rapidly progressive dementia, delusions, and autonomic seizures with pilomotor erection suggested a preliminary diagnosis of limbic encephalopathy. At that time, cerebrospinal fluid (CSF) analysis including the following was performed: herpes simplex virus (repeated three times with negative results), varicella zoster virus, cytomegalovirus, Epstein-Barr virus, HIV, human T-cell lymphotropic virus 1/2, JC virus, Borrelia burgdorferi (Lyme disease), Tropheryma whippleii (detected but not confirmed in a second sample), Treponema pallidum, and Cryptococcus neoformans. All results of these tests were negative. No growth of any bacteria or fungi was detected. The patient? protein electrophoresis result was also normal. His 14-3-3 protein test result was negative. The patient had a slightly reduced amyloid-帣42 level (543 pg/ml). However, his tau protein level was normal (210 pg/ml). His CSF was also analyzed using an established radioimmunoassay in a reference laboratory. Intracellular antibodies (Hu, Ri, Yo, and Ma2) and surface antibodies (VGKCs KV1.1, KV1.2, and KV1.6) were assessed. Negative results were obtained for the intracellular antibodies. According to the relative values provided by the reference laboratory, the results of the VGKC-Ab test were positive. Twenty-three months before the patient? death, cerebral imaging by 2-deoxy-2-[fluorine-18]fluoro-d-glucose integrated with computed tomography-positron emission tomography (18F-FDG-PET/CT) was performed according to the following acquisition protocol. Fluorodeoxyglucose (FDG) (簣5.3 MBq/kg) was injected intravenously under euglycemic (6-hour fast, capillary blood glucose <140 mg/dl) and standardized resting (eyes open, reduced ambient noise) conditions. After 1 hour of uptake time, images were acquired by positron emission tomography-computed tomography (PET-CT) using a Biograph Duo lutetium oxyorthosilicate PET/CT scanner (Siemens Medical Solutions, Knoxville, TN, USA), followed by dedicated brain PET-CT image acquisition (10-minute acquisition). Next, three-dimensional iterative reconstruction was used for the brain PET images (iterations = 6/subsets = 16/full width at half maximum = 2 mm/matrix = 256). Finally, computed tomographic images were used for attenuation correction of PET data. The cerebral FDG-PET performed 23 months before the patient? death showed low FDG uptake in the frontal and temporal lobes, especially in the right hemisphere, with relative preservation of the posterior cingulum (Fig. ). Six months before the patient? death, he presented with normal-pressure hydrocephalus. A ventricular peritoneum derivation (VPD) was inserted. A small improvement in walking was observed, but it lasted no more than 2 weeks after the VPD implant. Three months before the patient? death, a second repeat cerebral FDG-PET scan using the same acquisition protocol as before showed diffusely low cerebral glycolytic activity with a marked increment in the severity of the metabolic deficit in the frontal and temporal lobes, as well as hypometabolism involving the parietal lobes and posterior cingulum (Fig. ). Three months before the patient? death, a biopsy done in the right axillary lymph node indicated a metastatic cholangiocarcinoma. The most common type of hilar extrahepatic cholangiocarcinoma is classified into four stages according to the Bismuth classification []. In our patient, this classification was considered stage IV. Two months before his death, a contrast-enhanced computed tomographic scan showed brain metastases in the right parietal cortex (Fig. ). A postmortem examination showed a cholangiocarcinoma with multiple metastases that included the lungs and lymph nodes. All other organs were studied during the autopsy, and the only primary neoplasia found was the cholangiocarcinoma; the other sites were metastatic, all exhibiting the same morphological aspects as the cholangiocarcinoma. The brain weighed 1300 g, and a catheter was well positioned in the lateral ventricle. There was mild cortical atrophy, ex vacuo dilation of the ventricles, and mild focal thickening of the cerebellar leptomeninges, which were infiltrated by the carcinoma, described as neoplastic epithelial cells (Fig. ). The morphological aspects were similar to the cholangiocarcinoma seen in the liver, histopathologically confirming the brain metastasis. There were no other changes in the cerebral cortex and white matter, except some thickened, hyalinized microvessels in the deep white matter with adjacent gliosis and calcification of vessel walls in the basal ganglia. Axonal peripheral neuropathy associated with microangiopathy, possibly related to diabetes, was also seen.
Dominik Ali	45	1986/11/12	413-330-1207	[email protected]	842 Tina Garden	A 74-year-old Japanese woman gradually developed general malaise and loss of appetite. In addition, arthralgia and pitting edema in her feet appeared. Her laboratory findings revealed an elevated white blood cell (WBC) count and C-reactive protein (CRP). She was admitted to our hospital for further examination. She had an operation for endometrial cancer at the age of 72 and had no family history. On admission, her blood pressure was 129/76 mmHg, heart rate was 101/minute, respiratory rate was 18/minute, and body temperature was 39.1 簞C. A physical examination revealed pitting edema on the dorsum of her hands and feet. Her neurological examination was unremarkable. However, she had difficulty with squatting and walking because of arthralgia in her proximal lower limbs. Laboratory testing showed a WBC of 11700/弮L, CRP of 6.7 mg/dL, erythrocyte sedimentation rate of 44 mm/hour, total protein of 5.8 g/dL, and albumin of 2.3 g/dL. Rheumatoid factor, anti Ro/SSA antibody, anti La/SSB antibody, anti Scleroderma 70 antibody, and anti cyclic citrullinated peptide antibody were negative. Serum interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF) were markedly increased to 285 pg/mL (normal; <2 pg/mL) and 1820 pg/mL (normal; 262簣228 pg/mL) [], respectively. Her human leukocyte antigen (HLA) typing included B7. A chest X-ray revealed mild pleural effusion on the right side. Electrocardiography showed a complete right bundle branch block without ST-T change. Echocardiography revealed a slight pericardial effusion surrounding her entire heart. She had a high fever, general malaise, and muscle weakness, and her laboratory tests revealed elevated inflammatory markers including WBC, CRP, and erythrocyte sedimentation rate. Because infectious disease was suspected, tazobactam-piperacillin 13.5 mg/day was started. On day 2, atrial fibrillation appeared on the electrocardiography monitor. A chest X-ray showed that the pleural effusion had increased in both lungs. Furthermore, echocardiography demonstrated increased pericardial effusion around her heart. On day 3, she developed respiratory failure, and oxygen administration was started by nasal cannula at a dose of 3 L/minute. Enhanced chest-abdominal computed tomography (CT) showed marked bilateral pleural and pericardial effusions without neoplastic lesions (Fig. ). Analysis of the pleural effusion revealed exudate with an increased protein level and neutrophils, but cytological evaluation of the pleural effusion revealed no evidence of malignancy. Cultures of blood and pleural effusion were all negative. Our patient fulfilled the diagnostic criteria of RS3PE syndrome including: 1) pitting edema of the hands and feet; 2) sudden onset of polyarthritis; 3) onset at age 50 years or older; and 4) negative serology for rheumatoid factors []. From day 6, she was treated with methylprednisolone administered intravenously at a dose of 1000 mg/day for 3 days. After steroid therapy, her fever rapidly improved, and blood tests revealed a decrease in CRP to 1.71 mg/dL. The pleural and pericardial effusions on chest CT decreased (Fig. ). When the pericardial effusion decreased, her atrial fibrillation disappeared. After steroid pulse therapy, oral prednisolone administration at a dose of 15 mg/day was started. Her respiratory failure immediately improved, and then she no longer required oxygen administration. A chest CT performed on day 38 revealed that the pleural and pericardial effusions had disappeared (Fig. ). On day 45, she was discharged. One year later, the prednisolone dosage was decreased to 8 mg/day, but the patient experienced no recurrence of symptoms. At the 1-year examination, no malignancy was found. Serum IL-6 and VEGF were significantly decreased to 1.3 pg/mL and 562 pg/mL, respectively.
Zelda Enriquez	31	1997/7/6	691-516-9228x7287	[email protected]	85222 Valerie Cliffs Suite 819	A 26-year-old male ex-Olympic gymnast and gymnastics coach presented to our emergency department with bilateral knee pain and inability to straight leg raise secondary to jumping from the roof of his single story home, where his legs gave way after he landed on his feet. This is in the context of landing a jump vault and hearing a ?opping sound??and sudden onset pain from his knees bilaterally 3 days prior. He had a background of chronic patellar tendonitis and a repair of his left patellar tendon for partial rupture 9 years previously. The patient was otherwise well and taking no regular medications. Physical examination revealed bilateral patella alta with a palpable defect superior to the tibial tuberosity; there were no significant joint effusions (Fig. ). Xray demonstrated high riding patella bilaterally. The diagnosis was confirmed on ultrasound, with the right proximal insertional fibres ruptured and 1.5 cm retraction (Fig. ). The right distal tibial insertion was intact. The left had a similar appearance with some calcified areas on the retracted fibres, which were believed to represent chronic calcification and/or avulsion fragments. The patient was consented for a bilateral patellar tendon reconstruction rather than a primary repair due to the poor quality of the tendons. Autograft was not considered as the injury was bilateral and due to the volume of remaining tendon fibres. The aim being to return him to his elite gymnastics as soon as possible. Surgery was performed by the senior author (PT). Bilateral midline incisions were made over both knees, the operative findings confirmed the sonographic diagnosis. The reconstruction was performed using a ?igure 8??pattern through a transverse patellar tunnel with screw fixation to two tibial tunnels. The knee was placed at 90簞 of flexion prior to tensioning of the ligaments. The remnants of the patella tendon where sutured to the LARS ligaments using a braided, absorbable suture. The patella tendon was repaired using 2x corkscrew anchors (medial and lateral). The wound was closed in layers achieving meticulous haemostasis (Fig. ). Post-operative Xrays indicated adequate screw and tibial tunnel placement (Fig. ). The patient was allowed to weight bear as tolerated and placed in extension knee splints for 2 weeks to minimize the chance of wound dehiscence. Rehabilitation with physiotherapy started on day 1 focusing initially on passive range of motion (ROM) exercises then on extensor and quadriceps strength. At the two-week wound review post-operatively the patient still had some ongoing pain and swelling after bending the knees, however there were no signs of wound infection. The ROM of the right knee was 0-85簞 and the left 0-45簞. At this appointment the patient was able to complete a straight leg raise bilaterally indicating an intact extensor mechanism. Two months post-operatively ROM was 0-125簞 bilaterally with no reported complications. At four months his ROM had improved to 0-130簞. At four years post-operatively, the patient is running a gymnastics club and actively coaching young gymnasts. He still experiences mild discomfort on descending stairs if carrying heavy loads but is otherwise painfree. He has not returned to training for competitive gymnastics. The patient claims he could return to some of the gymnastics skills but would struggle with vault and floor exercises secondary to lack of training. His Tegner Lysholm Knee score is 80, Modified Cincinatti rating score is 77 and Mohtadi-QOL Knee score is 58. Physical examination demonstrates well healed scars, no ligamentous instability or pain on palpation of the patellar tendon and bilateral ROM from 0-130簞 (Fig. ).
Elisha Bonilla	22	1998/3/19	711.922.3815x05487	[email protected]	431 Morales Knolls	A 47-year-old male reported a seven day history of influenza-like symptoms, including general malaise, chills, dry cough, chest-pain, shortness of breath, and temperatures of up to 39.5 簞C, which did not respond to paracetamol or ibuprofen. The patient worked as a veterinary researcher in a laboratory where experimental intranasal infections with C. abortus were developed in sheep. The patient carried out these infections using a suspension of C. abortus sprayed into both nostrils with a multi-dose spray pump, as described previously []. Ten days after the sheep infections, the patient showed the first symptoms. The initial medical examination identified crackling sounds during lung auscultation. A chest x-ray showed areas of consolidation of the lower lobe of the left lung, indicating a pan-lobar pneumonia (Fig. ). Blood and biochemical tests revealed a non-significant increase in mean corpuscular hemoglobin (MCH), as well as a slight increase in urea and creatinine levels: 50.3 and 1.3 mg/dl respectively. By contrast, the C-reactive protein level was very high, 8.9 mg/dl, normal values being <0.5 mg/dl. These high values suggested a bacterial infection rather than a viral infection, in which case the values would be lower []. Serological analyses with several ELISA tests (all of them from Vircell Microbiologist, Granada, Spain) using specific IgG and IgM monoclonal antibodies (MoAb) were negative against Mycoplasma pneumoniae, Legionella pneumophila (serogroup 1-7), Rickettsia conorii and Chlamydia pneumoniae. Due to the suspicion of C. abortus infection, serum and sputum samples from the patient were sent to a laboratory at the Animal Health Department of the Murcia University (Spain) for further analysis. A C. abortus-specific serological test, performed using an ELISA kit (ID Screen Chlamydophila abortus indirect multi-species, IDvet), revealed high IgM and IgG optical densities (OD) in the serum samples (Table ). The serum samples were tested with a peroxidase-conjugated anti-human IgG or IgM (Sigma), and the OD were determined (DigiScan with DigiWin Software, ASYS Hitech). In addition, two serum samples from asymptomatic work colleagues were analyzed with the same ELISA kit. As might be expected, both colleagues showed antibody production against C. abortus, but with a lower OD than the patient, especially for IgM production (Table ). It was therefore assumed that antibody production on the part of the patient was due to a clinical episode and not to a progressive sensitization to C. abortus. C. abortus was isolated from the sputum using a McCoy cell monolayer, as described previously []. Positive cell cultures were identified by an immunofluorescence assay using the C. abortus-specific anti-MOMP FA2H10 MoAb []. The MoAb was produced using BALB/c mice and purified by immunoafinity, and was chosen because it is specific for an oligomer of the outer membrane protein of C. abortus (named as C. psittaci, serotype 1). C. abortus DNA was detected in the sputum sample and also in the cell culture isolates by PCR analysis. Total DNA was extracted with a commercial kit (DNeasy tissue kit, Qiagen) and was used as a template for a C. abortus-specific PCR, using primers pmp-F (5??CTC ACC ATT GTC TCA GGT GGA-3?? and pmp-R821 (5??ACC GTA ATG GGT AGG AGG GGT-3?? for the target gen pmp 90/91 [], which allow the sensitive amplification of 821-bp length DNA fragments. In order to confirm the specific presence of C. abortus in the sputum sample as well as in the cell isolates, the amplicons were sequenced (ABI Prism 3130). The PCR products showed 100 % identity to the C. abortus AB7 strain sequence obtained using MEGA5 software and BLAST簧 program. This C. abortus strain was the same strain as that used in the experimental infections in ewes carried out by the patient []. Once diagnosed, the patient was treated with antibiotics (levofloxacin, initial intravenous dose of 500 and 500 mg orally every 24 h for 10 days; and clarithromycin 500 mg every 12 h for 10 days), mucolytic carbocysteine lysine (2.7 g orally every 24 h), glucocorticoids (beclomethasone dipropionate inhaler 100 弮g every 12 h) and non-steroidal anti-inflammatory drugs (paracetamol 1 g orally in case of fever or pain). This treatment resulted in successful recovery of the patient in 2 weeks without sequels.
Romina Rocha	45	1983/1/5	001-938-618-9773x546	[email protected]	4466 Garcia Fort Suite 863	A 27- month-old girl was referred to Bambino Ges羅 Children's Hospital in 2009 after a 1-week history of vomiting associated to mild intermittent strabismus. Ophthalmologic evaluation revealed bilateral papilledema. Magnetic Resonance Imaging (MRI) showed diffuse brainstem and spinal leptomeningeal enhancement (Fig. ??. CSF analysis was unremarkable. Tuberculosis (TB) was not confirmed by a complete work-up. Nonetheless, antitubercular treatment was started based on the MRI findings. After 10 days the patient was transferred to the Intensive Care Unit for a salt wasting syndrome. A new MRI demonstrated hydrocephalus (Fig. ) and progression of leptomeningeal enhancement (Fig. ??. A new CSF examination was done and showed neoplastic cells with large cytoplasm and prominent nucleoli (Fig. ) positive for S100. Therefore, antitubercular therapy was discontinued and a ventriculoperitoneal shunt was placed because of the progression of neurological symptoms. Moreover, a spinal intradural biopsy was performed: histological examination showed pleomorphic cells with vesicular nuclei, eosinophilic nuclear pseudoinclusion and moderate cytoplasm (Fig. ). Immunohistochemistry showed intense positivity for MelanA, suggesting the diagnosis of primary leptomeningeal melanomatosis. No signs of cutaneous melanosis were observed. Chemotherapy was started, including temozolomide, cis-platinum, vindesine and peg-interferon alfa-2b. MRI was obtained every two months showing stable disease until the sixth course of chemotherapy when progression was found. At that time, radiation was associated to peg-interferon alfa-2b but the tumor rapidly spread to chest and abdomen. General clinical conditions progressively worsened and patient died 11 months after diagnosis. Molecular investigations were performed post-mortem on tumor tissue and revealed absence of BRAFV600E, GNAQQ209 and GNA11Q209 mutations but the presence of a NRASQ61K mutation.
Onyx Gomez	43	1996/2/24	001-801-380-7157x97324	[email protected]	69137 Manning Pike	Patient 1, a 49-year old male (Table ), presented with gait disturbance and vertigo. MRI evaluation revealed hemangioblastoma at the right superior cerebellum. A year after excising the mass, bilateral 2.2??.0 cm tumors were found in both kidneys. Eye examination revealed a hemangioma-like lesion in the right eye. In addition, CT of the abdomen revealed a pancreatic neuroendocrine tumor (NET). Genetic testing showed that the patient harbored a novel frameshift mutation, p.Met54Glyfs*77, in the VHL gene. One of his daughters and his son were heterozygous for the same mutation. His 35 year-old daughter had 3 types of VHL-related tumors: Cerebellar hemangioblastoma, RCC in the right kidney lower pole, and pancreatic NET which invaded the superior mesenteric artery and superior mesenteric vein. His son, who was 38 years old, visited an emergency room presenting with a generalized tonic-clonic seizure. On evaluation, the brain MRI showed a well-defined mass in the left parietal area, which was pathologically found to be a meningioma.
Natalie Vang	30	1991/11/21	818-511-4161x167	[email protected]	49749 Makayla Ridges Suite 380	Patient 2 was a 25-year-old male (Table ) who reported frequent headaches and dizziness. MRI of the brain revealed a 0.9-cm nodular lesion at the medullocervical junctional level. This patient underwent midline suboccipital craniotomy. A heterozygous missense mutation was identified in the VHL gene: p.Glu70Lys. There was no family history. This patient regularly visited the clinic, and no other VHL-related tumor developed.
Jimmy Neal	32	1980/12/9	979-287-3018	[email protected]	24921 Michelle Forest Apt. 165	Patient 3 was a 46-year-old female who was the index patient in family 3 (Table ). She visited the clinic with a 3-month history of blurred eye vision in her left eye. Examination revealed RHB in her left eye. VHL analysis revealed the heterozygous missense mutation p.Glu70Lys. Since her sister had suffered from RHB, the mutation was considered to be passed down from her parents. Her son, a 19-year-old male, inherited the same mutation, but no VHL-related symptoms were present. He underwent surveillance for VHL manifestations. RHB was the only VHL-related phenotype in this family.
Talia Gould	44	1996/10/21	+1-513-311-2781x424	[email protected]	245 Tanya Lodge Suite 352	Patient 4 was a 20-year-old female (Fig. ). Through routine eye examination, multiple RHBs in both eyes were found. We analyzed the VHL gene and found that the patient was heterozygous for the p.Glu70Lys mutation. Her mother had been treated for RHB but refused to undergo genetic analysis. Further genetic testing and family history assessment was unavailable for this family. No other VHL-associated symptoms were observed in patient 4 and her mother. In family 5, two siblings (Table ) underwent surgical resection for CHB at the ages of 12 and 19. Abdominal CT revealed RCCs in these siblings. The family history revealed VHL manifestations in their father who died in 2013 and had RCC, PCC, and CHB (Table ). The proband (patient 5) harbored an in-frame deletion p.Phe76del affecting exon 1 in the VHL gene. This in-frame deletion appeared to run in this family.
Blaine Poole	22	1983/7/20	(330)722-8361x95380	[email protected]	31826 Todd Groves	Patient 7 was 14-year-old male who experienced frequent abdominal distension and intermittent headaches. At a routine health check-up, multiple pancreatic cystic lesions and RHB in the temporal disc side of his left eye were found. MRI of the brain showed a tiny well-enhanced nodule at the cervicomedullary junction with an adjacent prominent vascular structure. The features of this patient? presentation were indicative of VHL. Although this patient had no family history, a heterozygous missense VHL mutation, p.Asn78Ser, was identified in patient 7.
Bonnie Schroeder	32	2005/4/8	+1-407-762-6528x4865	[email protected]	1356 Phillips Parks	Patient 8, a 38-year-old male, showed mild imbalance with gait difficulty. MRI of the brain revealed a 3.5-cm cystic mass in the cerebellum, which was pathologically found to be hemangioblastoma. When this patient underwent CT, which included the pancreas and kidney, multiple pancreatic cysts and renal cysts were found. A VHL c.464-1G > T splicing mutation was detected in patient 8 and there was no family history of VHL disease in his family.
Izaiah Fox	24	2002/6/26	6843727771	[email protected]	73039 Bell Keys	Patient 9 was a 47-year-old male (Table ). He presented with dizziness and headache, which he had experienced for the 3 months prior to examination. After systemic examination, multiple VHL related tumors were revealed, including RCC, PCC, and CHB. VHL analysis detected a heterozygous missense mutation, p.Arg167Trp. He had a family history of VHL-related tumors, including CHB and RCC. His brother was diagnosed with CHB and his niece was diagnosed with CHB, RHB and RCC. The same mutation was identified in his niece.
Juliette Bernard	45	1999/12/2	(855)474-8906x991	[email protected]	5648 Taylor Circles Apt. 003	Patient 10 (Table ), a 59-year-old female, presented with indigestion, anorexia, and nausea. This patient underwent abdominal CT; multiple renal cysts, a hypervascular mass in the pancreatic body, and a cystic mass in the pancreatic head and tail were found. Pylorus preserving pancreaticoduodenectomy was performed in this patient. Two months after surgery, gait disturbance appeared. MRI of the brain revealed multiple enhancing masses in both cerebellums, which were pathologically found to be hemangioblastoma. Based on suspicion of VHL, we sequenced the VHL gene in this patient; a heterozygous missense VHL mutation, p.Arg167Gln, was confirmed. None of her family members were affected.
Jair McConnell	45	1992/9/1	001-861-959-2378x441	[email protected]	22379 Brown Forest	A 60-year-old non-smoking white male, with an alcohol history of 2-3 drinks per day, was diagnosed in July 2013 with squamous cell carcinoma (SCC) of the right retromolar trigone with extension into the adjacent buccal mucosa. He was treated with primary surgery on September 12, 2013, consisting of a right lateral pharyngotomy and segmental resection of the right posterior mandible and adjacent buccal mucosa, and a right selective neck dissection followed by left free fibula osteoseptocutaneous flap reconstruction. Final pathology showed metastatic keratinizing SCC measuring 0.96 cm with a 0.5 cm depth of invasion. There was an invasion of the bony mandible into the cortex and medulla. There was no lymphovascular space or perineural invasion and all margins were free of cancer, with the closest being 0.18 cm medially. One of 12 lymph nodes in right Level I and one of one facial lymph nodes were positive for metastatic disease, the latter with extranodal extension. No cancer was found in any of 20 lymph nodes from Levels 2-4. Pathologic stage was T4aN2b with an R0 resection. After multidisciplinary tumor board discussion, it was recommended that he receive adjuvant radiation therapy (RT) with concurrent cisplatin chemotherapy. Starting six weeks postoperatively, he received a radiation dose of 6,000 cGy in 30 fractions over six weeks to the tumor bed and involved nodal regions via a 5-field IMRT plan using 6 MV photons, along with a q3 weekly bolus of cisplatin, 100mg/m2. He completed all adjuvant treatment on December 3, 2013, uneventfully and without unplanned delays or unexpected toxicities. His last clinical follow-up appointment on January 28, 2015 revealed no evidence of locoregional recurrence and no significant late treatment toxicities. In February 2015, he presented to the emergency room with acute shortness of breath, palpitations, and a recent history of air travel. Electrocardiogram (ECG) showed rapid atrial fibrillation. CT scan of the chest, using a pulmonary edema (PE) protocol, showed a small pericardial effusion, measuring up to 1.3 cm in width, and moderate-sized, uncomplicated bilateral pleural effusions. There was a single 0.9 cm short axis prevascular lymph node, which was felt most likely to be reactive. He was discharged with arrangements for an outpatient cardiology follow-up. His atrial fibrillation proved to be difficult to control, in part because of intolerance of beta blockers due to pre-existing asthma. On July 1, 2015, he re-presented with shortness of breath on exertion and peripheral edema. Initial bloodwork revealed the N-terminal prohormone of brain natriuretic peptide (NT-proBNP) was elevated at 1650 (0-300) with mild high sensitivity troponin elevation at 20 (0-14). ECG showed a septal infarct, right axis deviation, and a marked ST segment abnormality. The rhythm was variant between sinus tachycardia and atrial flutter. After admission, an echocardiogram was performed, and a large mass was identified in the right ventricle. The following day he underwent pulmonary function tests (PFTs), which showed both FVC and FEV1 < 50% of predicted values. A CT scan of the neck showed stable post-treatment changes and no evidence of primary or residual tumor, while CT of the chest, abdomen, and pelvis revealed the growth of the prevascular mediastinal lymph node to 1.7 x 1.2 cm. Also noted were hepatic venous congestion and the right ventricular mass. Retrospectively, this was thought to have been visible as a small abnormality on the CT/PE study from 4.5 months earlier. Cardiac MR (Figures -) showed the mass to be 6.7 x 3.8 x 4.5 cm and centered on the right ventricular free wall. There was a narrowing of the tricuspid valve and mass extension into the right atrium. A large pericardial effusion was associated with this mass. A biopsy was attempted via right heart catheterization through the right internal jugular vein under transthoracic echocardiogram guidance. Repeated attempts were unsuccessful and yielded only normal myocardial tissue because of catheter deflection as it entered the right ventricle. Diagnostic thoracentesis of his left pleural effusion showed only rare atypical cells. After much consideration and discussion with the patient about potential benefits and risks, it was decided to proceed with an open biopsy. On July 14, 2015, he underwent sternotomy with direct visualization and biopsy of the pericardium and the right ventricular mass. Pathology showed metastatic poorly differentiated squamous cell carcinoma arising from within the myocardium of the right ventricular free wall (Figures -). A full immunohistochemical panel revealed the tumor to be p63, 34BE12, E-cadherin, pancytokeratin (PCK), and cytokeratin 5/6 (CK5/6)-positive. It was negative for melanin, S100, WT1, CD34, and CD45 and, hence, felt to be consistent with metastatic disease from his original oral cancer. Shortly after biopsy, palliative radiotherapy with 2,000 cGy in five fractions was discussed with the patient. The goals of treatment would have been to try and improve his cardiac function. Risks were discussed, including potential worsening of arrhythmias and catastrophic cardiac rupture due to regression of a tumor that was replacing much of the cardiac wall. He appreciated the risks and uncertain benefits and consented to treatment as described. Unfortunately, after discharge, he began decompensating again and, hence, pursued comfort measures only. He passed away within one week of this decision and exactly one month after presenting in heart failure.
Denise Wilkinson	19	1997/2/17	001-568-888-4100	[email protected]	415 Hatfield Mountain	A 67-year-old Caucasian woman admitted to hospital due to a mass on the scalp. This lesion was present for almost a year. It was a semimobile cyctic mass that elevated the scalp. There was no change in the skin color. Its dimensions were 1.5 ? 1 ? 0.6 cm. The laboratory, clinic, and radiologic findings (head x ray) of the patient were normal. It was evaluated a benign lesion such as lipoma or epidermal cyst by surgeon due to a small semimobile mass and no erosion of the skull. It was excised by a local surgery excision. The skin is sent as a different part from this excision material. In pathological examination, there were solid and cystic spaces including tubuloalveolar, ductal, and papillary structures protruding into cistically dilated lumina (Fig. ). These structures were lined by atypical epithelial cells including hyperchromatic, pleomorfic, and big nucleus (Fig. ). Necrotic areas and mitotic figures were present (Fig. ). There was no epithelial invasion. These histopathologic findings were compatible with ADPA. For elimination of a metastatic primary papillary carcinoma focus such as lung, breast, thyroid, etc. An immunohistochemical study was performed. EMA, PanCK, and CEA were positive. SMA, Calponin, and P63 were negative. These immunohistochemical dyes support diagnosis of ADPA. In the examination for elimination of probably a metastatic focus, TTF1 for lung, ER and PR for breast, CK19 and HBME1 for thyroid, and GFAP for brain were negative. In clinical investigation before pathologic report for the ADPA, it is approved that there was not any mass in lung, breast, thyroid, brain, and ovary. The patient is in the 4th month in the postoperative period and there was no pathologic finding.
Leonard Boyer	21	1999/6/20	+1-265-806-9792x0851	[email protected]	0362 Guerra Flats	A 19-year-old female patient with a history of congenital afibrinogenemia. According to the French law, in case of retrospective study, the ethical approval was not necessary. She was admitted to the emergency department of a local hospital with acute abdominal pain and nausea. Her genetic fibrinogen mutation was allele 1: FGG IVS2??c>G, allele 2: FGG IVS2??c>G. She was being treated monthly with fibrinogen concentrates, tranexamic acid (during menstrual periods), and oral contraception. She had suffered from massive bleeding 7 years ago, diagnosed as hemoperitoneum complicating an ovarian cyst rupture. On clinical examination, her abdomen was tender but not tense, she was afebrile and hemodynamically stable. There was no history of trauma. Her laboratory tests showed: hemoglobin 13.5 g/dL, hematocrit 40%, liver enzymes: aspartate transaminase 196 U/L, alanine transaminase 239 U/L, bilirubin 14 弮m/L, International Normalized Ratio 8.4, activated cephalin time of 180 seconds (ratio 5.4), a platelets count of 234,000 mm??, a white blood cells count of 17,000 mm??, a C-reactive protein of 19 mg/L. Abdominal ultrasonography revealed an unexpected large liquid picture measuring 10 cm by 5 cm over hepatic segments number VI, VII, and VIII. Abdominal computed tomography (CT) confirmed this suspicion of hematoma (Fig. ). A few hours later, she exhibits tachycardia, increased abdominal pain, and hypotension. Hemoglobin decreased to 5 g/dL. Fluid replacement therapy with crystalloids was started. Packed red blood cells and fresh frozen plasma transfusion were given. At the same time, fibrinogen concentrate (3 g) was administered (Clottafact [1.5 g/100 mL] LFB Biomedicaments, France). Decision was made to transfer the patient to a tertiary referring university hospital for endovascular embolization. The surgeon decided to implement abdominal packing as damage control therapy, to ensure safe conditions before transferring the patient. Active bleeding continued during the intervention and norepinephrine was introduced. At that time, 10 units of blood suspensions, 6 units of fresh frozen plasma, 4.5 g of fibrinogen concentrates, and 2 g of tranexamic acid were given. Selective catheterization of the 2 branches of the right hepatic artery was successfully performed (Fig. ). On day 2, the patient underwent further surgery to remove abdominal packing but subcapsular hepatic hematoma was still bleeding and another packing therapy was necessary. Fibrinogen concentrates were infused just before this 2nd surgery to optimize coagulation disorders. The removal of abdominal packing was possible on day 4, without any abdominal bleeding (plasmatic fibrinogen 1.9 g/L before surgery, Von Clauss coagulation micromethod[). Three grams of fibrinogen concentrates and tranexamic acid were administered at the beginning of the 3rd surgery. The time course of plasma fibrinogen concentrations is described in Fig. . Simultaneously, she presented an early ventilator acquired pneumonia (methicillin sensitive Staphylococcus aureus). Despite an appropriate intravenous antimicrobial use, fever and ileus remained and white blood cells increased to 35,000 mm??. On day 6, abdominal CT scan showed abundant pelvic peritoneal effusion and diffuse infiltration of the peritoneum, suggesting a postoperative peritonitis. Laparotomy was performed with abundant cleaning of the peritoneal cavity and drainage (plasmatic fibrinogen was 3.1 g/L after infusion of 4.5 g of fibrinogen concentrates). Antimicrobial therapy was switched to imipenem, vancomycin, and amikacin. No septic shock occurred. Purulent peritoneal effusion was infected with methicillin resistant Staphylococcus haemolyticus allowing de-scalation to vancomycin alone for 10 days. In the postoperative period, no bleeding occurred, bowel transit was normal, and drainage tubes were removed quickly. No thrombo-embolic adverse event occurred. Thrombo-embolic prophylaxis by low molecular weight heparin was introduced on day 7, after prophylactic intermittent legs compression during the 1st week. The patient was discharged to surgical ward on day 11.
Chaya Atkinson	45	1993/10/3	200.746.3518x10645	[email protected]	4465 Roberto Neck	In September 2015, a 36-year-old, emaciated, Hungarian man presented with a 3-month history of widespread, red, rust-colored papules and nodules partially erosive and crusted as well as a 4 cm wide, prominent, skin-colored, subcutaneous tumor at the left zygomatic arch (Fig. ). The oral mucosa was free of lesions. The patient was occasionally homeless in Austria, his travel history was unremarkable and he reported intravenous drug use. The latter comorbidity and the clinical presentation prompted HIV rapid testing, which provided a positive result. HIV infection was confirmed by Western blot and quantitative HIV PCR (4.70 log copies/mL). The CD4+ cell count was 47 cells/弮L (CD4+/CD8+ ratio: 0.06), indicative for severe immunosuppression. In addition, the patient was HCV positive and the viral load was 2.62 ?106 copies/mL (genotype 1a). The white blood cell count was within normal range, and the patient was subfebrile. Related to the rust-colored papules, BA, vascular tumors-like multiple pyogenic granulomata (PG), and HIV-associated Kaposi sarcoma were suspected. A biopsy of the nodular skin lesions from the dorsum revealed solid, abundant telangiectatic vessels in the upper dermis with prominent endothelial cells surrounded by neutrophils and lymphocytes, compatible with BA and PG. Immunohistochemical staining for human herpes virus 8 was negative. The Warthin?tarry stain showed clusters of microorganisms. High levels of serum IgG antibodies against B henselae and B quintana were detected in an indirect immunofluorescence assay (Euroimmun, L羹beck, Germany) strongly suggesting BA. For direct pathogen identification, an EDTA blood sample and tissue samples of the nodular skin lesions were analyzed in PCR assays. DNA was extracted and processed from both samples using a peqGOLD Tissue DNA Mini Kit (Peqlab) according to the manufacturer's protocol. DNA isolates were amplified with a real-time PCR targeting the ssrA gene of Bartonella spp.[ An ABI7900 cycler (Applied Biosystems, Foster City, CA, USA) was used with a thermal profile of initial enzyme activation at 95簞C for 2 minutes, 45 cycles of denaturation at 95簞C for 15 seconds, followed by annealing and elongation at 61簞C for 1 minute. Bartonella spp. DNA was detected in all samples. Amplicons for sequence analysis were generated in conventional PCR. The PCR products were loaded onto a 1% agarose gel and amplicons of size 301 bp were purified using the QIAquick Gel Extraction Kit (Qiagen, Austria). DNA sequencing was carried out by Eurofins Genomics DNA sequencing service (Eurofins Genomics GmbH, Ebersberg, Germany). The forward and reverse DNA sequences obtained were used for homology searches in the National Center for Biotechnology Information (NCBI) database BLAST search program (). The skin tissue was further cultured on Columbia blood agar plates (Biomerieux, Vienna, Austria) at 37簞C in an atmosphere of 5% CO2 and 95% humidity. After 4 weeks of culture, microbial growth was detected by clinical characteristics and selected colonies were propagated by culture via dilution streak plating. Single colonies were then picked and sub-cultured, and the DNA was purified and tested by a Bartonella-specific PCR assay.[4] Sequencing (BLAST analysis) demonstrated a clearly positive result for B quintana. Contrast-enhanced computed tomography (CT) scans of the head, chest, and abdomen (Fig. ) revealed hepatosplenomegaly and several abscesses located in lung, stomach wall, pancreatic tail, subcutaneous tissue, and the large lesion (4.5 ? 2.8 cm) at left zygomatic arch already eroding the adjacent bone. No pathological findings were seen on x-ray of the long bones or on echocardiography. In order to exclude a different pathology of the facial subcutaneous mass, such as Burkitt lymphoma, a biopsy was performed. Histology revealed neutrophil infiltrations with no evidence for a malignant process. In addition, DNA was extracted from the paraffin-embedded tissue for broad spectrum fungal PCR (MycoRealFungi, Ingenetix, Vienna, Austria) and no pathogens could be identified. The same tissue was tested for B quintana by PCR as described above and the result was positive. Silver impregnation staining showed bacilli. In view of the advanced immunosuppression, a screening for other opportunistic infections was performed. Atypical and typical mycobacterial infections were excluded by negative cultures from blood, urine, and skin biopsies. The TBC-specific interferon-gamma release assay and Cryptococcus neoformans antigen were also negative. Among several blood cultures from venous blood only one was positive for Staphylococcus epidermidis. After 23 days of treatment with doxycycline 100 mg bid, an improvement of the skin lesions, partial regression of the facial tumor and resolution of fever were observed. The HIV viral load had decreased to 2.02 log c/mL after initiation of antiretroviral therapy with emtricitabine/tenofovir 200/245 mg qd and dolutegravir 50 mg qd. A follow-up CT scan could not be performed, because the patient abandoned the hospital and was lost for follow-up.
Duke Harmon	43	1997/2/23	525.782.5611	[email protected]	01841 Alicia Lakes Apt. 886	A 60-year-old man with a 3 ? 3 cm calculus in the left renal pelvis was scheduled for percutaneous holmium laser lithotripsy. He weighed 65 kg and was 172 cm tall. He had a history of mitral valve-replacement surgery and was on warfarin therapy. He also had a history of asthma for more than 20 years with severe pulmonary decompensation. International normalized ratio (INR) at admission was 2.26. He was shifted from warfarin to low molecular weight heparin therapy. INR decreased to 1.23 on the day prior to the operation. Because of pulmonary decompensation and the need for intensive anticoagulation therapy, he was a high-risk case for both general anesthesia and neuroaxial block. After careful discussion with multidisciplinary team and due deliberation and with the consent of patient and his family, PCNL was performed under ultrasound-guided PVB.
Maren Sloan	33	2001/12/5	+1-466-850-2591x720	[email protected]	972 James Streets Apt. 464	A 67-year-old man with multiple calculi in the right renal pelvis and calyces was scheduled for PCNL. His weight and height were 60 kg and 163 cm. He had a history of stroke sustained 6 months prior to admission, which resulted in hemiplegia. He also had a history of chronic obstructive pulmonary disease. Preoperative pulmonary function tests revealed severe obstructive ventilatory impairment. Preoperative electrocardiogram indicated atrial fibrillation. 24-h Holter monitoring revealed long RR interval (>3 seconds, 3 times). Temporary pacemaker was implanted before the operation. This patient was at increased risk for general anesthesia because of poor pulmonary function. He had a relative contraindication to neuroaxial block because of hemiplegia. Based on our successful experience with PVB in the previous patient, we decided to adopt the same technique for this patient. With the consent of patient and his family, ultrasound-guided PVB was performed. PCNL was successfully performed with minimal interference with cardiopulmonary function.
Ocean Velasquez	22	1994/2/9	-5989	[email protected]	82989 Deleon Mountains	A 27-year-old man with staghorn calculi was scheduled to undergo a second PCNL. His weight and height were 70 kg and 172 cm. He had a history of lumbar vertebral fracture and had a relative contraindication to neuroaxial block. He had undergone PCNL under general anesthesia one week earlier. Postoperatively, he experienced severe nausea and vomiting. He refused to accept general anesthesia this time. With the agreement of patient, we chose ultrasound-guided PVB for him.
Esme Flowers	36	2000/12/11	+1-862-528-6404x7618	[email protected]	4407 Matthew Turnpike	A 43-year-old female patient referred to our hospital? emergency department in August 2014 with complaints of fever and severe pain in right breast. She noticed a soft lump and mild pain in her breast a week back. The intensity of pain increased gradually and for the last 2 days and her temperature increased. The patient had a history of 5 years of RA. The patient was not pregnant or lactating. She had three children with the last one delivered 6 years back. On physical examination, the right breast was tender and swollen. A mobile, sensitive, soft, fluctuating mass of around 4 by 5 cm located in the right lower quadrant was palpable. The mass was not fixed to the upper skin. The upper skin was warm and erythematous. There was no nipple retraction or discharge from the nipple. Axillary lymph nodes were not palpable. The patient? respiratory, digestive, nervous system physical examinations were normal. Physical examination of the musculoskeletal system revealed swelling in 2nd and 3rd proximal interphalangeal joints of the right hand and swelling and limitation of motion in the right knee. Ultrasonography (USG) of the right breast revealed a heterogeneously hypoechoic deep-seated irregular collection of approximately 40?38 mm size. The patient was diagnosed with a breast abscess. The abscess was drained by USG guided drainage, and oral amoxicillin clavulanic acid 625 mg bid therapy was started empirically. The drained fluid was sent to medical microbiology laboratory in a tightly capped, sterile container. On macroscopic examination, it was observed that the material had yellowish cream colour and dense consistency. Gram staining of the material showed abundant polymorphonuclear leukocytes and gram-negative bacilli. The material was inoculated on 5 % sheep blood and eosin methylene blue (EMB) agars plates. One of the sheep blood agar plates was incubated under anaerobic conditions. After incubation at 37 簞 C for 24 h smooth, straight-edged colonies without hemolysis grew on sheep blood agar. On EMB agar, lactose-negative colonies were detected which tested negative for oxidase production. These colonies were identified as Salmonella species by Phoenix BD (Becton, Dickinson, USA) automated system and Salmonella group by Maldi-TOF MS (bioM矇rieux, France). The microorganism was identified as Salmonella enterica serotype Typhimurium according to the Kauffmann-White scheme by using specific antisera (Difco, Becton, Dickinson, USA). No anaerobic bacteria were isolated. The microorganism was found susceptible to ampicillin, ciprofloxacin, trimethoprim-sulfamethoxazole, erythromycin, chloramphenicol, ceftazidime, and ceftriaxone by antibiotic susceptibility testing with Phoenix BD (Becton, Dickinson, USA) automated system. The patient was called back to the surgical outpatient clinic to obtain a detailed history. It was learned that 2 months ago she had diarrhoea for approximately 3 days and did not receive any antibiotic treatment. The patient had RA for 5 years and was on prednisone therapy. There was no history of contact with animals or trauma to breasts. There was no history of similar swelling in the other breast; no history of diabetes mellitus, hypertension, bronchial asthma, and tuberculosis. When the patient? laboratory tests were examined leukocyte count was 12,400/mm3 (83.9 % neutrophils), hemoglobin was 13.3 g/dL, fasting blood glucose was 108 mg/dL, erythrocyte sedimentation rate was 31 mm/h, CRP was 32 mg/l, RF was 28 IU/ml and anti CCP was 267.9 U/mL. Alkaline phosphatase, amylase, aspartate aminotransferase, alanine aminotransferase, gamma-glutamyl transpeptidase, bilirubin levels were within normal limits. HBsAg, anti-HCV and anti-HIV (ETI-MAX 3000 analyzer; DiaSorin S.p.A., UK), anti-HBc (Cobas 6000 analyzer, Roche Diagnostics, USA) tests were found to be negative. Chest X-ray was normal. Stool, urine, and blood samples were obtained for culture and found negative for Salmonella spp. Gruber Widal test was also found negative. Figure shows a timeline of events. The patient was fully treated with the surgical draining of the breast abscess under general anaesthesia and oral ciprofloxacin 500 mg twice daily. At the end of 2 weeks, the patient was fully healed with no clinical signs of abscess. The control examination with USG after 4 months showed no sign of recurrence of the abscess.
Saul Fields	30	2005/4/5	+1-344-984-7701x56045	[email protected]	74650 Brian Locks Suite 559	A 57-year-old woman had underlying systemic iron deficiency anemia. She presented to our outpatient clinic with abdominal pain in March 2013. Panendoscopy and colonoscopy were performed and no organic lesions were detected in the upper and lower GI tract, and only gastritis was noted. However, the abdominal discomfort could not be eliminated and it worsened. Because of progressive abdominal pain, the patient was transferred to the emergence department after 1 week. Abdominal computed tomography (CT) (Fig. ) revealed a marked, approximately 10-cm mass in the pancreatic head. The pancreatic head tumor was initially diagnosed and she was then admitted for further examination. During hospitalization, laboratory analysis did not show hyperbilirubinemia and elevated amylase or lipase levels. The levels of tumor markers including carcinoembryonic antigen, carbohydrate antigen 19-9, and carbohydrate antigen 125 were within normal limits. CT-guided aspiration of the cystic component was performed, and cytological analysis showed only some macrophages. We performed surgical exploration on March 22, 2013. During the operation, we found that the lesion was approximately 10 cm in size with cystic and solid components (Fig. ). The tumor appeared to originate from the pancreatic head and presented as a cystic hematoma protruding downward into the mesocolon. We performed partial cystectomy for hematoma evacuation, and tumor tissue was obtained from the pancreatic head for pathologic analysis. Examination of frozen tissue section did not indicate any malignancy. Finally, gastrojejunostomy bypass was performed without radical resection of the tumor. The patient showed good postoperative recovery. The final pathologic analysis confirmed the DT diagnosis and immunohistochemical staining showed focal positivity for smooth muscle actin and desmin but negativity for Mdm2, CDK4, and CD34. The tumor showed strong positivity for beta-catenin (Fig. ). The patient received one 200-mg tablet daily of the non-steroidal anti-inflammatory drug (NSAID) celecoxib for half a year. We obtained a follow-up abdominal CT scan in October 2013 after 6 months of celecoxib treatment. The CT images showed a marked regression of the existing pancreatic head mass and complete disappearance of the cystic lesion. Therefore, the dose was modified from one tablet daily to one tablet every other day with the same dose of celecoxib starting from the date of the 6-month follow-up until now. The abdominal CT scan obtained at the 18-month follow-up in September 2014 showed no local recurrence (Fig. ). There is no side effect noted by the patient in the period of celecoxib treatment. The patient is still receiving celecoxib treatment for the control DT. DT originates from the abnormal proliferation of myofibroblasts. It is a very rare disease and presents with a benign pathology; however, it exhibits local invasiveness indicating clinical malignancy. The DT location can be classified as extra-abdominal, abdominal, or intra-abdominal. Genetic screening for defects showed that mutations in the FAP gene were a risk factor that was more frequently associated with intra-abdominal DT []. Other studies showed that intra-abdominal DT frequently had pelvic and mesenteric origins. DTs originating from pancreas are very rare. To date, only 12 cases of pancreatic DT have been reported [?. Among these articles, only three of them reported DTs that originated in the pancreatic head, and all patients underwent tumor resection [?. The present case is the fourth case of DT originating from the pancreatic head; however, it is the only case treated by medication and achieved completed remission. The clinical presentation of most patients with DT is usually asymptomatic and otherwise presents non-specific abdominal pain. DT diagnosis could not be confirmed via laboratory analysis or radiological images. Therefore, the definitive diagnosis of DT could only be based on histological and immunohistochemical findings []. In present case, DT diagnosis was confirmed by the final immunohistochemistry staining that showed strong positivity for beta-catenin and focal positivity for smooth muscle actin and desmin. The current first-line treatment for DT is complete resection with free margins. Sometimes, performing resection with wide, free margins for the prevention of tumor recurrence is difficult because of the locally invasive behavior of the tumor. Chemotherapy, molecular-targeted therapy, and radiotherapy are considered in cases with high surgical risk. Another case that was successfully treated with NSAIDs was previously reported []. Further analysis showed the stabilization of the beta-catenin protein in DT caused by mutation in either the APC or beta-catenin gene []. If mutated, beta-catenin accumulates in the cell and activates the T cell factor, which triggers the transcription of target genes including COX-2 []. Therefore, an elevated COX-2 protein level was observed in DT; COX-2 partially regulates proliferation through beta-catenin stabilization []. Therefore, the degradation of this protein or reduction of the nuclear accumulation of beta-catenin might decrease tumor growth. A molecular model suggests an interaction between NSAIDs and beta-catenin. However, the mechanism of beta-catenin inhibition by NSAIDs has not yet been elucidated. In the present case, the patient achieved complete remission on treatment with a COX-2 inhibitor, celecoxib. The initial plan was to treat the patient with tamoxifen; however, owing to its side effect, we did not proceed with this treatment option. At the end of the 2-year follow-up, the patient showed no tumor recurrence with maintenance therapy using celecoxib. Tanaka et al. [] reported that a patient who received etodolac, another COX-2 inhibitor, only achieved partial remission. NSAIDs indeed have a cytoreductive effect on DTs. However, different types of NSAIDs appear to have different efficacies in DT size reduction. Although resection is the only radical treatment option for DT according to the current consensus, medical therapy also has an important role in the treatment of patients with comorbidities or in those where resection is contraindicated. Chemotherapy has a relatively high complication rate for patients, and the results of radiotherapy treatment were not optimistic. Therefore, NSAIDs offer another treatment option for DT with fewer side effects. However, additional clinical trials and molecular analyses should be performed to test the effect of NSAIDs in the treatment of DTs.
Annie Lu	42	1977/12/1	(243)269-7178x495	[email protected]	39070 Hanna Park Apt. 365	A 72-year-old Japanese woman was referred to our hospital by her general practitioner with a 10-day history of swelling and pain in her right forearm without a previous history of trauma. A physical examination revealed that her right forearm was tense and swollen from the area directly proximal to her wrist to immediately below her elbow (Fig. ). She complained of increasing pain with passive extension and slight numbness of her fingers. Computed tomography revealed a low-density area in the muscles of the anterior aspect of her right forearm (Fig. ). Laboratory investigation showed a white cell count of 184.5 ? 109/L, including 1 % blast cells, 2 % eosinophils, 6.5 % basophils, 2.5 % promyelocytes, 14 % myelocytes, 18 % metamyelocytes, 44 % neutrophils, 7.5 % monocytes, and 4.5 % lymphocytes. In addition, her hemoglobin level was 66 g/L, and her platelet count was 3610 ? 109/L. Routine coagulation parameters revealed slightly prolonged prothrombin time (15.9 seconds, international normalized ratio 1.28). However, her activated partial thromboplastin time (32.6 seconds) was within the normal range of 24.0 to 35.0 seconds, as was her bleeding time (90 seconds). Plasma fibrinogen and D-dimer levels were slightly elevated to 390.8 mg/dL and 3.3 弮g/mL, respectively (normal 200 to 380 mg/dL and 0 to 1.0 弮g/mL, respectively), although her fibrin degradation product level was within normal range (8.2 弮g/mL). The von Willebrand factor (vWF) antigen level was normal (111 %), and the vWF ristocetin cofactor activity, at 70 %, was undiminished. Multimeric analysis of vWF did not show a decrease of large vWF multimers. Furthermore, factor 8 and 13 activities, at 82 % and 72 % respectively, were not reduced. Laboratory values are summarized in Table . She did not have any apparent skin and mucosal bleeding tendency such as petechial hemorrhage. Bone marrow aspiration revealed hypercellularity with marked myeloid proliferation, but only 3 % of the cells were blasts (Fig. ). These findings led to the presumption of a myeloproliferative disorder (MPD) such as CML, polycythemia vera, or essential thrombocytosis (ET). Because the level of her neutrophil alkaline phosphatase score was markedly reduced to 49 (normal 150 to 350), we deemed CML to be the most likely diagnosis. Cytoreductive therapy with hydroxyurea (1500 mg/day) was initiated as the most credible therapy to reduce her platelet count, because we could not rule out the possibility of MPD which does not respond to tyrosine kinase inhibitors. An urgent orthopedic opinion was obtained, and compartment syndrome was confirmed after the compartment pressure was found to be 39 mmHg. Emergency fasciotomy for decompression of the anterior compartment was conducted, which confirmed a tense compartment and pathologically proven hematoma in the right anterior compartment. There was no evidence of leukemic cell infiltration. Persistent local bleeding developed during the latter part of the operation despite no surgical cause. After decompression, the wound was left open and managed with a wet dressing. Sustained postoperative oozing of blood resulted in hypovolemic shock and required massive red cell concentrate transfusion for hematologic and cardiovascular resuscitation. She continued to require 4 to 8 units of red blood cell concentrate on a daily basis (in Japan, 1 unit of red cell concentrate is derived from 200 mL of donated whole blood). Chromosome analysis of her bone marrow cells demonstrated a karyotype of 46,XX,t(9;22)(q34;q11.2) (Fig. ). In addition, fluorescence in situ hybridization detected a BCR?BL fusion signal in 93 % of the cells (Fig. ). Thus, she was diagnosed with chronic-phase CML []. Following this definitive diagnosis, dasatinib therapy (100 mg/day) was initiated. Although there were no signs of improvement in the oozing of blood while her platelet count remained high, her bleeding tendency began to improve after her platelet count decreased to less than approximately 1000 ? 109/L. During her clinical course her coagulation parameters remained stable. Although her D-dimer level, which reflects the dissolution of blood clots, remained slightly elevated (0 to 3 弮g/mL) for a period of time, her prothrombin time and fibrinogen levels soon recovered to their normal range. Complete hemostasis was achieved 7 days after the fasciotomy, and the fasciotomy site was closed on postoperative day 20. She continued dasatinib therapy, with good hematological and molecular responses. She obtained complete cytogenetic response and major molecular response at 12 and 18 months, respectively, after the initiation of dasatinib administration.
Duncan Kane	36	1996/11/4	(997)623-3807x853	[email protected]	4781 Parker Mills	A 69-year-old male presented to the otolaryngology office with complaints of right sided neck mass and otalgia. His past medical history was significant for 50 years of smoking, regular alcohol use, and colonic adenocarcinoma managed with a colectomy several years prior. He was diagnosed with T4aN2aM0 hypopharyngeal carcinoma involving the right pyriform sinus with a single 4 cm metastasis to the right level V. He was offered surgical resection followed by GPU reconstruction and planned adjuvant radiotherapy. Of note, during surgical planning, it was felt that the mediastinal esophagus was likely not involved with the tumor. Thus, tumor resection was expected to produce a circumferential pharyngeal defect extending into the cervical esophagus, but not the mediastinal esophagus. Faced with such a defect, to avoid the morbidity associated with entering the abdominal cavity, many surgeons would advocate for reconstruction with a tubed cutaneous free flap rather than with the GPU []. However, at our institution one of the authors (D.W.A) working alongside the thoracic surgery team has been able to achieve better functional outcomes with the use of GPU as compared to reconstruction with tubed cutaneous free flaps. After careful consultation with the thoracic surgery team, a joint decision was made to pursue GPU reconstruction. A laryngopharyngectomy and right modified radical neck dissection were performed without complication. Following the resection, the thoracic surgery team proceeded with the esophagectomy and gastric mobilization. Gastric mobilization was hindered by intrabdominal adhesions related to the previous colectomy as well as dilated gastric veins related to apparent liver cirrhosis. Nevertheless, a well-vascularized and tensionless gastropharyngeal anastomosis was attained and a jejunostomy tube inserted. Postoperatively, the patient was managed in the intensive care unit due to difficulty weaning from the ventilator. His early postoperative course was complicated by sepsis, and an anastomotic leak was considered despite serosangouinous neck drains and no wound breakdown. He was managed conservatively with antibiotics until postoperative day 7, when he lost vacuum on the negative pressure suction drain in the neck. Dehiscence was confirmed using a water-based dye. The patient was then taken to the operating room and found to have circumferential necrosis of the proximal GPU extending inferiorly into the upper mediastinum (Fig. ). The necrosis was debrided until well-vascularized gastric mucosa was reached. A large defect remained extending from the distal oropharynx to the proximal superior mediastinum. The reconstructive options to re-establish the continuity of the alimentary tract in this patient were severely limited. Due to the patient? history of colonic resection and recent gastric pull up, intra abdominal tissue transfer, such as jejunal transfer or colonic interposition were not available. The two remaining options included a vascularized free tissue transfer or creating a controlled pharyngeal fistula and over sewing the proximal stomach. A 20 cm by 15 cm elliptical ALT flap was chosen as the donor free tissue transfer, and was folded on itself in conical design to reconstruct a neopharynx (Fig. ). Postoperatively, the patient spent 22 days in the intensive care unit and another month in the hospital undergoing rehabilitation and addressing psychosocial issues. His jejunostomy tube was removed prior to discharge as he was supporting himself nutritionally with a pureed diet. An endoscopic view of the ALT anastomosis one month post reconstructive surgery is shown in Fig. . At four months recovery he remains on an oral diet. His laryngostoma is shown in Fig. .
Ellianna Griffith	34	1991/12/20	-11682	[email protected]	46337 Juarez Club Apt. 872	A 20-year-old female who presented with an intermittent fever for two years and double lower limb numbness and weakness for one month was admitted to hospital in January 2015. In April 2013, she underwent a right lung pneumonectomy for ?ung cancer,??while the postoperative pathology referred to a ?ecrotizing granulomatous mass.??Seven months after the operation, a computed tomography (CT) scan showed an enclosed outstanding mass in the right chest wall (Fig. ). Thereafter, the patient was hospitalized three times due to a repeated fever and multiple bump biopsies from the enclosed outstanding mass; however, there was no clear diagnosis. One month before this admission, in addition to a fever, the patient suffered from worsened lower limb numbness and weakness, difficulty walking, and dysuria. In the past, she was healthy and had good nutrition. A physical examination showed an abnormal and oncotic hard mass (10 cm ? 5 cm) that protruded from the right chest wall. A 0.3-cm-diameter external fistula was seen in the central part of the pack (Fig. ), which oozed a nonodorous, yellowish turbid liquid. Coarse sounds without moist rales or rhonchi were heard with left lung auscultation. Superficial feeling of the abdominal wall was decreased from the T7 and T8 level, while a deep feeling was present. Muscle strength for both lower limbs was decreased: 1簞 right and 3簞 left. Muscle tension of both lower limbs was also diminished. The bilateral Babinski sign was positive, but concavity swelling of the lower limbs existed. Laboratory tests showed a white blood cell count of 18.21 ? 109/L, a hemoglobin level of 82 g/L, a platelet level of 509 ? 109/L, a C-reactive protein concentration of 98.00 mg/L, an erythrocyte sedimentation rate of 98.00 mg/L, and negative 1-3-帣-D glucan and glactomannan (GM) tests. Post right pneumonectomy, the thoracic CT scan revealed uneven density flake soft tissue shadows in the right chest cavity, multiple sizes of nodules in the left lung, a soft tissue mass (12.5 cm ? 5.9 cm) around a small pneumatosis in the right chest wall, and a reduced density in the right liver lobe near the diaphragmatic top (January 2015, Fig. ). The thoracic magnetic resonance imaging (MRI) results showed that a space-occupying lesion existed in the right pleural and chest wall with the T3-6 vertebral body and the right rib damaged, invading the spinal channel and spinal cord (January 2015, Fig. ). A biopsy through the right chest wall mass and thoracic mass by fistula of chest wall showed broad nonseptate hyphae with right-angle branching, consistent with mucormycosis (Fig. ). The mucormycosis was widely disseminated, invading the lung, skin, liver, vertebrae, and spinal cord. The patient quickly developed type II respiratory failure. A noninvasive ventilator assisted breathing, and intravenous amphotericin B (AmB) was administered. When the dose of AmB increased to 0.5 mg/kg/day, with the cumulative dose reaching 150 mg, mental disorders (agitation, fear, etc.) and cardiac arrhythmias occurred. The adverse events disappeared when AmB was changed to liposomal AmB. After treatment for 28 days, the patient? body temperature became normal and her respiratory function as well as lower limb sensory and motor function recovered. The size of the right chest wall mass became smaller, and the fistula dried. Laboratory and imaging examinations indicated that the lesions of the lung, liver, and thoracic vertebrae were significantly smaller. The patient was discharged back to the local hospital for further antifungal treatment and had recovered by the 3-month follow-up exam, and plastic surgery was suggested to repair the thoracic wall.
Franklin McCann	33	1983/3/8	6775247359	[email protected]	65036 Carpenter Causeway Suite 408	A 40-year-old woman was referred to our strabismus clinic with a complaint of double vision. She had already been diagnosed with hyperthyroidism, and her thyroid function had normalized after 2 months of treatment with antithyroid medication; subsequently, 2.5 mg carbimazole was used as a maintaining dose. She did not take systemic steroid medication, because her dysthyroid ophthalmopathy was not severe (Clinical Activity Score, CAS 2) and there was no aggravated symptomatic sign. She subsequently developed worsening diplopia and proptosis in her left eye 1 month prior to presentation. At the initial examination, her visual acuity was normal in both eyes (20/20), and 2 mm exophthalmos was noted in her left eye. She exhibited left hypotropia of 35 prism diopters (PD) in the primary position with marked limitation of left eye elevation (Fig. ). Asymmetric enlargement of the inferior and medial rectus muscles was demonstrated in her left eye on computed tomography (CT), and was compatible with dysthyroid ophthalmopathy (Fig. ). The forced duction test (FDT) revealed restriction in the upward direction in her left eye. She described a highly predictable fluctuating pattern of diplopia, that spontaneously switched at approximately 8 o?lock every night. To differentiate the causes of the fluctuating diplopia, such as myasthenia gravis, we performed laboratory tests, including a determination of acetylcholine receptor antibody titer; all results were normal. Based on her description, we recommended that she keep a diary to record her diplopia episodes and have her picture taken during episodes of both mild and severe diplopia. At the next visit, during which she exhibited relatively mild diplopia, cover testing revealed left hypotropia of 7 PD in the primary position with mild limitation of left eye elevation (Fig. ). Her diary clearly revealed the ?very-other-day??pattern of diplopia. During the 6 months since the first visit, her thyroid function remained normal and the proptosis of her left eye decreased. A cycle involving diplopia-free days was gradually predominant. Finally, the left hypotropia of 7 PD remained, but without a cyclic pattern. Her diplopia was well compensated with mild chin elevation.
Joyce Fisher	30	1988/9/10	847.563.0146	[email protected]	83255 Griffin Cliff	The patient is an 11-year-old male with attention deficit hyperactivity disorder (ADHD) who presented with an unintentional 20 lb. weight loss that was initially attributed to ADHD medication. Exam was notable for cervical lymphadenopathy. Laboratory workup showed leukocytosis with left-shifted granulocytes (WBC 210,000 cells/弮L; 65 % neutrophils, 6 % bands, 5 % lymphocytes, 3 % monocytes, 2 % eosinophils, 3 % metamyelocytes, 13 % myelocytes, 2 % promyelocytes, 1 % blasts), normocytic anemia (Hgb 10.8 g/dL, MCV 92 fL), elevated LDH (1,858 U/L), and elevated uric acid (7.0 mg/dL). Abdominal CT scan showed splenomegaly. A bone marrow study revealed hypercellular marrow (>95 %) with increased myeloid cells at all stages of maturation, increased eosinophils, scattered basophils, few erythroid progenitors, so-called dwarf megakaryocytes, and sea-blue histiocytes (Fig. ). Blasts were <5 % morphologically. Flow cytometry identified a population of atypical CD3+ MPO- TDT+ T lymphoblasts (9 % of total events). IHC of the bone marrow core biopsy confirmed these latter findings, showing individual and clusters of CD3+ TDT+ T lymphoblasts comprising ~10 % of overall cellularity and 20??0 % of cells in restricted areas (Fig. ). RT-PCR from peripheral blood was positive for the BCR-ABL1 p210 transcript. Cytogenetic analysis of the bone marrow showed 46,XY,t(6;9;22)(p22;q34;q11.2)[]/46,sl,inv(3)(q21q26.2)[], confirming the presence of a variant three-way translocation generating the BCR-ABL1 fusion. The presence of a subclone (11 of 20 cells analyzed) with inv(3)(q21q26.2) suggested disease progression. Following identification of this inversion and validation of a novel MECOM antibody, combined IHC for MECOM and CD3 was performed on the bone marrow core biopsy. Interestingly, the CD3+ population and the MECOM-overexpressing population were non-overlapping (Fig. ), indicating the T lymphoblastic transformation was independent of the acquisition of inv(3)(q21q26.2). Based on these findings, a diagnosis of CML with T lymphoblastic transformation was rendered. The patient was started on hydroxyurea and allopurinol, followed by single-agent treatment with imatinib (500 mg daily). Repeat bone marrow studies on day 25 of treatment showed restoration of trilineage hematopoiesis and normal cellular morphology, with abnormal T lymphoblasts comprising 1 % of total cellularity by flow cytometry. Subsequent to this study, he was transitioned from imatinib to dasatinib (100 mg daily) due to the development of oral ulcers, and continued to improve clinically on TKI alone. A third bone marrow biopsy and aspirate at day 54 showed 0.02 % T lymphoblasts. Peripheral blood quantitative RT-PCR analysis at three months showed a 1.4 log reduction of BCR-ABL1 transcripts (4.3 % IS units) (Table ). Despite this response to TKI monotherapy, a matched unrelated stem cell donor was identified and transplantation is scheduled given the high-risk features of his disease.
Gael Conner	22	1987/3/4	570.413.0302x29658	[email protected]	17176 Wells Ways Suite 082	A 74-year-old female noted visual impairment in her left eye. The best corrected visual acuity (BCVA) was 0.25 in the left eye. Fundus photography (Fig. ) shows retinal hemorrhage with a white lesion superior to the optic nerve. FA revealed staining in the early phase (Fig. ) and leakage in the late phase (Fig. ). The OCT showed a round cavity with a hyperreflective wall and a dark lumen in the area of the RAM (Fig. ). Cystoid retinal edema was adequately visualized in the en face OCT image (Fig. ). The OCT angiogram also showed the RAMs. An interruption of blood flow in the area of the RAM was observed in the superficial retinal OCT angiogram (segmented with an inner boundary at 3 弮m beneath the internal limiting membrane and outer boundary at 15 弮m beneath the inner plexiform layer) (Fig. ). The deep retinal OCT angiogram (segmented with an inner boundary at 15 弮m beneath the inner plexiform layer and the outer boundary at 70 弮m beneath the inner plexiform layer) and the OCT angiogram of the outer retina showed high blood flow in the RAM (Fig. and ). Using cross-sectional OCT angiography, it is possible to visualize the level of blood flow in the RAMs (Fig. ).
Alondra Sloan	40	1990/4/12	(519)256-2951	[email protected]	4652 Crystal Falls Apt. 153	A 68-year-old female noted acute visual loss in her left eye, the BCVA was 0.1. Fundus photography, FA and OCT angiography findings of a superotemporal macroaneurysm with intraretinal hemorrhage are presented in Fig. -. The superficial retinal OCT angiogram shows reduced blood flow in the RAM (Fig. ). Blood flow was detected in the same area in the deep retinal OCT angiogram (Fig. ) while no flow was detected in the outer retinal OCT angiogram (Fig. ). In this case, the RAM must have its origin in the inferior part of the vessel, as the saccular RAM can be perfectly visualized in the deep retinal OCT angiogram (Fig. ). Two months after intravitreal anti-vascular endothelial growth factor therapy it was not possible to visualize the RAM despite varying the segmentation (Fig. - ).
Ocean Griffith	39	1983/1/17	540.580.8048x6514	[email protected]	0028 Arnold Extension	A 72-year-old female developed a deterioration of visual acuity in the left eye. The RAM is superotemporal in relation to the fovea, as in Case 2 (Fig. ). Using information from FA, SD OCT and OCT angiography (Fig. -), two main parts can be determined in the RAM: a smaller part (Fig. ) located mainly above the retinal vessel with blood flow seen in the superficial retinal OCT angiogram (Fig. ) and a fusiform part located below the retinal vessel (Fig. ) with blood flow seen mainly in the deep retinal OCT angiogram (Fig. ). The shape of the RAM is shown best on the early fluorescein angiogram (Fig. ). The cross-sectional OCT angiography visualizes the level of blood flow in the RAMs (Fig. ).
Alicia Grimes	37	1977/11/2	2813678694	[email protected]	376 Maddox Plaza	This 29 years old female presented with 1 week history of low grade intermittent fever with constitutional symptoms and a persistent worsening of cough for the same duration. Her fever did not associate with chills or rigors and she did not have hemoptysis. She was previously healthy and she was a housewife who engaged herself actively in gardening at her own backyard. She did not have a contact history or a past history of tuberculosis. She had no history of foreign travel. She did not have any other symptoms to note. On examination, she was average built with a BMI of 24 kg/m2. She was febrile. She was neither pale, nor she had lymphadenopathy or organomegaly. Her pulse was 120 bpm and blood pressure was 86/60 mmHg on admission. There were few scattered crackles in her lungs. There were no signs of focal consolidation, effusions evident on examination. She did not have skin ulcers, cellulitis or skin abscesses. She had no features of meningism, focal neurological deficits and had normal fundi. Her investigations showed a haemoglobin of 11.2 g/dl with neutrophil leukocytosis (total 18,000/繕l with 82 % neutrophils) with platelet count of 540,000/繕l. Her ESR was 60/1st h and CRP was 458 繕/l (<6). Chest radiograph showed bilateral patchy shadows involving both lung fields (Fig. ). Her high-resolution computed tomography (HRCT) scan showed multiple bilateral cavitary lesions in the upper, middle and lower zones of lungs without air-fluid levels (Fig. ). We administered broad spectrum intravenous antibiotics (Ceftriaxone 2 g 12 h and Clindamycin 600 mg 8 h) for 5 days, in spite of which she continued to have high fever spikes and showed clinical deterioration. Her blood and urine cultures revealed negative results. Since her Mantoux test and sputum smear and culture for acid fast bacilli (AFB) were negative, we proceeded to perform an early fibro-optic bronchoscopy. Bronchoscopy was normal and the broncho-alveolar lavage fluid (BALF) specimens were negative for AFB, Pneumocystis jiroveci and fungi. All BALF cultures including bacterial, fungal and mycobacterial were performed and special cultures were requested for melioidosis due to poor response to initial broad spectrum antibiotics. Surprisingly, culture for melioidosis using Ashdown? agar became positive for Burkholderia pseudomallei, while all other cultures were negative. On Ashdown? agar, the colonies showed typical purple, wrinkled morphology after 72 h of incubation. Melioidosis antibody titre (Indirect Haem-agglutination Assay) was detected to be highly positive at a titre of 10,640. Her transthoracic and trans-esophageal echocardiogram did not show vegetations. Autoimmune panel with ANA and ANCAs were negative. Contrast CT chest and abdomen did not reveal any visceral lymphadenopathy or abscesses. Her HIV, TPHA/VDRL, HbA1c (5.5 %), renal, liver functions and serum immunoglobulin levels were all normal. After detecting BALF cultures and antibody titre positivity for Melioidosis, we changed the antibiotics to high dose intravenous Meropenem (2 g 8 h). She showed excellent response for these antibiotics within few days and demonstrated a marked clinical improvement and radiographic resolution. After 7 days of intravenous antibiotics, melioidosis antibody titers reduced to 2750 and subsequently to 160 after 4 weeks. We continued her intravenous antibiotics for a month and discharged her on oral Doxycycline (100 mg 12 h) for another 24 weeks. In review of the patient, her recovery was uncomplicated after completion of the course of antibiotics.
Harlan Moon	32	1980/7/6	2922112331	[email protected]	813 Jennings Isle Apt. 663	A 17-year-old man complained of diarrhea and bloody stools and was referred to our hospital in 2000. His family had no particular disease. He was diagnosed with total-type UC by total colonoscopy (TC). The patient had no primary sclerosing cholangitis. Mesalazine at 2250 mg/day was prescribed. In June 2005, he underwent TC. Immunohistochemical analysis of a rectum biopsy showed LGD with an overexpression of p53 protein. The following month he complained dyspnea and edema of the whole body and he gained 4 kg in weight. He was consulted by a nephrology physician. Laboratory data showed total protein of 4.5 g/dl, albumin of 1.4 g/dl, total cholesterol of 352 mg/dl, creatinine of 1.7 mg/dl, and blood urea nitrogen of 43 mg/dl in the serum. His urine showed proteinuria 3.6 g/day. He was diagnosed with NS according to the criteria. Biopsy of the kidney demonstrated a minimal change in NS histologically. Prednisolone at 35 mg/day was initiated. Cyclosporine at 250 mg/day and mizoribine at 150 mg/day were added because of steroid resistance. His symptoms disappeared after 3 months of medication. Mesalazine had not been administered to the patient since 2008. Prednisolone was tapered to 12.5 mg. TC was performed in 2013 and a stenotic lesion was detected in the rectum. Biopsy of the rectum showed LGD histologically. In July 2014, he complained of abdominal distension. Abdominal X-ray showed distension of the colon and an abdominal computed tomography (CT) scan showed a rectal tumor associated with swellings of multiple abdominal lymph nodes. He was admitted to our hospital. Laboratory data at admission is shown in Table . Serum carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA19-9) levels were markedly elevated. Urinalysis did not show occult blood, proteinuria, and glycosuria. TC showed a round shaped elevated lesion in the rectum with an ulcer on top (Fig. ). A colonoscope was passed through the stenosis site and inflammation of the colorectal mucosa was mildly active. Biopsy of the rectal tumor showed well-differentiated adenocarcinoma with mucin production. Overexpression of p53 protein was observed immunohistochemically. Magnetic resonance imaging (MRI) showed multiple swellings in the rectal mesentery lymph nodes and lateral pelvic lymph nodes. Transverse colostomy was performed under general anesthesia. As KRAS gene mutation was not detected in the biopsy specimen, the patient received a total of two cycles of modified FOLFOX6 and panitumumab [] over 2 months as neoadjuvant chemotherapy. Adverse effects of chemotherapy were peripheral neuropathy (grade 1) and skin rash in the face (grade 1). Repeated CT scan and MRI did not show shrinkage of the tumor indicating stable disease. Five weeks after the completion of chemotherapy, the patient underwent a total proctocolectomy with a stapled ileal pouch anal-canal anastomosis, total mesorectal and bilateral pelvic lymph node dissection, and temporary loop ileostomy. The patient fared well in the postoperative course. The resected specimen showed an irregular shaped, round, elevated mass that was 3 cm in diameter at the rectum (Fig. ). Background colorectal mucosa was flat with mild inflammation confirming UC. Histological examination of the specimen revealed mucinous adenocarcinoma with well and moderately differentiated adenocarcinoma. Over expression of p53 protein was observed immunohistochemically (Fig. ). Metastases were observed in 25 lymph nodes including the mesorectal and bilateral pelvic lymph nodes. Colorectal mucosa showed active UC with cryptitis and crypt abscess histologically. The pathological stage of the carcinoma was pT3N2bM1a, stage IV, according to the TNM classification of UICC, 7th edition []. The patient was discharged from the hospital after receiving 1 cycle of modified FOLFOX6. He received additional 7 cycles of modified FOLFOX6 in 4 months at the outpatient unit. Adverse effects of postoperative chemotherapy were peripheral neuropathy (grade 2). The patient is currently alive without evidence of recurrence of the carcinoma 12 months after colectomy. We report a case of an UC patient where disease was associated with advanced rectal carcinoma. UC was associated with NS after treatment of UC with mesalazine for 5 years. Firwana et al. reported a case of NS after mesalazine treatment for Crohn? disease []. He also reviewed six case reports of NS after the treatment of UC with 5-amino-salacylic acid derivatives. All six patients were given steroids and improved. However, the relationship between mesalazine and NS is currently unclear. Kiran et al. reported that carcinoma was present in 29 % of UC patients with preoperative HGD compared with 3 % in those with LGD []. Thus, the risk of carcinoma in UC patients with HGD is substantial. In the current case, the patient showed advanced rectal carcinoma 8 years after LGD was detected by surveillance colonoscopy. He received steroids and immunosuppressants for the treatment of NS. However, these reagents might accelerate the progression of rectal carcinoma from dysplasia. The standard surgical procedure for patients with UC is restorative proctocolectomy with construction of an ileal pouch. Colectomy for UC patients with carcinoma requires sufficient lymph nodes resections []. In this case, 25 metastatic lymph nodes including the pelvic lateral lymph nodes were resected surgically. It is very rare that so many metastatic lymph nodes were detected in resectable sporadic or colitic carcinoma cases without distal organ metastases. This patient was under immunosuppressive conditions induced by the steroid and immunosuppressant treatment for NS. The possibility exists that this immunosuppressive condition was precipitated the aggressive lymph node metastases. Advanced carcinoma affects the lifespan of UC patients. In a study conducted in Japan, Watanabe et al reported that patients with CRC-associated UC showed poorer survival rates than those with sporadic CRC in the advanced stages, while no difference was observed in the early stages []. It is important to detect dysplasia and carcinoma at an early stage by surveillance. LGD with an overexpression of p53 protein was detected in this patient by surveillance colonoscopy. p53 mutations have been shown to occur at an earlier phase in the progression of UC-associated neoplasia []. It had already developed to an advanced carcinoma when he underwent the follow-up colonoscopy. This is a rare case, and there are still problems concerning the method of surveillance and follow-up colonoscopy.
Naya Cantrell	20	1986/2/21	690-932-9652	[email protected]	83696 Patricia Center	A 42-year-old man who had been treated for depression for 3 years visited our clinic because of right motor weakness and aphasia that had worsened progressively over 1 month. Manual muscle testing (MMT) revealed right motor weakness of 3/5 in the upper limb and 4/5 in the lower limb and he had total aphasia. Head magnetic resonance imaging (MRI) (Fig. ) demonstrated multiple cystic lesions in the left temporal lobe. These cysts had low signals in T1-weighted (T1WI) and diffusion-weighted images (DWI), and high signals in T2WI. The parenchymal components around the cysts showed slightly high signals in DWI. The parenchymal portions and cyst walls were enhanced by gadolinium dimeglumine. A pronounced left to right midline shift caused by the mass effect of the cysts and brain edema was evident. Neuroradiologists commented that the most possible diagnosis was GBM and malignant oligodendroglioma should be a differential diagnosis considering the patient? age. The tumor was partially removed and the largest cyst opened under general anesthesia (the white arrows in Fig. indicate the resected portion.). The tumor was yellowish and soft and the fluid in the cyst was weakly xanthochromic. Histological examination of the resected tissue resulted in a diagnosis of GBM (Fig. ). One month after surgery, radiation of 60 Gy (2 Gy ? 30 times) and oral temozolomide (TMZ) treatment (75 mg/m2) for 42 days were initiated []. After this treatment, the right lower extremity? motor function recovered to 5/5 on MMT; however, the right upper limb motor weakness and aphasia did not improve with the treatment. One week after completion of irradiation and TMZ treatment, the patient was discharged to home and continued to take TMZ treatment for 5 days every 4 weeks. Three months later, he was brought to our emergency room because of deterioration in consciousness. His Glasgow coma scale was 4 (E1M1M2), and MRI demonstrated enlargement of several of his cysts, causing uncal herniation (Fig. ). The patient? parents refused further treatment on his behalf and he died 5 days after this admission. An autopsy was performed 6 h after his death with the permission of the patient? parents. Histological examination of the operative specimen resulted in a diagnosis of GBM, and this diagnosis was based on the following characteristic features; high cellularity, pleomorphism with giant cells, endothelial proliferation, pseudo-rosette formation, necrosis (Fig. a, b, c), and a high Ki-67 index of 20 % (Fig. d). On gross morphological examination, the cysts in the tumor shrank after formaldehyde fixation and the parenchymal portion of the tumor was clearly identified. An absence of destructive tumor invasion of the white matter indicated it was not aggressive (Fig. ). On low magnification of hematoxylin and eosin (HE) stained sections, tumor necrosis was evident but the boundary between the tumor and normal surrounding tissue was well-defined (Fig. ). Examination under higher magnification confirmed well-defined tumor boundaries (Fig. a, b, c). The histologic appearance was heterogenous and pleomorphic giant cells were readily identified (Fig. d). Clear xanthomatous cells with foamy cytoplasm were apparent (Fig. e) and were strongly positive on silver staining (Fig. f). The tumor was strongly glial fibrillary acidic protein positive (Fig. g) and Ki-67 index was extremely low as 1 % (Fig. h).
Harris Randolph	42	1982/3/20	(314)569-3276x7170	[email protected]	8530 Rodriguez Walks	A 61-year-old male with a history of alcoholism and open cholecystectomy was admitted to our hospital for hematemesis. On examination, he was tachycardic and tachypneic. His blood test results were as follows: total white blood cell count 16.1 ? 103/L, platelet count 9.3 ? 104/L, C-reactive protein level 10.3 mg/dL, blood urea nitrogen level 30 mg/dL, serum creatinine level 1.37 mg/dL, and endotoxin level 7.5 pg/mL. Upper gastrointestinal endoscopy revealed a left-sided rupture of the lower esophagus (Fig. ), and a chest CT scan revealed pneumomediastinum (Fig. ), which was suggestive of the intra-mediastinal type of Boerhaave? syndrome. The patient underwent emergency surgery 24 h after the onset of his symptoms. A 7-cm upper midline incision was made through the skin for the hand-assisted laparoscopy technique. Two 5-mm ports were produced in the left hypochondrium and left flank. A 12-mm port for a video camera was made in the left upper quadrant. Under hand-assisted laparoscopy, while expanding a field of vision of the esophageal hiatus, the abdominal esophagus was encircled and the left diaphragmatic crus was dissected with ultrasonically activated scalpel. Subsequently, intraoperative endoscopy was performed; we identified the site of the esophageal rupture at the same level as diaphragm. A longitudinal perforation of the left side of the esophagus measuring approximately 2 cm was clearly visible (Fig. ). A cloudy fluid collection with gastric juice and blood clots was seen around the site of perforation in the mediastinum. The associated crushing injury was mild, and the rupture site was closed with a two-layered suture under hand-assisted laparoscopy (Fig. ). The closed site was not covered by the omentum or any other tissue. A leakage test was performed. Ultimately, a drain was placed in the posterior mediastinum, and a feeding jejunostomy was constructed. The patient recovered without any complications. He was transferred from the intensive care unit to a general ward in their progress from being critically ill to recovering. A diatrizoate meglumine esophagogram obtained at 9 days after the operation did not show any extravasation. He was discharged from hospital at 3 weeks after the operation. Boerhaave? syndrome involves the spontaneous transmural rupture of the esophagus and was first described in 1724 by Dr. Herman Boerhaave []. Despite considerable improvements in diagnostic, therapeutic, and intensive care techniques, esophageal perforation remains a life-threatening condition and is associated with high morbidity and mortality rates (ranging from 10 to 50 %) []. It is considered to be caused by a rapid increase in pressure within the esophagus, e.g., during vomiting, together with the failure of the cricopharyngeus muscle to relax. Such ruptures often occur at sites of anatomical weakness, e.g., the part of the left posterolateral wall of the lower third esophagus located 2 to 3 cm from the gastroesophageal junction []. In cases of esophageal rupture, the most important aim of any treatment should be immediate closure of the esophageal tear. There have been several reports about cases in which Boerhaave? syndrome was successfully managed with a self-expandable metallic stent [?. Recently, endoscopic closure using a clipping device has also been reported [?, but surgical treatment is usually selected, as advanced mediastinal intrapleural contamination by the contents of the gastrointestinal tract is often seen at the initial examination. Regarding surgery for spontaneous esophageal rupture, previous reports have suggested that most procedures are performed via a thoracotomy or laparotomy, and there have been few reports about camera-assisted operations being performed for spontaneous esophageal rupture. A search of PubMed using the keywords ?horacoscopy,???aparoscopy,??and ?oerhaave? syndrome??revealed that camera-assisted operations have been performed for spontaneous esophageal rupture in seven cases [?, and simple suture closure of the perforated site under thoracoscopy or laparoscopy was carried out in six of these cases [?? ]. Ashrafi et al. state that indications for the minimally invasive technique for spontaneous esophageal rupture are limited to patients with stable hemodynamics, without signs of escalating sepsis, and without concomitant conditions contraindicated for surgeries, including laparoscopic or thoracoscopic surgery []. However, Vaidya et al. reported that they selected a thoracoscopic approach for a patient with septic shock []. Thus, at present, there are no clear strategies for the selection of endoscopic treatment. Previous studies, including ours, reported that the perforations were 2?? cm in diameter, suggesting that endoscopic surgery is well indicated for patients in whom the perforation site is small, but that, in patients in whom the perforation site is so large that it must be closed with sutures and covered by the omentum or any other tissue, open-chest or abdominal surgery needs to be performed to secure sutures without sticking to endoscopic surgery. The advantages of endoscopic surgery include the following: (1) it is minimally invasive because it minimizes the injury to the chest and abdominal walls; (2) it carries a low risk of wound infection; and (3) it provides a wider surgical field. On the other hand, its disadvantages are as follows: (1) the operative time is prolonged; (2) the removal of contaminants may be insufficient; and (3) the surgeon needs to be skillful in the manipulation of sutures. As for the approach used to gain access to the mediastinum, the transabdominal approach is considered to be more useful than the transthoracic approach as it causes less operative stress and allows a drain to be inserted into the appropriate part of the posterior mediastinum, the use of the greater omentum, and the addition of an intestinal fistula. In the present case, as the perforation did not open into the thorax, we selected the transesophageal approach. Patients with esophageal perforations are often in a poor physical condition, and camera-assisted operations, which take a considerable amount of time, are not suitable for such cases. However, at our hospital, camera-assisted approaches have been selected for standard esophageal and gastric operations since 2003. In the current case, we selected a laparoscopic approach, even though the patient required an emergency operation, based on the assumption that the enlarged view that this would provide would allow us to definitively identify and close the perforation site. In addition, since the patient had a history of cirrhosis, and preoperative CT revealed marked enlargement of the left hepatic lobe, we predicted inadequate widening of the surgical field at the esophageal hiatus under complete endoscopic visualization and performed hand-assisted laparoscopic surgery. Successful lifting of the lateral hepatic segment and traction of the stomach by the surgeon? left hand enabled us to close the perforation in a wide surgical field. Concerning the closure of the perforation site, it has been reported that dysraphia is more likely to occur in cases in which 24 h or more have passed since the onset of the condition [], but as the enlarged view provided by the laparoscope revealed that the perforation site was small in diameter and did not exhibit necrosis or severe crushing, we performed simple closure under hand-assisted laparoscopy, and the patient? postoperative course was favorable. It is not possible to conduct a comparative study of the various methods that can be used to treat spontaneous esophageal rupture in cases that require emergency surgery, as the number of cases is limited. Therefore, the further accumulation of cases is necessary, but as camera-assisted operations are minimally invasive, such procedures should be considered under certain conditions (e.g., depending on the skill level of the surgeon and whether the institution has the appropriate facilities to perform such operations).
Kailey Whitaker	37	2000/1/23	271-685-5421	[email protected]	4075 Johnson Causeway Apt. 408	An 18-month-old boy without previous health problems presented with abdominal pain, vomiting, and poor appetite for 6 days. The medical history revealed that he had been given a multivitamin preparation once daily (50.000 International Unit (IU) of vitamin D and 10.000 IU of vitamin A) by his pediatrician for a wide anterior fontanelle for about three months. The physical examination on admission revealed only agitation. Vital signs were normal. Serum calcium was 11.5 mg/dL (normal 8??0.4), phosphorus was 4.3 mg/dL (normal 4.5??.5), alkaline phosphatase (ALP) was 91 IU (normal 60??21), creatinine was 0.5 mg/dL, 25-hydroxyl-vitamin D (25-OHD) levels were 2271 ng/mL (normal 30??00), and parathyroid hormone (PTH) was <3 pg/mL (normal 4.6??8.1). Urinary calcium/creatinine ratios were 1.1 mg/mg (normal <0.21). The patient was treated with intravenous hydratation of 150 mL/Kg/day and furosemide at 2 mg/kg/day, with a diet with low calcium and phosphorus content. Renal ultrasonography showed medullary nephrocalcinosis with symmetric bilateral involvement (); so prednisolone 1 mg/kg/day was also added to the treatment regimen. The ECG, hearing test, and ophthalmological examination were all normal, in particular there was no ?apilledema.??With this therapy, the total calcium level decreased rapidly and we observed a clear improvement in clinical conditions. When he was discharged, his 25-OHD level was still elevated at 630 ng/mL and his calcium concentration was 9.5 mg/dL. His discharge instructions included avoidance of products containing vitamin D. On follow-up examination at 1 month the patient was normocalcemic with normal urinary calcium excretion and 25-OHD levels were at 150 ng/mL. The patient is being followed up for nephrocalcinosis.
Keith Hanson	26	2005/4/18	(742)729-1746x995	[email protected]	5968 Michael Cliffs Suite 808	An 88-year-old man was admitted to the hospital with worsening malaise, fever, and weakness. The patient had a prior medical history of bilateral hip prosthesis, high blood pressure, and hypercholesterolemia. In the months preceding his admission, he had developed a right-sided hip pain. On the day of admission, his family had found him lying on the floor, too weak to walk. His blood pressure was 94/22 mmHg, heart rate 64 beats/min, and temperature 38.9簞C. The examination of his head, neck, heart, lungs, and abdomen did not reveal anything abnormal. He had no wound nor sore. The mobilization of his right hip showed a limited range of motion without pain. Laboratory analysis revealed leukocytosis with a white blood cell count of 15.3 ? 109/L (normal: 4 ? 109/L to 11 ? 109/L), a normal hemoglobin level of 135 g/L (normal: 132 g/L to 170 g/L), and platelet count of 155 ? 109/L (normal: 130 ? 109/L to 400 ? 109/L). The creatinine was at 151 弮mol/L (normal: 58 弮mol/L to 110 弮mol/L), aspartate aminotransferases (AST) were at 63 U/L (normal: 17 U/L to 59 U/L), creatine phosphokinase (CPK) was at 669 U/L (normal: 55 U/L to 170 U/L), C-reactive protein (CRP) was at 230 mg/L (normal <10 mg/L), and procalcitonin was at 1.1 ng/mL (normal <0.1 ng/mL). Four sets of aerobic and anaerobic blood cultures were collected on two consecutive days. A preliminary diagnostic of septic arthritis of the patient's right hip was suspected and a computed tomography (CT) scan of the pelvis was ordered. It showed no asymmetry or fluid collection around the hip prosthesis. The chest X-ray was read as normal. The patient was kept under observation, without antibiotic therapy. The four anaerobic blood culture bottles became positive after 29 hours of incubation. Their Gram stain revealed the presence of Gram-negative rods. The culture showed the bacillus to be anaerobic, Gram-positive, and sporulated, typical of a Clostridium species. No other organism was isolated. A commercial anaerobic manual identification panel (RapID ANA II System, Remel, Lenexa, KS, USA) identified the bacillus as Clostridium butyricum, with >99.9% of probability. However, the isolate could not be identified by matrix-assisted laser desorption ionization-time-of-flight mass spectrometry (MALDI-TOF MS) (Bruker, Madison, WI, USA), after a complete extraction. Both empirical antibiotherapy with intravenous piperacillin-tazobactam and the search for the source of infection were initiated. A CT scan of the abdomen and pelvis showed multiple stones in the gallbladder without cholecystitis and no ascites nor colonic masses. A transthoracic echocardiogram revealed an aortic valve sclerosis without vegetation. A colonoscopy revealed three benign polyps without any sign of colitis or cancer. The patient defervesced after four days of intravenous piperacillin-tazobactam. On the 6th day of antibiotherapy, he was switched to oral amoxicillin for eleven more days. He was discharged from the hospital still weak, using a walker. Four months later, he had recovered his strength and was able to walk with a cane. He had no relapse of fever nor worsening of his right hip pain. Due to the discordance in identification between the RapID ANA II System and MALDI-TOF MS, the bacterium was sent to the Laboratoire de Sant矇 Publique du Qu矇bec (LSPQ) for further characterization. It was designated LSPQ-04253 and was identified by 16S rRNA sequencing using the BigDye Terminator v3.1 Cycle Sequencing Kit on an ABI 3130xl Genetic Analyzer (Applied Biosystems, Foster City, CA, USA) []. Its nucleotide sequence was determined and showed 99.2% identities with its orthologous sequence in Clostridium lavalense type strain CCRI-9842T. To identify the taxonomic neighbors of the clinical isolate, the 16S rRNA gene sequences were used for an initial BLAST search () against GenBank. Phylogenetic and molecular evolutionary analyses with closely related Clostridium species were performed on 1,344 nucleotides with MegAlign簧 version 10 (DNASTAR, Madison, WI). Multiple sequence alignment and phylogenetic analysis using 16S rRNA gene sequences of LSPQ-04253 (GenBank accession number KX024579) and those of C. lavalense CCRI-9842T and CCRI-9929, C. asparagiforme DSM15981T, C. bolteae WAL16351T, C. citroniae CCUG52203T, C. clostridioforme ATC2553T, C. aldenense CCUG52204T, and C. butyricum VPI3266T revealed that LSPQ-04253 clustered with the two C. lavalense strains and was distant from C. butyricum VPI3266T (). The antibiotic minimal inhibitory concentrations were determined using the agar dilution method [] and E-test only for vancomycin and teicoplanin. The bacterium was susceptible to clindamycin, cefoxitin, meropenem, metronidazole, piperacillin-tazobactam, vancomycin, and teicoplanin and intermediate to penicillin (). No vanB gene was found in isolate LSPQ-04253 as described in the type strain CCRI-9842T. The vanB gene is carried by a mobile genetic element (transposon Tn5382) in the type strain CCRI-9842T that could be missing in isolate LSPQ-04253.
Mariana Burton	35	1985/5/6	970.506.0647	[email protected]	00528 Jackson Circle	A 62-year-old male patient presented in January 2011 with cough and hemoptysis, microhematuria, polyarthritis of both ankles, knees, and left wrist, bilateral lymphadenopathy, and pulmonary nodules revealed by a CT-scan (). This nonsmoker had worked from 1974 to 1986 in a uranium mining being exposed to silica dusts. His ANCA titer was 1 : 640 with a specificity of PR3-ANCA at 193 U/mL. Neither rheumatoid factors nor anticyclic citrullinated peptide (CCP) was detectable. Although a kidney biopsy was inconclusive, the diagnosis of granulomatosis with polyangiitis was suspected, and cortisone treatment resulted in an amelioration of symptoms. A thoracoscopically resected pulmonary lesion from the left upper lobe (, arrow) revealed necrotic granuloma with centrally located silicoanthracosis consistent with silicosis. No vasculitis was found in lung or kidney at that time. In February 2011, the patient had a resection of a clear cell carcinoma in the upper pole of the right kidney (pT1 cN0 cM0 G2). Meanwhile, methotrexate and nonsteroidal antirheumatic drugs (NSAR) were added due to ongoing and immobilizing polyarthritis, leading to a rapid and significant improvement of inflammation and pain. While ANCA titers gradually decreased over time to 1 : 40, and PR3-ANCA titers almost normalized, antinuclear antibodies showed a constant level of 1 : 80. However, complement factors and also IgG levels showed normal values throughout the entire clinical course. A follow-up thoracic CT scan (January 2014) revealed new bilateral multiple round lesions (). Histological specimens provided by transbronchial forceps biopsy could only reveal fibrotic areas with anthracosis and birefringent crystals by compensated polarized light microscopy, corroborated by the aforementioned pathological diagnosis of anthracosilicosis. During the following short time period of 6 months, the patient developed an increasingly nagging cough with repeated expectorations of putrid and musty material several times a day. A CT scan (June 2014) showed a massive bilateral progression of nodules in size and number (Figures and ), further aggravating the patient's symptoms. A bronchoscopic forceps biopsy with radial endobronchial ultrasound (R-EBUS) under fluoroscopic guidance from the largest lesion (), but also from other lesions, was performed; however, these biopsies were not diagnostic. Therefore, three representative nodules of the right lung were thoracoscopically resected and appeared as whitish, star-shaped lesions on the pleural surface, highly suggestive of metastases (). Inside, they showed a white-greyish surface with dark spots and of crumbly consistency. Histology confirmed anthracosilicotic dust bands, regional necroses, and vasculitis. However, there were no necrobiotic granulomas (Figures ??. As a new finding, vasculitis with fragmentation of the internal lamina elastic wall was found (, arrow), and therapy with rituximab was started. In parallel, therapy with NSAR and methotrexate was stopped, leading to reoccurrence of intolerable joint pain and polyarthritis within several weeks. X-rays of the hands showed hook-like osteophytes in the metacarpophalangeal (MCP) joints, cartilage calcification, and joint space narrowing and subluxation of MCP joints but no RA typical features such as joint erosions. These findings were highly suggestive of calcium pyrophosphate crystal deposition (CPPD) disease as a cause of the patient's polyarthritis. The patient was restarted on NSAR and methotrexate and improved rapidly. At a follow-up visit in October 2015, the patient reported a clear improvement of the polyarthritis and respiratory symptoms.
Zander Whitaker	23	1984/10/30	889-897-4652	[email protected]	7076 Preston Wall	A 41-year-old unemployed, single woman with suspected insulinoma and a 13-year history of refractory schizophrenia (diagnosed according to The Diagnostic and Statistical Manual of Mental Disorders IV Criteria) was transferred from a mental hospital to the inpatient psychiatric unit of the Osaka General Medical Center (OGMC) for persistent hypoglycemia (fasting plasma glucose = 50~60 mg/dL) combined with elevated levels of immunoreactive insulin (IRI; 12.7 弮U/mL) and C-peptide immunoreactivity (CPR; 4.1 ng/mL). Although the patient did not present with any relevant physical or mental symptoms, she had previously been hospitalized five times for aggravation of psychotic symptoms. On this admission, she received antipsychotics (paliperidone 12 mg/day, bromperidol 36 mg/day, and levomepromazine 200 mg/day), mood stabilizers (sodium valproate 1200 mg/day), and benzodiazepines (flunitrazepam 2 mg/day and brotizolam 0.5 mg/day). While her psychiatric condition did not improve, side effects such as extrapyramidal symptoms and persistent hypoglycemia were observed. Blood tests for insulinoma were requested, with the following results: fasting plasma glucose (FPG) = 51 mg/dL and immunoreactive insulin (IRI) = 12.7 弮U/mL. The results of clinical diagnostic tests for insulinoma were as follows: Fajans index (IRI/PG; PG: plasma glucose) = 0.24 (insulinoma > 0.3), Grunt index (PG/IRI) = 4.01 (insulinoma < 2.5), and Turner index (IRI ? 100/PG-30) = 60.4 (insulinoma > 50). Her blood test results did not meet the criteria for insulinoma under the Fajans and Grunt indexes. Chest and abdominal computerized tomography (CT) did not reveal tumor lesions. Therefore, we suspected drug-induced hypoglycemia instead of insulinoma and promptly discontinued the benzodiazepines. However, we resumed oral administration of benzodiazepines when the symptoms of hypoglycemia did not resolve. We then reduced the dose of paliperidone, after which the symptoms of hypoglycemia gradually improved (). Although some of the patient's other medications could affect blood glucose levels (e.g., bromperidol and levomepromazine), they were not changed. Diabetes management therapy, including blood glucose monitoring and diet therapy, was initiated after discontinuation of paliperidone because we detected glycophilia. Since the hyperglycemia persisted, we performed an oral glucose tolerance test (OGTT) to evaluate for abnormal glucose tolerance, and the following results were obtained: fasting: 61 mg/dL; 30 min: 204 mg/dL; 60 min: 172 mg/dL; 90 min: 64 mg/dL; and 120 min: 77 mg/dL. She was diagnosed with reactive hypoglycemia in the early stage of diabetes.
Ivanna McMahon	36	1993/8/17	+1-980-255-0211x8441	[email protected]	676 Smith Way	A 50-year-old Asian woman known for end stage kidney disease secondary to IgA nephropathy received a kidney transplant from a 50-year-old deceased donor. The pretransplant crossmatch (CDC) was negative despite a PRA value of 25% and a complete HLA mismatch (in B and DR). The cold ischemia time was 7 hours and the patient was induced with basiliximab and methylprednisolone. Subsequent maintenance immunosuppression consisted of tacrolimus, mycophenolate, and prednisone. After transplant, the recipient had delayed graft function requiring three dialysis sessions. Ultimately, the clinical course was favorable and she was discharged with a good renal function (creatinine value of 128 弮mol/L (1.48 mg/dL)). One week after discharge, the patient was readmitted for graft dysfunction with a rise in creatinine to 194 弮mol/L. An urgent biopsy showed moderate glomerulitis and the presence of inflammatory cells in peritubular capillaries, but C4d was negative. As C4d-negative antibody-mediated rejection (AMR) was not a recognized entity in 2013; she was only treated with intravenous pulses of methylprednisolone and the creatinine stabilized at 160 弮mol/L. A few weeks later, an analysis revealed the presence of a donor specific antibody (DSA) (DQA0302) with MFI of 1800. This DSA was present before the transplant (MFI 1600) but we were not aware of its presence at the time of transplant. The DSA titers decreased mildly after initial treatment (MFI 900). At 3 months after transplantation, despite a CMV infection treated with ganciclovir, renal function remained stable. However, after 5 months, the recipient presented again with severe acute graft dysfunction (creatinine up to 400 弮mol/L) but, this time, there were signs of thrombotic microangiopathy (TMA): thrombocytopenia, decreased haptoglobin, increased LDH, decreased fibrinogen, and hemolytic anemia. A second biopsy was performed and it showed an acute active type 2 AMR with lesions of TMA, peritubular capillaritis, and glomerulitis (negative Cd4) associated with a grade IA cellular rejection (). Immunohistochemistry for CMV was negative. She was then treated aggressively with a combination of pulse steroid therapy (3 doses), thymoglobulin (2 doses), plasmapheresis (3 exchanges), intravenous immune globulin (4 doses), and rituximab (4 doses). Two weeks after the intensive treatment, despite a stabilization of renal function (creatinine of 200 弮mol/L), signs of AMR (TMA) still persisted in a repeat biopsy (3rd biopsy) and DSA remained elevated (MFI 1300). We decided to treat the recipient with 3 doses of eculizumab (1200 mg, 900 mg, and 900 mg) (). Following the therapy, blood markers of TMA significantly improved (). However, because of the heavy immunosuppressive state, the patient suffered from multiple infections: CMV reactivation, pulmonary aspergillosis, and Clostridium difficile colitis. DSA titers were not repeated after eculizumab treatment. The last graft biopsy (8 months after transplant) showed ongoing AMR and progression of chronic lesions (moderate tubular atrophy and interstitial fibrosis) (). However, transplant glomerulopathy and peritubular capillary basement membrane multilayering were not seen in the electron microscopy (EM). One year after transplant, renal function declined progressively (creatinine up to 250 弮mol/L) and haptoglobin gradually decreased, but platelets were normal and there was only mild anemia. All infectious complications resolved.
Jakob Browning	40	1980/11/23	001-632-280-1561x3997	[email protected]	3284 Gregory Stream Suite 635	A 60-year-old man was admitted to our hospital with the primary symptom of confusion. He lived in the dormitory of a factory that had a public bathhouse, in which bath water was circulated through a sterilizer system and reused. His medical history was unremarkable. He did not use any drugs but had a 60 pack-year history of smoking. Four days before admission, he had developed cough. On the day of admission (Day 1), his colleague noticed that he could not stand by himself and had slurred speech; thus, he brought him to the emergency department of our hospital. On admission, he was restless and could not obey our orders. His temperature was 40.1簞C, blood pressure was 154/82 mmHg, and pulse rate was 102 beats/minute. His oxygen saturation was 90% with oxygen supplementation at 4 L/minute using a facial mask. Pulmonary auscultation revealed coarse crackles in his right lung. Laboratory tests revealed a normal white blood cell (WBC) count but detected neutrophilia (WBC count, 8.36 ? 103/弮L; neutrophils, 96.5%), hyponatremia (Na, 128 mEq/L), a markedly increased creatine kinase (CK) (3254 IU/L), and increased fibrinogen/fibrin degradation products (FDP) (31.2 弮g/mL). Chest CT revealed extensive patchy shadows mainly in the right upper lung lobe. Based on these findings, an intravenous broad-spectrum antibiotic (tazobactam/piperacillin, 18 g/day) was initiated. On Day 2, a Legionella urinary antigen test was positive for Legionella pneumophila serogroup 1, suggesting a Legionnaire pneumophila infection. The patient's clinical presentation and rapid progression of respiratory failure supported this diagnosis. Thus, intravenous levofloxacin (500 mg/day) and azithromycin (500 mg/day) were initiated. On Day 3, the patient required a respirator, and low-dose hydrocortisone and sivelestat sodium hydrate, a selective neutrophil elastase inhibitor, were initiated. The patient's respiratory condition gradually improved, and he was extubated on Day 17. However, even after the cessation of sedatives, the patient demonstrated a variety of neurologic symptoms. He was awake but was disoriented, was dysthymic, and could not speak. He was able to lift his upper limbs but exhibited muscle weakness in his right upper and lower limbs. He could not move his limbs smoothly and showed a lack of coordination in the finger-to-nose test. Subsequent MRI of the brain revealed no abnormalities (). Cerebrospinal fluid (CSF) analysis revealed that the CSF cell counts, protein and glucose concentrations, and immunoglobulin G index were all normal. Gram staining and bacterial culture of the CSF were also negative. One week after the initial examination, the patient exhibited improvements in limb movement, but his disorientation remained. Electroencephalography showed diffuse slow activity. SPECT of the brain using a technetium-99m-ethyl cysteinate dimer (99mTc-ECD) was performed on Day 23 and showed multifocal hypoperfusion mainly in the frontal and temporal lobes (). Thereafter, his consciousness gradually became clearer without any treatment, and he was able to speak simple words. Six weeks after the initial examination, a second SPECT scan was obtained and revealed that the hypoperfusion in the frontal and temporal lobes had improved to almost normal levels (). At that time, laboratory tests revealed that most values, including FDP, had returned to normal limits. He recovered the strength in all 4 extremities by Day 60, and the patient was able to walk and speak falteringly when he was discharged on Day 68.
Princess Bass	27	1980/3/8	(952)901-7052x191	[email protected]	76990 Harris Wells Apt. 278	A 59-year-old man was admitted to our hospital for difficulty in breathing. He had a medical history of subdural hematoma caused by an accident but had been well without any neurologic abnormalities. He lived in the same dormitory and used the same bathroom as the patient in Case 1. He did not drink alcohol regularly but had a 20 pack-year history of smoking. He had developed a cough 2 days before admission and had experienced a headache and tremors the following day. On admission (Day 1), his body temperature was 36.9簞C, and chest auscultation revealed normal respiratory sounds. He was confused, and neurologic examination revealed disorientation and bilateral intention tremor. Laboratory tests revealed an increased WBC count (20.19 ? 103/弮L), increased C-reactive protein (21.16 mg/dL), mild hyponatremia (Na, 132 mEq/L), mild liver dysfunction (aspartate aminotransferase, 59 IU/L; alanine aminotransferase, 54 IU/L), mildly increased CK (240 IU/L), and an increased D-dimer (5.12 弮g/mL). Chest radiography and CT showed left lower lobe consolidation. A Legionella urinary antigen test was positive for Legionella pneumophila serogroup 1. Thus, the patient was diagnosed as having Legionnaires' disease, and intravenous levofloxacin (500 mg/day) and azithromycin (500 mg/day) were initiated. On Day 10, the patient's chest radiography showed improvement, and he was afebrile. However, his consciousness was still impaired, and his Mini-Mental State Examination (MMSE) score was 19/30. Brain CT showed a left subdural hematoma with a mild mass effect and a pituitary tumor, but acute lesions were not observed. Thus, we suspected that his neurologic symptoms were caused by the Legionella infection. On Day 13, SPECT of the brain using 99mTc-ECD revealed multifocal hypoperfusion mainly in the parietal, precuneus, and posterior cingulate cortices and in the right cerebellum (). His symptoms gradually improved without any treatment, and his MMSE score increased to 23/30. The patient had mild disorientation and slurred speech when he was discharged on Day 24.
Landen Spence	22	1992/6/22	-4024	[email protected]	151 Tyler Valley Suite 146	A 40-year-old woman in the 27th week of her fourth pregnancy was referred to our rheumatology clinic because of a three-week history of a skin rash, arthralgia, and weak positive anti-nuclear antibody test by immunofluorescence assay (ANA-IFA). Her previous three pregnancies were uneventful. She did not have a history of recent infections nor a potential exposure to toxic or medical agents. Physical examination on admission revealed bilateral periorbital edema and erythema on her eyelids (heliotrope rash) as well as diffuse rash on the face (). She had bilateral erythematous macules on the extensor surfaces of the metacarpophalangeal and proximal interphalangeal joints (Gottron's sign). There was also symmetrical and proximal muscle weakness in the upper and lower extremities. Other physical examination findings were unremarkable. Laboratory findings were as follows: serum creatine kinase (CK): 2138 U/L (normal: 29??00), lactate dehydrogenase: 520 U/L (normal: 140??80), aspartate aminotransferase: 113 U/L (normal: <35), and alanine aminotransferase: 58 U/L (normal: <35). ANA-IFA was weakly positive at 1/100 titration (homogenous pattern). Anti-Ro52 was positive, but antibodies to Jo-1 or other extractable nuclear antigens were negative. Serum anti-double stranded DNA, rheumatoid factor, and complement levels were within normal ranges. Serum creatinine level and urine analysis were normal. Erythrocyte sedimentation rate was 31 mm/h and serum C-reactive protein level was 17.9 mg/L (normal 0??). Electromyography of the right deltoid muscle revealed fibrillations and small polyphasic motor unit action potentials. Magnetic resonance imaging showed diffuse edema in thigh muscles (). She declined muscle biopsy. Investigations for occult malignancies including breast ultrasound, pelvic and abdominal ultrasound, peripheral blood smear, and fecal occult blood test were all negative. Serum CA-125 level was 6.5 U/mL (normal 0??5). She was diagnosed with dermatomyositis according to Bohan and Peter's criteria [, ] and started on methylprednisolone 32 mg/day orally. Five weeks later, however, no improvement was noted in muscle strength, skin rash, or serum CK level. Her blood pressure was 180/120 mmHg and she had developed bilateral pretibial edema. She was diagnosed as having preeclampsia and her pregnancy was terminated by an emergency caesarean section at the 32nd week of the gestation. She gave birth to a 1800 g male infant with no apparent congenital malformation. In postpartum period, we searched the mother for the presence of anti-phospholipid antibodies. Anti-cardiolipin and anti-帣2 glycoprotein-1 antibodies (immunoglobulin G and M isotypes) were found to be negative. Lupus anticoagulant test could not be performed due to technical limitations. After delivery, her muscle strength and rash improved rapidly. The dosage of methylprednisolone was tapered off. At postpartum 6th week, she was on methylprednisolone 8 mg/day and serum CK levels were within normal limits. There was only mild facial rash left, but heliotrope rash and Gottron's sign had completely resolved.
Aislinn Malone	37	1985/6/30	629.836.9150x6140	[email protected]	9537 Ashley Station	The patient is a 24-year-old male with a history of Down syndrome who was brought to our hospital from Yemen by his brother after 10 days of fever (39.5簞C), productive cough, shortness of breath, and right side chest pain. He developed the symptoms after few days of being found eating a sandwich that he hid in an organic fertilized soil of his house backyard. He was brought to our hospital in Jordan after he failed a course of moxifloxacin for assumed diagnosis of community acquired pneumonia. On presentation he was still complaining of same symptoms, and he denied any jaundice, abdominal pain, nausea, vomiting, or diarrhea. On physical examination he was alert, oriented, sweaty, and in mild respiratory distress. His vital signs were as follows: temperature 38.3簞C; pulse 110 bpm; respiratory rate 26 bpm; blood pressure 90/45 mmHg; and O2% of 82% on room air. He had cracked lips, protruded tongue, erythematous, and dry mucous membranes of the oropharynx. Chest exam revealed significant decreased air entry and tactile vocal fremitus with crackles and minimal wheezes on the right side and normal heart sounds with no murmurs or rubs or gallops. His abdomen was soft and nontender with no evidence of organomegaly. He was immediately resuscitated with IVF bolus and oxygen supplementation; his chest X-ray revealed right sided obliteration of costophrenic angle and displaced right lung (). CT scan showed right sided pleural effusion with pocket mainly in lateral aspect and in the oblique fissure, multiple gas bubbles with air fluid levels, and partial atelectasis of right middle and lower lobes that are medially displaced (). Abdomen ultrasound and CT scan showed no evidence of obvious gross liver pathology. He was diagnosed with right sided pneumonia complicated with multicystic empyema, and he underwent thoracotomy with drainage, decortication, and chest tube placement. Light microscopic examination of both pleural fluid and bronchoalveolar lavage sample revealed Entamoeba cysts and trophozoites. Microbiology revealed negative acid-fast stain or fungal infection. Aerobic and anaerobic blood culture showed no growth. Colonoscopy was done and biopsy revealed mixed inflammatory infiltrate suggestive of infection with no demonstrable amoeba. And stool analysis ?3 was also negative for Entamoeba cyst or trophozoites. Serological test was not done due to unavailability. The patient was treated with metronidazole 750 mg three times daily; luminal agent was not given due to unavailability in Jordan. His clinical condition significantly improved after 48 hours of antibiotic treatment. On one-month follow-up visit he was free of symptoms, and he returned back to Yemen.
Ruben Berry	22	2005/5/17	844.409.7849x9284	[email protected]	15448 Heather Camp	A 4-year-old girl was referred to our center with presentation of abdominal pain. Diagnostic sonography revealed an intussusception in this patient. Surgery was performed and a large mass was seen in ileocecal area. After the mass biopsy, Burkitt's lymphoma stage IIb was diagnosed since other organs were not involved. Chemotherapy was performed in accordance with the intermediate risk group protocol named LMB89. The first course started with one dose of COP (CPM 300 mg/m2 vincristine 1 mg/m2 and 60 mg/m2 prednisolone; prednisolone was continued for 7 days). One week later, the second course was administered with COPADM1 (CPM IV 250 mg/m2/dose, MTX 3000 mg/m2, doxorubicin 60 mg/m2, Vincristine 2 mg/m2, and 60 mg/m2 Prednisolone; prednisolone was continued for 5 days). Three days after the second chemotherapy course, the patient experienced febrile neutropenia. In spite of appropriate antibiotic therapy ceftazidime 800 mg IV, vancomycin 250 mg IV, and fluconazole 50 mg were administered with supportive therapy. The patient deteriorated and suffered from massive lower GI bleeding. Her blood test is described in . Since our patient suffered from Burkitt's lymphoma, she did not experience b symptoms. The patient received several units of pack cell, 0.25 unit/kg platelets, 15 cc/kg fresh frozen plasma, and some doses of cryoprecipitate for more than 3 days which is explained in . Different abdominopelvic sonographies, which were complemented with CT scan, showed pancolitis. Treatment was administrated with octreotide at a dose of 1 弮g/kg/h. It is worth mentioning that octreotide administration in this situation counts as an off-label indication but, because of its perfect efficacy at esophageal bleeding, we used it for second-line therapy, but no clinical response was observed. After 72 hours of refractory and sever bleeding, the patient was switched to 90 弮g/kg injection of AryoSeven. Bleeding was successfully controlled 1 hour after administration of a single dose of AryoSeven. Vital signs were stabilized. The lowest haemoglobin concentration at the time of blood haemostasis was 6 g/dL and no more decrease in hemoglobin concentration was observed. The patient received 10 cc/kg pack cell (PC) two times as a result of the latest severe bleeding and low hemoglobin. Patient's PT, PTT, and INR are declared in . In order to identify the source of bleeding, the patient underwent endoscopy and colonoscopy after her general condition was stabilized. No specific finding, except for a severe mucosal fragility, was found. Her last lab data are presented in , 12 hours after administration of AryoSeven. Patient's chemotherapy was continued, her primary disease was at remission, and there was no sign of GI bleeding ever since. For other chemotherapy cycles, GCSF administration was performed to prevent febrile neutropenia. The levels of fibrinogen were not evaluated during the study because the required equipment was not available.
Annabelle Jenkins	23	1990/12/8	(723)834-5293	[email protected]	4727 Boyle Ranch Apt. 274	Patient 1 was a 10-year-old girl admitted with 2 days of fever, retroorbital headache, vomiting, and myalgia. Her white blood cell (WBC) count on the 3rd day was 2.2 ? 103 with 65% neutrophils and platelet count of 117,000. Alanine transaminase (ALT) was 17.8 and aspartate transaminase (AST) was 55.2. Treatment was commenced as dengue fever in febrile phase. By the 4th day, she became sleepy and lethargic and the level of consciousness gradually deteriorated. She was moved to high dependency care for further management. On neurological examination, she had increased tone in all four limbs with cogwheel rigidity. The lowest Glasgow coma scale documented was 10/15. Her vital parameters and serial packed cell volume readings remained stable without signs of fluid leakage. Lowest WBC count (1.9 ? 103) was reported on day 5 of the illness and lowest platelet count was 50,000/mm3 on day 6. Liver enzymes deteriorated (ALT: 357, AST: 478) and serum albumin was 38 g/dL and INR was 1.17 on day 6. Tender liver was palpable 2 cm below the costal margin. Renal functions (sodium: 142 mEq/L, potassium: 4.2 mEq/L, blood urea: 7.6 mmol/L, and serum creatinine: 52 弮mol/L) remained normal. Serum calcium remained between 2.08 and 2.69 mmol/L. Primary dengue infection was suggested by the presence of IgM antibodies and absence of IgG antibodies to dengue virus. Dengue NS-1 antigen and the viral load were not performed due to lack of laboratory support. A CT scan revealed hypodense areas in bilateral thalamic and basal ganglia regions (). Cerebrospinal fluid examination did not reveal any cells and had normal protein levels (42 mg/dL). Over the next 2 weeks following the acute illness, she showed complete recovery with no residual neurological disability.
Declan Salinas	39	1999/6/15	8865838571	[email protected]	353 Jacqueline Manors Suite 933	Patient 2 was a 3-year-old girl who presented with 3 days of continuous high fever, intermittent abdominal pain, vomiting, and retroorbital headache. There had been one episode of gum bleeding on the first day of illness. She had been apparently a well child without any significant illnesses in the past. WBC count done on day 2, on request of the general practitioner, was 8.15 ? 103 with 85% neutrophils and platelet count was 120,000 mm3. She had right hypochondrial tenderness with just palpable liver on day 5. ALT and AST were 16 mEq/L and 69.7 mEq/L, respectively, on day 3 and they increased to 29.7 mEq/L and 122.2 mEq/L on day 5. Initial C-reactive protein was 6 mg/dL and remained normal. She was monitored for vital signs including urine output and packed cell volume (PCV). Baseline PCV was 32 and the highest noted was 35. The lowest platelet count documented was 67,000/mm3 and the lowest WBC count was 2.27 ? 103. She did not show signs of fluid leakage. However, there was a second episode of gum bleeding on day 5 with normal clotting profile (INR: 1.1 and APTT: 35). She was positive for IgM and negative for IgG antibodies for dengue virus. On the 5th day of illness, she developed tremors in her upper limbs. She had cogwheel rigidity, mask like face, and staccato speech. Power was grade four in all four limbs with normal reflexes. CT scan of brain revealed diffuse hypodense areas in bilateral basal ganglia and thalamus with predominant involvement of right side (). During the first 48 hours after the onset of neurological symptoms, she showed rapid deterioration and became aphasic with severe paucity of movements. Cerebrospinal fluid examination showed 2 lymphocytes without any polymorphs and normal proteins. She recovered dramatically and showed complete recovery within 3 weeks after onset of illness.
Royalty Schultz	19	1986/8/21	226-921-8447	[email protected]	68410 James Neck	A 34-year-old Nigerian man presented to the Mental Health/Psychiatry Clinic following a referral from the Surgical Outpatient Department on account of persistent testicular pain of a psychogenic nature and an insistence on a bilateral orchidectomy. History shows bilateral testicular pain of six-year duration, initially affecting the left testes alone, with the pain radiating to the perineum and pelvis, associated with pins and needles sensation and said to have affected his sleep and sex life. Although there is a positive history of multiple heterosexual partners, barrier contraception in the form of condoms was practiced. There was no history of fever and other constitutional features, undescended testes, childhood mumps, steroid Prune belly syndrome, perineal trauma, scrotal sores/or itching, exanthematous and pigmentary changes to the scrotum, change in testicular consistency, penile discharge, and pain on micturition and masturbation. Testicular pain is, however, associated with inability to perform the sexual act as well as masturbation. Due to the persistence of symptoms, he proceeded to General Hospital Ahoada, Rivers State, Nigeria, where a battery of unknown tests were carried out (involving drawing blood and several X-rays and ultrasound scans), results did not detect any abnormalities, drugs were prescribed, and he cannot recall their names; however, the pain persisted. Subsequently, he proceeded to a tertiary center (BMH, PH), where further tests were done and drugs were administered on an outpatient basis; however, the pain was persistent. He then proceeded to Navy Hospital, Borokiri, Port Harcourt, Nigeria, where an abdominal X-ray as well as abdominal and testicular ultrasound scans was done, as well as a thorough physical examination of the external genitalia; he was told that nothing was wrong with him and drugs were administered, but the symptoms were persistent. Similar findings were reported when he went to a tertiary health facility, UPTH. He also went to unorthodox, tradomedical practitioners in a quest for a permanent solution; several unknown concoctions were administered to him as well as incantations, all to no avail. He came to this facility in 2010 (after relocating to Bayelsa State, Nigeria); the battery of tests aforementioned earlier were made, including semen analysis (obtained via masturbation, a fact, he had earlier denied but is present in his medical record), and he began complaining a feeling that the right testis was ascending up into his stomach; a surgery (nature of surgery unknown to him) was performed in March, 2014; on recovery and discharge, pain persisted, with no worsening or relief; he claims that dribbling of urine began after the surgery. There were associated feelings of worthlessness following the pain symptoms, loss of ?anliness/sexual ability,??suicidal ideation, and suicidal attempts of 6-year duration, which has increased in intensity, frequency, and duration and progressively worsened after visits to doctors/several hospitals did not provide relief. Feeling of worthlessness was associated with low energy, loss of interest in pleasurable activities, cessation of self-employed work, loss of income, decreased willpower to live, loss of self-esteem, and feeling of extreme helplessness; however, patient claims he has a zest for life. Suicidal ideation is of 5-year duration; following failed attempts at obtaining a cure after consulting unorthodox medical practitioner, he began thinking about taking poisons to end his life; however, he did not tell anyone about his thoughts; there were no associated delusions, hallucinations, or other disturbances perceptions. Suicidal attempts began 4 years earlier; he ingested ?oisonous??plants, battery acid, and ?aygon??insecticide. There is no history of attempts to end his life by violent means, self-mutilation, or ingestion of sleeping pills. Suicidal attempts were not revealed to anyone; however, the last ?ttempt??to end his life occurred 3 weeks prior to psychiatric consultation, with his mother privately reporting that he came to her privately, weeping bitterly and showed her some ropes he had in his possession for the purpose of killing himself, but he never carried through with the plan; he summarily denied this but later admitted it as a fact. Feeling of loss of ?anliness??was of 5-year duration and began after he noticed that he could not have sex anymore due to the intense pain. This was associated with an increased desire to have his testicles removed, in order to be relieved of the pain. Although he considers himself as male, he insists he feels unmanly and incomplete; there was, however, a strong insistence that he is not a female on further probing during history taking. However, no history of increased activity, agitation, restlessness, aimless wandering, increased libido, irrational talk, loss of appetite and sleep disturbances as well as hearing voices, social withdrawal, paucity of self-care, violent behavior, gathering of debris, or any abnormal behavior. Past psychiatric history revealed that there was a positive history of psychoactive drug use, specifically, Cannabis, which he used via smoking of dried leaves wrapped in a strip of paper; he began using it at age 16/17 (17 years ago); he was introduced to it by friends and a desire to experiment and rebel; he used drugs independently and with friends; habit was sustained by his allowance and stealing from his parents; drug use continued for a few years and ceased in his early twenties. In his mid-twenties, in 2006 (9 years ago), he became socially withdrawn, left the house for no apparent reason, and began living alone; then he disappeared for 3-4 months (he later admitted he travelled to Niger in a failed bid to emigrate to Europe) and returned home voluntarily, only to begin living in uncompleted buildings and talking irrationally, with history of provoked violent attack on a shopkeeper after being stigmatized as ? mental case.??Subsequently, in 2008, he was admitted to the Neuropsychiatric Hospital, Rumuigbo, and a diagnosis of ?ubstance Use Disorder??was made; several investigations were done and drugs administered; he was discharged after a month and a fortnight. Follow-up was regular for two months; however, he defaulted because he claimed he was not ?ental??and because of the side effects of drugs. There is no history or evidence of a relapse following discharge. In the past medical history, there was no history of hypertension, epilepsy, asthma, diabetes mellitus, sickle cell disease, neurocutaneous disorders, and erectile dysfunction. Family history showed that he is the only male as well as the first of four children in a monogamous setting. However, his mother had 2 children in a later marriage after the divorce of his parents, making a total of 6 children, 3 full sisters and 2 half-sisters. His mother is a middle aged matron with tertiary level of education. His father is deceased, had tertiary level of education, and was a senior civil servant with the Ministry of Agriculture. He was not close to his father and appeared to have had a troubled relationship with him following the divorce of his parents; his father died three years earlier, at age 61, from an unknown medical condition. The personal history revealed that the patient completed his basic education. However, he did not pass the UME with good scores and failed to obtain a university admission, and he was said to be an average student. He enrolled in a part time program, in the National Open University, 2 years earlier, but did not complete the course because of his present illness. Subsequently, he said he has received training as an electrician and construction worker. He claims to be a single father of an 18-year-old boy (with elaborate information about the nonexistent child); however, his mother denies this and he later admitted he told us this so that we would remove his testes since he has a child. Social history showed that, in the past, he occasionally drank alcoholic beverages but has seldom done so for the past seven years. He smoked tobacco in cigarette form occasionally. Patient seldom interacts with people except his family members whom he lives with; he initially said he lives alone but his mother said he lives with her and he admitted this. Psychosexual history as elicited showed that adrenarche was attained at age 14 while onset of nocturnal emissions began at age 17. First sexual activity was with a female partner, experience was pleasurable and exciting, and subsequent experiences have been pleasurable and in almost all cases barrier contraception in the form of male condoms was used. The patient insisted that his sexual orientation and love interest has been strictly heterosexual. He has seldom had any early morning erections over the last 6 years and cannot recall having experienced any nocturnal emissions within the same period. He denies any masturbation as well as orgasm/ejaculation; however, semen for analysis was obtained via masturbation without the use of pornography. In the forensic assessment history, patient has not been convicted for any crime, sentenced to jail, or jumped bail; however, he has been arrested thrice and detained for minor offences such as moving at night during a curfew. Premorbidly, leisure activities included watching football matches; he formed deep ties in relationships, had few friends, and was introverted/quiet. His prevailing mood was happy. Appearance and behavior showed a well dressed and well groomed young man (with attention paid to minute details such as manicured nails and perfectly styled hair) appearing appropriate for age, maintaining good eye contact, and holding an umbrella with depressed posturing. The tone of speech was low to moderate tone, reactive to circumstances, coherent, and relevant. The mood was sad and affect was depressed. Perceptual abnormalities present included somatic hallucination. There were no disorders of the thought process or formal thought disorder detected. Under cognition, patient was fully oriented to time, place, person, and circumstance. Attention, concentration, comprehension as well as calculator ability, and abstraction were within normal range. In addition, immediate recall, long and short memory are within normal range. Judgement was good and insight was partial (he did not believe the drugs will be of any benefit and his case is hopeless). Primary diagnosis was hypochondriasis with comorbid secondary diagnosis of severe depression with psychotic features. Patient was managed using the biopsychosocial model. Drug treatment was done using tricyclic antidepressant (Amitriptyline) and antipsychotic medication (Olanzapine). Psychotherapy carried out involved cognitive behavioral therapy. Finally a letter was sent to SOPD about diagnosis and unsuitability for bilateral orchidectomy as requested. Initial improvement (evidenced by reduction in pain intensity) was seen for the first 3 weeks; however, pain has been persistent. There was insistence on a bilateral orchidectomy during his last visit.
Cody Bryant	36	1989/9/19	943.483.2141x38393	[email protected]	0201 Tammy Spring	A 20-year-old Caucasian male presented to the cardiac clinic for evaluation of chest pain and elevated blood pressure. His chest pains were sharp, occurred randomly, and were mostly nonexertional. He also had an abnormal EKG demonstrating nonspecific ST/T wave changes (see ). His past medical history was unremarkable. He is a smoker and drinks alcohol socially and denied illicit drug usage. An echocardiogram performed in the office was normal, with the coronary arteries arising from the appropriate cups. A cardiac computed tomography angiography (CCTA) was performed revealing anomalous origin of the right artery from the left coronary cusp. The proximal portion had an acute-angle take-off with an intramural course with a 70??0% stenosis. The vessel also coursed between the aorta and pulmonary arteries (see ).
Parker Payne	44	2005/6/30	001-239-958-9513x9029	[email protected]	28269 Anderson Lock	A 68-year-old female consulted the Department of Oral, Maxillofacial and Plastic Surgery with a painful swelling on the right infraorbital side of the face (). Intraorally, no signs were noted (), apart from scars in the region of the unattached gingiva of the maxilla (). The patient underwent a Caldwell-Luc operation at the age of 13 on both sinuses due to chronic maxillary sinusitis. She had a mastectomy for breast cancer following irradiation 31 years previously. MRI and CT found a cystic lesion of the right maxillary sinus with orbital floor elevation. In the left maxillary sinus, a cyst was detected as well (). Bony defects were visible in the nasal part of the right sinus and the orbital floor (). At operation, after incision of the gingiva, no bony wall of the right maxillary sinus was evident (). The cystic tissue was adherent to the buccal soft tissue and the infraorbital nerve. Cystectomy was performed with infraorbital nerve preservation and cystic tissue was removed intact (). A sinuscopy revealed minor damage of the orbital floor; the medial sinus wall was missing after Caldwell-Luc surgery. No further reconstruction was necessary. A pack was inserted in the inferior nasal meatus for 5 days. On the left side, cystic tissue was removed in the same way as described above and a pack in the inferior meatus was removed after 3 days. Clinical signs and histological report confirmed a postoperative bilateral maxillary cyst (Figures and ). No diplopic images were stated but the patient complained about hypoesthesia of the right infraorbital nerve. After half a year, CT control showed no recurrence. Sensory disturbances disappeared during the follow-up examinations within one year after removal of the cysts.
Edward Duncan	36	1990/6/23	(826)420-6118x032	[email protected]	4437 Cunningham Island	A 7-year-old girl complained of pain in her left thigh, and she was limping. She had no history of trauma, disease, or surgery. She had left thigh pain for 3 weeks before visiting the outpatient clinic. The ranges of left hip motion were as follows: flexion, 130簞; abduction, 30簞; extension, 10簞; internal rotation, 45簞; and external rotation, 10簞. The circumferences of the right and left thighs were 29.5 cm and 28.5 cm, respectively. Plain radiographs obtained at the initial visit showed no abnormalities (). T2-weighted magnetic resonance imaging (MRI) scan of the hip joints showed no abnormal findings, except left hip joint effusion (). TS was diagnosed on the basis of the patient's medical history and imaging findings, and we continued follow-up. She was on bedrest for 4 weeks, and we instructed her to avoid weight bearing during this time. However, her left thigh pain and limping did not improve. The ultrasonic joint spaces (distance from the anterior cortex of the femoral neck to the front of the capsule [the width of the joint space]) measured 6.0 mm on the right side and 8.7 mm on the left side at 4 weeks after the initial visit. Joint effusion was diagnosed on the basis of anechoic findings of the left hip joint space. T1-weighted and T2-weighted MRI scans of the hip joints showed a linear low-signal-intensity region at the left femoral capital epiphysis 2 months after the initial visit (). Although no abnormalities were found, follow-up continued. The ultrasonic joint space measured 11.6 mm on the left side 2 months after the initial visit (). Synovitis was diagnosed on the basis of echoic findings of the left hip joint space. A culture of the joint aspirate showed negative results. The laboratory data showed no abnormalities. The ultrasonic joint space measured 12.3 mm on the left side 3 months after the initial visit. Hence, we determined that synovitis progressed. Plain radiograph showed bone resorption at the left femoral capital epiphysis 3 months after the initial visit (). Bone scintigraphy findings showed a cold-in-hot pattern at the left femoral capital epiphysis (). We diagnosed the patient as having LCPD on the basis of these imaging findings. We instructed her to wear a hip abduction and non-weight-bearing brace. We categorized the disease as Catterall group II and Herring group B according to the findings from plain radiographs 8 months after the initial visit (). Plain radiographs showed a continuous subchondral bone 15 months after the initial visit. We removed her brace and continued follow-up. Plain radiographs showed good remodeling of the necrotic area at the left femoral capital epiphysis 7 years after the initial visit. She did not have a lower limb discrepancy, left thigh pain, or limping ().
Elise Hayes	41	2002/10/9	(652)671-3598x397	[email protected]	660 Karen Key Suite 434	A 7-week-old boy presented with a cystic swelling of the left tongue tip and inferior side of the tongue. The cyst was discovered on ultrasound at 28 weeks of gestation. An immediate postnatal MRI revealed a well-defined midline structure in the anterior half of the tongue of 3.6 cm (Figure ). Because the cyst was asymptomatic, surgery was delayed until the age of 1 year.
Legend Hutchinson	41	1982/9/27	466.672.2560x075	[email protected]	792 Butler Drive Suite 492	A 2-year-old boy was referred to our department for macroglossia. Clinical examination showed a cystic midline protrusion of the tongue tip and corpus and a tongue ankylosis. MRI confirmed a well-demarcated cyst of 4.6 cm (Figure ). At the age of two, two connected cysts were resected. A lingual frenulotomy was performed.
Jamie Houston	38	2001/8/18	668.852.0681x498	[email protected]	7782 Hall Branch Apt. 319	A 14-month-old girl was referred to us with an infection of the floor of the mouth following attempted needle aspiration of a suspected ranula 1 week earlier, not ameliorating under Amoxicillin?lavulanic acid. The swelling had been noticed since the age of 2 months. The infection cooled down after partial needle aspiration of the liquid content, relieving pressure and facilitating penetration of antibiotic treatment with Piperacillin/tazobactam (Tazocin簧) and metronidazole (Flagyl簧). After remaining asymptomatic for 7 months, the swelling recurred. A prophylactic antibiotic treatment with Amoxicillin was started and the operation date was accelerated.
Sylas Lucas	22	1990/12/6	336.988.8215	[email protected]	53591 Megan Vista Apt. 052	A 29-year-old man consulted us with a slowly progressive swelling, medially in the floor of the mouth, since 10 years. Clinical examination showed a soft well-defined cyst medially in the flour of the mouth and descending submentally. MRI showed a 7-cm bilocular midline cystic lesion in the floor of the mouth.
Phoenix Haynes	30	1995/4/21	001-447-321-0180x2376	[email protected]	8113 Barber Mews	A 55-year-old female patient with serious bilateral maxillary molar eruption visited the hospital to secure space for mandibular molar prosthetics. For diagnosis, she received clinical and radiological examinations (Fig. ). She had serious bilateral maxillary molar eruption (bilateral vertical dimension on the first molar 0 mm), bilateral mandibular molar edentulous condition, lack of proper vertical dimension for prosthetic treatment, and multiple dental caries, and retained dental root were observed. PMSO was planned to restore decreased vertical dimension due to serious eruption of the bilateral maxillary molar, and we decided to shift the molars in a posterior-superior direction by approximately 7 mm from the maxillary and mandibular right first premolar. Incision was conducted in accordance with local analgesia under general anesthesia, and a buccal flap was formed. Horizontal osteotomy was performed from the bilateral first premolar to the first permanent molar, while vertical osteotomy was conducted on the mesial surface of the bilateral first premolar. After palatal osteotomy, the maxillary posterior segment was separated, and excessive bone fragments were removed. The segmented bone fragments were shifted using the wafer, which was made before operation, and then they were fixed using the mini plate and screws. And the operation was finished following intra-oral suture and intermaxillary fixation (Fig. ). At 2 weeks, stitch-out and removal of the intermaxillary fixation were conducted, and at 4 weeks, the wafer placed on the maxillary teeth was shifted. Then, implant placement and prosthetic treatment were done (Fig. ). One, 2, and 6 months after the surgery, we confirmed stable occlusion and no complications such as necrosis of the osteotomy site.
Kason Reed	44	1980/5/16	691.707.1983x16842	[email protected]	104 Ronnie Mountain	A 43-year-old female patient visited the hospital for treatment of the erupted maxillary right molar and the molar width discrepancy and protruded upper lip. She was able to bite just in the left due to excessive eruption (downward canting, 3 mm) of the maxillary right molar and scissor bite in clinical and radiological examinations (Figs. and ). Over eruption and scissor bite of right maxillary molar teeth, maxillary and mandibular hypo-growth (SNA 75.68, SNB 71.41), maxillary arch length discrepancy (maxillary inter molar width 46 mm, mandibular intermolar width 40 mm) (Fig. ), acute nasolabial angle (90簞), and protruded upper lip (upper lip E-line 1.95) were found, and for surgical treatment, Le Fort I osteotomy and PMSO were performed. According to the analysis and surgical planning, total setback 5.5 mm, posterior impaction 3.0 mm, and medial shift of maxillary right molar 5.0 mm was planned. Le Fort I osteotomy was conducted in accordance with local analgesia under general anesthesia. After maxillary down fracture was performed, maxillary setback was secured using the intermediate wafer. And then, vertical osteotomy and palatal osteotomy were conducted between the maxillary right lateral incisor and maxillary right canine, and osteotomy of the maxillary right molar was finished (Fig. ). Medial shift of the segmental bone fragment was identified using the final wafer, and then it was fixed using the plate and screws, and intra-oral suture and intermaxillary fixation were carried out (SNA 67.66, SNB 66.41). At 2 weeks, stitch-out and intermaxillary fixation were removed, and at 4 weeks, the wafer was removed. She continued postoperative orthodontic treatment and she had stable occlusion (Figs. and ). Maxillary arch length discrepancy (Fig. ) was also effectively decreased (maxillary inter molar width 41 mm, mandibular inter molar width 40 mm), enabling the patient to chew with bilateral molars and satisfying the lateral profile of the upper lip (upper lip E-line ??.82).
Valentina Boyer	20	2003/12/26	+1-427-763-9590x701	[email protected]	263 Leslie Ford	A 54-year-old male with past medical history of end-stage renal disease on hemodialysis, hepatitis C, ischemic stroke, depression, and anxiety was brought to the emergency department with the complaints of fever (100.2簞F) and increased surgical site pain in left thigh. He underwent creation of a left superficial femoral artery to greater saphenous vein prosthetic AV graft 1 day prior to admission. Few hours after the procedure, he started having increased surgical site pain, erythema, and small vesicles at the margins of wound. On examination, the left thigh AV fistula site staples were intact. There was mild erythema, tenderness, and vesicles at margins. Serous discharge was noted from vesicles but no purulent drainage from wound area. Lab investigations showed creatinine level of 12.6 mg/dL and a potassium level of 6.4 mEq/L at the time of admission prompting hemodialysis. On admission, his vitals were normal and white blood cell (WBC) count was 10 000/cumm. The next day, his left thigh wound pain and swelling worsened concomitant with erythema, tenderness, and purulent discharge. Blood cultures were taken and drainage sample was sent for culture and sensitivity. His WBC count increased to 13 000 and erythrocyte sedimentation rate was 45; therefore, on suspicion of AV graft infection he was started on vancomycin 1.25 g intravenously given after each hemodialysis session. The vancomycin trough level was therapeutic, and blood cultures and drainage culture were negative. Despite the antibiotic therapy, his symptoms worsened with each passing day. Repeat blood and drainage cultures were consistently negative. Computed tomography scan of pelvis showed extensive edema and congestion of the subcutaneous fat extending from the left external oblique musculature inferiorly along the lateral aspect of the pelvis into the left upper extremity (). Because of worsening symptoms, he underwent exploration of left thigh AV graft and samples were collected from perigraft tissue and perigraft fluid for culture and sensitivity. Once again, all cultures came back negative. Over the next couple of days, he started having purulent drainage from multiple locations along the skin overlying the graft (). The decision was then taken to surgically remove the graft. The drainage sample from excised graft was also negative for bacteria. After graft removal, his symptoms markedly improved with complete resolution of symptoms in 7 days. In the meantime, he was given topical emollients for symptom relief only. Due to abrupt onset of symptoms within hours after AV graft placement, the diagnosis of AV graft contact dermatitis was made.
Zeke Moss	33	1988/5/5	001-592-930-6414	[email protected]	9408 Dougherty Club	A 78-year-old woman presented with a 2-week history of progressive lower limb weakness and bilateral numbness from her hips to her toes. Her primary care physician referred her for physical therapy, which did not improve her strength. Her symptoms continued to worsen until she became bed bound and presented to the hospital 2 months after the initial onset of symptoms. Her past medical history included stage IIIC ovarian adenocarcinoma 17 months prior to presentation. At that time, her Ca 125 level was 1612 units/mL. She received 6 cycles of neoadjuvant chemotherapy with carboplatin and Taxol, followed by exploratory laparotomy, bilateral salpingoopherectomy, splenectomy (due to concerning lesions in the spleen), and 3 cycles of chemotherapy. Two subsequent abdominal computed tomography (CT) scans showed no evidence of recurrence and Ca 125 levels had decreased to normal. On exam she had left lower extremity weakness; 2/5 in both hip and knee flexion and extension, 4/5 in dorsiflexion and plantar flexion. Right lower extremity strength was 0/5 in both hip and knee flexion and extension, and 3/5 in dorsiflexion and plantar flexion. Ca 125 levels were normal. Cerebrospinal fluid analysis indicated nonspecific inflammatory changes and no sign of infection. Spinal magnetic resonance imaging (MRI) showed an intramedullary lesion at T11-T12 (). Positron emission tomography CT revealed focally increased FDG avidity at T11-T12. A laminectomy and biopsy of the lesion were performed; pathology indicated metastatic ovarian cancer (). The patient received high-dose corticosteroid therapy for 1 week after her presentation. She was discharged to a rehabilitation facility and underwent outpatient radiation therapy with 30 Gy in 10 fractions of 300 cGy each, from T7 to L1. She tolerated the treatment well and gained slight improvement in sensation and weakness, but was unable to ambulate. She received a subsequent 6 cycles of chemotherapy with Taxol and carboplatin. On completion of this chemotherapy, her weakness improved with motor strength in the left lower extremity 4/5, and in the right lower extremity 3/5 in all muscle groups. MRI of the thoracic spine 4 weeks after the last cycle showed widespread bony metastases but no spinal cord compression. An MRI of the cervical spine showed no enhancing lesions. A CT of the abdomen and pelvis was without masses in the pelvis. Ultimately, patient gained improvement in her lower extremity strength, but she did not regain full function.
Bianca Valdez	23	1983/7/21	(264)635-0630x58262	[email protected]	2935 Williams Stravenue Suite 254	A 70-year-old male with past medical history of hypertension only presented to the emergency department with left hand weakness and numbness for past 7 hours. He was unable to extend his wrist and fingers and had loss of sensation and paresthesia on the dorsum of the left hand. He had experienced a similar episode 3 days ago, which resolved in a few hours; the patient had initially attributed these symptoms to old age and muscle spasms. Today his symptoms did not resolve in the past few hours, hence he decided to come to the emergency room for further investigation. He denied any dizziness, lightheadedness, loss of consciousness, slurring of speech, ambulatory dysfunction, or difficulty maintaining balance. He denied any weakness or loss of sensation in any other extremity. His home medications were amlodipine, benazepril, and vitamin D3. He did have a family history significant for stroke and diabetes mellitus. He denied smoking or alcohol or drug abuse. His vitals were temperature 36.9, heart rate 98, respiratory rate 20, blood pressure 140/65, oxygen saturation 98% on room, weight 68 kg, height 170 cm. On examination he was alert and oriented in time, place, and person. His gait and cranial nerves were intact. Motor examination demonstrated weakness of left wrist and finger extensor muscles with a muscle strength grading of ??. Strength in the left bicep, brachoradialis, tricep muscle, flexors of the fingers and wrist was normal. Sensory examination demonstrated mild decrease in sensation on the dorsal aspect of the left hand, predominantly in the anatomical snuff box. Strength and sensation in the other extremities were normal. Other systemic examinations were unremarkable.
Kyler Camacho	37	1984/11/8	472-921-1674	[email protected]	988 King Street Apt. 099	A 22-year-old female university student with no medical and psychiatric history spontaneously presented to the emergency unit of our university hospital, complaining of speech disorders, physical and mental fatigue. Four days earlier she had developed a stuttering episode with concentration difficulties. A side effect of a recently prescribed antihistaminic drug (cetirizine) was suspected, and the medication was stopped. She was discharged home. Two days later, she was readmitted to the hospital because of an increase in subtle chorei form movements (perioral, hands), as well as memory impairment. She also presented with decreased mobility, lack of right hand grip, paraesthesia of the left hemibody, dysarthria, and tinnitus. illustrates the intensity of neurological and psychiatric symptoms throughout the different phases of the disease. In the following days, her orofacial dyskinesia worsened and she developed a swallowing disorder, speech deterioration, vomiting, wide pupils and facial flush, right upper limb weakness associated with paranoid delusions, as well as auditory and olfactory hallucinations. Over the following 2 weeks she developed delirium and increased impairment of consciousness, requiring endotracheal intubation and ventilation in the ICU. A large differential diagnosis was considered, including most common causes of encephalitis: viral, autoimmune, paraneoplastic and toxic-metabolic. Electroencephalogram (EEG) performed shortly after inpatient admission (2 weeks after the initial symptoms) was suggestive of anti-NMDAR encephalitis, showing poorly reactive rhythmic delta activity over the frontal regions []. This was confirmed by a positive anti-NMDAR antibody test in cerebrospinal fluid and serum (Serum: 1/100, Cerebrospinal fluid: 1/2). A nodule in the right ovary observed by ultrasound and removed by laparoscopy did not show a tumoural lesion by histology (no teratoma). During the following months, the patient, while unconscious and mechanically ventilated, received specific immunological treatments (high-dose steroids, 15 cycles of plasma exchange, 8 cycles of cyclophosphamide, 4 cycles of rituximab, and 4 cycles of intravenous immunoglobulin), with high-dose midazolam (and at times propofol) sedation. Although we did not formally identify a clinical or electrographic seizure, she received non-sedating intravenous antiepileptic treatment (valproic acid and lacosamide), because the rhythmic delta activity by EEG represented an ictal correlate []. Her clinical course was characterized by hemodynamic, breathing, cardiac arrhythmias, infectious, metabolic, and nutritional dysfunctions. Throughout this long ?nresponsive phase??there was no contact with the patient. Sometimes she opened her eyes but remained mute and unresponsive to visual stimuli. Orofacial and arm dyskinesia were regularly present upon weaning of the sedation, and her EEG showed a rhythm delta over the frontal regions alternating with a disorganized, relatively fast activity. After several months of being in an unresponsive state in the ICU under continuous immunotherapy, the patient started to improve. Limited contact became possible: she opened her eyes and responded to simple orders. She was finally extubated eight months after her admission. After awakening, the patient presented with an acute confusional state but her neurological status improved slowly. Over the following weeks, she was slow and hypomimic with ataxic movements. However, delirium persisted with episodes where she was mute, a kinetic, and unresponsive to verbal commands. For four months the patient presented with severe psychopathological manifestations. Episodes of psychomotor agitation with overt aggressive behaviour were very challenging, including throwing objects around the room, slapping her hands on the bed, trying to pull out the feeding tube, sometimes requiring sedation (intravenous midazolam and propofol) and even physical restraint for short periods. Psychotic features were clearly recognizable upon speaking indicating paranoid delusions as well as auditory and olfactory hallucinations. She was also tremendously anxious or perplexed, and refused contact (e.g. actively kept her eyes closed) with the ICU staff or even with her family. Affective elements were also present, including depressive withdrawal, suicidal ideation culminating in attempted self-harm by knife or strangulation with a cable. She also presented with severe insomnia and nightmares. Interactions with her caregivers were marked by irritability and emotional manifestations, recurrent refusal of care and feeding, and even requests to be killed or that we let her die. This highly challenging situation for the ICU team required that an additional nurse be hired allowing ?ne to one??continuous supervision of the patient to prevent accidents. Besides midazolam and propofol she received quetiapine XR (orally through a nasogastric tube). Despite benzodiazepine and antipsychotic treatments, the patient remained impulsive and aggressive with psychotic symptoms for several weeks. An improvement occurred after 1 month when quetiapine XR was increased to 600 mg/day. In parallel, physiotherapy was started in the ICU to improve swallowing and reduce feeding difficulties. A specific team (not the C-L team) delivered these therapies. The neuropsychological rehabilitation therapies were used to target attention disorders, immediate and episodic anterograde memory, and language disorders such as dysarthria, lack of words and ideomotor apraxia. Four months after coma resolution (12 months after admission), we observed a significant improvement that was chronologically linked to a decrease in plasma anti-NMDAR antibodies. A further increase of quetiapine allowed a low tapering of benzodiazepines, at which time, there was a complete disappearance of psychomotor agitation. However, hallucinations as well as feelings of sadness, anger, and fluctuating suicidal ideation were still present. Five months after coma resolution, the patient was transferred to a psychiatric unit for management and treatment of the residual psychotic symptoms. Her autonomy increased and she better engaged in social interactions with therapists and other patients. She was collaborative in psychiatric consultations, physiotherapy and occupational therapy activities. She began to take better care of her body, applying make-up or asking for an appointment with the hairdresser. Contacts with family and friends were more frequent and a source of pleasure. She started to make plans for her future (wish to be discharged home and return to her university studies). After 2 months at the psychiatric unit, the psychotic and affective symptoms gradually resolved. At that point, cognitive deficits were still present, with attention disorders, deficits in immediate and episodic anterograde memory, language disorders (dysarthria, lack of words, paraphasia and agrammatism), and ideomotor apraxia. The patient was transferred to a neurorehabilitation unit (NRU) for about 6 weeks. She was trained by neuropsychological tasks and showed improved interference management, information retention in episodic and verbal memory, and mental calculations. Furthermore, she benefited from intensive ergotherapeutic and physiotherapeutic approaches. In particular, ergotherapy by NRU staff focused on cognitive, speech-language deficits, communication skills and social-motivational occupations. The clinical evolution was remarkably good, with an improvement in cognitive and executive functions and social organizational activities. Sixteen months after her first contact with our hospital, the patient was discharged home. She was followed up by liaison psychiatry consultation once a week. She took quetiapine (300 mg/day) treatment for 6 more months, which was then successfully discontinued. Nine months after discharge, the patient and her parents reported social, personal and family stability. The patient resumed university attendance and successfully passed her exams. She has little recollection of the 16 months of admission and treatment, particularly the ICU phase. At the last follow-up, 2 years after the event, she only complained about the tracheostomy scar.
Armani Brady	39	1978/6/8	(370)912-9439x61169	[email protected]	56851 Shelby Mews	The first patient is a 30-year-old female less than one month post-partum who presented with rapid onset obtundation. MRI/V revealed superior sagittal sinus thrombosis with venous infarctions in both frontal lobes. The patient was started on a heparin drip. Her exam declined while on heparin, and she required intubation. A CT scan demonstrated increased cerebral edema as well as progression of the previously identified thrombus into the right transverse sinus (Figure ). She was brought to angiography for possible intervention secondary to failed medical management. This confirmed that the occlusion had spread to the right transverse sinus. Both stent retriever (Solitaire FR, Medtronic) and aspiration (ACE, Penumbra, CA) were attempted without success. A 0.010-inch microcatheter (Eschelon 10, Medtronic) was placed in the anterior third of the superior sagittal sinus. The bolus dose of IV tPA was calculated to be 10 mg. This was diluted in a 500 ml bag of heparinized saline and set up for a ten-hour infusion at 100 ml per hour for a dose of 1 mg/hr in 50 ml/hr of fluid. We chose this dose after reviewing Rahman et al., and picking a dose on the low side of the spectrum provided. Unfortunately, the catheter became clogged after only three hours. She was taken back to angiography where persistent occlusion was demonstrated. To prevent further clogging of the catheter, the 0.010-inch microcatheter was swapped out for a larger 0.027-inch microcatheter (Marksman, Medtronic). The tPA dose was doubled to 20 mg and dissolved in a 1 liter bag of heparinized saline. The infusion was then 2 mg/hr in 100 ml/hr of fluid. This infused the entire ten hours without clogging. CTA revealed a recanalized sinus still with thrombus and no new hemorrhages (Figure ). Heparin was then restarted. On discharge one month later, she was neurologically intact.
Reed James	20	2000/5/11	846-348-8998x4720	[email protected]	484 Mueller Dam Apt. 883	The second patient is a 70-year-old female with a diagnosis of multiple myelomas who presented with a seizure. MRA/V revealed a right temporal venous infarct and an occluded right transverse sinus (Figures -). She was started on a heparin drip. Seven days later, she experienced a neurological decline and required intubation. Repeat imaging revealed occlusion of the superior sagittal sinus, the vein of Galen, the torcula, as well as both transverse sinuses. There was some minor edema in the thalami bilaterally. Given her precipitous decline, it was decided that if any recovery were possible, acute intervention was needed, and waiting for an MRI to rule out thalamic infarction was not feasible. She was taken to angiography and both stent retriever and aspiration thrombectomy were attempted without recanalization (Figures -). A 0.027-inch microcatheter was placed in the thrombus in the sagittal sinus, and 20 mg of tPA was dissolved in a 1 liter bag of normal saline and infused at 2 mg/hr for ten hours. She was brought back to angiography at the completion of the infusion, which revealed recanalization (Figure ). MRI confirmed bilateral thalamic infarcts. After twenty-four hours of no improvement in her clinical exam, her family elected to withdraw care.
Quinn Hale	28	1992/5/18	001-602-871-0783x67090	[email protected]	373 Heather Brook Apt. 742	The third patient is a 40-year-old female who presented with a seizure. A CT scan revealed bilateral temporal venous infarcts and superior sagittal sinus, the straight sinus, vein of Galen, and the right transverse sinus (Figure ). She was intubated for airway protection and started on heparin. On day two, she became hemiplegic with CT evidence of an extending thrombus but no new hemorrhage. She was taken to angiography where both stent retriever and aspiration thrombectomy were attempted without recanalization. A 0.027-inch microcatheter was placed in the anterior third of the sagittal sinus, and 20 mg of tPA was dissolved in a 1 liter bag of normal saline and infused at 2 mg/hr for ten hours. CTA at the completion of the infusion revealed recanalization (Figure ). She was discharged two weeks later with 3/5 strength on the left upper and lower extremity and was neurologically intact three months later at her follow-up.
Ezequiel Ward	45	1987/7/31	(946)462-3816x16332	[email protected]	364 Tom Shores	A 24-year-old white man presented to the authors institution with progressive kyphosis and severe back pain. The patient had undergone a previous thoracic fusion operation at the age of 17 for Scheuermann's Kyphosis, which was complicated by a postoperative infection requiring hardware removal and brace immobilization for approximately 1.5 years. Exertion, lifting, and attempting to maintain horizontal gaze worsened his symptoms while rest was the only method to improve his pain. On physical examination, the patient was 5 feet 5 inches, 190 lbs., and demonstrated severe thoracolumbar kyphosis (Figure ). He had well healed operative scars of the posterior midline as well as a right thorax. His neurological exam was normal. Deep tendon reflexes and straight leg raising tests were all normal. Imaging revealed severe focal lumbar kyphosis of 69 degrees at L2-L3 (Figure ). There was evidence of a robust posterior fusion mass from T10-T12 and from L1-S1 with pseudoarthrosis and spinal stenosis at the T12-L1 level (Figure ). The original operative plan was T4-pelvis instrumentation with a Ponte osteotomy at L2 and laminectomy at T12-L1 for stenosis at the area of pseudoarthrosis. This was to be followed by a posterior vertebral column resection (VCR) of L2 for correction of the deformity. The operation commenced with the placement of pedicle screw instrumentation from T5 to S1, pelvic fixation using standard iliac screw technique, and finally with transverse process hooks at T4. Pedicle screws were placed above and below the level of pseudoarthrosis at T12-L1 and originally left out of L2 in preparation for the VCR. A functional laminectomy with removal of fibrocartilaginous scar at T12-L1 was then performed. A Ponte osteotomy was completed at the L2-L3 disc space, and preparation for an interbody fusion started. Once the blunt paddles were placed within the disc space and sequentially dilated, the space became mobile, and a complete correction of deformity was demonstrated on intraoperative fluoroscopy (Figure ). Two titanium cages were filled with autograft bone and implanted in the disc space. Additional screws were then implanted into L2, and two titanium rods were contoured to provide maximum lordosis over the corrected spine followed by placement of the bone graft (Figure ) in the interlaminar spaces as well as the intertransverse gutters. Due to the intact laminae at L5 and S1 enabling a large surface area for interlaminar fusion of the L5-S1 segment, it was not felt that an L5-S1 interbody graft was necessary (as has been recommended in long segment thoracolumbar fusions). The patient experienced a postoperative ileus that resolved in four days with conservative measures. A CT angiogram of the abdomen was performed and did not support any acute abdominal pathology or mesenteric ischemia. The patient did not experience any other perioperative complications. The patient was discharged on postoperative day eight. At the six-month follow-up, his posture was normalized, and he regained horizontal gaze, helping him achieve ambulation without the use of his previous walking device (Figure ).
Ariana Sanford	31	2004/4/28	001-683-637-8055x08227	[email protected]	74943 Catherine Brooks Suite 039	A 36-year-old man was referred after failure of internal fixation to an open fracture (Gustilo?nderson IIIA) of the distal meta-diaphysis of the left femur 5 months earlier. This initial injury was managed by emergency debridement, irrigation and distal femoral locking plate fixation. At presentation with the non-union, the patient had healed scars with no evidence of sepsis. The painful non-union was evident clinically and associated with a varus deformity of the femur in the region of the fracture site. Local and systemic staging confirmed the patient to be smoker with no other co-morbidities. Radiographs displayed a broken locking plate and a femoral non-union with a 12簞 varus and 5簞 procurvatum deformity (Fig. ). Knee motion was reduced, with a passive range of motion from full extension to 50簞 flexion. No evidence of infection was found after routine biochemical investigation and confirmed after intra-operative sampling. Surgery consisted of plate and screw removal through an exposure along the entire length of the plate followed by circular external fixator application (TL-Hex, Orthofix SRL, Verona, Italy) using the ?ings first??method. Proximal fixation consisted of three hydroxyapatite coated half pins secured to a 5/8th ring and an arch. Distal fixation consisted of one 1.8 mm tensioned transverse wire and two hydroxyapatite half pins secured to a full ring (Fig. ). The non-union site was left undisturbed, and no bone graft used. After a latency period of 7 days, gradual correction was achieved over 6 days. This included 5 mm of distraction at a rate of 1 mm per day to facilitate reduction. Final anatomical alignment in the coronal and sagittal plane was confirmed on radiographs. Functional rehabilitation was encouraged with the assistance of a physiotherapist during the correction and consolidation phases. Full weight bearing was allowed from the first post-operative day. Pin track care followed our standard protocol and included twice daily cleaning with an alcoholic solution of chlorhexidine [, ]. The only complications encountered during the treatment period were minor pin track infections. One half pin developed a Checketts and Otterburn stage II infection that responded to oral antibiotics []. The tensioned wire developed a stage III infection at a late stage of treatment. The wire was removed without further complications. Radiographs confirmed solid union with exuberant callus formation after 13 weeks. The external fixator was removed when painless weight bearing on a dynamized frame was achieved. At last follow-up, 9 months after frame removal, no deformity had occurred at the union site and knee range of motion had improved at full extension to 90簞 flexion (Fig. ).
Truett Coleman	39	2000/7/25	(482)334-2985x945	[email protected]	5510 William Streets Suite 523	A 54-year-old man presented with a history of epigastric pain and melena over the last few days. The patient did not have hormone-related symptoms. He had received treatment for a myocardial infarction at the age of 51 with an antiplatelet therapy; therefore, we considered that there was a possibility of tumor bleeding by antiplatelet therapy. Blood biochemical examinations indicated slight anemia. Serum carbohydrate antigen 19-9 and carcinoembryonic antigen (CEA) levels were not elevated. The 24-h urinary 5-hydroxyindoleacetic acid (5-HIAA) level was 3.2 mg. Upper gastrointestinal endoscopy showed a bulging papilla with bleeding (Fig. ). A biopsy was not performed at that time because of the active bleeding. Endoscopic examination on day 7 revealed an approximately 10-mm mass of the ampulla of Vater with a superficial ulcer (Fig. ). Pathological findings of the endoscopic biopsy of the ampulla revealed a well-differentiated NET. Immunohistochemically, the tumor stained positive for CD56, chromogranin A, and synaptophysin. An upper gastrointestinal series showed an ampullary mass without any obvious lesions of the jejunum (Fig. ). An enhanced abdominal computed tomography (CT) scan revealed a 10-mm hypervascular tumor at the ampulla of Vater and a 41-mm multilocular cyst adjacent to the wall of the jejunum near the ligament of Treitz (Fig. ). The wall of the multilocular cyst showed the same enhancement patterns with the tumor. The patient underwent magnetic resonance imaging that was negative for visceral metastasis. About the cyst, the preoperative definitive diagnosis was not provided. The patient was referred to our hospital for treatment of the NET. We performed a pylorus-preserving pancreaticoduodenectomy with regional lymph node dissection. The operative time was 497 min, and the volume of blood loss during the surgery was 1220 ml. The resected specimen was macroscopically a 9-mm white solid tumor (Fig. ). Tumor cells confined to duodenal mucosal layer. There was a 52-mm cyst in the superior mesentery adjacent to the wall of the jejunum (Fig. ). Microscopically, the tumor consisted of small-sized round cell proliferations with a solid nest pattern (Fig. ). The cyst preserved the structure of the lymph node and was the superior mesenteric lymph node metastasis of the tumor (Fig. ). We performed D2 lymph node dissection, and there was no metastatic lymph node except for the superior mesenteric lymph node. Immunohistochemically, the resected specimen revealed that the tumor and the lymph node metastasis were positive for chromogranin A (Fig. ) and CD56 and negative for synaptophysin. The Ki-67-labeling index of the tumor cells determined with MIB-1 was 2.0 %. The final diagnosis was sporadic non-functional NET G1 of the ampulla of Vater (pT1N1M0 stage IIIB). The patient was discharged 36 days after the operation. He has had no recurrence for 4 years after surgery. No further treatment was administered. NETs of the ampulla of Vater are rare and difficult to diagnose [, ]. Jaundice (60 %) and abdominal pain (40 %) are the most frequent symptoms. Upper gastrointestinal bleeding is a rare presentation (<3 %) [, ]. In our case, the patient presented with abdominal pain and melena. On admission, upper gastrointestinal endoscopy revealed a bulging papilla with active bleeding. When we performed gastric endoscopy again 7 days later, we found a 10-mm mass of the ampulla of Vater with a superficial ulcer. Diagnosis of NETs is established by histological and immunohistochemical analysis of endoscopic biopsy specimens []. NETs of the ampulla of Vater usually appear as submucosal masses that are small and spherical with a smooth surface and an intact duodenal mucosa. Consequently, superficial biopsies are negative and deeper biopsies are required for a diagnosis [, ]. In our present case, we could get the biopsy specimen from the mass because it had an ulcerated surface. Thus, the correct diagnosis of a NET was established preoperatively. However, we could not diagnose the cyst adjacent to the wall of the jejunum as a lymph node metastasis at the time of resection. Previous reports have suggested that the biological behavior of ampullary NETs is distinct from that of duodenal NETs and they are more aggressive []. Randle and colleagues reported that ampullary NETs were larger, higher grade, and higher stage and had a higher rate of lymph node metastasis than duodenal NETs []. The incidence of lymph node metastases in patients with resected ampullary NETs and duodenal NETs was 72.9 and 48.4 %, respectively. In the case of ampullary NETs, even in tumors smaller than 2 cm, a high percentage have lymph node metastases [, ?. Nikou et al. reported that lymph node metastases were found in two cases of ampullary NET with tumor sizes of 1.0 and 1.2 cm, respectively []. In the present case, the histologic examination revealed metastasis to distant lymph nodes despite the tumor only being 9 mm in diameter and within duodenal mucosal layer. These findings suggest that there is no correlation between tumor size and metastatic potential in ampullary NETs. We consider that an anatomical reason is one of the reasons why NETs at the ampulla of Vater have high incidence of metastasis. Ampullary carcinoma with perisphincteric or duodenal submucosal invasion showed more frequent lymph node metastasis and a greater tumor recurrence rate than tumor limited within the sphincter of Oddi muscle []. Moreover, the perisphincteric and duodenal submucosal space is relatively small and closer to the next layering of duodenal proper muscle and the pancreas. Thus, the malignant potential of perisphincteric and/or duodenal submucosal invasion may be greater than that of other gastrointestinal tract tumors. Moreover, previous studies have reported that lymph node metastasis is difficult to detect on preoperative imaging [, ]. Although some authors report the existence of lymph node metastasis of NETs is not correlated with patient prognosis, this could be due to metastasis to the liver or some other organ, which is an important prognostic factor for NETs. Therefore, radical resection with lymph node dissection is recommended as a treatment of ampullary NETs regardless of tumor size [, , , , ]. We performed a pylorus-preserving pancreaticoduodenectomy with regional lymph node resection and also resected a 52-mm lymphatic metastasis adjacent to the wall of the jejunum. Despite the lymph node involvement, the Ki-67-labeling index was low (2 %) and the patient has had no evidence of recurrence for 4 years after surgery. Randle et al. indicated that tumor size was correlated with poor prognosis, but the presence of positive lymph nodes was not a predictive outcome in resected ampullary NETs []. Untch et al. reported that only tumor size and tumor grade were associated with recurrence []. Thus, we can perform radical resection and completely remove the tumor with good clinical outcomes.
Julia Allison	33	2005/6/18	001-556-931-6453x01066	[email protected]	61411 Annette Parkways Suite 299	A 57-year-old woman with no past medical history presented at our emergency department (ED) with 1 day of posterior neck pain, followed by weakness in her left upper limb and both lower limbs, and inability to pass urine since that morning. There was no history of trauma to the head or spine. Two weeks prior to the ED visit, she experienced severe neck ache and consulted a chiropractor, who performed manipulation and needle acupuncture of her back, with relief of her initial symptom. On examination, the patient was afebrile, with a blood pressure of 116/55 mmHg, a pulse rate of 108 beats per minute, a respiratory rate of 18 breaths per minutes and an oxygen saturation of 97 % in room air. Palpation of her abdomen revealed a bladder distended to the level of the umbilicus. Neurological examination disclosed weakness of the left upper limb and spastic paraparesis. Power in the right upper limb was full. There was also decreased sensation from the level of the neck downwards but with sparing of the right upper limb. A lax anal tone was obtained on digital rectal examination. X-rays of the cervical and thoracolumbar spine showed disc narrowing at the C5/C6 and C6/C7 levels, and grade 1 spondylolisthesis of L4 on L5. The white blood cell count was 30.13 ? 109 cells/L (normal range, 4??0 ? 109 cells/L) with neutrophilia, and the C-reactive protein and erythrocyte sedimentation rate levels were 391 mg/L (0.2??.1 mg/L) and 119 mm/h (3??0 mm/h), respectively. Her procalcitonin level was 2.4 弮g/L (<0.5 弮g/L). Magnetic resonance imaging (MRI) of the spine showed multiple spinal abscesses in the posterior epidural space from C5 to T1 and from L4 to L5. The former measured 0.7 ? 1.3 ? 5.1 cm, displacing the cervical cord anteriorly and compressing it at C7-T1, with a high T2 signal intensity in the cord (see Fig. ). There was also a high T2-TIRM signal in the C6-7 vertebral bodies and intervening disc with abnormal enhancement, in keeping with osteomyelitis and discitis. This was also seen in the lumbar spine (see Fig. ). In the lumbar spine, another collection measuring 0.4 ? 1.2 ? 3 cm was noted in the right posterior epidural space along the right lamina of L4/L5. Together with a disc bulge, this compressed the thecal sac and cauda equina (see Fig. ). She was admitted to the orthopaedic surgery high dependency unit, and intravenous (IV) antibiotics were commenced. Surgical decompression of the spinal cord and drainage of the epidural abscesses was performed early the next day. Blood cultures and intra-operative fluid cultures grew pan-sensitive Staphylococcus aureus. Following advice from the infectious disease specialist, the patient completed 6 weeks of IV penicillin G. Repeat blood cultures taken 1 week after initiation of antibiotics were negative. Post-surgery, she underwent intensive physical therapy for a month, after which she was discharged independent without aid in the community. A follow-up MRI scan performed 4 months later showed resolution of all the abscesses. A spinal epidural abscess (SEA) is a pyogenic infection of the potential space between the vertebral body and dura mater of the spine. Although rare, their incidence has been rising gradually over the past decade, possibly due to increasing intravenous drug abuse, an ageing population with multiple comorbidities and more frequent use of spinal instrumentation. SEAs can be either primary or secondary. Primary SEAs result from haematogenous spread of pathogens from a distant focus to the epidural space, while secondary SEAs occur after spinal trauma, injections, surgery or direct inoculation of a pathogen into the epidural space []. In a literature review of 40 studies published from years 2000 to 2013 [], the most common symptoms and signs reported in SEA were neck and back pain, fever and neurological deficits. Altered mental state and incontinence were also documented. However, presentation can be ambiguous and variable, resulting in delayed diagnosis. The most common predisposing factor is diabetes mellitus. Others include alcoholism, renal failure, intravenous drug abuse, chronic inflammatory conditions, bacteremia and previous spinal intervention. The neuroimaging study of choice is MRI. It visualizes soft tissues as effectively as computed tomography (CT) myelography, with a comparable sensitivity for SEA. It also provides superior identification of perimedullary lesions with no risk of introducing pathogens into the thecal sac. MRI is able to differentiate between SEAs and other spinal lesions such as subdural abscesses and tumours. The classical description of an SEA on MRI is the collection in the epidural space which is iso- or hyperintense on T1 images which enhances with gadolinium contrast, and a T2 image with a non-homogeneous and hyperintense signal []. A systematic review of 12 studies published after 1999 [] reported S. aureus as the most common causative pathogen, followed by Streptococcus species. SEA formation following acupuncture is rare, and a literature search yielded only seven such cases between 1998 and 2015, summarized in Table . There is variability in patient profile, clinical presentation, spinal level of involvement, causative organism and treatment. Acupuncture is an early form of medical intervention that originated in China thousands of years ago and continues to be practised today, especially in Asian countries. Studies report its clinical benefits for chronic painful musculoskeletal disorders, headaches and hypertension. Major complications are uncommon, with an incidence of 0.55 per 10,000 individual patients in 12 prospective surveys []. The most common infection is hepatitis B (>60 %) due to transmission via contaminated needles. Only three cases of spinal infection were reported in this review. Treatment of SEA includes antibiotics for systemic manifestations and surgery for local disease control. One literature review of 28 case series acknowledges that SEAs are rare, with significant variation in their causes, anatomical locations and rate of progression, making it difficult to build a strong evidence base to stratify patients to the most effective treatment algorithm []. However, there is relative consensus that patients with acute or progressive neurological deficit, progressive deformity, spinal instability, or disease progression despite antibiotics require surgical intervention []. When surgery is required, a posterior laminectomy is the most common approach. Less invasive methods of surgical evacuation include CT-guided needle aspiration, hemilaminectomy and interlaminar fenestration, which report promising results. For extensive SEA, suction irrigation catheters passed through end- or intermediate-level laminectomies or multilevel unilateral fenestrations may be performed to avoid a multilevel laminectomy []. Based on the best available evidence, medical management should be considered in the following patients []:Those who are unfit for surgery (medically unstable, serious comorbidities) Complete paralysis for >48 h without significant concern for an ascending lesion (surgical risks likely to outweigh the chance of functional recovery) Risk factors for failure of medical treatmentDiabetes mellitis Bacteremia White blood cell count >12.5 ? 109 cells/L C-reactive protein >115 mg/L Ring enhancement of lesion on MRI Methicillin-resistant S. aureus infection Although the period of antibiotic therapy is debatable, this should last at least 4 weeks because studies have shown a 25 % rate of relapse in patients who were treated for less than 4 weeks []. The patient and treating physician should bear in mind that even in the absence of risk factors, there is no guarantee that medical treatment will not fail and that the patient will not require surgery eventually. The risk of failed medical management has been reported to be between 8.3 and 17 %. The best predictor of post-operative neurological outcome is preoperative neurological status [].
Dennis Steele	43	1987/11/29	+1-639-731-4669x8841	[email protected]	272 Davis Junctions Apt. 443	A 64-year-old woman with an unremarkable medical history was admitted to a nearby hospital for a high fever and diarrhea that had lasted for 1 week. She had vision loss in her right eye, exacerbation of renal dysfunction (serum creatinine level, 4.96 mg/dL), and thrombocytopenia (platelet count, 14,000/弮L) within 48 h from initial presentation. Therefore, she was transferred to our institution for treatment of severe infectious disease. On admission, she was alert and had no alteration of mental status, no hypoxia on room air, and normal vital signs (temperature, 36.7 簞C; blood pressure, 126/80 mmHg; pulse rate, 74 beats/min; respiratory rate, 12 breaths/min). However, she had almost complete ablepsia of the right eye. Laboratory data showed severe inflammation (white cell count, 21,700/弮L; C-reactive protein level, 28.2 mg/dL; procalcitonin, 46.4 ng/mL), liver dysfunction (aspartate transaminase, 147 IU/L; alanine transaminase, 345 IU/L), thrombocytopenia (platelet count, 32,000/弮L), an increased serum creatinine level (1.35 mg/dL), and coagulopathy (fibrin/fibrinogen degradation product, 56.6 弮g/mL; D-dimer, 24.2 弮g/mL; prothrombin time-international normalized ratio, 1.15)(Table ). Computed tomography showed a low density area in the right lobe of the liver, and she was diagnosed with a liver abscess (Fig. ). Broad-spectrum antibiotic therapy with meropenem (3 g/day) was immediately started. Endogenous endophthalmitis of the right eye was also diagnosed, and emergency vitrectomy was performed on the day of admission. The blood culture at admission showed K. pneumoniae, and the antibiotic therapy was changed to ceftriaxone (2 g/day) on day 4 because all of the cephalosporin had high susceptibility (Table ) and ceftriaxone was known to penetrate to the liver in high concentration, which was continued to day 14. Percutaneous drainage of the liver abscess, which was unresolved by medical treatment, was performed after coagulopathy improved on day 6 (Fig. ). The culture of the drainage fluid also showed K. pneumoniae. The drainage tube was removed on day 14, and the patient was discharged in a good general condition on day 22, with an additional 2-week course of oral levofloxacin on days 14??8. However, she had complete ablepsia of the right eye. Capsular probing via PCR for the presence of magA (serotype K1) and K2A (serotype K2) genes was performed by using the magA-specific primers (forward, 5??GGTGCTCTTTACATCATTGC-3?? and reverse, 5??GCAATGGCCATTTGCGTTAG-3?? and k2A-specific primers (forward, 5??CAACCATGGTGGTCGATTAG-3?? and reverse, 5??TGGTAGCCATATCCCTTTGG-3?? as previously described[]. The virulence-associated gene rmpA was also screened using PCR by using the rmpA-specific primers (forward, 5?? ACTGGGCTACCTCTGCTTCA-3?? and reverse, 5?? CTTGCATGAGCCATCTTTCA-3?? as previously described []. The reaction mixtures of these samples were kept at 95 簞C for 5 min, followed by 35 cycles of 95 簞C for 1 min, 50 簞C for 1 min, and 72 簞C for 1 min and then 72 簞C for 7 min. The K2A gene, which is consistent with the K2 serotype, was detected by PCR. PCR was also positive for the virulence-associated gene rmpA. To assess for the presence of hypermucoviscosity, a string test was performed on the organism grown in 5 % sheep blood agar. The formation of a mucous string of >5 mm in length after touching a colony with a loop was considered positive []. The isolated K. pneumoniae had a positive string test consistent with a hypermucoviscous phenotype (Fig. ). We present the case of invasive liver abscess syndrome with endogenous endophthalmitis caused by the K2 serotype of K. pneumoniae extending to complete ablepsia despite improvement of the pathophysiology of severe sepsis. One systematic review reported K. pneumoniae had been the most common causative organism (27 %) in endogenous bacterial endophthalmitis. Following other organisms were Staphylococcus aureus (10 %), Pseudomonas aeruginosa (6 %), Group B streptococci (6 %), and Neisseria meningitidis (5 %) []. Therefore, use of broad-spectrum antibiotics must be considered until identification of causative microorganism. This case of a primary liver abscess caused by the definite K2 serotyping of K. pneumoniae is the first adult case in Japan. This invasive syndrome caused by K. pneumoniae serotypes K1 or K2 has been reported mainly in Southeast Asian countries, especially in Taiwan. We found around 50 cases with liver abscess caused by K. pneumoniae serotype K2 all over the world [, ]. However, in Japan, only three cases?wo in elderly men infected with the K1 serotype and one in a 7-year-old child with the K2 serotype?ave been reported [?, when we searched PubMed and the Igaku Chuo Zasshi databases for papers published between Jun 01, 1970, and Dec 31, 2015, by using combinations of the following keywords: ?lebsiella pneumoniae,???iver abscess,???1??or ?2,??except minutes and selected articles about this invasive syndrome published by Japanese authors. Almost 30 cases of primary liver abscess caused by K. pneumoniae with endogenous endophthalmitis have been reported in Japan (all reports in Japanese); we conducted a search of the medical literature published using the Igaku Chuo Zasshi database and ?iver abscess,???ndophthalmitis,??and ?lebsiella pneumoniae??as search terms. In addition, we could find two more Japanese literatures related to invasive liver abscess syndrome when we searched Igaku Chuo Zasshi database by using following keywords: ?lebsiella pneumoniae,???iver abscess,??and ?mpA??[, ]. The K1 and K2 serotype were suspected in all cases because of the clinical features, but examination of the serotype has never been reported. Therefore, an invasive liver abscess caused by the K1 or K2 serotype of K. pneumoniae may not be rare in Japan. The virulence-associated gene rmpA was positive in our case. rmpA is not an independent factor contributing to a liver abscess, but it aids in capsule synthesis. One report showed that all K. pneumoniae strains that cause liver abscesses and abscesses at other sites are rmpA-positive. rmpA has been confirmed as a gene that regulates capsular polysaccharide synthesis []. Thus, in our case, rmpA was probably associated with liver abscess formation.
Rylie Dickerson	22	1990/1/18	(763)464-3781	[email protected]	4943 Kennedy Rapid Suite 020	A 79-year-old woman visited a local internal medicine clinic because of intermittent right upper quadrant abdominal pain. The patient was admitted to our hospital due to suspected acute cholecystitis and had Murphy? sign on admission. The patient was being regularly treated for diabetes, hypertension, and dyslipidemia, but she did not smoke or drink alcohol. Additionally, the patient had a history of breast cancer diagnosed 22 years previously that had been treated with mastectomy. Routine laboratory evaluation showed white blood cell count at 10,100/弮l, total bilirubin at 7.9 mg/dl, aspartate aminotransferase (AST) at 113 U/l, alanine aminotransferase (ALT) at 2 U/l, and C-reactive protein (CRP) at 30.38 mg/dl. We regrettably did not check tumor markers. The patient underwent ultrasonography (US) and computed tomography (CT), with and without contrast, for screening. The US images revealed many small stones and sludge in the gallbladder (Fig. ). CT images similarly revealed a thick-walled gallbladder containing multiple stones (Fig. ). Multiple 3-cm-sized round nodular lesions with heterogeneous enhancement were also noted in the peri-cholecystic and peri-pancreatic area, which were suggestive of metastatic lymph nodes (Fig. ). Percutaneous transhepatic gallbladder drainage was then performed, as an alternative interim strategy before surgery. Abdominal symptoms subsequently improved, and endoscopic ultrasound-guided fine needle aspiration of enlarged lymph nodes resulted in a diagnosis of small cell carcinoma or adenocarcinoma. However, we could not identify the primary lesion. Therefore, our surgical procedure included radical cholecystectomy with partial liver resection together with lymph node dissection. Pathology of the resected specimen showed that the entire gallbladder was tumorous. Additionally, the tumors invaded into the liver (Fig. ). Histopathological examination of the tumors revealed neuroendocrine carcinoma, small cell type (Fig. ). The tumor stage of this patient was IVb, T3aN1M1. On immunohistochemical study, the tumor cells showed positive staining for chromogranin and negative staining for synaptophysin (Fig. ). Postoperatively, the patient developed progressive jaundice unrelieved with biliary stenting and died 13 weeks after the surgery. Neuroendocrine tumors (NETs) usually appear in the gastrointestinal and bronchopulmonary systems. There are no neuroectodermal cells in the gallbladder mucosa. Therefore, primary gallbladder neuroendocrine tumors are postulated to arise from either a multipotent stem cell or neroendocrine cells in intestinal or gastric metaplasia secondary to cholelithiasis and chronic cholecystitis []. In neuroendocrine neoplasms of the gallbladder, SCC is extremely rare: although 1002 cholecystectomy cases have been performed at our hospital since 1997, the case described was our first of gallbladder SCC. In recent years, gallbladder SCC has attracted increasing attention for improved understanding and diagnosis. As shown in Table (compiled from our literature survey), the average age of diagnosis is 64 years, with a male-to-female ratio of 1:1.9. In most cases of gallbladder SCCs, patients typically present with recurrent right upper quadrant pain. Less frequent presentations include abdominal mass, weight loss, and ascites []. Gallbladder SCCs are commonly associated with stones, with cholelithiasis found in 49 % of cases (Table ). A preoperative diagnosis of gallbladder SCC is very difficult because the presentation generally consists of nonspecific symptoms. And, despite the development of imaging studies, it is also still difficult to diagnose gallbladder SCC by ultrasonography, CT, or magnetic resonance imaging. Therefore, the diagnosis is often made incidentally at the time of cholecystectomy performed for cholecystolithiasis or polyps. Recent studies indicate that the role of 18F-FDG positron emission tomography (PET)/CT might prove to be useful in diagnostic imaging for the detection of gallbladder SCC []. However, we regrettably do not have a PET-CT scanner in our hospital. Gallbladder SCC usually presents as a large mass containing extensive necrosis with marked propensity for invasive submucosal growth []. Histopathologically, about 80 % of cases are pure SCC and the remaining 20 % are combined SCC (Table ). Microscopically, the WHO classification defines small cell carcinomas as neuroendocrine tumors with >20 mitoses/2 mm2 (mean 75/10 hpf) and small cell cytological features []. Immunohistochemically, the tumor cells expressed neuroendocrine markers, such as chromogranin A, synaptophysin, and/or CD56. In comparison to NETs which usually are diffusely positive for neuroendocrine markers, SCCs show more focal staining []. Surgical treatment remains the best curative option and appears to prolong life, with chemotherapy adding a marginal advantage. The operative procedures that we deem appropriate range from cholecystectomy alone to extensive surgical resections, including regional lymph node clearances and hepatic lobectomy. However, therapeutic options are limited because the disease presents in an advanced stage. In fact, stage IV is found in 67 % of cases (Table ). In cases of nonresectable tumors, the primary management is chemotherapy, and the chemotherapeutic agents of choice are cisplatin, etoposide, and 5-fluorouracil. Actually, there are few papers that showed specific chemotherapy regimens [, ]. Surgical treatment and chemotherapy are found in 74 and 59 % of cases, respectively (Table ). The role of radiotherapy remains undefined due to paucity of data []. As shown in Table , distant metastasis at presentation is present and is most often located in adjacent lymph nodes (70 %) and the liver (56 %). Median survival for gallbladder SCC is only 8 months. Based on SEER data, gallbladder SCC has no survivors at 10 years, in contrast to the 10-year survival of 36 % for carcinoid tumors [].
Flynn Lowe	42	1983/8/22	254.922.1462	[email protected]	600 Clark Mews	A 13-year-old female patient, who was previously diagnosed with Melnick-Needles Syndrome (OMIN 309350) at eight years of age, sought dental follow-up at the Specialized Pediatric Dentistry Clinic of the Brazilian Association of Dentistry in the state of Alagoas, Brazil, accompanied by her mother. The diagnosis was based on pathognomonic and radiographic findings: small face with a prominent brow, exophthalmia, micrognathia, cranial hyperostosis, poor alignment of the teeth, short upper limbs, bowed long bones (especially the humerus and tibias), metaphyseal thickening, genu valgum, shortened distal phalanges, narrow pelvis and shoulders, tapered ribs with irregularities, elongation of the vertebrae, and kyphoscoliosis (Figures , , and ). The extraoral photographs of the face revealed the prominent brow, exophthalmia, and micrognathia (Figures and ). Intraoral examination revealed considerably poor tooth alignment, with upper dental crowding and anterior deep bite (Figures , , and ). The panoramic radiograph revealed hypoplastic coronoid processes of the mandible, left stylohyoid ligament suggesting ossification, and diverse dental development anomalies, such as maxillary anterior crowding, agenesis of tooth 15, retention of teeth 65 and 85, impacted teeth 33, 37, 43, and 46, and semi-impacted tooth 36 (). A teleradiography was requested due to cephalometric analysis, in which we observed that there was no proportionality between the effective lengths of the maxilla and mandible, according to the length of the mandible condyle from the point Co to the point Gn. According to McNamara, for a given size jaw there is a directly proportional mandible size (). The patient was submitted to prophylaxis, advice regarding oral hygiene and topical application of fluoride. As immediate treatment, the primary teeth retained in the arch were extracted due to the fact that these teeth were impeding the normal eruption of the permanent successors. The patient will continue in medical and dental follow-up with orthodontic accompaniment and there is a probability that she will undergo orthognathic surgery or mandibular distraction after the growth phase. She is also being assisted by a program focused on the prevention of dental caries, periodontal disease, and occlusal problems.
Amari Rodriguez	25	2001/10/19	235.919.0867	[email protected]	56959 Larry Ranch	A 77-year-old woman was transferred to the intensive care unit (ICU) of our hospital with altered mental status that was thought to be secondary to carbon dioxide retention caused by aspiration pneumonia. In the ICU, she progressed into hemodynamic shock. A central venous cannulation was attempted via the right internal jugular vein. Before performing the central venous cannulation, her heart rate was averaging 70??0 beats per minute. Following the cannulation, her heart rate suddenly dropped to 20 beats per minute and the cardiac tracing on the monitor showed a complete heart block (CHB). A stat electrocardiogram (EKG) was obtained which confirmed complete heart block with an escape rhythm (). Atropine was administered which failed to correct the heart rate. A chest radiograph showed the tip of the catheter in the superior vena cava. A transcutaneous pacemaker was immediately placed followed by a temporary transvenous pacemaker placement. Her previous EKG revealed a preexisting left bundle branch block (LBBB) (). 24 hours following this event, the CHB resolved and she was back in sinus rhythm with a LBBB. The transvenous pacemaker was removed. An echocardiogram showed normal left ventricular function with an ejection fraction of 55%. Since the CHB was transient and was thought to be due to mechanical inhibition of the right conduction system caused by guide wire insertion, further electrophysiologic studies were not felt warranted. Following this, she had a complicated medical course but she eventually recovered and was discharged in a stable condition without a pacemaker.
Henry Sweeney	37	1987/6/27	342-509-3750	[email protected]	3684 Jon Keys Apt. 635	A 50-year-old female with a past medical history of SLE diagnosed 20 years ago, ESRD on hemodialysis and atrial fibrillation on anticoagulation, presented to the emergency department after a syncopal episode. The patient was sitting on the side of her bed when she suddenly lost consciousness falling to the floor striking the right side of her face. She sustained significant facial trauma; however there were no signs of intracranial hemorrhage on CT scan of the brain. She described palpitations with chest pressure prior to the event. Initial electrocardiogram (ECG) reported by emergency medical services did not reveal any acute arrhythmias, such as TdP. Due to the complaints of chest pressure she was evaluated for cardiac ischemia utilizing cardiac biomarkers, CKMB and Troponin I. A total of three sets were completed every eight hours apart; all studies were reported within normal limits ruling out cardiac ischemia. Troponin I values were less than 0.045 ng/mL and CKMB values were all less than 0.5 ng/mL. ECG on presentation revealed 1st-degree AV block with corrected QT (QTc) interval of 689 ms (). Patient's ECG from two years prior showed a baseline prolonged QTc interval of 500 (). She was evaluated for reversible causes of QT interval prolongation including electrolyte derangements, medications, and SLE flare. Her electrolytes were within normal limits including potassium (3.9 mmol/L), magnesium (2.3 mg/dL), phosphorous (4.2 mg/dL), and calcium (9.0 mg/dL). C3 levels were low, with normal C4 and ESR, and anti-dsDNA was negative making SLE flare unlikely. Patient continued to have dialysis on her normally scheduled days. After evaluation by electrophysiology there was concern for presumed TdP due to her clinical presentation and grossly abnormal QTc interval. She was placed on mexiletine 150 mg BID and was initially recommended to have defibrillator implantation to protect against sudden cardiac death from cardiac arrhythmias. Evaluation of the patient's medications revealed that she was taking HCQ for the past two years for treatment of refractory SLE. HCQ was discontinued and the patient's QT interval slowly decreased to a QTc interval of 500 over the next 7 days (). She was monitored on the telemetry unit for one week and did not have any further arrhythmic events. After further discussion with the patient's Cardiologist and Rheumatologist she had been noted to have a prolonged QTc interval of approximately 500 ms while on therapy with HCQ. She was trialed off the medication for two months without resolution of the QT interval abnormality, possibly due to undiagnosed underlying SLE cardiomyopathy. The patient's ejection fraction on echocardiography one year prior was estimated at 65% with Doppler parameters consistent with abnormal relaxation suggesting heart failure with preserved ejection fraction. Implantable defibrillator placement was discussed with the patient prior to admission due to the prolonged baseline QTc interval. However, she was reluctant to proceed as she did not want indwelling devices due to concern for possible Permacath placement in the future for dialysis if her arteriovenous fistula malfunctioned. The risks and benefits of continuing HCQ were discussed with the patient, and she decided she wanted to continue the medication due to well controlled SLE flares. She had noted adverse reactions to steroid therapy and limited treatment options for SLE due to her underlying renal failure. We discussed the risks of recurrent syncopal episodes if she was to continue HCQ therapy, and the high risk for intracranial bleed while on anticoagulation if she was to have another syncopal episode. Final recommendations were made to proceed with defibrillator placement if she was to continue with HCQ to protect against life threatening arrhythmias. She had a subcutaneous defibrillator placement and was to be restarted on HCQ as an outpatient with dose reduction and close monitoring of QTc interval. At three-month follow-up the patient remained off HCQ as after discussion with her Rheumatologist the risks related to syncope and possible intracranial bleed while on anticoagulation treatment greatly outweighed the benefits of HCQ therapy. She continues to be closely monitored for SLE flares, and treatment decisions are to be strategized as clinical issues arise. Subcutaneous implantable defibrillator interrogation showed no recorded events at three months. Her ECG continues to show a prolonged QTc interval of 508 ms. Due to the prolonged QTc interval she is still at high risk of ventricular arrhythmias despite discontinuation of HCQ. Therefore, the subcutaneous implantable defibrillator is providing secondary prevention of sudden cardiac death due to prolonged QT interval.
Yara Bernal	18	1994/1/26	393-900-0729x19710	[email protected]	7147 Adams Glen Suite 277	A 12-year-old child, male, was admitted to the Department of Neurology in Beijing Chaoyang Hospital. Three weeks before his admission, he received the vaccination of hepatitis B. He was with symptoms of myasthenia of limbs and alteration of consciousness. He also had high fever, with the body temperature maintained between 38簞C and 38.5簞C. He was with no symptoms of headache, dizziness, nausea, vomit, sphincter dysfunction, and optic neuritis. Four years ago, he had suffered from essential thrombopenia, which relapsed two years ago. He had no history of toxic substance, allergy, operation, trauma, blood transfusion, and inheritance history. He was a full-term baby, with normal vaginal delivery. His mother had suffered from a disease of allergic purpura. Physical examination on his admission showed that he was with somnolence and uncooperative. His pupils were equal in size and round. The optic nerve was normal. Bilateral light reflexes retained. Muscle strength of limbs grading (II level) was found with hypomyotonia. Sensory tests were uncooperative. Abdominal reflex and cremasteric reflex were negative. The signs of bilateral Babinski and Gordon were positive. Ankle clonus was also detected. Neck resistance was also found. On admission, the results of blood test were as follows. WBC level (15.8 ? 109/L) and the proportion of neutrophils (85.2%) were markedly increased. Procalcitonin was 0.05 ng/mL, which indicated no bacterial infection. C-reactive protein was increased, which was 1.14 mg/dL (0??.8 mg/dL). ESR was 50 mm/H (2??5 mm/H). IgG was 1750 mg/mL (751??560 mg/mL). IgA, IgM, C3, and C4 were normal. Sputum cultures of bacteria, fungus, virus, and tuberculosis were negative. Total protein and albumin in blood were decreased. Globulin and total bilirubin were normal. The cerebrospinal fluid (CSF) examinations showed increased pleocytosis (52/弮L) and leucocyte count (40/弮L). The CSF was composed of 90% mononuclear cells and 10% polynuclear cells. Pandy test was negative. Total protein was normal. Glucose (4.77 mmol/L) was slightly increased (2.5??.4 mmol/L). Chloride (115.1 mmol/L) was slightly decreased (118??29 mmol/L). Oligoclonal band was found. The aquaporin 4 antibody was negative. Bacteria, mycobacterium tuberculosis, virus (e.g., herpes simplex encephalitis virus, cytomegalovirus, and Epstein-Barr virus), and fungal cultures from CSF and blood serology, with PCR also performed, were negative. Three days after his admission, MRI of brain and spinal cord also revealed some abnormal findings. His brain MRI showed widespread abnormal signals on FLAIR image (). Spinal cord MRI showed that there were abnormal multifocal, strip long T1 and T2 signals at the cervical and intumescentia lumbalis (). Furthermore, evoked potential such as brainstem auditory evoked potentials and somatosensory evoked potential also showed some abnormal changes in this case. The visual evoked potential was normal. With treatment with high-dose methylprednisolone and intravenous immunoglobulin and also with some antibiotic and antiviral therapy, he showed a dramatic improvement of the clinical and CSF results. About four months later, he recovered completely and there was no relapse during three years of follow-up.
Eithan Felix	19	1992/2/10	+1-503-859-4646x7199	[email protected]	47759 Ryan Street	A 61-year-old right hand dominant housewife sustained an injury to her right upper limb following a low energy mechanical fall. The patient attended the Emergency Department (ED) due to significant right arm pain, swelling, and deformity. No neurovascular deficit was documented on initial clinical assessment. X-rays confirmed a short spiral fracture of the humeral diaphysis with slight angulation (). A Bohler-U cast was applied and repeat X-ray demonstrated excellent alignment on both anteroposterior and lateral views (Figures and ). On return from the X-ray department the patient reported new onset of paraesthesia in the right hand and difficulty moving her fingers. On detailed questioning, the patient admitted to very slight alteration of sensation at the thumb base and posterior aspect of the forearm prior to application of the Bohler-U cast. Examination confirmed MRC grade 0/5 power in wrist and finger extensors and reduced sensation at the base of thumb and posterior aspect of the forearm in keeping with a dense radial nerve palsy. Peripheral pulses were easily palpable and capillary refill was normal. Due to the onset of new neurologic deficit following fracture reduction and cast application, the clinical concern was of iatrogenic nerve injury. Urgent surgical exploration was performed via an anterolateral approach. Intraoperatively, the radial nerve was found to be interposed in the fracture (). The nerve was in continuity, though there was evidence of significant contusion. Following careful extraction of the radial nerve, the fracture was reduced and stabilised using a 10-hole AO (Arbeitsgemeinschaft f羹r Osteosynthesefragen) large fragment dynamic compression plate and interfragmentary compression screw (). Postoperatively, there was no immediate improvement in radial nerve function. A custom-made thermoplastic wrist extension splint was initially fitted. Examination at one week from date of surgery demonstrated some early return of sensation in the distribution of the superficial branch of the radial nerve though the dense motor deficit remained unchanged. An early rehabilitation programme consisting of passive range of movement exercises and dynamic splinting was commenced under the supervision of departmental hand therapists. Full active shoulder and elbow range of movement exercises commenced 4 weeks from date of surgery. Examination at 3 months demonstrated MRC grade 3/5 finger extension and wrist extension. At 3 months from date of surgery, the fracture also appeared radiologically united. Complete recovery of radial nerve function was evident at 8 months (MRC grade 5/5). highlights our patient's recovery process as described above.
Paisleigh Pope	22	1991/4/3	001-539-738-0825	[email protected]	925 King Underpass	An 80-year-old man developed sudden severe back pain with no history of trauma, and then he tried to walk. However, due to weakness and numbness in his legs, he could not walk and collapsed to the ground. He presented to an outside hospital with paraplegia, and then he was transferred to a tertiary center for further management. On admission, neurological examination of his motor power was reduced (0/5) in both lower extremities (paraplegia), tendon reflexes in both lower limbs had increased, Babinski responses were positive, there was anesthesia/hypoesthesia for light touch, and there was hypoesthesia below the thoracal (T5-T6) dermatome. His knee- and ankle-jerk reflexes were absent bilaterally. There were urine and stool incontinence. The complete blood count (hemoglobin, hematocrit, and platelet count), biochemistry profile (kidney function, liver function, proteins, and glucose), prothrombin time, and INR were normal, and he did not have additional comorbidities (hypertension, diabetes mellitus, etc.). In addition, he did not use an anticoagulant therapy. At the time of admission to emergency, only subcutaneous low molecular weight heparin (0.3 mL) was conducted. A high-dose steroid treatment was given on the day of his admission (bolus 30 mg/kg administered over 15 minutes with maintenance infusion of 5.4 mg/kg per hour infused for 23 hours). The Computed Tomography (CT) scan was not diagnostically helpful. Magnetic resonance imaging (MRI) revealed an intramedullary mass with signal intensity surrounded by a zone of high and low signals on both T2 weighted images (Figures , , and ). Unfortunately, because of the lack of time, we did not have contrast-enhanced MRI or spinal angiography, so we could not eliminate the vascular pathologies. Because of mass effect, there was obstruction of cerebrospinal fluid and syrinx. After written informed consent was obtained from the patient and his family, an operation was performed. The patient underwent the operation on the day of admission. After thoracic vertebra 5 and 6 laminectomy, microscopic dural opening was revealed, a small paramedian myelotomy was performed at the level, and blunt dissection and aspiration of the hematoma were done. A hematoma was completely removed (). There were no vascular abnormalities and tumors. The pathology report confirmed the diagnosis of intramedullary hemorrhage. After the operation, there was no recovery of sensory or motor functions, and he began receiving rehabilitation treatment.
Gunnar Spencer	34	2000/3/13	908-527-2014x294	[email protected]	1763 Elizabeth Fork	A 43-year-old male construction worker presented with left medial ankle pain. He fell and twisted the left ankle at the age of 25 years. Fibular fracture was diagnosed using simple X-rays and then he was admitted to the emergency department for open reduction and internal fixation using a fibular plate at a hospital near the accident location. Five years later, left ankle pain developed and the range of ankle motion became limited, so he underwent resection of bone hyperplasia. Thereafter, the left ankle became intermittently painful, which persisted for about one year. The initial examination at our hospital (18 years after the ankle fracture and 43 years old at this time) revealed tenderness along the posterior tibial tendon and a left ankle range of motion from 10簞 of dorsiflexion to 60簞 of plantarflexion. He was overweight at that time (his body mass index was 35.6 kg/m2). The left foot was relatively flat, but too much toe sign was not visible and he could perform left single-leg heel raises. X-rays revealed bone hyperplasia between the distal tibia and fibula (). Three-dimensional computed tomography (3D-CT) revealed tibial bone hyperplasia along the posterior tibial tendon (). The posterior tibial tendon had spotted isointensity in regions of low intensity on T1-weighted magnetic resonance (MR) images and spotted high intensity in low-intensity T2-weighted images (). Injecting lidocaine into the posterior tibial tendon sheath relieved the pain. Thus, we diagnosed posttraumatic posterior tibial tendinitis. We planned a tendoscopic procedure instead of an open procedure because an open procedure needs opening the tendon sheath and postoperative immobilization such as a cast or brace. The patient underwent left posterior tibial tendoscopy using a 2.5 mm 30簞 arthroscope under spinal anesthesia. We used the two main portals described by van Dijk in 1997 []. The distal portal was 2 cm below and anterior to the medial malleolus, whereas the proximal portal was 2 cm posterior and superior to the medial malleolus. An ultrasound machine (Noblus; Hitachi Aloka, Tokyo, Japan) and a high-frequency linear probe (L64 linear probe 18?? MHz; Hitachi Aloka) are prepared with a sterile ultrasound probe cover and sterile gel. We used an ultrasonography in order to introduce a scope easily, reliably, and safely. Irrigation solution was injected into the posterior tendon sheath under long-axis view along the posterior tibial tendon of sonography just before incising the skin (). We opened the tendon sheath via the skin incision by blunt dissection using a hemostat under sonography. Tendoscopic findings revealed tenosynovitis and fibrillation on the surface of the tendon. We cleaned and removed the synovium surrounding the tendon using an arthroscopic shaver system and a radiofrequency wand for small joints (). Moreover, we deepened the groove for the posterior tibial tendon using a bone cutter/shaver so as the posterior tibial tendon can move smoothly. After groove deepening, there was one- or two-millimeter space around the tendon. Weight-bearing ambulation was permitted one day after surgery and the patient returned to full-time work in the construction industry six weeks after surgery. At the most recent review two years after surgery, he had no medial symptoms and he was capable of all types of heavy work despite occasional slight pain related to sinus tarsi syndrome. His preoperative and postoperative scores on the American Orthopaedic Foot and Ankle Society (AOFAS) ankle-hindfoot scale were 71 and 90 points, respectively. Each author certifies that his or her institution approved the human protocol for this investigation and that all investigations were conducted in conformity with ethical principles of research. The patient and his family were informed that data from the case would be submitted for publication and gave their consent.
Alyssa Park	43	1995/8/21	7172847976	[email protected]	7980 Spence Inlet Suite 033	A 48-year-old man presented with symmetrical arthritis of the wrists, metacarpophalangeal and proximal interphalangeal joints, elbows, and knees. He had had intermittent inflammatory back pain since the age of 40 years. His brother suffered from psoriasis. The physical examination revealed alopecia areata, which he said he had had for a long time, but he had never had psoriasis or other skin manifestations. Radiography showed bilateral sacroiliitis but no erosions of hands or feet. His serology was notable for a high-titer positive anti-SSA antibody of ??40 kU/L (ref. <7), a low-titer positive anti-dsDNA antibody of 14 kIU/L (<10), and a positive IgM-rheumatoid factor of 19 kIU/L (<10). Anti-CCP and HLA-B27 were negative, and proteinuria was not present. However, he had no symptoms of systemic lupus erythematosus or Sj繹gren syndrome. A diagnosis of spondyloarthritis was made, and his symptoms improved on NSAID and prednisolone. However, he developed diabetes mellitus, and sulfasalazine was prescribed. A week after starting sulfasalazine, he presented with erythema of the trunk and neck. A cutaneous drug reaction was suspected, sulfasalazine was immediately stopped, and methotrexate was prescribed instead. Three weeks after stopping sulfasalazine, he was admitted because of fever, cough, dyspnea, loss of appetite, night sweats, and weight loss. The erythema had spread to the extremities with a dark red, confluent maculopapular exanthema, and bullae and denudation of the epidermis in large patches of the back were now observed (). MRI of the sacroiliac joints and lumbar spine was consistent with bilateral sacroiliitis but showed no active inflammatory changes. His serology was negative for hepatitis B, hepatitis C, and HIV and not indicative of current infection with EBV, CMV, or parvovirus. Anti-dsDNA antibody had increased to 43 kIU/L, and he had proteinuria 0.7 g/day. He was managed with intravenous antibiotics and continued on prednisolone 7,5 mg qd, and his skin gradually improved. After 3 weeks, high fever recurred, erythroderma with small pustules on the trunk and extremities was observed, and he developed TEN-like lesions of 10% of body surface area (), mostly the back and nates. He was transferred to an intensive care unit due to Staphylococcus aureus sepsis and stabilized with intravenous immunoglobulin (IvIg), intravenous antibiotics, methylprednisolone 100 mg qd, and Flamazine cream. After recovering from the sepsis, PET-CT revealed multiple pathologically enlarged lymphatic glands with increased FDG uptake in the neck and axillary and inguinal regions, near porta hepatis, and in the retroperitoneum. A lymph node biopsy showed large areas of histiocytosis but was not diagnostic for dermatopathic lymphadenopathy or hemophagocytosis. Skin biopsy was consistent with TEN-like lesions, showing full thickness necrosis of the epidermis and pigment incontinence (). His mucous membranes were not involved. Bone marrow biopsy showed an increased number of myelopoietic precursor cells, sparse erythropoiesis, and slight interstitial infiltration of B and T lymphocytes but no malignancy. Renal biopsy showed focal mesangial hypercellularity and 1 of 15 glomeruli with sclerosis, a faint linear reaction for IgG, and a slight unspecific reaction in IgM and IgA but no complement deposition, and this was not found to be indicative of glomerulonephritis.
Daxton Tate	28	1987/5/13	(997)251-5095	[email protected]	7008 Karen Via Suite 058	Our patient is a 70-year-old Japanese man with diabetes. He was admitted to our hospital 4.5 years ago for further investigation and treatment of recent-onset insulin allergy. He had a 30-year history of type 2 diabetes mellitus and an approximately 6-year history of insulin injection administration, with an average glycated hemoglobin (HbA1c) level of 7.0 %. He reported a past history of myocardial infarction, hypertension, and dyslipidemia and had undergone coronary artery bypass grafting at 68 years. During that surgery, intravenous protamine sulfate was administrated, which induced anaphylaxis. At that time, 4.5 years prior to the current presentation, he was receiving 100 mg/day aspirin, 100 mg/day clopidogrel, 100 mg/day imidapril, 20 mg/day isosorbide mononitrate, 5 mg/day nicorandil, and 10 mg/day pravastatin, and these had been stable for more than 3 years. In addition, he was being treated with 20 U/day insulin lispro and 8 U/day insulin glargine, which had also been unchanged for approximately 3 years. Finally, he had been receiving 0.9 mg/day voglibose to supplement his insulin therapy for approximately 5 years. Approximately 5 months before his current presentation, he reported that he had started to develop wheals with redness and itching at the injection site, which occurred immediately after injecting insulin. Of note, these symptoms only occurred following the use of insulin lispro and not after insulin glargine. Over the subsequent months, part of the surface of the wheals had hardened and further investigation was performed. Two months before his current admission, blood samples were taken that revealed high serum levels of human insulin-specific immunoglobulin E (IgE; 4.21 U/mL; normal range <0.35 U/mL), hypereosinophilia (total white blood cell count, 6000/弮L; total eosinophil granulocyte count, 780; percentage, 13 %), and a high level of total IgE (403 IU/mL; normal range <173 IU/mL). A pathology sample was taken 2 months before his admission, which revealed eosinophilic infiltration in the hardened part of the injection site (Fig. ). Therefore, just before admission, he was diagnosed with insulin allergy and was switched from insulin lispro plus glargine to insulin aspart plus glargine. He was admitted to our hospital for treatment of his poor glycemic control and to evaluate his residual ability of insulin secretion. On admission, his physical examination was largely unremarkable (height, 157.1 cm; body weight, 53.0 kg; body mass index, 21.5 kg/m2; body temperature, 36.5 簞C; blood pressure, 104/62 mmHg; and pulse, 75 beats/minute, regular), although there was induration with slight redness of his abdomen. His laboratory data at that time are shown in Table . Of note, his fasting plasma glucose level was 346 mg/dL, and his HbA1c level was 12.3 %. In addition, we detected high serum insulin, anti-insulin IgG antibody, and anti-insulin receptor antibody levels. Although there were no further symptoms of local allergy (wheals, redness, and itching) after injecting with insulin aspart, his postprandial hyperglycemia continued at approximately 350 mg/dL. Therefore, he was switched to insulin glulisine (24 U/day) plus glargine (22 U/day) after admission. On day 17 of admission, his 24-hour blood glucose profile was 88, 241, 241, 295, 297, and 292 mg/dL at 7:30 a.m., 10:00 a.m., 11:30 a.m., 2:00 p.m., 5:30 p.m., and 8:00 p.m., respectively, with an energy intake of 1600 kcal. On days 18 and 19, his urinary excretion of C-peptide was 42.8 to 51.5 弮g/day (normal range 40 to 100 弮g/day). On day 19, a glucagon stimulation test was performed, and his serum C-peptide response ranged from 3.2 to 3.6 ng/mL (Table ). On day 22, although his high glucose levels persisted, he was discharged because we had confirmed his ability to secrete insulin. Although his hypereosinophilia was not resolved before his discharge, he had no further allergic symptoms (wheals, redness, and itchiness) at the injection site for glulisine, and the area of induration had almost disappeared. Figure summarizes his clinical course before and after the introduction of liraglutide. After discharge, although glulisine and glargine were continued, his glycemic control worsened, with fasting and postprandial hyperglycemia levels of approximately 200 and 300 mg/dL, respectively, and high serum insulin levels persisted (Fig. ). Therefore, 2 months after his admission, we added 0.3 mg liraglutide, discontinued the insulin glulisine, and added 2 mg/day glimepiride and 500 mg/day metformin to decrease his total insulin requirements. However, he also started to develop intermittent induration at the site of insulin glargine injection, and although there was no evidence of wheals or redness, his hypereosinophilia persisted. Based on these findings, we reduced the dose of glargine (32??2??6??0 U/day) and increased the dose of liraglutide (0.3??.6??.9 mg/day; Fig. ). This was followed by improvements in his HbA1c, serum insulin, and eosinophil levels; therefore, we decided to completely discontinue the insulin glargine after 3 months of the liraglutide treatment. On his new regimen of 0.9 mg/day liraglutide in combination with oral antidiabetic agents, his HbA1c level was maintained at approximately 7.0 %. The induration had almost completely disappeared 1 month after the insulin injections were discontinued. In addition, his body weight decreased by 1.7 kg after 2 months of liraglutide treatment. Table indicates his diabetes-related medical history. We also elucidated the changes in the characteristics of anti-insulin IgG antibodies before and after treatment with liraglutide. Scatchard analysis of the anti-insulin IgG antibodies in our case indicated that there was a high binding capacity and a low affinity constant for the high affinity sites before liraglutide therapy (Fig. ). After 1 year of treatment with liraglutide, the Scatchard plot indicated that the binding capacity decreased and the affinity constant for high affinity sites increased (Fig. ). Of interest, the anti-insulin receptor antibodies had completely disappeared within 1 year after the injection of liraglutide.
Skye Goodman	28	1979/4/11	791.620.1237	[email protected]	679 William Springs	The patient was a 65-year-old Japanese man. The cutaneous and inguinal specimens after fixation in 10 % neutral buffered formalin were embedded in paraffin for histological or immunohistochemical examinations. Furthermore, we prepared more than 10 step-serial H&E staining sections of the entire primary site (right sole), and absolutely confirmed the absence of residual foci of MM cells [, , ]. All immunohistochemical stainings were carried out using Dako Envision kit (Dako Cytomation Co., Glostrup, Denmark) according to the manufacturer? instructions. [?. We counted the numbers of CD4 (Dako, diluted 1:1)?nd CD8 (Nichirei Biosciences Inc., Tokyo, Japan, diluted 1:1)?ositive infiltrating T-lymphocytes in the cutaneous pigmented lesion (right sole) in 10 randomly selected fields of per section (original magnification: ?400) [?. All histological and immunohistochemical slides were evaluated by two independent observers (certified surgical pathologists in our department) [?. Agreement between observers was excellent (>0.9) for all antibodies investigated as measured by interclass correlation coefficient. For the few instances of disagreements, a consensus score was determined by the third board-certified pathologists in our department [?. All values are expressed as the mean 簣 SE. Significant differences were analyzed using Student? t test. Values of P < 0.05 were considered to be statistically significant. The patient had an unremarkable previous medical history, except for essential hypertension. The patient was admitted to hospital due to an approximately 2-months history of inguinal growing mass and pain. There had been no history of malignancy, immunosuppressive disorders, use of immunosuppressive medications, or unusual infections, including acquired immunodeficiency syndrome (AIDS). He had noticed a gradually growing, hemorrhagic and verrucous hypopigmented macule since approximately 2 years before, and it turned to be a solitary bluish to black patch with surrounding depigmentation, accompanied by recent decrease in size, measuring up to 20 mm in the right sole (Fig. a). There were no other primary cutaneous lesions identifiable by his history or careful physical examination that could represent the original MM. In parallel, he suffered from a rapidly growing and painful black-pigmented mass lesion with surface skin ulcer at the right inguen especially for recent 2 months (Fig. b), partly extending to the right scrotum and thigh (Fig. b). A pelvic CT scan showed a relatively well-defined huge and low-density mass with peripheral enhancement, measuring approximately 12 ? 10 cm (Fig. c). CT scans of the head, neck, chest and abdomen disclosed no definite evidence of neoplastic foci or other metastases in the lymph nodes or other organs. Laboratory data, including blood cell count, chemistry and tumor markers, were within normal limits. Within the post-operative 2 years with additional treatment of chemotherapy, including dacarbazine, nimustine, cisplatin, tamoxifen, (DAC-tam) and interferon-帣, recurrence of small (less than 5 mm) MMs in situ was noted in the right foot, waist and back at 1 month after the operation. But, surprisingly, the patient had neither local invasion nor metastases of the other sites, respectively, and he was alive and well. The surgical cutaneous specimen from the right sole (Fig. a) showed a hyperpigmented lesion made up of an aggregation of melanophages with surrounding variably sclerotic fibrosis mainly in the middle to deep layer of dermis. On high-power view, these aggregated round to oval cells had bland-looking small nuclei and abundant melanin-pigmented cytoplasm (Fig. b). In addition, reactive vascular networks were occasionally seen in these fibrous stroma, associated with mild interstitial and perivascular lymphocytic infiltrate (Fig. c). The covering non-disordered epidermis exhibited attenuated and atrophic change with absence of rete ridge, carrying no in situ MM cells within our thorough examination (Fig. a, c). Interestingly, the peripheral epidermis sometimes showed exocytosis of focal nested lymphocytes (Fig. c). Nevertheless, there was no MM cells infiltration from the epidermis to dermis, subcutis in all of the prepared step-serial H&E staining sections. Based on these features, we first diagnosed it as a benign cutaneous lesion, such as blue nevus. Immunohistochemically, these melanophages were strongly positive for CD68 (KP-1; Dako, diluted 1:100) (Fig. b), whereas completely negative for S-100 protein (Dako, diluted 1:900), HMB45 (Enzo Life Sciences Ltd., New York, diluted 1:100), and Melan A (NOVOCASTRA, 1:50) (data not shown). Furthermore, we counted the numbers of CD4- and CD8-positive infiltrating T-lymphocytes in this cutaneous lesion (Fig. d). Intriguingly, CD8-positive T-lymphocytes were markedly larger than CD4-positive lymphocytes in number (CD4: 1.8 簣 0.5 per 1 high power field vs. CD8: 54.6 簣 5.2 per 1 high power field; P < 0.0001) (Fig. d). Next, gross examination of the surgical specimen from the inguinal to scrotal and femoral mass showed that the huge, relatively well-demarcated and multi-nodular tumor, measuring approximately 13 ? 11 ? 7 cm, had gray-whitish to yellow-whitish cut surfaces with hemorrhagic and yellowish necrotic foci (Fig. a). Microscopically, the inguinal tumor consisted of a diffuse proliferation of markedly atypical large cells (Fig. b) having hyperchromatic, pleomorphic mono- to multi-nuclei, and abundant eosinophilic to sometimes clear cytoplasm, appearing predominantly as single cells, admixed with a large number of likely pre-existing lymphocytes (Fig. c). These proliferating tumor cells involved and erased the remarkably enlarged lymph node tissue, partly surrounded by fibrous capsule (Fig. b). On high-power view, the large tumor cells occasionally had melanin pigments in the cytoplasm and often showed autophagy (Fig. c). In immunohistochemistry, these malignant cells were specifically positive for melanocytic markers, such as HMB45 (Fig. d) and Melan A (Fig. d), and S-100 protein (data not shown), whereas negative for CD68 (data not shown). Based on all the clinicopathological features, we confirmed that these proliferating atypical cells were derived from MM, and finally made a diagnosis of complete regression of primary cutaneous MM on the right sole, associated with distant inguinal lymph node metastasis of MM.
Philip Pope	25	1987/5/11	529.515.1841x34364	[email protected]	371 Lopez Passage	A 20-year-old woman with diagnosis of ASs was referred to our department for reduced vision in her left eye (LE) of 2 weeks onset; she was negative for pseudoxanthoma elasticum or any other systemic disease. The patient signed a comprehensive consent form according to Good Clinical Practice guidelines, before proceeding with all examinations and treatments. Her best-corrected visual acuity (BCVA) was 20/20 in the right eye (RE) and 20/100 in the LE, with no signs of inflammation in the anterior chamber and vitreous of either eye. Fundus biomicroscopy revealed ASs in both eyes (BE). RE had no evidence of inflammation at the fundus observation (Fig. ). Interestingly, only the LE had multiple discrete grey-white lesions (dots) scattered over the fundus, from the paramacular area up to the mid-periphery, and the macula had a granular appearance (Fig. ). Fluorescein angiography (FA) (HRA, Heidelberg Engineering, Heidelberg, Germany) indicated mild optic disk leakage with some hyperfluorescent changes scattered throughout the fundus (paramacular area and mid-periphery). No CNV was detected (Fig. ). Indocyanine green angiography (ICGA) (HRA, Heidelberg Engineering, Heidelberg, Germany) disclosed late hypofluorescent lesions scattered at the posterior pole and in the mid-periphery (Fig. ); we interpreted these as signs of zonal outer retinal inflammation. Spectral-domain optical coherence tomography (SD-OCT) macular scans showed disruption in the photoreceptor layer (Fig. ). Automated static threshold perimetry indicated visual field defects mainly located paracentrally and temporally (Fig. ). On the basis of all these findings a diagnosis of ASs and coincident MEWDS was made. The patient was prescribed oral prednisone (1 mg/kg) for 7 days then half the dosage for another 7 days. Two weeks later, LE BCVA improved up to 20/25, with resolution of the MEWDS findings, except for the granular appearance at the macula; RE BCVA was 20/20 with no signs of inflammation at the fundus evaluation. Two months later, the patient returned because of sudden vision loss in her LE (20/100). FA indicated CNV in the paramacular area in LE. FA and ICGA showed no signs of choriocapillaris inflammation in BE (Fig. ). We proposed to the patient an intravitreal injection of ranibizumab as an off-label treatment option. She signed informed consent and was given a single injection of ranibizumab (0.5 mg/0.05 mL) following the normal procedure. One month after the injection BCVA improved up to 20/40, the CNV showed regression (Fig. ), and there was no need for retreatment up to her latest follow-up visit 1 year after the injection, when BCVA had improved up to 20/25. ASs are often complicated by the appearance of CNV [, ]. To date there are no reports of AS associated with acute ocular inflammation. MEWDS is a unilateral inflammatory disease, which usually resolves spontaneously, with full recovery []. Here we describe a patient with ASs, who was diagnosed with coincident MEWDS. Two months after this diagnosis she developed CNV, which was effectively treated by intravitreal ranibizumab. In the current literature there are only six cases of CNV that developed after (from 4 weeks to 13 years) the diagnosis of MEWDS [?? ]. Only two were effectively treated by intravitreal anti-vascular endothelial growth factor (VEGF) [, ]. In all other cases the visual loss was permanent despite treatment [?. The case described is unusual: the CNV occurred in a patient with ASs, 2 months after the diagnosis of MEWDS. Although both ASs and MEWDS may contribute to the onset of CNV, the patient? young age, good prognosis after intravitreal ranibizumab and the presence of MEWDS involving the macular area may indicate an inflammatory etiology of the CNV. Though the pathophysiologic mechanism remains unclear, it has been suggested that alterations of Bruch? membrane or the outer retinal barrier caused by choroidal inflammation may be associated with ischemic and/or inflammatory CNV []. It has also been hypothesized that the inflammatory processes induce the release of chemokines that favor angiogenesis []. This patient was in fact younger than the mean age at which CNV usually develops associated with ASs [, ]. Moreover, in this case a single intravitreal ranibizumab injection had a lasting effect (up to at least 1 year), as shown by FA and OCT, and by the maintenance of good BCVA. Our results are in agreement with Rouvas et al. who have described the good responses to intravitreal treatment with ranibizumab for inflammatory retinal diseases []. This might be explained by the different nature of the CNV, in which inflammation may have played an important pathogenic role, compared to other CNVs secondary to ASs which tend to be associated with a worse visual prognosis, and need more injections [].
Aurelia Rosas	19	1994/5/6	448.903.7196x996	[email protected]	631 Donald Dale Apt. 937	The patient, a 16.3 year-old girl, was in excellent health, both physically and emotionally, with no known adverse drug reactions or allergies and good oral hygiene. The major concern of both the patient and the referring general dentist was the persistence of several deciduous teeth. Possible systemic, endocrine, metabolic or genetic-syndromal disorders as possible etiologic factors could not be detected by a physician and endocrinologist, to which the patient was referred prior to orthodontic examination. In addition, there was no family history of eruption failure of permanent and persistence of deciduous teeth. The first deciduous upper molar on the right side (tooth 54) was extracted 1 year earlier by the referring general dentist, but no spontaneous eruption of the permanent first premolar could be achieved within this timeframe (Table ). Thus and due to no previous referral by the general dentist or presentation of the patient for orthodontic planning, treatment was started quite late. The clinical examination and study model analysis (Figs. and ) in conjunction with the panoramic radiograph (Fig. ), taken by the referring general dentist, showed initially nine persisting deciduous teeth ??all deciduous canines and molars of the upper jaw and those on the right side of the mandible, minus the already extracted upper right deciduous molar. All upper permanent canines and premolars were impacted and malpositioned. The lower right canine, which was severely rotated, was also impacted to a higher and the lower right first and second premolars to a lesser degree, whereas the corresponding teeth on the lower left side were in good occlusion and showed no signs of impaction. No adequate root resorption of the deciduous teeth could be detected radiologically with exception of the lower deciduous molars on the right side. Radiographically the impacted teeth showed no evident signs of ankylosis. Some physiological spacing of the incisors was present and both the first permanent molars as well as the deciduous canines were in good occlusion and had a class I relationship with a shallow curve of Spee in both jaws. The second permanent molars were also in occlusion and all four third permanent molars present radiologically within their germ stage. No reduction of the extraction space due to a mesial or distal drifting of the neighboring teeth was evident. The patient also showed a lower alveolar midline deviation to the right side (about 2 mm), most likely due to the unilateral failure of eruption at the lower right side, which caused a more mesial eruption and inclination of the lower left permanent canine and the lower incisors, as evidenced by the gap formed between the canine and lower left premolar. 3D CBCT diagnostic imaging was declined by the patient and her guardian due to concerns about a potential health risk by the radiation exposure. The cephalometric analysis according to Ricketts (Table , Fig. ) revealed a vertical dolichocephalic growth type, hyperdivergent jaws and a large mandibular plane angle, thus a further vertical development had to be avoided during treatment. In addition, the mandible was found to be prognathic with an ortho- to retrognathic upper jaw, resulting in a slight skeletal class III, which was dentally compensated (class I molar relation). Both approximately normally inclined upper and lower incisors were distinctly protruded, whereas Ricketts??E-line indicated a retrusion of the upper lip due to the proganthic lower jaw. Furthermore, overjet and overbite were increased. The radiologically evident complete root development of all permanent impacted teeth with closed apices in conjunction with the persisting deciduous teeth without distinct signs of root resorption in absence of genetic-systemic causes formed the basis for our diagnosis of idiopathic multiple bilateral impactions, confirmed by the failure of spontaneous eruption of the first upper left premolar after extraction of the corresponding deciduous predecessor. The main treatment objective was to align the nine impacted teeth at the occlusal level by means of controlled guided traction after their surgical exposition and ligation and after extraction of the preceding deciduous teeth. Secondary objectives were a levelling of the arches, a correction of the lower dental midline shift, establishing a physiological overjet and overbite and achieving a stable and functional occlusion. We opted for treatment with a fixed multi-bracket appliance in conjunction with the Easy-Way-Coil System??(EWC) []. To minimize treatment time, a simultaneous alignment of all impacted upper six permanent teeth was planned (4 weeks discrepancy in start of treatment between right/left side). Vertical and horizontal relations as well as dental class I were to be kept stable during treatment and the risk of root resorption at the second incisors was minimized by excluding them from the appliance. Due to the higher treatment complexity of the maxilla, treatment within the mandible was started at a later date. The patient and her legal guardians were informed and motivated about the necessity of good oral hygiene and compliance during the treatment as well as about the expected duration of treatment and the risks involved - particularly root resorptions and failure of eruption, which would necessitate a later implant and restorative treatment. Informed consent was obtained and ethical regulations according to the Declaration of Helsinki (1964) and its later amendments were observed at all times. The best choice of treatment was discussed and considered both with the patient and an oral surgeon and a combined surgical-orthodontic approach with simultaneous traction of the impacted teeth by means of an Easy-Way-Coil??was deemed to be most promising and time-efficient. The most simple approach to treat multiple impactions would have been to just extract the persisting deciduous teeth and wait for a spontaneous eruption. However, the advanced age of the patient, the complete root development and the already failed attempt by the general dentist made a success of this approach seem unlikely. An alternative method described by Schmidt and Kokich [] to extract the deciduous teeth and only expose the impacted teeth surgically without traction to facilitate spontaneous eruption, which for canines usually occurs after 6?? months [], was rejected by the patient for cosmetic reasons. This also excluded the possibility of a surgical luxation of the impacted teeth to facilitate tooth movement. In addition, the impactions were considered to be too deep for an exposure without traction to be successful. For this reason, we also opted for a closed instead of an open eruption technique in this case [, ]. If the impacted teeth were ankylosed, an extraction and consecutively implant-restorative treatment would be indicated. This option was considered as potential alternative, if the primary therapy failed in case of confirmed or developing ankylosis or invasive cervical root resorption during treatment, whereas at baseline no ankylosis of the impacted teeth could be confirmed. In this case a mesialisation of the upper permanent molars by means of skeletal anchorage and a mesial sliding appliance [] could have been used to reduce the resulting gaps, enable an occlusal alignment of the third molars and reduce the number and extent of necessary implants and restorative treatment. A sequential extraction of persisting deciduous teeth [] and also sequential traction of the impacted teeth individually as described by Krey et al. [] could have reduced the risk of extensive tooth loss in case of alignment failure. This approach, however, was rejected due to the unwillingness of the patient to accept prolonged treatment times, her already advanced age for treatment and a persisting deciduous dentition. The patient rather preferred implant-restorative treatment in case of treatment failure. At the beginning of treatment brackets (slot 0.018?? were bonded only to the upper central incisors and bands were placed on the first molars. These were connected by a transpalatinal arch (Burstone, 0.032??? 0.032??stainless-steel) for anchorage. Second molars were stabilized by connecting them with the first molars with a passively bonded partial arch-bow (0.017??? 0.025??stainless-steel) (Fig. ). At the level of the second transversal palatal ridges one mini-screw of the BENEfit??system (2.0 ? 11 mm, dental line, Birkenfeld, Germany) [] was inserted into the palate to carry a screwed standard abutment with a welded 1.1 mm stainless-steel wire. This wire was shortened, bent to the upper central incisors in shape of a U and bonded to their palatal surface (Fig. ). The upper central incisors and first molars were then connected with a 0.017??? 0.025??stainless-steel arch-wire (straight-wire technique) within a passive closed coil spring (Fig. ). The lateral incisors were intentionally left out of the fixed appliance to avoid any root movement and contact with the impacted canines. Treatment was started at the upper right quadrant. After extraction of the persisting deciduous teeth a mucoperiosteal flap was raised and the impacted permanent teeth were surgically exposed up to the assumed cemento-enamel junction. Three EWC??appliances were bonded to the individual teeth at the palatal surface (canine) and at the buccal surfaces (premolars) and directed towards the arch-bow used for anchorage (Fig. ). The mucoperiosteal flap was then repositioned and fixated with surgical sutures (closed eruption technique). After 1 week the sutures were removed and the EWC??springs activated. The direction of traction was chosen to be disto-buccal for the canines and buccal for the premolars. A suitable anchorage spot was chosen on the circular arch-bow and a gap introduced into the passive closed coil spring by gently closing a ligature cutter at this location. This way a displacement of the ligature wire used for connecting the EWC??spring with the arch-bow was effectively prevented. The correctly aligned EWC??springs were then shortened to create a gap of exactly 2 mm (canine) and 1 mm (premolars) between arch-bow and the spring. By gently closing the ligature cutter 1 mm proximal to the shortened end of the EWC??spring, an eyelet consisting of the terminal three coils was formed, which was connected seamlessly to the gap formed at the arch-bow coil spring by means of a ligature wire (Fig. ). The EWC??spring activation of 2 and 1 mm ensured a predictable force level of 0.32 N and 0.16 N, respectively. Four weeks later the same procedure was performed at the contralateral upper left jaw side (Fig. ). At the same time the EWC??springs at the right side were reactivated by clipping 2 mm (canine) and 1 mm (premolars) off the springs at their anchorage point and re-fixating them with a ligature wire (Fig. ). This procedure was repeated every 4 weeks at both upper jaw sides until the impacted teeth erupted. Then the EWC??springs were removed and lingual buttons attached to the occlusal surfaces of the premolars (Fig. ) and buccal surfaces of the canines (Fig. ). The teeth were then further moved in buccal direction by means of a PowerTube??5 (Ormco B.V., Netherlands) until brackets could be bonded to the labial surfaces (Fig. ). Final extrusion and levelling of all impacted teeth was achieved by a superelastic Nickel-Titanium (NiTi) arch-wire initially (Fig. ) and subsequent rigid TMA (Titan-Molybdenum-Alloy) arch wires. After the deciduous teeth had been extracted in the lower jaw, the permanent premolars at the lower right side erupted spontaneously (Fig. ). A fixed multi-bracket appliance was bonded in preparation for aligning the impacted lower right canine, which was performed in analogy to the technique used in the upper jaw (surgical exposure, 0.017??? 0.025??stainless steel anchorage arch-bow with passive closed coil spring, EWC??system and 2 mm activation). To prevent intrusion and tipping of the incisors at the lower right side, the continuous arch-wire was separated and only the lateral tooth segment used for anchorage (Figs. and ). After removing the upper skeletal anchorage, remaining spaces in the upper dental arch were closed and incisors slightly re- and intruded with a T-loop bow (0.017??? 0.025??TMA, Fig. /i) to achieve physiological overjet and overbite. The lower alveolar midline deviation was corrected during levelling by uprighting the mesially inclined lower right canine into the gap to the first premolar with consequent distalisation of incisors aided by a buccal power chain placed on top of the arch-bow from the lower right second incisor to the left first premolar (Fig. ). Total treatment time was 22.8 months. The required time for aligning the upper jaw from surgical exposure to finishing with a 0.017??? 0.025??TMA arch-bow was 19.6 months with a total 26 sessions in the office. The corresponding treatment time for the lower jaw was 20 months with a total of 19 office visits. All impacted teeth could be successfully aligned within the levelled dental arches. Class I canine and molar relation were maintained at both sides (Figs. and ) and the patient (19.7 years) was happy with the treatment result. The cephalometric analysis after the end of treatment (Table , Fig. ) as well as the superimposition of pre- and posttreatment tracings (Fig. ) showed a slight intensification of the mesial skeletal tendency to a skeletal class III jaw relation (dentally compensated), caused by anterior rotation of the mandible, whereas the vertical hyperdivergence of the upper and lower jaw decreased by posterior rotation of the maxilla and anterior rotation of the mandible. The protrusion of the upper, but not lower incisors, both normally inclined, was reduced during treatment. Upper incisors were intruded. The lower alveolar midline deviation could be successfully corrected and a physiological overjet of 2.5 mm and overbite of 2.2 mm was achieved. The retrusion of the upper lip remained unchanged during treatment due to the still prognathic mandible. For retention purposes, the patient was instructed to wear a removable Hawley-retainer for 24 h a day within the first 6 months and then to gradually reduce wear time (only at night, every second night etc.). In addition, a coaxial annealed retainer wire (Penta-One??0.0215?? Masel Orthodontics, Carlsbad, USA) was bonded to the palatinal surfaces of the lower incisors and canines due to the severe rotation of the aligned lower right canine and thus increased risk of rotational relapse []. A total of nine deciduous teeth were extracted and seven permanent teeth surgically exposed, ligated and aligned. The two initially impacted permanent premolars at the lower right side erupted spontaneously after extraction of the deciduous molars and did not require additional traction. Post-treatment clinical examinations and radiographs (Fig. ) showed no indications of avital or traumatized teeth, root resorption, periodontal problems (probing depths < 3 mm for all teeth and locations) or other iatrogenic damages. At the end of treatment the patient was referred to the oral surgeon for possible extraction of the third molars, since proper eruption of the lower right molar seemed unlikely due to the radiographically evident lack of space and problematic angulation.
Remi Coleman	38	1993/12/11	804-452-5531	[email protected]	47369 Reyes Park Apt. 708	A 23-year-old woman, gravida 1, para 0, at unknown gestational age presented to our emergency department with lower abdominal pain, vaginal spotting, and lightheadedness for 2 days. The patient? medical history was significant for morbid obesity with body mass index, history of Chlamydia infection and pelvic inflammatory disease at age 15, and laparoscopic cholecystectomy at age 19. Physical examination including a transvaginal ultrasonographic evaluation revealed a ruptured ectopic pregnancy. The patient was hemodynamically stable and underwent a laparoscopic right salpingectomy. The procedure was performed in a standard ?ow??lithotomy position (using Allen簧 stirrups). Care was taken not to flex hips or knees beyond 90簞, with hip abduction less than 45簞 and neutral hip rotation (Fig. ). The patient was in Trendelenburg position to allow for adequate visualization of the pelvis. Pneumatic compression devices on both calves were in place throughout the procedure. Skin-to-skin surgical time was 90 min due to the need for lysis of omental adhesion and the presence of pelvic adhesive disease consistent with the patient? prior surgical and gynecologic history. Intraoperative findings and pathologic evaluation confirmed the diagnosis of a ruptured ectopic pregnancy. Immediately upon awakening from general anesthesia, the patient complained of severe bilateral calf pain. Initial evaluation of the lower extremities revealed no compression marks, ecchymoses, erythema or edema, and the peripheral neurovascular exam was unremarkable. Serum electrolytes were within normal limits. Serum creatine kinase (CK) was elevated at 22,760 units/L (Norm: 38??76 units/L), consistent with rhabdomyolysis. An urgent bilateral lower extremity Doppler ultrasound was obtained which ruled out a deep venous thrombosis. The patient was treated symptomatically with analgesics and muscle relaxants. As her pain continued to escalate, a concern for acute compartment syndrome (ACS) was raised and the orthopedic surgery service was consulted. Based on the high level of suspicion for ACS in light of the clinical exam and exacerbating pain out of proportion, the patient was taken back emergently to the operating room by the orthopedic surgery team for bilateral lower extremity four-compartment fasciotomies. Surgery was accomplished approximately 8 h after her initial laparoscopic surgery. Intraoperatively, the diagnosis of ACS was confirmed. All muscles in the four compartments in bilateral legs were viable on clinical exam and bovie stimulation testing, without any signs of muscle necrosis. The patient required two additional staged surgical procedures for scheduled 2nd looks, soft tissue debridement and delayed primary wound closures. Her serum CK decreased to 1278 units/L prior to discharge and she did not sustain any renal failure or crush syndrome throughout the hospitalisation. The patient? serum creatinine remained in a normal range of 0.6??.8 mg/dL (Norm: 0.5??.0 mg/dL). She was discharged home on postoperative day four after definitive wound closure, with home physical therapy arranged. She had an uneventful further recovery, with pristine wound healing, no infection and a normal neurovascular status on bilateral lower extremities on follow-up exam. By 3 months after discharge from the hospital, she had regained full unrestricted function and a normal quality of life. We present a rare case of bilateral lower extremity acute compartment syndrome that developed in a healthy 23-year-old female after undergoing a laparoscopic salpingectomy for ruptured ectopic pregnancy in the usual lithotomy position. This case highlights a rare complication that has been previously associated with lithotomy or hemilithotomy positioning [, , , ]. Lower extremity ACS is a pathologic condition in which increased tissue pressure within a closed osseofascial space compromises blood circulation and normal function of tissues within the compartment leading to tissue hypoxia and necrosis [, ]. If left untreated, patients with ACS can develop muscle contracture, sensory deficits, paralysis, possible need for limb amputation, and potentially multi-system organ failure secondary to crush syndrome [, ]. The osseofascial lining of the four lower extremity compartments (anterior, lateral, superficial and deep posterior) form an enclosed environment of muscle, blood vessels and nerves with limited ability to expand or accommodate increased volume or pressure [, ]. ACS in the postoperative state occurs as a result of intraoperative compression with a prolonged decrease in arterial pressure and/or increase in venous pressure, followed by reperfusion []. Local ischemia can subsequently damage endothelial cells resulting in leakage of proteins and fluid into the interstitial space. The subsequent increase in interstitial pressure elevates compartmental pressures, thus perpetuating the cycle of hypoperfusion and tissue ischemia []. Specifically, in the lithotomy position, ischemia occurs from compressive forces of the external leg holsters and diminished blood flow from leg elevation and kinking of the popliteal artery, leading to ischemia/reperfusion injury with subsequent compartment syndrome []. When the lower extremities are taken out of lithotomy position and elevation, limb reperfusion occurs and can lead to injury with the formation of oxygen free radicals and cytokines that perpetuate endothelial damage and interstitial edema []. In addition to lithotomy position, other risk factors associated with ACS after surgery include: ankle dorsiflexion, Trendelenburg position, leg holders, length of surgery greater than 2 h, intraoperative hypotension or hypovolemia, and epidural analgesia []. Bauer and colleagues recently published two articles documenting cases of lower extremity ACS following gynecologic surgery in the lithotomy position and found that the vast majority of ACS cases occurred following procedures lasting longer than 4 h [, ]. Other studies have demonstrated that calf compartment pressure increases at a rate of 1.1 mmHg per hour in the lithotomy position []. In addition, elevation of the leg above heart level (?igh??lithotomy positon) has been associated with increased risk of compartment syndrome []. Intermittent pneumatic compressive devices used for the prevention of deep venous thrombosis intraoperatively, as applied in the patient presented in this case report, have also been identified as causative factors for ACS []. We postulate that lithotomy with adjunctive Trendelenburg position was likely the contributory root cause of bilateral lower extremity ACS in the young 23-old patient described in this case report. Multiple technical tricks have been described to prevent ACS in lithotomy position, including intraoperative repositioning of the legs, avoiding pressure in the popliteal fossa and kinking of the popliteal artery by avoiding knee flexion beyond 90簞 [, ]. Lower extremity ACS remains a clinical diagnosis, and immediate recognition and surgical management are paramount in avoiding long-term impairment and poor outcomes []. The most common clinical symptom is represented by uncontrolled pain out of proportion and exacerbated pain by passive stretch of the toes and ankle joint []. Measurement of the compartment pressure can aid in the diagnosis, albeit there is dispute among orthopedic surgeons regarding the pressure threshold for the diagnosis of ACS and subsequent need for fasciotomy. The most effective method of diagnosis of ACS remains a clinical diagnosis requiring a high level of suspicion by the treating physician. Given the short duration of our patient? procedure and lack of risk factors with the exception of lithotomy position and her full recovery after immediate recognition and surgical management, our case highlights the imperative of understanding the pathophysiology of ACS in lithotomy position and raising a high level of suspicion in a patient with postoperative calf pain after gynecological procedures.
Julia Barnett	41	1994/11/28	+1-317-889-1058x0747	[email protected]	104 Hanson Mills	A 60-year-old man who was admitted with persistent diarrhea and a 10 kg weight loss in 17 months. After a tentative diagnosis of coeliac disease he was followed in another hospital for 8 months. His laboratory data revealed hypoproteinemia and elevated soluble interleukin-2 receptor (sIL2R). Anti-gliadin and anti-transglutaminase antibodies, serum-specific markers for coeliac disease, were negative []. Abdominal computed tomography (CT) revealed thickening of the jejunal and ileal walls and mild dilatation. On upper GI endoscopy, a reddish depressed lesion with erosion was seen in the gastric body; in the second portion of the duodenum, the edematous mucosa was accompanied by villous atrophy (Fig. ). Upper GI endoscopy with narrow-band imaging (NBI) showed an edematous mucosa, while magnifying endoscopy with NBI revealed a cerebriform or flat pattern with villous atrophy in the second duodenal portion (Fig. , ). Capsule endoscopy demonstrated a diffuse edematous and granular mucosa with villous atrophy and circumferential ulcers in the jejunum and ileum. On lower GI endoscopy, an edematous mucosa with small erosions at the ileocecal valve were present (Fig. ) whereas the findings along the whole colorectal mucosa were nearly normal. Biopsy specimens from the gastric, duodenal, jejunal, and cecal mucosae showed diffuse intramucosal invasion by small to medium-sized atypical lymphocytes with irregular hyperchromatic nuclei, in addition to many atypical IELs (Fig. , ). Immunohistologically, the atypical lymphocytes were positive for CD103, TCR-帣F1, CD3, CD7, CD8, CD56, and TIA1. Other biopsy specimens from the duodenal second portion showed villous atrophy and chronic inflammatory infiltrates. The T-IELs comprising the infiltrates were positive for CD103, CD3, CD7, CD8, and TIA1 but negative for CD56 and CD5. Evaluation of the colorectum showed colitis with an increased number of T-IELs, all of which were of the same phenotype (Fig. ). A bone marrow biopsy revealed scattered infiltrates of CD103-, CD3-, CD8-, CD56-positive and CD5-negative atypical lymphocytes; these accounted for ~30 % of the nucleated cells. The patient was treated with a chemotherapy regimen of CHASE (cyclophosphamide, cytarabine, etoposide, and dexamethasone) and underwent autologous stem cell transplantation. Despite a partial response to chemotherapy, he died of sepsis 3 years after disease onset.
Stephen Crawford	38	2000/7/29	+1-247-630-5330x415	[email protected]	68529 Garcia Common Apt. 713	A 40-year-old woman who was admitted with diarrhea and a weight loss of 6 kg in 3 months. Her laboratory data revealed hypoproteinemia and an elevated sIL2R level. On upper GI endoscopy, the gastric mucosa was nearly normal in its appearance whereas the mucosa of the duodenal second portion was edematous. Antegrade double-balloon endoscopy of the duodenal third portion and the jejunum showed a diffuse edematous mucosa, erosion, and ulcerative tumors in both. On lower GI endoscopy, an edematous mucosa and reddish polypoid lesions were seen in the terminal ileum together with an edematous colorectal mucosa. Biopsy specimens from the duodenal third portion, intestine, and colon showed a diffuse, intramucosal infiltrate of medium-sized atypical lymphocytes with coarse chromatin together with many atypical IELs. Other biopsy specimens from the duodenal second portion (Fig. , ) and intestine revealed chronic inflammatory changes with villous atrophy and an abundance of CD103-, CD8-positive, CD56-negative T-IELs. Scattered infiltrates of CD3-, CD4-, and CD8-positive, CD103- and CD56-negative lymphocytes were identified on bone marrow specimens but there was no evidence of bone marrow invasion. She was treated with a chemotherapy regimen of THP-COP (pirarubicin, cyclophosphamide, vincristine and prednisolone) but 10 months after the initial diagnosis was readmitted to our hospital with acute abdominal pain due to intestinal perforation. A partial jejunal resection revealed multiple and circumferential ulcerated tumors with or without perforation; the mucosa surrounding the tumors was characterized by fold thickening with granular changes (Fig. ). Histologically, a diffuse infiltrate of medium-sized atypical lymphocytes accompanied by atypical IELs was present in the mucosa, in the peripheral zones of the tumors (Fig. ). Enteropathy-like lesions with villous atrophy and an abundance of IELs were evident in the mucosal layer outside the main tumors. Preoperative biopsy specimens obtained from the ascending colon to the rectum revealed colitis, with increased CD3- and CD8-positive and CD56-negative T-IELs (Fig. , ). She died of the disease 2 months after surgery.
Aubree Singh	39	2002/2/20	452.293.3862x02255	[email protected]	02640 Tucker Bridge Suite 155	A 50-year-old woman who was admitted to our hospital for abdominal distension. Her laboratory data revealed an elevated sIL2R. On abdominal CT, long segmental thickening of the jejunal and ileal wall with dilatation was evident. Upper GI endoscopy revealed an edematous and reddish granular mucosa with white villi in the duodenal second portion (Fig. ). On lower GI endoscopy, a diffuse granular mucosa with villous atrophy in the terminal ileum, an edematous mucosa with multiple erosions in the ascending and sigmoid colon, and reddish longitudinal ulcers in the rectum were seen (Fig. ??. Biopsy specimens from the duodenal second portion, intestine, and colorectum revealed a diffuse intramucosal infiltrate of medium-sized atypical lymphocytes with many atypical IELs (Fig. , ). Other biopsy specimens from the duodenum, jejunum, and colorectum indicated chronic inflammatory changes of the propria mucosae and increased CD3- and CD8-positive, CD56-negative T-IELs. An invasion by CD3-, CD8-, and CD56-positive atypical lymphocytes was seen on the bone marrow specimens. She was treated with cyclophosphamide, doxorubicin, vincristine, and prednisolone and high-dose methotrexate/cytarabine followed by allogeneic stem cell transplantation. Despite a partial response to chemotherapy, she died 9 months after disease onset.
Louis Hubbard	29	1983/4/16	737.962.2658	[email protected]	16952 Steven Road	A 70-year-old man admitted for nausea. His laboratory data revealed pancytopenia and hypoproteinemia. Helicobacter pylori IgG antibody was positive in serum. Abdominal CT showed a 30-mm-diameter tumor in the duodenal second portion and thickening of the jejunal wall. On 18F-fluorodeoxyglucose (FDG) positron emission tomography, there was FDG uptake in the duodenal second portion. Upper GI endoscopy revealed pinhole-like severe stenosis, a submucosal tumor at the anal side of the stenosis, and edematous mucosa in the duodenal second portion. Lower GI endoscopy showed a granular mucosa of the terminal ileum and edematous or normal-looking mucosa in the colorectal mucosa. Duodenitis with villous atrophy and abundant IELs was evident on biopsy specimens from the duodenal second portion taken outside the tumor. The tumor itself consisted of small to medium-sized atypical lymphocytes both among the epithelial cells and in the mucosal layer. The cecal mucosa was characterized by a severe infiltrate of atypical IELs (Fig. ). Other biopsy specimens from the duodenum, descending colon, and rectum showed chronic inflammatory changes with increased CD3- and CD8-positive, CD56-negative T-IELs (Fig. , ). Infiltrating atypical lymphocytes of the bone marrow were positive for CD3, CD8, CD103, and TCRC帠M1, but negative for CD56. He was treated with a chemotherapy regimen of SMILE (dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide). After chemotherapy, abdominal CT revealed a reduction of the tumor. Despite a partial response to chemotherapy, he died of sepsis 9 months after disease onset.
Rosie Long	45	1984/4/7	(605)855-2969x080	[email protected]	59420 Garza Course	A 47 year-old female had been complaining of asthenia and a significant weight loss in the previous 2 months. She was smoker (about 20 cigarettes per day) and had no underlying diseases such as diabetes or hypertension. Test to detect Helicobacter pylori was negative. After gastroendoscopy and other explorations, such as computerized axial tomography scan, histological analysis of the tumour confirmed the diagnostic of gastric adenocarcinoma: diffuse adenocarcinoma with signet-ring cells. The proband died during the study. Some of her relatives presented previous history of malignant colorectal polyp (one sister), hyperplastic gastric polyp (a nephew) or gastric cancer (great-uncle) (Fig. ). Thus, search for CDH1 germline mutations was conducted in the patient and in other members of the family (three sisters, a great-uncle and a nephew of the proband), after genetic counseling and informed consent. In order to determine the expression level of the protein, paraffin tissue samples were subjected to immunohistochemical staining with monoclonal antibody against E-cadherin (clone 36B5, Leica Biosystems, UK) in an automated Bond system in combination with Bond Polymer Refine Detection (Leica Biosystems, UK) according to manufacturer instructions. Genomic DNA extraction was carried out from peripheral blood by using QIAamp DNA Blood Mini Kit, and from paraffin embedded tissue (from proband? gastric biopsy) with QIAamp DNA FFPE Tissue Kit; following manufacturer instructions. In the proband, RNA was also isolated from peripheral blood with QIAamp RNA Blood Mini Kit and from tissue using RNeasy FFPE Kit, and then stored at ??0 簞C. The CDH1 gene was studied by analyzing the entire coding sequence, including intron?xon boundaries, by PCR and sequencing. The presence of the mutation was confirmed at RNA level in the proband material. Pathogenicity of the new variant was predicted by in silico analysis with bioinformatics tools such as sorting intolerant from tolerant (SIFT) and Mutation Taster. ExAC browser of Broad Institute, 1000 Genomes database and dbSNP138, as well as, the human gene mutation database (HGMD), Leiden open variation database (LOVD) and ClinVar databases were checked to assess the presence/absence of detected alterations in variations repositories. A novel heterozygous deletion in exon 9 of CDH1 gene (NM_004360.4:c.1220_1220delC; NM_004360.4 (CDH1_i001): p.(Pro407Glnfs*10)), has been found in the proband and subsequently in other family members. This variant was not found in 100 healthy controls and it is not present in 1000G, ExAC and dbSNP, pointing out that this variant is not common in population. To the best of our knowledge this variant has not been previously described, and it is not included in ClinVar, HGMD or LOVD. Variant has been included in ClinVar database (SCV000266475). This mutation, generates a premature stop codon at position 407 giving rise to a truncated protein, that leads to a pathogenic variant (Corso et al. ). The presence of the mutation was corroborated both, at DNA and RNA level in peripheral blood. The mutation was not found in 100 control samples. The variant was considered pathogenic/disease causing by in silico predictors SIFT and Mutation Taster. Furthermore, this variation is considered to be pathogenic, according to American College of Medical Genetics and Genomics (ACMG) interpretation: null variant in a gene where loss of function is a known mechanism o disease, absent in population databases, protein length changing variant, co-segregation with disease in multiple affected family members in a gene definitively known to cause the disease, and patient? phenotype highly specific for gene (Richards et al. ). One of the sisters (subject IV-2), who was found a malignant colorectal polyp at the age of 49, presented also the deletion, and her son (nephew of the proband; (subject V-1)) harboured also the mutation and had been previously diagnosed with hyperplastic gastric polyp. Neither of the other relatives tested harboured the mutation and they were healthy, but for the great-uncle (subject III-1) who had been suffered indeterminate-type GC and did not present the mutation. There were other relatives which had been suffered hyperplastic colon polyps (subjects II-4; III-10), GC (subject II-2; III-2), and other kind of cancer (subjects II-3; III-7), but samples were not available for this study. The sister, carrier of the mutation, and asymptomatic at the time of this study, underwent PTG. Her son (subject V-1), refused the procedure and is under annual endoscopic surveillance (recently, hyperplastic gastric polyps has been detected). It was not possible to establish the origin of this mutation with the data collected. It should be necessary to test more relatives in order to determine in which point its origin is. In the tumor cells of proband? gastric biopsy, the immunohistochemical expression of E-cadherin was absent or frankly reduced. In contrast, normal continuous membranous staining for E-cadherin was evidenced in gastric tumor cells of great-uncle (Fig. ).
Jace Hughes	26	1982/12/24	233.650.5852x1913	[email protected]	0452 Vasquez Terrace	A 59-year-old man was admitted to our hospital for the treatment of a rectal tumor. The tumor was found via colonoscopy during an examination at a medical checkup. The colonoscopy revealed an ulcerated tumor at the anterior rectum, 4 cm from the anal verge. Histopathologic examination revealed moderately differentiated adenocarcinoma. CT and MRI scans showed that the rectal tumor was contiguous with the prostate and the left seminal vesicle. PET-CT showed no evidence of metastasis. The carcinoembryonic antigen (CEA) level was elevated at 105.1 ng/mL. After neoadjuvant chemoradiotherapy (CRT) (S-1 100 mg/m2/6 weeks, 2 Gy*25/5 weeks = 50 Gy), CEA level decreased to 24.3 ng/mL but the size of the tumor and the degree of invasion were unchanging (Fig. ). From the CT and MRI, the invasion to adjacent organs was still undeniable; therefore, we thought that TPE was appropriate surgery for this patient from the point of view of curability. We performed LTPE 8 weeks after chemoradiotherapy. The patient was placed in the Trendelenburg lithotomy position. The surgeon and the cameraman stood on the patient? right side. Five ports were placed as follows: 12-mm ports at the umbilicus and the lower right quadrant and 5-mm ports at the upper right and left and lower left quadrant. First, the inferior mesenteric artery and vein were skeletonized, clipped, and divided with adequate lymph node dissection. After dissecting the mesentery of the sigmoid colon, the sigmoid colon was transected with a linear stapler (Endo GIA, purple, 60 mm; Covidien, Norwalk, CT, USA). Next, the left ureter was dissected to the level of the ureterovesical junction, where it was clipped and divided. The surgeon moved to the patient? left side, and the same procedure was performed for the right ureter after mobilizing the cecum to the level of the duodenum. We did not place ureteral catheters. The superior hypogastric nerve was divided at the level of the aortic bifurcation. The surgeon moved to the patient? right side again and dissected the posterior rectal space. After recognizing the left internal iliac vessels, the left vas deferens was clipped and divided. The branches of left internal iliac artery were clipped and divided until the entry point to Alcock? canal. Left en bloc lateral lymph node dissection was performed, and the same procedure was then performed on the right side. Bilateral obturator nerves were preserved. After dissecting the dorsal cavity of the rectum to the level of the levator ani muscle, the prerectal peritoneum was incised and the medial ligament was clipped and dissected. The Retzius space was dissected, and the dorsal vein complex (DVC) was exposed after incising the bilateral puboprostatic ligament and the endopelvic fascia (Fig. ). At first, we tried to dissect the DVC with a linear stapler (Endo GIA, purple, 60 mm), but we were unsuccessful because of the thickness of the DVC. The black staple, used for thick tissue, was insufficient to dissect it. We dissected the DVC carefully with Ligasure??Maryland (Covidien), taking care to not cause bleeding of the cutoff stump. The urethra was exposed and dissected with a linear staple (Endo GIA, camel, 45 mm). The levator ani muscle was divided laterally at its origin from the tendinous arch. After the patient? legs were elevated, the surgeon and the first assistant moved to the anal side and closed the anus with double purse-string suture. After the perianal skin was incised, a multiple access port (GelPoint簧Mini; Applied Medical, Rancho Santa Margarita, USA) was set using the transanal minimal invasive surgery (TAMIS) technique. Three small ports were installed, and perirectal tissue and muscles were dissected until we reached the abdominal cavity under the pneumoperirectum (Fig. ). The entire specimen was removed from the perineal opening, and the perineal opening wound was closed (Fig. ). An ileal conduit and a sigmoidostomy were then constructed extracorporeally through the extended umbilical incision. The total operative time was 760 min, and the estimated amount of bleeding was 200 ml. The postoperative course was good, and the patient was discharged on postoperative day 15. Histopathological analysis revealed no apparent invasion of the tumor to adjacent organs, and the pathological stages were ypT3 and ypN0. Currently, laparoscopic technique is used for various surgeries; thus, opportunity to perform laparoscopic surgery has increased for many surgeons. The laparoscopic approach has advantages not only for patients but also for surgeons, i.e., less pain, smaller wounds, earlier recovery, and a magnified view. LTPE is a challenging and complicated operation, but some studies have reported on the safety and feasibility of it not only for urologic or gynecologic malignancies [, ] but also colorectal malignancies [, , , ]. This is our initial experience with LTPE, and so far, we have not encountered any complications. We have routinely performed lateral lymph node dissection for locally advanced lower rectal cancer, and we believe that this previous experience enabled our current success. Upon histopathological analysis, no apparent invasion of the tumor to adjacent organs was observed, but this was difficult to detect before the surgery; therefore, this patient should have been treated by TPE. This case did not include a posterior invasion, so the surgery time was comparatively short. We did not reconstruct the perineal defect, change the patient? position, or place ureteral catheters after dissecting the ureters. We believe that these factors contributed to shortening the surgery time. We did not monitor the urine volume after dissecting the ureters, but intraoperative and postoperative complications did not occur. During the anal-side procedure, we used the TAMIS technique. With the TAMIS technique, we could keep a good visual field even after penetration to the abdominal cavity because the pressure of pneumoperirectum can be kept by the pneumoperitoneum. In addition, the perineal wound becomes smaller, which may lead to reduction in the perineal surgical site infection, and pnuemoperirectum can reduce the amount of bleeding. In the middle of the procedure, the wound retractor broke due to contact with the blade of the Sonicision??(Covidien) and the pneumoperirectum could not be kept thereafter, which will require further attention.
Samantha Allen	36	1990/12/18	274-697-9505x9976	[email protected]	2312 Neal Falls Apt. 187	A 73-year-old Japanese male presented to our hospital with dyspnea and swelling of the lips and eyelids which occurred 30 minutes after eating boiled broccoli. He had no history of allergic reactions to latex or fruits. The patient's clinical findings suggested he was experiencing an immediate-type hypersensitivity reaction to broccoli, such as oral allergy syndrome (OAS) or PFAS. Therefore, tests for allergic reactions including the CAP fluorescent enzyme immunoassay RAST (CAP-FEIA RAST) test and a skin-prick test (SPT) were performed. A blood test revealed an elevated level of serum immunoglobulin E (IgE: 681 IU/mL; normal range: 0??70 IU/mL) and IgE antibodies specific for mugwort (0.83 UA/mL; normal range: 0??.34 UA/mL) were detected in the CAP-FEIA RAST test, although no IgE antibodies specific for almond or mustard were detected. An SPT was then performed using the prick-to-prick technique with samples of mugwort, broccoli, cauliflower, cabbage, mustard, almond, and peanut. The results of the SPT were assessed as false positive (+), positive (2+), strongly positive (3+), and most positive (4+) when the average wheal diameter was more than 25%, 50%, 100%, and 200%, respectively, of a positive control induced by 10 mg/mL of histamine dihydrochloride 30 minutes after administration []. Positive reactions were observed for mugwort (3+), raw broccoli (4+), and mustard (3+) (). Heated broccoli (4+) and heated mustard (3+) also showed positive reactions. The test was negative for cauliflower, cabbage, almonds, and peanuts. Based on the clinical symptoms and the results of the allergy tests, the patient was diagnosed with MMAS due to broccoli consumption. The patient was therefore advised to avoid consuming broccoli and mustard. He has not experienced any symptoms of MMAS since.
Carter Patterson	38	1993/6/14	(688)710-3155x06442	[email protected]	982 Nicole Walks Suite 680	A 68-year-old woman went to a dermatologist for an opinion regarding the thickening and yellowing of the nail plate on the fifth toe of the left foot, which had appeared gradually over the past 2 years. She had no history of prior traumatism, and the lesion was pain-free. Clinical examination showed a thickening of the nail plate, a xanthonychia, and distal splinter haemorrhages of the nail plate on the 5th toe (). Dermoscopy revealed visible holes in the thick portion of the nail plate. All other nails on both the hands and the feet were lesion-free. She had no relevant medical history. Analysis of two mycological samples was negative. For diagnostic purposes, a nail avulsion under local anaesthesia was performed, which revealed the presence of a villous tumour on the nail matrix (). The tumour was excised, and after eight months of follow-up, the nail regrew without any signs of relapse or ungual dystrophy.
Kaylee Vega	26	2004/8/8	972.236.9314x1766	[email protected]	722 Justin Shoal Suite 772	A 46-year-old African American woman presented to the emergency room with respiratory distress requiring intubation. She was found to have severe pulmonary edema on initial evaluation. A transthoracic echocardiogram showed global hypokinesis of the left ventricle and severe systolic dysfunction, with an ejection fraction of 30%. A myocardial perfusion test showed no evidence of stress-induced ischemia, and she was diagnosed with nonischemic cardiomyopathy. Her physical appearance was remarkable for prominent loss of subcutaneous adipose tissue in the extremities and trunk but excessive adipose tissue deposition in her neck with severe acanthosis nigricans suggestive of partial lipodystrophy. Sequence analysis of LMNA gene showed a heterozygous missense mutation at exon 8 (c.1444C>T) causing amino acid change, p.R482W. Her glycosylated hemoglobin was 5.8, indicating that she had prediabetes. One year later a coronary angiogram performed due to retrosternal chest pain showed mild coronary artery disease in left anterior descending artery (LAD) and right coronary artery with an ejection fraction of 25%. The patient was continued on medical therapy. At this point she was diagnosed with diabetes mellitus (glycosylated hemoglobin 6.6%). A lipid profile showed total cholesterol 215 mg/dL, triglycerides 260 mg/dL, low-density lipoprotein 124 mg/dL, high-density lipoprotein 39 mg/dL, and very low density lipoprotein 52 mg/dL. Primary hyperparathyroidism and euthyroid multinodular goiter were also diagnosed. She continued having multiple recurrences of chest pain. A nuclear perfusion scan showed inferior wall scar with moderate amount of peri-infarct ischemia. A series of coronary angiograms performed during a period of 12 months showed significant coronary artery disease: firstly of right coronary artery (90%) that required stenting followed by LAD (80%) that was stented, distal left main coronary (), proximal left anterior descending and obtuse marginal (OM) managed by coronary artery bypass graft (CABG) surgery. An angiogram performed for recurrence of chest pain 4 months after the CABG showed inadequate flow from the left internal mammary artery graft to the LAD but patent saphenous vein grafts to the OM and posterior descending artery. Stenting was done across the left main to the LAD. She improved clinically following this procedure. Two years after her initial presentation, she had asymptomatic transient complete heart block, which was thought to be related to 帣-blockers. This was managed conservatively. Four years after the initial presentation, she had symptomatic sinus node dysfunction with bradycardia. She had episodes of sinus arrest with idioventricular escape rhythm. Since she had persistent left ventricular dysfunction with an ejection fraction of 25%, an implantable cardioverter-defibrillator (ICD) was done. Nine months later she had erosion of the ICD pulse generator in the absence of infection and needed explanation followed by reimplantation on the contralateral side. Six years after the initial presentation she was diagnosed with symptomatic typical cavo-tricuspid isthmus dependent atrial flutter that required ablation. Seven years after the initial presentation, she developed left bundle branch block and underwent upgrade of the ICD to a biventricular cardioverter defibrillator. The patient stated that her deceased mother, a son, and her grandson may have the disease. We offered genetic studies for the son and grandson but both declined to have testing.
Aidan Boyer	32	1983/7/9	001-787-856-6926x481	[email protected]	004 Martha Forges	A 27-year-old white man with an unremarkable medical and family history presented to our emergency department with nausea, vomiting, fever reaching 38.8簞C, and bloody-mucoid diarrhea 10 to 13 times a day for the past 2 days. In that period, Salmonella had been found in some meat cultures in the city center of Sivas and an endemic diarrhea presenting with the same clinical manifestations had been defined. He stated that he had eaten from the meat that had previously been shown to contain Salmonella. His blood pressure was 120/70 mmHg and his pulse rate was 78/minute, but other physical examination findings were normal. His baseline biochemical analysis was unremarkable except for leukocytosis of 21.6?103/mm3. Stool microscopy demonstrated 7 to 8 red blood cells per high-power field and 1 to 2 white blood cells in some fields. No growth was detected in his blood or stool cultures. He was hospitalized in the infectious diseases clinic for gastroenteritis, and he was started on ciprofloxacin 500 mg treatment twice daily. One week after hospitalization, his laboratory results were as follows: blood urea nitrogen 75 mg/dL, serum creatinine 10.4 mg/dL, lactate dehydrogenase (LDH) 1539 U/L, total protein 4.5 g/dL (5.7 to 8.2), and albumin 2.8 g/dL (3.2 to 4.8). His urine analysis results were normal. He was transferred to our nephrology department because he was anuric, and the possibility of HUS was under consideration. In that period, his blood pressure was 130/80 mmHg, pulse rate was 84/minute, and body temperature was 36簞C; there were no pathologic findings in his physical examination. There was no growth in the specific stool culture performed for enterohemorrhagic Escherichia coli. Two days later, his hemoglobin (Hb) concentration dropped to 10 g/dL, direct Coombs test was negative, his reticulocyte ratio was 6 %, and there were schistocytes in his peripheral blood smear. His serologic tests were negative for anti-nuclear antibodies, cytoplasmic anti-neutrophil cytoplasmic antibodies, hepatitis B surface antigen, antibodies to hepatitis B surface antigen, antibodies to hepatitis C virus and human immunodeficiency virus, perinuclear antineutrophil cytoplasmic antibody and anti-glomerular basement membrane results; his C3 was 0.511 g/L (0.9 to 2.1) and C4 was 0.151 g/dL (0.1 to 0.4). A urine analysis performed 2 weeks later when his urine output began to increase, revealed +4 proteinuria and normal microscopy, whereas micro-total protein in 24-hour urine was measured as 18 g/day. In addition, valsartan 160 mg/day and prednisolone 1 mg/kg/day was added to the treatment because his blood pressure increased (maximum to 170/100 mmHg), and hemodialysis and plasma exchange were commenced for aHUS plus acute renal failure. His hemodialysis therapy was discontinued after eight sessions because his creatinine levels decreased and urine output increased. Plasma exchange was performed for a total of 12 sessions until his platelet count and LDH levels returned to normal. He also received a red blood cell transfusion (two units) for symptomatic anemia (Hb 7 g/dL, hematocrit 22 %). The pathology report of the samples obtained through renal biopsy was consistent with C3 glomerulonephritis; therefore, azathioprine 100 mg/day was added to his treatment. A renal biopsy specimen stained with hematoxylin and eosin revealed six glomeruli with increased mesangial cells and matrix, and minimal focal interstitial lymphocytic inflammation. There was no pathologic finding in the tubules and blood vessels (Fig. ). Direct immunofluorescence staining of the tissue sections revealed a total of eight glomeruli, which showed only mild to moderate mesangial granular staining for C3; no immunofluorescent staining was detected with immunoglobulin A, immunoglobulin G, immunoglobulin M, C1q, and fibrinogen. Congo red staining was negative. One month later, his serum creatinine was found to be 1.1 mg/dL, LDH was 232 mg/dL, his platelet count was 190,000/mm3, Hb was 12.8 g/dL, albumin was 3.5, C3 was within the normal ranges, and the micro-total protein in 24-hour urine was 1.3 g/day.
Chaya Phelps	21	1989/1/23	(865)410-4764x73067	[email protected]	23831 Jennifer Circles	A 37-year-old woman purposely came to the outpatient department of our district hospital in the North West Region of Cameroon for a refill of her propranolol medication. She reported taking the drug routinely for palpitations. On further inquiry, she reported being well until 12 months prior to presentation when she suddenly became aware of her heartbeat, initially on moderate exertion and then even at rest. It had been recurrent and lasting several minutes each time it occurred. It progressively became associated with dyspnoea initially on moderate exertion and then on mild exertion, such that she had to quit farming which was her main source of income. After consulting at various health facilities in the North West Region of Cameroon, she was prescribed irregular and alternate daily regimens of 40 mg propranolol and 0.25 mg digoxin which she had been taking for 12 months at the time she presented at our hospital. Although the medications conferred her some relieve, she noticed a progressive weight loss over the 12-months period prior to consulting at our hospital, from 85 to 58 kg despite an abnormal increase in her appetite for food. She also experienced frequent watery stools (averagely six times daily). About 6 months prior to presentation, she complained of heat intolerance and profuse sweating resulting in sleeping with minimal clothing. In addition, she noticed spontaneous resting tremors of her hands which started at the same time as the heat intolerance. Three months prior to presentation at our hospital, she noticed a painless lump on her neck that progressively increased in size. On reviewing her medical records, we noticed a number of investigations requested at various hospitals which she visited during the 8 months preceding her presentation at our hospital. These included electrocardiographs, echocardiograms, HIV tests, full blood counts, fasting blood sugar, thyroid hormone assays, which were all without particularity but for the electrocardiography results which always showed a sinus tachycardia. She has no known history of exposure to radiations or family history of malignancies. Physical examination revealed a chronically ill-looking middle aged woman. Her eyes were normal (Fig. ). Her voice was clear. She had fine resting tremors of her hands with her arms outstretched. There was a left anterolateral neck mass measuring 4 ? 3 cm (Fig. ). The mass was rubbery, mobile, non-tender, moved with swallowing, and not fixed to overlying or underlying tissue. No bruit was heard over the mass. There was no palpable cervical lymphadenopathy. Her vital signs were normal but for regular respiratory and pulse rates of 35 breaths/min and 104 beats/min respectively. Her BMI was 20.1 kg/m2. There was discrete bilateral pedal pitting oedema. The rest of the physical examination was without notable findings. In view of this presentation, a presumptive diagnosis of HT was made and we placed her on propranolol, 40 mg twice daily. Our hospital was not equipped with the necessary diagnostic tools, so we referred the patient to a regional hospital which is about 10 km from our locality. To confirm our diagnosis, we requested for a functional [serum T3, T4, Thyroid stimulating hormone (TSH)] and structural (ultrasound) assessment of the thyroid. The patient was counter-referred to us with the following results:Functional assessment of the thyroid: levels of serum T3, T4, and TSH (Table ). Structural assessment of the thyroid (ultrasound scan): The left thyroid lobe appeared enlarged, heterogeneous, with a fairly iso-echoic, well-defined homogenous solid mass (3.6 ? 1.8 ? 2.9 cm in size). The right lobe was without particularity. No cervical lymphadenopathy was observed. In view of these findings, we concluded on a primary HT most likely due to a toxic uninodular goiter. Again, due to the limited resources in our hospital, we referred the patient to a hospital which is over 30 km from our locality for initiation of a course of methimazole. She was placed on 60 mg methimazole daily, 4 weeks after which she underwent a left thyroid lobectomy. The resected lobe (Fig. ) was sent for histopathology. The postoperative course was uneventful. The lobe weighed 33.5 g and was received in formalin as two grey-tan soft tissue fragments: a large (6.5 ? 5 ? 3.5 cm) and a small (3 ? 1.5 ? 1 cm) fragment. Cutting demonstrated a red tan cut surface with a 3 ? 2.3 ? 2 cm nodule inside the bigger piece. Microscopic examination of representative sections of the large fragment with a full thickness section of the nodular structure revealed a nodular proliferation of enlarged pale cells with marginated chromatin and overlapping nuclei. Pink ?ubble gum??like colloid was focally noted. The lesion was partially encapsulated and displayed areas with fibrosis and more follicular appearance of the aggregates. Based on these, a histological diagnosis of PTC (pT2N0M0) was made. We monitored the patient through scheduled regular visits and referrals. Figure shows the Incision site on the anterior aspect of the neck 2 weeks after lobectomy. We observed a progressive decline in hyperthyroid symptoms and signs: the palpitations regressed as well as the diarrhoea and polyphagia; the heat intolerance regressed and 1 month postoperatively, her weight increased from 58 to 68 kg.
Hamza Palmer	23	1988/5/20	2236771279	[email protected]	131 Berry Lodge Suite 009	A 67-year-old man with untreated diabetes mellitus developed dizziness, followed by dysarthria and dysphagia, 4 days before admission to our hospital. On admission, he was fully conscious, and the physical examination was almost normal except for hypertension and tachycardia in a regular rhythm. Neurological examination revealed equal and round pupils with a normal light reflex. His extraocular movements were full range without diplopia, but he showed gaze-evoked horizontal nystagmus, dysarthria, and dysphagia. He had no complaint of dysgeusia. Muscle tonus was normal without obvious paralysis in all four limbs. Examination of the sensory system revealed hypothermia and hypoalgesia with normal tactile sensation over the left side of his face and right half of his trunk and limbs. Vibration and position sensation were normal in all four limbs. He presented with ataxia in his left upper and lower limbs. Tendon reflexes were normal in his upper limbs and absent in his lower limbs without the extensor plantar reflex. Brain magnetic resonance imaging (MRI) demonstrated hyperintensity in the left side of the dorsolateral medulla with diffusion weighted imaging (DWI) with hypointensity on apparent diffusion coefficient (ADC)-map (Fig. ). He was diagnosed as having lateral medullary syndrome and was treated with oral clopidogrel (continued during admission), intravenous argatroban for 3 days, edaravone for 9 days, and hydration with normal saline (1.5 L/day). Hematological examination showed normal serum sodium levels of 139 mEq/L on admission, however, the serum sodium levels decreased gradually, and 9 days after admission (13 days after onset), he developed asymptomatic hyponatremia (109 mEq/L) with a decreased serum osmolarity of 223 mOsm/L (normal range, 275 ??295 mOsm/L). The turgor in all four limbs was normal, and he did not present with obvious thirst. Thyroid, renal, and adrenal functions were normal, but increased serum levels of antidiuretic hormone (ADH) (6.9 pg/mL; normal range, 0.3 ??4.2 pg/mL) were observed. Urine osmolarity was 668 mOsm/kgH2O (normal range, 100 ??1,300 mOsm/kgH2O), and urine sodium was 121 mOsm/L (normal range, 40 ??90 mOsm/L). Thoracoabdominal CT images showed no abnormalities, such as mass lesions or lymphadenopathy. He was diagnosed as having SIADH based on the diagnostic criteria of Bartter and Schwartz []. He was treated with fluid restriction and intravenous furosemide injections. Laboratory examination on the 23rd day after admission showed almost normal serum sodium levels (136 mEq/L) with normal serum ADH levels (1.2 pg/mL). His neurological symptoms, such as dizziness and dysarthria, also gradually resolved, and thereafter, hyponatremia was not observed.
Juniper Kim	24	1996/7/27	754.407.3829	[email protected]	81296 Torres Court Suite 275	A 29-year-old white male farmer sought medical attention because of left upper-quadrant abdominal discomfort and unintentional weight loss of 8 kg. His initial clinical examination revealed light pallor and splenomegaly, 10 cm below his left costal margin. However, no lymphadenopathy or hepatomegaly was detected. Laboratory analysis showed a leukocyte count of 38 ? 109/L, a hemoglobin level of 10.5 g/dL, and a platelet count of 289 ? 109/弮L. He denied fever or drenching night sweats and was not taking medication. He was referred to our Department of Hematology, at our university hospital, where a bone marrow aspiration and biopsy were performed. Microscopic examination showed a left-shifted granulopoiesis, and the Ph + was found with no other chromosomic aberrations. BCR-ABL fusion transcript was identified by polymerase chain reaction (PCR) and both transcripts (b2a2 and b3a2) were present. The diagnosis of CML in chronic phase was made: Sokal score 0.85 (low risk <0.8; moderate risk 0.8 to 1.2; high risk >1.2) and Hasford score 741 (low risk <780; moderate risk 780 to 1480; high risk >1480). He was initially treated with hydroxyurea, which was later substituted by imatinib 400 mg daily starting in early January 2013. We observed a slow progression of the splenomegaly until complete spleen regression by 3 months. The first reevaluation was made at 3 months of imatinib (early response). During that period he showed no symptoms and gained weight (4 kg). Early response was detected through a cytogenetics analysis and quantitative polymerase chain reaction (qPCR) assessment. There was no cell growth to perform an analysis of the Ph + through the G-band method. Fluorescent in situ hybridization revealed 3.5 % of cells with the gene fusion, while PCR assessment was not conclusive due to the low quality of deoxyribonucleic acid (DNA). He was considered an optimal responder. He persisted on imatinib 400 mg for 6 months before the agent was discontinued because of pancytopenia. His neutrophil count fell to 0.5 ? 109/L, platelets dropped to 85 ? 109/弮L, and hemoglobin was 6.4 g/dL (Fig. ). Transfusion was not necessary. After imatinib cessation, we waited 1 week until a new hemogram was done. Because there was no improvement in his hemogram we decided to examine his bone marrow. A bone marrow study was performed showing complete remission of CML and numerous Leishmania amastigotes within the macrophages (Figs. and ). IgM antibody to Leishmania K39 antigen was positive. He was admitted to our hospital and began treatment with pentavalent antimonials. His hemogram started to improve at 10 days of leishmaniasis treatment and normalized by 2 months. New bone marrow aspirate showed no more leishmaniasis after 60 days of therapy. Complete recovery of symptoms and examinations (bone marrow and hemogram) was seen after that time (Table ). Acute cardiac toxicity required suspension from treatment. An electrocardiogram showed prolongation of the QT interval and bradycardia. The medication was stopped when his heart rate dropped below 45 beats per minute; it was replaced by amphotericin B regimen. After 3 months, TKI therapy was resumed and he again showed adequate response to imatinib. The last PCR showed deep molecular response (MR 4.0).
Roman Hanson	35	2004/8/28	5165696685	[email protected]	7001 Christina Street Suite 397	A 33 year-old woman presented with a history of moderate abdominal pain and recurrent nausea. Recently, she experienced lancinating pain in her right leg and hot flashes. She did not report unintended weight loss, fatigue or fever. Previously, she had undergone body contouring plastic surgery after a weight loss of 35 kg. She carried a levonorgestrel-releasing intrauterine device (IUD) for several months. Computed tomography (CT) revealed a retroperitoneal mass adjacent to the right psoas muscle. Since a retroperitoneal sarcoma was suspected, the patient was referred to our institution. The laboratory test yielded normal blood levels of chromogranin A, neuron-specific enolase, CA15-3, and CA125. Urine and blood samples did not show elevated production of catecholamines, vasoactive intestinal peptide or androgens. Gynaecological examination including transvaginal ultrasound showed normal findings and confirmed the correct position of the IUD. Magnetic resonance imaging (MRI) demonstrated a solid tumour (68 ? 35 ? 28 mm) between the anterior circumference of the right psoas muscle and the anterior surface of the third and fourth lumbar vertebra as well as laterally extending to the to the right ureter, the inferior vena cava and right common iliac vein (Fig. ). A CT-guided percutaneous core needle biopsy was performed to allow a histological diagnosis before surgery. The core biopsies contained only mature tissue with bundles of Schwann cells. Prominent, but also mature, ganglion cells were within the stroma, and there were no other histological signs of malignancy. Therefore, histopathological examination assumed a GN (Fig. ). Institutional interdisciplinary tumour board recommended a primary surgical resection. Prior to surgery, a double J stent was placed in the right ureter. Explorative laparotomy exposed the paravertebral tumour extending to the lower pole of the right kidney. The uterus and the adnexa with ovaries were without any pathological findings. After ureterolysis, the tumour was cautiously dissected from adherent structures such as the right common iliac vein and the kidney which were both not infiltrated by the tumour. Finally, the tumour was completely removed by means of blunt and sharp dissection and bipolar coagulation under a surgical microscope (the microscope has been used to save most of the branches of the lumbar plexus and nerves of the sympathetic trunk which was partly incorporated into the GN). Neuropathological examination of the tumour established the diagnosis of a typical GN with extremely low proliferation (Ki67 less than 1 %) and no evidence of a ganglioneuroblastoma. Within the tumour, a ganglion with mature ganglion cells was embedded and a peripheral nerve was observed being penetrated by the tumour (Fig. ). The postoperative course was uneventful. After surgery, the patient reported heat sensations of stocking distribution in the right lower leg. Follow-up 4 months after surgery still revealed a small hyperthermic area and stocking-like heat sensations in the right lower leg that was interpreted to relate to a lesion of the sympathetic trunk partly incorporated into the GN. MRI at follow-up did not show tumour recurrence. Clinical manifestations of GNs are diverse. In most cases, symptoms result from compression or displacement by the growing tumour and represent disturbances such as disruption of venous flow [] and lower abdominal pain []. Most GNs are located mediastinal or retroperitoneal. In addition, a primary intraosseous GN of the sacrum has been described as a rare manifestation []. GN usually represents a non-secreting tumour. Occasionally, GN may release hormones that can cause diarrhoea, sweating and arterial hypertension []. Tumours may also cause tenesmuses and weight loss []. In our case, we additionally observed lancinating pain in a leg most likely caused by irritation of branches of the lumbar plexus running along the anterior surface of the psoas muscle. Important differential diagnoses of retroperitoneal masses like the GN are retroperitoneal soft tissue sarcoma, and retroperitoneal fibrosis, and rarely lymphoma, a primary germ cell tumour, or metastatic testicular cancer. The complete removal of the GN is described as adequate therapy with an excellent prognosis. Nevertheless, surgical morbidity has to be considered. In a retrospective series of 146 children with GN, surgical tumour resection resulted in an excellent long-term survival, but, nevertheless, 22 of the 146 patients (15 %) suffered surgery-related complications, of which two were fatal and seven were severe (e.g. Horner syndrome, rupture of thoracic aorta and thoracic haemorrhage) []. Residual GN after incomplete resection remains stable for years without malignant transformation []. In addition, Retrosi et al. did not observe recurrences after incomplete excision in a series of 23 children with thoracic (n = 14), abdominal (n = 7) or pelvic GN (n = 3) []. Surgical complication rate reached 30 % []. Therefore, Retrosi and De Bernadi recommended less radical surgery in childhood GN to reduce surgery-related morbidity and mortality [, ]. Even a watchful waiting could be an option in selected cases after secured diagnosis. Interdisciplinary discussion and image-guided biopsy prior to surgery is recommended to prevent unnecessary expanded surgical approaches with significant postoperative morbidity. From our experience, we would suggest surgical resection of GN, but with a less radical approach in order to preserve the adjacent organs and structures where appropriate.
Mariana Lin	43	1996/10/28	333-931-6595x0388	[email protected]	53918 Sparks Harbor Suite 081	A 27-year-old white man was hospitalized on 17 September 2014 with dyspnea and cough, which had started the day before, and a 1-month history of small-volume hemoptysis, which had aggravated in the last 24 hours. He also reported a history of weight loss, corresponding to 12 % of his corporal mass in the past year, but no other accompanying symptoms. He reported a cigarette smoking habit of 4 pack years, having occasionally smoked recreational drugs (cannabis). He was also professionally exposed to chemical toxics: thinners. There was no previous history of renal or pulmonary disease. No family disease was known and he had no other relevant findings in his past medical history. On examination at the time of admission he was apyretic, his respiratory rate was 29 breaths/minute, pulse rate was 118 beats/minute, and blood pressure (BP) was 151/86 mmHg. We observed pallor of his skin. Pulmonary auscultation revealed rales in his right hemithorax and left base. A chest X-ray (CXR) showed bilateral infiltrates (Fig. ). An analytical study (Table ) showed a normocytic normochromic anemia with a hemoglobin level of 7.2 g/dL and leukocytosis with normal renal function and coagulation times. His erythrocyte sedimentation rate was 34 mm/hour and procalcitonin level was 0.309 ng/mL at the time of admission. A urine analysis had no erythrocyturia or eosinophiluria. His oxygen saturation was 96.8 % on 21 % oxygen with no signs of carbon dioxide (CO2) retention and no electrolytic or acid-base changes. Chest computed tomography (CT) revealed a reticulonodular infiltrate of both lungs with extensive ground glass appearance (Fig. ). He was initially treated with aminocaproic acid 3 g orally every 8 hours and codeine 20 mg orally every 6 hours, having also completed 7 days of daily ceftriaxone 1 g administered intravenously and daily azithromycin 500 mg administered orally. Transfusion of two blood units was performed with an increase in hemoglobin count up to 8.9 g/dL (Table ) without complications. His serum urea and creatinine levels were 43 mg/dL and 0.78 mg/dL, respectively, at this time. A bronchoscopy performed on the second day revealed hematic traces with no other alterations or apparent lesions (Fig. ). On the fourth day of admission, he developed deterioration of ventilatory parameters and of respiratory rate (32 breaths/minute) needing oxygen supplement via high flow mask. Due to respiratory failure with a partial pressure of oxygen (pO2) of 48.3 mmHg and an oxygen saturation of 83.6 % on 100 % oxygen, he was transferred to our intensive care unit (ICU), but did not require mechanical ventilation. Sputum cultures were negative, as were the rapid urine antigen tests for Legionella pneumophila and Streptococcus pneumoniae. DNA analysis for Mycobacterium tuberculosis was negative, as were the studies for Influenza A, Influenza B, and Influenza H1N1, and the serologic markers for human immunodeficiency virus, hepatitis C and hepatitis B. Direct and indirect Coombs test were both negative. An echocardiogram was performed showing no significant findings. Serologic tests for auto-antibodies (antinuclear antibodies, anti-neutrophil cytoplasmic antibodies, and anti-GBM antibodies) were negative. After 5 days, he was transferred out of our ICU with a good clinical and analytical evolution (Table ). At the end of the tenth day his general state deteriorated with fatigue and hematuria (327 erythrocytes/L) and in 3 days he developed deterioration of renal function with a serum creatinine level of 4.55 mg/dL and a serum urea level of 189 mg/dL, with recurrence of hemoptysis and anemia, showing a hemoglobin value of 6.1 g/dL (Table ). His pO2 value dropped to 55.5 mmHg with an oxygen saturation of 89.4 % on 50 % oxygen. Immunosuppression with daily prednisolone 1g administered intravenously was initiated. An urgent bronchoscopy was repeated after new transfusion of three blood units, showing no evidence of lesion again. His hemoglobin level rose to 9.1 g/dL after the transfusion. A renal biopsy was performed 24 hours after the bronchoscopy and showed fibrinoid necrosis in glomeruli (19 glomeruli were assessed) and cellular crescents in 26 % of glomeruli (Fig. ). Immunofluorescence revealed a linear deposition of IgG, compatible with GS. Immunosuppressive therapy with daily cyclophosphamide 120 mg orally was added. Because of renal failure with a serum creatinine level of 5.13 mg/dL, he was transferred to a referral center where he underwent 21 sessions of plasmapheresis and four sessions of hemodialysis. Four weeks after initial presentation, his serum creatinine level was 1.8 mg/dL at the time of his discharge from the reference center. Over the course of 6 months, he completed treatment with daily prednisolone 60 mg orally and daily cyclophosphamide 100 mg orally, maintaining regular follow-up with out-patient nephrology, with currently no need of hemodialysis. No further episodes of hematuria or hemoptysis have been reported so far.
Conor Rush	25	1992/10/20	656.575.3912x35380	[email protected]	1436 Powers Port Suite 237	We report the case of an 8-year-old girl of Colombian origin and mixed race ancestry (Mestizo) who is the product of the first pregnancy of non-consanguineous parents; her mother was 32-years old and her father was 31-years old at the time of her gestation. Her mother? prenatal history shows no evidence of teratogen exposure or any other relevant exposures or pathologies. Ultrasound reports during weeks 14 and 25 of gestation showed no morphological alterations. Delivery care was performed at 38 weeks of gestation by spontaneous vaginal delivery, after which bilateral clubfoot and heart murmur were identified. An echocardiogram showed the presence of an atrial septal defect of 7.4 mm with a left to right shunt, dilated coronary sinus, subaortic interventricular communication of 4.8 mm and 6.8 mm with a left to right shunt. She underwent surgery for her congenital heart disease at 51-days old due to the presence of dyspnea, fatigue, anorexia, and diaphoresis. Currently she has a unicameral pacemaker. She is currently 8 years of age, and has the following clinical findings: delayed psychomotor and language development, cognitive deficits, anthropometric measurements within normal percentiles, upward slanting palpebral fissures, divergent strabismus, rotated and low-set ears, tall and broad nasal bridge, flat philtrum, bifid uvula, posterior cleft palate, increased anteroposterior diameter of the thorax, scoliosis, and umbilical hernia. Her extremities evidenced clinodactyly of her fifth bilateral fingertips, bilateral ulnar deviation, right thumb with the presence of two interphalangeal grooves, thenar hypoplasia, gait abnormality, bilateral clubfoot (corrected), bilateral sandal gap, nail split of the second toe of her right foot, and a social personality (see Figs. and ). Complementary studies were conducted: magnetic resonance imaging (MRI) of her brain evidenced brain stem and corpus callosum hypoplasia, and cortico-subcortical atrophy; thoracic spine MRI showed a left middle thoracic scoliotic curve (see Fig. ). Electroencephalogram had an abnormal vigil tracing with frequent repetitive discharge points, isolated biphasic spike, associated with right frontotemporal high voltage slow waves. Hip radiography confirmed bilateral coxa valga and hip subluxation; foot and ankle radiography documented a varus foot, her first metatarsal talus mechanical axis was altered by lateralization of the astragal axis, and the presence of ankle valgus. G-banding karyotyping was performed in 20 metaphases, which reported a 46,XX female; subsequently, chromosomal analysis was performed by CGH array that showed a loss of approximately 3.608 Mb on chromosome 21q22.3, and a copy number gain of 12.326 Mb on chromosome 7q35q36.3 (see Fig. ); these alterations affect approximately 112 and 186 genes, respectively (see Table ). Both copy number changes are terminal, which suggests an unbalanced translocation between chromosomes 7 and 21, resulting in a derivative chromosome 21. Complementary studies were performed with both parents using fluorescence in situ hybridization (FISH). The results showed our patient? mother has a reciprocal translocation between chromosomes 7 and 21 ish t(7;21)(q35;q22.3) (RP11-10L20-, RP11-75J5 +; RP11-75J5-, RP11-10L20 +).
Maleah Dudley	26	1989/12/26	8676576904	[email protected]	0006 Miller Wells	A 51 year old woman with a 12 year history of hypertension developed hypokalemia (as low as 2.2 mmol/L). Her blood pressure was controlled with amlodipine 10 mg once daily and she received potassium supplements to maintain eukalemia. Her serum aldosterone was 2832 pmol/l with undetectable plasma renin activity. Her aldosterone to renin ratio (ARR) was therefore estimated at greater than 28,000 pmol/l/ng/ml/h (normal less than 2000 using renin activity of 0.1 ng/ml/h to avoid over-inflation). Her serum creatinine was 75 umol/l with eGFR of 85 ml/min. Abdominal CT showed a 3.5 cm low density left adrenal mass and adrenal vein sampling confirmed left lateralization with lateralization index values of 23:1 and 28:1 pre and post cosyntropin infusion. She underwent left adrenalectomy for what was reported as a 15.6 g adrenocortical adenoma on pathology. The post-operative biochemical course is presented in Table . At 2 weeks post-operative follow up, her blood pressure was 124/80, serum potassium 4.5 mmol/L and serum creatinine 52 umol/l without any medications. Her serum aldosterone was less than 70 pmol/l and renin activity of 0.36 ng/ml/h yielding ARR less than 194. However, at 6 weeks post-operative, she presented with serum potassium of 6.7 mmol/l, serum creatinine of 152 umol/l associated with persistent diarrhea (determined to be non-infectious) and clinical volume depletion. After fluid resuscitation she was started on fludrocortisone 0.1 mg daily and up-titrated to 0.1 mg bid over a 1-week period. At the higher fludrocortisone dose she became normokalemic and her serum creatinine decreased to 134 umol/l (Table ). One month later, her serum potassium continued to be 4.8 mmol/l and serum creatinine 112 umol/l and so a dose reduction in fludrocortisone was attempted but at 0.1 mg per day, her potassium promptly rose to 5.6 mmol/l with creatinine 140 umol/l. Hyperkalemia persisted in the fluid replete state. She was not on any medications causing hyperkalemia. In the context for fludrocortisone responsive hyperkalemia we did not calculate TTKG (trans tubular potassium gradient). The fludrocortisone dose was increased again with similar normalization of potassium and drop in serum creatinine but her resultant blood pressures rose to 160/100. Another attempt was made to decrease the fludrocortisone to 0.1 mg daily which was initially tolerable but by 2 months on this dose, her serum potassium was again found to be 6.4 mmol/l with creatinine 152 umol/l. This was transiently treated with higher dose fludrocortisone but the patient felt unwell on the medication, complaining of abdominal pain and so the drug was stopped in favour of furosemide 20 mg daily. Off of the fludrocortisone, her blood pressures were 135/88 but there was resultant hyponatremia, mild hyperkalemia and a sharp rise in serum creatinine to 188 umol/l and so the furosemide was stopped in favour of a return to fludrocortisone once more. Again, her potassium normalized to 4.5 mmo/l with drop in serum creatinine to 127 umol/l. Nephrology was consulted and the patient started on sodium bicarbonate 650 mg tid with cessation of fludrocortisone. During the last 8 months of such therapy, her blood pressures have been normal (122/88), potassium levels have been near-normal (5.2 mmol/l) with stable renal function (creatinine 160 umol/l, eGFR 31 ml/min).
Colter Montoya	33	1980/9/10	885-305-3802x04041	[email protected]	2274 Gibson Row	A 60 year old man with a 30 year history of hypertension and serum potassium as low as 2.9 mmol/l was referred for assessment. At presentation he required five antihypertensive agents to control his blood pressure which ranged from 130-150/80-100. His medications were perindopril 8 mg once daily, irbesartan/hydrochlorothiazide 300/25 mg once daily, nifedipine 60 mg twice daily and carvedilol 6.25 mg twice daily. After temporarily stopping carvedilol, irbesartan/hydrochlorothiazide and perindopril, his serum aldosterone was 1041 pmol/l with plasma renin activity of 0.2 ng/l/s yielding an aldosterone to renin ratio (ARR) of 5205 pmol/l/ng/ml/h (normal less than 2000). CT abdomen showed a multinodular left adrenal gland with a 1.3 cm dominant nodule. Adrenal vein sampling (AVS) showed left lateralization with lateralization indices of 4:1 and 12:1 pre and post cosyntropin infusion. Preoperative serum creatinine and GFR were 77 umol/l and 60 ml/min respectively. He underwent left adrenalectomy for 1.5 cm adrenocortical adenoma in the setting of background adrenal hyperplasia. At 4 weeks follow up his blood pressure was 128/80, serum creatinine 77 umol/l, serum aldosterone less than 70 pmol/l with renin activity of 0.31 ng/l/s yielding ARR of less than 225 pmol/l/ng/ml/h. At that time his irbesartan/hydrochlorothiazide was discontinued and nifedipine was reduced to 60 mg daily instead of twice daily and he continued to take carvedilol and perindopril. At 2 months follo up his serum potassium was found to be 6.0 mmol/l and serum creatinine 141 umol/l. He admitted using four pills of Ibuprofen for a fall related injury. At that visit his ACE inhibitor was stopped. A day later his serum creatinine came down to 121 umol/l but potassium remained at 5.9 mmol/l. He was started on fludrocortisone 0.05 mg daily. Two months later his serum creatinine was 115 umol/l and potassium 5.3 mmol/l. He did not have clinical signs of volume depletion and no other apparent reason for high potassium was found; therefore he was switched to furosemide 20 mg once daily. He required loop diuretic to normalize his serum potassium for 6 months after which it was stopped as his serum potassium remained 4.5??.9 mmol/l without further intervention. At that time his only medications were nifedipine 60 mg daily and carvedilol 6.25 mg daily. At 15 months post-operative, he was successfully weaned off of carvedilol and his only continuing medication was nifedipine 30 mg daily with average blood pressures of 136/90.

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