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patient_info_and_summary

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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
Kamryn Kaur	19	1990/4/13	001-304-817-4272	[email protected]	175 Alvarado Divide Suite 775	A 52 year old woman with type 2 diabetes and congenital right kidney aplasia was referred with a 12 year history of hypertension and hypokalemia as low as 2.3 mmol/l. At presentation she required amlodipine 10 mg once daily, telmisartan 80 mg once daily and spironolactone 25 mg once daily to maintain normotension and eukalmeia. Off of spironolactone, her aldosterone was 1157 pmol/l and direct renin was 3 ng/l yielding ARR of 368 pmol/l/ng/l (normal < 60 using the newer direct renin assay). CT abdomen showed 1.5 cm hypodense left adrenal mass. AVS showed left lateralization with lateralization index of 20:1 pre and post stimulation. Preoperative GFR was 69 ml/min and serum creatinine of 80 umol/l. She underwent adrenalectomy for what was reported as 2 cm adrenal cortical mass with no surrounding hyperplasia. Two weeks after the surgery she presented to emergency department with intermittent nausea and vomiting. Her serum potassium was 6.3 mmol/l and serum creatinine was 150 umol/l. After fluid resuscitation her serum potassium dropped to 5.1 mmol/l but creatinine persisted at 160 umol/l. She was started on sodium bicarbonate 650 mg bid. At 2 weeks follow up her serum creatinine was 208 umol/l and potassium remained at 5.1 mmol/l. She did not require any more antihypertensive medications and her blood pressure was 140/70. She was started on fludrocortisone 0.05 mg once daily and at 1 week follow up lab work her serum creatinine reduced to 170 umol/l with serum potassium of 5.2. She is currently on fludrocortisone 0.05 mg once daily.
Augustine Bautista	32	1992/10/5	360.774.6480x759	[email protected]	19087 Carlos Oval	A 6-year-old Iraqi boy of Arab-Asian ethnicity who had developed cerebral palsy (CP) with mental retardation and epilepsy after encephalitis at the age of 9 months. Since that time, he had been treated with oral VPA (30 mg/kg/day, twice a day) to control the seizures. His family history was unremarkable apart from that his next older brother had acute lymphoblastic leukaemia and died due to severe infection a year and a half before the birth of this case of KS. Our patient was the youngest among 4 siblings of healthy second-cousin parents. The other 2 older siblings are healthy. Our patient suffered from recurrent respiratory tract infections and was hospitalized twice. The latest episode was on December 1st, 2013 when he was 6 years-old. Although he had middle lobe atelectasis, he responded well to a 1-week treatment with non-specific antibiotic and his chest roentgenogram showed improvement. One week after being discharged from the hospital, painless skin lesions appeared in the form of a bluish-purple plaque (blue-violet to black hue) on the middle and little toes of his left foot and extended to the upper lateral sole area (Fig. ). Physical examination revealed a spastic quadriplegic type of CP, mentally subnormal with open mouth, mouth breathing, and misalignment of the teeth with malocclusion. He was in full-body hypertonia with legs being affected more than arms and hyperreflexia with clonus. Systemic examination of the chest revealed harsh vesicular breath sound and normal heart sounds. His abdomen was soft, with no organomegaly, mass or free fluid. The investigations performed included complete blood count, blood film, erythrocyte sedimentation rate, coagulation profile, blood chemistry, liver and renal work-up, urinalysis, chest x-ray and ultrasound examination of the abdomen were all normal. Serological screening for hepatitis B and C as well as HIV was negative. In January 2014, palpable bluish-purple nodules appeared on the left leg and then on the right leg. The skin discoloration extended to the plantar surface, and edema of the left foot became obvious (Fig. ), but peripheral arterial pulse and the perfusion in the lower extremities were intact. A skin biopsy was taken on February 15th, 2014 from the lesion on the left foot, thereafter the patient experienced pain. During February and March 2014, new skin nodules appeared in an ascending pattern: 2 around the groin, 2 on the left arm, 1 in the right sub-axillary area, and on the face (medial angle of left eye and both ear cartilages) (Fig. , ). Small cervical lymphadenopathy also became palpable. Meanwhile, a purple, slightly elevated lesion erupted on the hard palate. He was kept on his VPA dose with no additional therapy apart from analgesic agents, as the family refused hospital admission. On April 3rd, 2014 the patient had developed sudden respiratory distress, and died before arrival to the hospital just 3 days before getting his final biopsy test results. The skin biopsy material was re-evaluated in Japan because of the lack of facilities for HHV-8 detection in Iraq. The results are presented in Fig. , the spindle cells reacted with monoclonal antibodies against CD34 and CD31 antigens. Additionally, these cells were positive for HHV-8, hence the diagnosis of KS was confirmed. The ?ARE??checklist is available as Additional file 1. This child? clinical course is summarized in the accompanying supplemental time-line file (Additional file 2).
Antonella Wong	26	1991/1/12	(641)865-7128x73112	[email protected]	0703 Hernandez Divide Apt. 492	A 66-year-old woman presented to our outpatient services in 2011 with the complaint of progressively puffy eyelids on both eyes in the most recent 3 months. Her past medical history showed hypertension, type 2 diabetes mellitus, ischaemic heart disease, and end-stage renal disease. Additionally, she had been undergoing haemodialysis three times a week since 2009 (3 years ago). With the history of chronic haemodialysis, in 2011, a computed tomography angiography (CTA) revealed an occlusion of the right subclavian vein at the junction with the superior vena cava (SVC), and there was a subtotal occlusion at the SVC orifice in the arteriography (Fig. ). Progressively, her appearance changed, with marked oedema of the face and neck, but without any obvious lymphadenopathy or palpable mass on the neck (Fig. ). Additionally, she experienced dyspnoea and intermittent nocturnal coughing. The patient received cataract surgery for both eyes 10 years ago. There was no use of corticosteroids in her medical records and no positive family history of glaucoma. A general inspection revealed the orthotropic primary ocular position without proptosis. The eyelids were swollen without tenderness or local heat, and initially, the intraocular pressure was normal. There was moderate congestion of the conjunctiva in both eyes; the corneas were clear, and the anterior chambers were deep and quiet. In 2013, two years after SVC syndrome was diagnosed, pneumatic tonometry revealed bilateral high IOP (22 mmHg OD, 23 mmHg OS) compared to a prior visit. Then, the IOP increased progressively to 30 mmHg, even with the combined use of brimonidine, dorzolamide and latanoprost, which was aimed at controlling IOP. At that time, the patient? best-corrected visual acuity (BCVA) was 20/60 in the right eye and 20/400 in the left eye. Gonioscopy demonstrated wide-open angles in both eyes without peripheral anterior synechiae or neovascularization of the angle. Upon fundus examination, the cup-to-disk ratio was found to have increased to 0.7 in both eyes after one and half years of treatment with an anti-glaucoma agent. Optical coherence tomography showed the superior and inferior thinning of the retinal nerve fibre layer (RNFL) in both eyes, which was worse on the left side. Specifically, the patient had an average RNFL of 65.57 弮m in the right eye and 47.51 弮m in the left eye. Standard automated perimetry showed a three-fourths quadrant depression in the right eye and more generalized depression in the left eye; the mean deviations were -17.57 and -27.07, respectively. Due to the progressive course of glaucomatous change, we suggested that the patient first have filtering surgery for the left eye. We performed a trabeculectomy, which resulted in a postoperative IOP reduction (from 30 to 12 mmHg) on the first day. No complications occurred intraoperatively and none was reported at the one-month post-operative review. However, over the next three months, although the filtering bleb significantly elevated, the intraocular pressure (IOP) increased gradually, reaching approximately 40 mmHg (Fig. ). Subsequently, this patient received the placement of one ExPRESS miniature glaucoma device P200 (Fig. ), which resulted in the lowering of the IOP (from 45 to 17 mmHg), and no further deterioration of visual acuity occurred 6 months after this intervention, while the IOP of the other eye still ranged between 33 and 35 mmHg. During the management of intractable secondary glaucoma, we reviewed the patient? past medical history and image data and found that the patient was susceptible to vascular thrombosis and stenosis, as evidenced by the high venous pressure observed during haemodialysis, and while creating the vascular shunt, repeated angioplasty and thrombectomy for venous outlet thrombosis were required. There were multiple attempts at creating vascular accesses during the initial 2 years. First, a right neck Hickman catheter was inserted for temporary haemodialysis access, after which a second was placed in her right arm, but failed within a short time. Third, an arteriovenous fistula (AVF) was created in the left arm with a radial arterial-cephalic vein anastomosis, which failed in three years and was replaced by an arteriovenous graft (AVG) between the brachial artery and the axillary vein in the left arm after 6 months. The patient had been under haemodialysis via AVG since then, but encountered acute occlusion once and received a balloon percutaneous transluminal angioplasty (PTA). In 2011, the patient was admitted once for SVC syndrome after an unsuccessful attempt to place a Hickman catheter from the right and left internal jugular vein to bypass the complete obstruction of the right subclavian vein and right brachiocephalic vein. PTA was performed unsuccessfully, despite the spontaneous resolution of oedema on the left side of the face. However, the patient presented with intermittent recurrence and indolent face and neck oedema afterwards. Over the next 3 years, marked SVCS, accompanied by uncontrollable IOP, led to irreversible glaucomatous optic neuropathy. Although femoral double lumen creation and venous bypass construction were suggested as alternative haemodialysis access routes, along with the ligation of the previous AVF in the left arm, the patient refused any cardiovascular surgical options.
Walter Copeland	40	1997/10/16	001-537-994-4949	[email protected]	369 Francis Harbors	A 32 year old married African female from the North west region of Cameroon, G4P4004 (four previous pregnancies, four term deliveries, no abortion, no preterm deliveries, and four living children), with a history of mild recurrent right iliac fossa (RIF) pain presented at our emergency service with an acute exacerbation of the pain over a 2 day duration and an episode of post prandial vomiting. She reported anorexia, and a low grade fever. She had no past history of a sexually transmitted infection. On examination, she was ill looking, tachycardic (pulse rate = 144 beats per minute) and febrile (maximum temperature = 38.4 簞C). She had a RIF tenderness, and positive Blumberg? (Rebound tenderness at the RIF), Rovsing? (increased pain at the RIF on deep palpation of the left iliac fossa), Obturator (abdominal pain on flexion and internal rotation of the hip) and Psoas (abdominal pain on passive extension or active flexion of the thigh at the hip) signs. A full blood count revealed a leucocytosis at 13,000 cells/mm3. A urinalysis and stool exam were normal and a pregnancy test was negative. The patient had a total Alvarado? score of 10 and a diagnosis of an acute appendicitis was further advocated by an abdominopelvic ultrasound which showed; an indistinct mass in the RIF, an inflammed appendix and collection of fluid in the pouch of Douglas. A diagnosis of an acute appendicitis associated with an indistinct RIF fossa mass was proposed, and an exploratory laparotomy via a midline infra-umbilical incision was performed. Intraoperatively, an inflammed, suppurative, and distorted right fallopian tube measuring 8 ? 4 cm, attached to the caecum and the terminal ileum by means of pseudo-membranes was found compressing and occluding the lumen of an inflammed appendix (Figs. , ). There was extensive damage of the right tube with complete obliteration of its lumen rendering saline irrigation and intraluminal adhersiolysis impossible. The left fallopian tube was also inflammed, measuring 4 ? 4 cm and discharging pus. The ovaries and uterus were macroscopically normal. A right salpingectomy and classical appendectomy were done. The pus from the left fallopian tube was drained, and the tube irrigated with normal saline followed by abdominal lavage. A pus sample was collected but could not be analysed due to financial constraints. The patient was therefore placed on intravenous (IV) antibiotics and the post-operative period was uneventful. The patient had positive Chlamydia Immunoglobulin M titres and a negative TPHA (Treponema Pallidum Haemagglutination Assay) test. She and her spouse were treated with doxycycline 200 mg once daily and metronidazole 500 mg 8 hourly upon discharge to complete a 14 day course of treatment. She returned for a follow up visit 2 weeks later with no further complaints.
Dayana Patel	45	2000/8/19	361.727.6383x816	[email protected]	39384 Kyle Throughway Apt. 625	A 46-year-old Syrian Alawis woman without a significant personal or familial medical history presented with a 1-month history of multiple sclerosis, fatigue, loss of weight, fever, and an elevated white blood cell (WBC) count. An initial evaluation revealed that she had anemia (8.5 g/dL), leukocytosis (total leukocyte count 134 ? 109/L), and thrombocytopenia (23 ? 109/L). She was pale and did not have lymphadenopathy. Our patient was transferred to the hospital because she was unconscious and making noise during breathing. Novel hematological parameters included anemia (8.2 g/dL), thrombocytopenia (29 ? 109/L), leukocytosis (229 ? 109/L), a plasma concentration of fibrinogen of 37 mg/dL (normal value, 200??00 mg/dL), and a prothrombin time of 18 s (normal value, 10.0??3.0 s). She received several blood transfusions. Our patient stayed in the hospital for 1 week. On the same day of treatment initiation with ATRA (45 mg/m2 daily dose), our patient died, 10 days after her diagnosis. An autopsy revealed death was due to hemolysis, intracranial hemorrhage, thrombocytopenia, and DIC. Cytogenetic and immunophenotyping analyses were also carried out. Our patient was diagnosed with APL according to the World Health Organization (WHO) classification and was considered high risk based on her WBC. Her brother gave consent for a scientific evaluation of her case and the study was approved by the ethical committee of the Atomic Energy Commission, Damascus, Syria. A chromosome analysis using GTG-banding was performed according to standard procedures [] before treatment with ATRA and revealed a karyotype of 46,XX,t(15;17)[8]/46,XX,t(1;2),t(15;17)[11]/46,XX [1] (Fig. ). Further studies were performed based on molecular cytogenetics (Figs. and ). Dual-color fluorescence in situ hybridization (D-FISH) using a specific probe for PML and RARA (Abbott Molecular/Vysis, Des Plaines, IL, USA) revealed the presence of the PML/RARA fusion gene on der(15) (Fig. ). Chromosomes 1, 2, 15, and 17 were studied with Whole Chromosome Paint (WCP) probes (MetaSystems, Altlussheim, Germany) [], which did not provide any information on the cryptic translocations (data not shown). Array-proven high-resolution multicolor banding (aMCB) [] was performed using probes corresponding to chromosomes 1 and 2, which were identified by GTG-banding as being involved (Fig. ). The following final karyotype was determined prior to chemotherapy treatment using a fluorescence microscope (AxioImager.Z1 mot, Carl Zeiss Ltd., Welwyn Garden City, UK) equipped with appropriate filter sets to discriminate between a maximum of five fluorochromes plus the counterstain DAPI (4',6- diamino-2-phenylindole). Image capture and processing were performed using an ISIS imaging system (MetaSystems, Altlussheim, Germany): 46,XX,t(15;17)(q22;q21)[8]/46,XX,t(1;2)(q42~43;q11.2~12),t(15;17)(q22;q21)[11]/46,XX[1]. Image capture and processing were performed using an ISIS imaging system (MetaSystems). Immunophenotyping was performed using a general panel of fluorescent antibodies against the following antigens typical for different cell lineages and cell types: CD1a, CD2, CD3, CD4, CD5, CD8, CD10, CD11b, CD11c, CD13, CD14, CD15, CD16, CD19, CD20, CD22, CD23, CD32, CD33, CD34, CD38, CD41a, CD45, CD56, CD57, CD64, CD103, CD117, CD123, CD138, CD209, CD235a, and CD243. In addition, antibodies to kappa and lambda light chains, IgD, sIgM, and HLADr were tested. All antibodies were purchased from BD Biosciences, San Jose, CA, USA. Samples were analyzed on a BD FACSCalibur??flow cytometer. Autofluorescence, viability, and isotype controls were included. Flow cytometric data acquisition and analysis were conducted by BD Cellquest??Pro software. Flow cytometric analysis of a peripheral blood specimen from our patient characterized this case as APL according to WHO classifications. The abnormal cell population (97 % of tested cells) was positive for MPO++, CD45+dim, CD34?? HLADr?? CD33+, CD13+, CD16?? CD64+, CD15+dim, and CD14??
Parker Boyle	28	1981/3/6	848-909-2740	[email protected]	45154 Crystal Ville	An 85 year-old Caucasian gentleman with hypertension, paroxysmal atrial fibrillation, hypothyroidism, stage 3 chronic kidney disease, mixed ischemic and non-ischemic cardiomyopathy, severe biventricular systolic dysfunction, secondary prevention cardiac resynchronization therapy device implant with defibrillator function, and frequent monomorphic VT presents with VT storm. Heart failure medical therapy was optimized and included beta-blockade. A prior trial of sotalol therapy provided no arrhythmia suppression. Amiodarone therapy provided some relief, particularly when augmented by mexilitine therapy, however drug-related lung and neurologic toxicities limited amiodarone use. Transvenous catheter VT ablation was attempted however the arrhythmia recurred a month later. On presentation, the patient had recurrent VT at 215 beats per minute. 12-lead electrocardiography revealed a monomorphic wide complex tachycardia with left bundle branch block like morphology, inferior axis, and late precordial transition. His arrhythmia precipitated frequent appropriate implantable cardioverter defibrillator anti-tachycardia pacing and shocks therapies. There was no evidence of ongoing reversible myocardial ischemia or metabolic and electrolyte abnormalities. Given his severe biventricular failure, prior sotalol and mexilitine failure, and drug-toxicities with amiodarone, antiarrhythmic options were limited. The patient was not a good candidate for a repeat ablation procedure or cardiac transplantation given his age and frail status. Having exhausted antiarrhythmic options, dronedarone therapy was considered for off-label use. After discussing potential benefits and risks regarding dronedarone use in the treatment of VT based on the limited data available in the scientific literature, the patient consented to an off-label trial of the agent at 400 mg twice daily. After only three doses, it was discontinued due to severe nausea and the development of multisystem organ failure including acute severe hepatotoxicity. This brief trial of dronedarone therapy had no appreciable effect of the patient? VT burden. The duration of the VT episodes was brief and not felt to be the cause of the patient? multi-organ failure that was temporally associated with the initiation of dronedarone therapy. Five days prior to dronedarone administration, the patient? serum alanine transaminase was measured at 59 U/L (normal 11??3 U/L), serum creatinine at 135 umol/L (normal 50??20 umol/L), and estimated glomerular filtration rate at 41 mL/min/1.73 m2 (normal >80 ml/min/1.73 m2). Two days after dronedarone administration a marked decline in kidney and liver function was observed with a serum alanine transaminase level increase to 1045 U/L, serum creatinine level of 231 umol/L, and estimate glomerular filtration rate of 21 ml/min/1.73 m2. Dronedarone therapy was subsequently discontinued. Unfortunately the patient? liver and renal failure continued to worsen despite drug discontinuation. He died eight days later from multi-organ system failure and metabolic shock.
Aliya Blair	21	1992/8/25	6598881065	[email protected]	354 Smith Stravenue	A 53-year-old white woman presented with increasing fatigue, loss of appetite, and severe epigastric pain radiating to her back. She had a history of MTC (pT2pN1b), diagnosed 25 years ago and treated by complete thyroidectomy and bilateral cervical lymph node dissection, followed by repeated surgical resection of lymph node metastases 19 and 12 years ago. No further specific treatment modalities (for example radiotherapy) had been employed. No genetic analysis had been performed for germline or somatic RET proto-oncogene mutations, nor for other somatic mutations (HRAS, KRAS, NRAS). On clinical examination, however, there was no indication of other manifestations of multiple endocrine neoplasia type 2 (MEN2), especially no clinical signs of pheochromocytoma. Since data on the RET proto-oncogene status have been missing in our patient, no risk category (moderate, high, highest) according to the revised American Thyroid Association Guidelines for the management of MTC [] had been assigned. However, based on the American Joint Committee on Cancer (AJCC) TNM-Classification, the MTC had been a stage IV A tumor (pT2pN1b) at the time of the initial complete thyroidectomy in our patient. Post complete thyroidectomy, she had hypoparathyroidism, which was treated with calcium and calcitriol. She received levothyroxine replacement therapy. Repeated biochemical and clinical evaluations during the course of the disease did not demonstrate any signs of paraneoplastic adrenocorticotropic hormone (ACTH) and/or corticotropin-releasing hormone (CRH) secretion. Medical therapy of the MTC included octreotide 20 mg every 4 weeks, which was switched to the tyrosine kinase inhibitor vandetanib 300 mg/day 11 month ago when computed tomography (CT) scanning revealed progressive mediastinal lymph node and diffuse and symptomatic pulmonary metastases. Standard laboratory tests in our patient revealed moderate leukocytosis (18.3 G/l) as well as slightly elevated serum CRP levels (12.58 mg/l); her serum lactate was 5.5 mmol/l. Pancreatitis was ruled out because her serum amylase and lipase levels were within normal range. An upper endoscopy was performed and demonstrated multiple centrally ulcerated lesions in her gastric mucosa of up to 4 mm in size (Fig. ). Two-phase contrast-enhanced multi-detector CT of her chest and abdomen revealed stable pulmonary and mediastinal lymph node metastases; however, there was a single new intrahepatic lesion of 4 mm size suspicious for liver metastasis. Contrast-enhanced multi-detector CT of her neck revealed multiple, new, centrally hypodense supraclavicular lesions up to 12 mm in size suggestive of lymph node metastases. In addition, multiple noduli within her stomach wall were apparent (Fig. , I?II). Three-phase bone scintigraphy showed no evidence of bone metastasis. Additional laboratory tests revealed a dramatic increase in her basal serum calcitonin levels from 563 pg/ml to 6487 pg/ml within 2 months, indicative of rapid tumor progress of the underlying MTC. Of interest, at the same time she had rather stable carcinoembryonic antigen (CEA) serum levels of 40.7 ng/ml (from 62.3 ng/ml 14 months earlier). Histologic and immunohistochemical analyses of gastric biopsy specimen demonstrated diffuse mucosal infiltration of MTC (Fig. ) with a KI-67 index of up to 40 %, proving metastatic disease of MTC to the gastric mucosa. Of note, while immunohistochemical analysis of calcitonin was strongly positive (Fig. ), staining for CEA demonstrated merely focal and weak staining (Fig. ); staining for thyroglobulin remained completely negative (data provided in Additional file ). She rapidly deteriorated and died due to multiorgan failure before third-line treatment could be initiated.
Troy Sullivan	28	2002/10/14	460-983-4661x21279	[email protected]	9704 Scott Island	A 37-year-old woman went to the clinic of a local hospital because of abdominal pain and hematochezia for one week. Colonoscopy examination showed a polypoid mass protruding into the rectum cavity, measuring approximately 5 cm in maximum diameter, with a rough and uneven surface (Fig. ). Then the patient underwent a partial rectectomy. The lesion was considered as a myogenic sarcoma, preferring pleomorphic leimyosarcoma by the referring pathologists. The postoperativ adjunctive therapy was not administrated . There was no evidence of recurrence or metastasis 8 months after surgery.
Melanie Jones	40	2005/7/17	001-682-453-0285x014	[email protected]	989 Fischer Skyway	The patient was a 55-year-old man who complained of abdominal pain and distension lasting for two days. Physical examination showed abdominal mass with tenderness. No obvious mass was found by colonoscopy. X-ray displayed intestinal obstruction. Computed tomography (CT) scan demonstrated a solid mass in the pelvic cavity (Fig. ). Clinically, the lesion was suspicious of gastrointestinal stromal tumor (GIST). Intraoperatively, the mass was found on the terminal ileum nearing the ileocecal junction. Partial ileal resection and appendectomy with intestinal anastomosis were performed shortly after. The lesion was originally diagnosed as malignant mesothelioma by the referring pathologist. After reevaluation of the H&E slides with application of immunohistochemistry and FISH analysis, the diagnosis of EIMS was finally rendered. The patient developed local recurrence 2 months after surgery. A re-excision of the recurrent tumor with adjuvant chemotherapy was performed. He was free of disease at 10-month follow up.
William Patton	23	1997/7/30	-7321	[email protected]	6876 Harris Keys Apt. 674	A 22-year-old young man presented with abdominal mass with intermittent abdominal pain for eight days and fever for five days. Subsequent CT scan revealed a solid heterogeneous mass of the right abdominal cavity, suspicious of GIST. After the admission, the patient underwent a complete excision of the abdomen mass and partial transverse colectomy soon. Intraoperatively, an enormous mass measuring approximately 20 ? 15 cm was identified, with multiple small omental or mesenteric nodules. Pathological examination of the submitted specimen showed that malignant tumor was suspected as GIST. To confirm the diagnosis, the pathological materials were sent to our department for further consideration. With the adjunctive study of immunohistochemistry and FISH, the lesion was re-diagnosed as EIMS. The patient developed local recurrence 2 months after surgery. The targeted therapy of ALK inhibitor (crizotinib) was administrated later. He achieved a partial response to the treatment. He was alive with disease at 14-month follow up.
Lorelei Nelson	44	1993/6/9	001-703-713-2217	[email protected]	46256 Daniel Mountain Suite 082	The patient was a 58-year-old woman who complained of a slowly enlarging and painless mass on the abdomen for two months. Physical examination showed an ill-defined solid mass of the right lower abdominal wall with no tenderness. Then she received a local resection. Intraoperative examination revealed that the mass was firm in consistency and infiltrated into the skeletal muscle of the abdomen wall. Pathologically, the tumor was considered as pleomorphic sarcoma by the referring pathologist. After surgery, the patient was treated with the additional chemotherapy. She developed local recurrence 2 months later and died of disease at 8-month follow-up.
Dylan Hudson	40	1995/7/2	931.643.0338x10215	[email protected]	6923 Jamie Rapids	A 15-year-old young woman complained of recurrent abdominal pain for four months and abdominal mass for four days. Physical examination revealed a well-defined solid mass of the right abdomen with mild tenderness. Ultrasonography displayed an abdominal tumor with heterogeneous internal echoes, measuring about 10.2 ? 7.5 cm. Subsequently, the patient received a complete excision of the abdomen mass and partial transverse colectomy. During the operation, the tumor was identified on the transverse colon and measured approximately 12 ? 8 cm, with multiple enlarged omental or mesenteric lymph nodes. The lesion was considered as fibrosarcoma with inflammatory cell infiltrates by the referring pathologist. After surgery, the patient did not receive the adjunctive therapy. She was free of disease at 7-month follow up.
Kamila Nichols	27	2003/12/28	(397)307-8203	[email protected]	10968 Carlson Street Suite 354	A 63-year-old man was referred to our hospital because of pain, redness and swelling in his left leg persisting for 1 month. At the onset of symptoms, he consulted the neighboring dermatological clinic where he was diagnosed with cellulitis. Antibiotics and nonsteroidal anti-inflammatory drug were administrated, but his symptoms did not improve. After 1 month, he was referred to our VTE specialist department. The patient had a history of hypertension, which was treated with a calcium channel blocker and an angiotensin receptor blocker. At the initial examination, his body mass index was 33 kg/m2, the vital signs showed a mildly elevated blood pressure (134/84 mmHg) and his peripheral oxygen saturation was 98 % in ambient air. The physical examination revealed a tender and swollen left leg. Laboratory test showed elevated levels of urinary acid (8.3 mg/dL) and blood glucose (116 mg/dL). His HbA1c was 5.7 %. Prothrombin and partial thromboplastin times were normal, although D-dimer concentrations were increased to 5.9 弮g/mL (normal, <1.00 弮g/mL). Plasma protein C, protein S, and antithrombin levels were within normal limits, and creatinine clearance was 113.8 mL/min, as calculated using the Cockcroft?ault method. Chest X-rays showed no abnormalities. Echocardiography was significant for mild concentric left ventricular hypertrophy with normal left ventricular function and showed no signs of increased right ventricular pressure. DVT was suspected because of the patient? symptoms and elevated D-dimer concentrations. Ultrasonography revealed a thrombus in the left but not in the right femoral vein (Fig. ), and computed tomographic angiography revealed clots in the right distal pulmonary artery. The maximum circumference of the thighs showed a swelling in the left leg (left calf: 47 cm, left ankle: 28.5 cm, right calf: 44 cm, right ankle: 27 cm). We instructed the patient to wear medical compression stockings and provided the anticoagulant treatment with subcutaneous injections of fondaparinux (7.5 mg once daily for 5 consecutive days on an outpatient basis), followed by oral administration of edoxaban (60 mg once daily from day 6). Different from western countries, the self- injection of fondaparinux is not allowed in Japan, so the patient was required to visit the hospital every day. After 2 weeks, swelling of the left leg improved, although redness and pain persisted. Subsequent ultrasound examination of the leg after 3 months of treatment with edoxaban revealed no clots in the left superficial femoral vein and soleus muscle vein. Moreover, D-dimer concentrations had dropped to normal levels, indicating decreased activation of secondary fibrinolysis. However, because some clots were still observed in the popliteal vein (Fig. ), treatment with edoxaban was continued. After 6 months, the swelling and redness in the lower leg had completely disappeared, and D-dimer concentrations remained normal. Ultrasonography showed further regression of the clots in the left popliteal vein, leaving only mural thrombi (Fig. ). Throughout the treatment period, doses of fondaparinux and edoxaban were not changed, and no bleeding complications occurred. The symptom of recurrence of pulmonary embolism was not observed during the clinical course. We suggested to end the edoxaban therapy after 6 month, but the patient hoped for a continuation of the anticoagulation therapy. Until recently in Japan, proper management of DVT required continuous antithrombotic therapy using unfractionated heparin followed by VKA administration within the optimal therapeutic range. In Japan, fondaparinux was approved in 2011. The approval of fondaparinux injections for VTE has relieved patients and physicians of the need for complex dose adjustments, 24-h infusion control, and blood tests every 6 h. Fondaparinux is associated with recurrent VTE and major bleeding rates similar to those occurring with intravenous unfractionated heparin (UFH) [, ]. The reasons for outpatient treatment in this case were that the patient was hemodynamically stable and did not suffer from renal failure, cancer, massive pulmonary embolism, heart failure, or bleeding. Furthermore, he had a good understanding about his illness. Edoxaban was approved in Japan prior to the world as an oral factor Xa inhibitor, and the ESC guidelines recommend the use of edoxaban following acute-phase parenteral anticoagulation therapy. Dabigatran also gained approval as an oral VTE treatment following acute-phase parenteral anticoagulation therapy by Europe and United States besides Japan. The RE-COVER and Hokusai-VTE trials mandated at least 5 days of heparin or low molecular weight heparin (LMWH) treatment prior to initiation of dabigatran or edoxaban [?. Dabigatran and Edoxaban both showed a no inferiority to warfarin for the prevention of recurrent VTE, and a significantly lower risk of major bleeding. Additionally, a lower proportion of the administered dose of edoxaban is eliminated via the kidneys (35 %) compared to dabigatran (85 %) [, ]. The pharmacokinetics of edoxaban were not affected by enoxaparin, whether administered concomitantly or 12 h apart []. Edoxaban is a once daily tablet, which is easier to swallow than dabigatran. In addition, dabigatran is not approved for VTE treatment in Japan. These reasons led us to choose this regimen using fondaparinux and edoxaban in this case. The ACCP guideline stated that in patients with acute DVT of the leg, the guideline suggest early ambulation over initial bed rest (Grade 2C), and that in patients with acute DVT of the leg and whose home circumstances are adequate, the guideline recommend initial treatment at home over treatment in hospital (Grade 1B). Unfortunately this patient couldn? be hospitalized, so we started the anticoagulant therapy carefully. According to the RIETE registry [], home treatment of DVT is appropriate in younger men with adequate weight who have no complications of heart failure, respiratory diseases, or cancer. Additionally, we suggest that patients must also understand their condition of illness, visit the hospital daily for fondaparinux administration at least 5 days, and put on and remove medical stockings by themselves or with the help of a family member. Moreover, other medical institutions, including specialist facilities that manage hemorrhagic events or pulmonary embolism, and clinics that cooperate with appropriate specialist institutions are appropriate alternatives to hospitals. The objectives of DVT treatment include the prevention of recurrence and regression of thrombotic clots, although echogenic masses tend to regress slowly []. The precise mechanism by which the factor Xa inhibitor edoxaban causes thrombus regression remains unclear, and no fibrinolytic activity of this agent has been described. Initiation of fibrinolysis occurs through a number of orchestrated interactions among fibrin and plasminogen as well as its activator and results in the generation of plasmin []. Therefore, the efficacy of edoxaban may reflect the potency of the fibrinolytic response relative to that of the coagulation response. Edoxaban reportedly exerts a stable anticoagulant effect compared with conventional drugs [, ] and serum D-dimer concentrations are widely used as a marker for secondary fibrinolysis following clot formation. In the present case, D-dimer concentrations rapidly normalized within 2 weeks and remained in the normal range at 6 months. However, clots continued to regress after the normalization of D-dimer concentrations, warranting the development of more sensitive markers of fibrinolysis during slow regression of thrombosis. In the Hokusai-VTE Study [], edoxaban was compared with VKA. A therapeutic range was achieved ??0 % of the treatment period and the reoccurrence of thrombosis was effectively prevented. Factor Xa inhibitors may provide more stable effects on the fibrinolytic system easily. The ESC guidelines recommend that treatment for DVT be continued for at least 3 months, and extensions of the treatment period should be considered depending on individual patient conditions. Our patient? VTE risk was idiopathic, which is why we treated him with NOACs for more than 3 months. In this case, symptoms improved after 3 months, and thrombotic clots remaining in the popliteal vein subsequently regressed further while continuing treatment, indicating the importance of thorough follow-up with ultrasonography and monitoring of subjective symptoms and blood parameters. Attention is described at the end. In this case, he was misdiagnosed with cellulitis at the onset. The initial misdiagnosis leads to delay of treatment. Cellulitis is an acute, spreading pyogenic inflammation of the dermis and subcutaneous tissue, usually complicating a wound, ulcer, or dermatosis. The area, usually on the leg, is tender, warm, erythematous, and swollen. The differential diagnosis of cellulitis is very important to DVT [].
Atlas Cortes	23	1999/1/2	-4447	[email protected]	056 Frazier River	The patient is a 32 year old female at 24 weeks of gestation who presented in January 2014 with bilateral submandibular swelling and pain with radiation to head, neck, chest, and back for 2 weeks. On her initial presentation she was found to have WBC 164 ? 109/L with a differential of 94.4 % lymphoblasts, platelets 27 ? 109/L, and hemoglobin of 10.4 gm/dL. A peripheral blood flow cytometry revealed abnormal immature B cell population that comprised 90 % of the total cells and were positive for CD10, CD19, CD22, CD34, Tdt, but negative for CD 20. These findings were characteristic for precursor B-cell ALL. Cytogenetics was negative for Philadelphia chromosome. Karyotyping showed a normal 46XX chromosome display. FISH was negative for BCR/ABL and MLL gene rearrangement on chromosome 11q23. Due to the pregnancy status, she underwent chemotherapy induction with the modified Linker regimen in 01/2014 [?. This included cyclophosphamide (1 gm/m2) on day 1, vincristine 2 mg IV on days 1, 8, 15, 22, 30, 37, and 44; daunorubicin 50 mg/m2 on days 1??, 30 and 31, and prednisone 100 mg po days 1??, 80 mg on days 5??8, tapered slowly to off on day 62. Cerebral spinal fluid was negative for malignant cells. On day 38, she had a bone marrow biopsy revealing a hypocellular marrow, erythroid hyperplasia, dyserythropoiesis and atypical immature B-cells consistent with residual disease of precursor B-ALL. Concurrent flow cytometry of the aspirate showed an immature B-cell population (1.8 % of total B cells) expressing CD19, Tdt. Clinically she was in complete remission by standard definition with platelets 140 ? 109/L absolute neutrophil count (ANC) 1.43 ? 109/L. She had C-section at gestation week 34 and delivered a healthy girl. The second cycle of chemotherapy began 1 week post-partum with standard hyperCVAD regimen B in 3/2014 [, ]. She had Ommaya reservoir placed and received intrathecal methotrexate twice per cycle following the standard protocol defined in the hyperCVAD regimen. This was followed by six more cycles of hyperCVAD. Shortly after completion of last hyperCVAD, she started to have progressively worsening cytopenia. In November 2014, a bone marrow aspiration and biopsy were done to evaluate her disease status. The flow cytometry study of the aspirate showed a 79 % lymphoblast population expressing the following markers: CD19, CD10, Tdt, and CD34. This was consistent with full relapse of her precursor B-cell ALL. Karyotyping was 46XX. The patient received re-induction chemotherapy with high dose cytarabine (3 gm/m2 daily IV over 3 h ? 5), and mitoxantrone (60 mg/m2 once) []. A repeat bone marrow aspirate and biopsy was performed in 1/2015 and revealed 43 % lymphoblasts. This confirmed persistent refractory precursor-B ALL. Salvage chemotherapy with CLAG regimen (cladribine, cytarabine and G-CSF) was started immediately [?. Four weeks later, a repeat bone marrow biopsy showed 68 % lymphoblasts, consistent with refractory disease to the CLAG regimen. In February 2015, she was treated with blinatumomab. She was started with 9 mcg/day. On day 3 of blinatumomab therapy, the patient started spiking fever to 102.7 簞F, with tachycardia and hypotension. The patient was transferred to the ICU for closer monitoring. Chemotherapy with blinatumomab was temporarily held for suspected cytokine release syndrome (CRS). The patient received one dose of tocilizumab 8 mg/kg IV once in addition to methylprednisolone. She required aggressive fluid resuscitation and was on vasopressors for 2 days. Blinatumomab was restarted 2 days later at 9 mcg/day. In total she received 9 days of blincyto at 9 mcg daily. The dose was increased to 28 mcg per day and continued to day 28. Her blood counts improved steadily. A bone marrow biopsy was performed. Flow cytometry of the aspirate revealed no immature B-cell population. Pathology revealed trilineage hematopoiesis with no increase in blasts. A concurrent peripheral blood counts showed platelets of 247 ? 109/L and ANC > 1 ? 109/L. At this point, the patient had a pathology documented complete remission (CR) after one course of single agent blinatumomab. Since she was not eligible for allogeneic hematopoietic stem cell transplantation (HSCT), maintenance chemotherapy with MTX, vincristine, pegylated asparaginase and dexamethasone was given. However, she developed acute pancreatitis after four cycles of the PEG-asparaginase-containing regimen. Upon recovery from the pancreatitis, a bone marrow biopsy confirmed that the patient remained in CR in August 2015. In September 2015, she was started on a 2-week cycle of blinatumomab as maintenance. Due to lack of convincing scientific data for using blinatumomab as maintenance treatment, further treatment with blinatumomab was stopped. She maintained normal blood counts and had normal activity. Unfortunately, in February 2016, her WBC increased to 16.4 ? 109/L, platelet counts decreased to 75 ? 109/L. Bone marrow biopsy at this point showed an abnormal immature B-cell population expressing CD19 (dim), CD22, CD10, CD34, TdT, HLA-DR, CD9 and CD38 (heterogeneous). The findings were consistent with relapsed preB-ALL. Since CD19 was still positive, blinatumomab was started. Two weeks later, the WBC rose to 74.4 ? 109/L, platelets down to 19 ? 109/L. Peripheral blood had 71 % blasts. Therefore, her ALL was refractory to blinatumomab re-treatment. At the time of this report, she was receiving additional treatment.
Lea Bowen	29	1984/12/11	001-558-442-0131x02341	[email protected]	355 Malik Turnpike Suite 442	The client, a single woman in her late 30s, presented for BATD exhibiting sadness, anger, poor familial relationships, feeling that her life was ?oring?? the wish to die, self-hatred, feelings that no one listens to her, and lack of compassion. She also reported recurrent thoughts about harming (and killing) her family members. The client lived with her brother and his family. The client had experienced significant trauma resulting from the Halabja chemical attack, where she lost her parents and was displaced to the town of Hawar with her brother. She was there for 7 months before she went to Iran for chemical treatment. The client visited a psychiatric hospital after the Halabja bombing, where she received psychotropic medication. With the onset of BATD, the client identified her core values and generated specific activities tied to those values. For instance, she valued compassion, sincerity, loyalty, honesty, and nurturing her brother's children as her own siblings. In accordance, she identified talking on the phone with her family, cooking, and helping them with their work as activities tied to these values. She also specified activities in line with her values of honesty, having good friends, loyalty, sincerity, learning new information, performing jobs appropriately, maintaining good relationships with employers, better self-care, psychological improvement, and completing daily chores. The CHW reported the client was motivated to participate in BATD and completed 12 sessions. Difficulties with her treatment included her brothers??resistance to her treatment given concerns about stigma including unintended consequences for the family if others became aware of her seeking treatment. This barrier, in addition to her demanding work schedule, hindered her ability to attend weekly sessions. As a solution, her treatment provider who arranged home and work visits spoke with her brother regarding treatment, which resulted in his willingness to allow her to continue treatment. As a result of BATD, the client experienced many positive changes in her life. Specifically, she reported improved relationships with her family, loving herself and the people around her, the desire to live and enjoy life, reduced sadness and anger, and improved job performance. Indeed, her brother witnessed these positive changes, and even asked to be a client himself. The focus of BATD on adherence to her daily goals helped engender the congruence of her lifestyle and value system, her motivation to treat her depression, and her perseverance and commitment to continue treatment despite logistical constraints. Furthermore, her treatment allowed her to obtain a referral to a psychiatrist and be properly diagnosed with epilepsy, which had been misdiagnosed previously.
Trevor Pope	33	2003/11/27	261.998.5603	[email protected]	3692 Richard Tunnel Suite 530	A 60-year-old Caucasian male with a past medical history of hypertension and coronary artery disease initially presented with acute onset left arm weakness and left facial droop. He was first evaluated by a local health care facility where a CT scan of his head was done and revealed a right frontal intraparenchymal hemorrhage with surrounding edema, mass effect, and midline shift. He was transferred to the neurosurgery service at our medical center for further management. The patient had been seen at an outside institution a week prior to admission for recent back pain and was found to have an elevated international normalized ratio (INR) of 6.1 at that time. He denied fevers, chills, shortness of breath, chest pain, early satiety, nausea, vomiting, or abdominal discomfort at admission. He had a history of hypertension and coronary artery disease with ischemic cardiomyopathy and left ventricular ejection fraction of 25% with a left ventricular aneurysm and apical thrombus for which he has been on chronic anticoagulation. Medications on admission included aspirin, lisinopril, allopurinol, metoprolol, niacin, simvastatin, warfarin, venlafaxine, and Ambien as needed. He had no known allergies. He lived with his wife, used to drink one or two beers a couple of times a week, and denied any recreational drugs use. One of his sons was diagnosed with acute lymphoblastic leukemia (ALL) at the age of 13 and was 21 years out from his treatment. He has no family history of hematological or other neoplastic diseases. The patient agreed to participate and was explained the nature and objectives of this study, and informed consent was formally obtained. No reference to the patient's identity was made at any stage during data analysis or in the report. His physical examination did not reveal any lymphadenopathy or splenomegaly. No focal neurological deficit was noticed. Initially, the patient? intracranial hemorrhage was thought to be in the setting of a supratherapeutic INR due to chronic anticoagulation with coumadin for left ventricular apical thrombus. However, his admission laboratory data showed leukocytosis with a white blood cell count (WBC) of 298,000/cmm with early forms (promyelocytes 7%, myelocytes 8%, metamyelocytes 4%, blasts 1%). The differential count at that time was neutrophil 61%, band 11%, lymphocytes 2%, basophils 2%, eosinophils 2% with platelet count of 76,000/cmm, hemoglobin of 10.1 gm/dL. Due to the concern of probable leukostasis secondary to leukocytosis contributing to the intracranial hemorrhagic stroke, the patient underwent leukapheresis (two units of whole blood phlebotomized) and received two units of red cell transfusion which resulted in a significant decline in WBC count to 228,000/cmm. Flow cytometry and FISH revealed 9:22 translocation; Philadelphia chromosome was consistent with the diagnosis of CML. A bone marrow biopsy also confirmed the diagnosis of CML, and there was no evidence of increased blasts in the bone marrow, indicating that he was in the chronic phase of CML. Quantitative PCR showed 43.1% B2A2/GUS. During hospitalization, aspirin and warfarin were stopped, and he was managed conservatively with platelet transfusions and close observation. He did not require surgical intervention and began rehabilitation therapy for his left hemiparesis. He was started on hydroxyurea (Hydrea) two grams daily titrated up to two grams twice daily followed by imatinib (Gleevec) 400 mg orally daily. The patient returned for a follow-up to the hematology clinic two weeks after discharge; labs showed a WBC of 3,500/cmm with no immature forms, and the differential count was neutrophil 60%, band 0%, lymphocytes 29%, basophils 1%, eosinophils 1% with platelets of 50,000/cmm, hemoglobin of 12.1 g/dL. Peripheral smear showed decreased platelet count with normal-looking neutrophils without any immature forms such as myeloblasts or promyelocytes and no evidence of dysplasia such as hypogranulated neutrophils, basophilic stippling in the red cells, or giant platelets. He was in hematologic remission, and the dose of Gleevec was decreased to 300mg daily from 400mg daily in the setting of thrombocytopenia that was initially attributed to Gleevec. The patient returned to the clinic after two months for follow-up; Gleevec was increased back to 400mg daily since there was no significant change in his platelet count by decreasing the dose, and his platelet count at that time was 65,000/cmm. He was then followed up in clinic every two months, and quantitative FISH demonstrated a complete cytogenetic response at nine months. The patient had a decrease in BCR-ABL PCR transcript on testing at twelve months follow-up from 43.1% to 0.29%, demonstrating a major molecular response. The patient returned to the clinic for an interval follow-up. Following several months of conservative management and evaluation of worsening lower back pain over five months that was initially attributed to long hours of driving, he had a CT scan done at an outside hospital which demonstrated a likely perforation of the rectosigmoid colon, and was hospitalized for management. Upon admission, Gleevec was discontinued with the identification of bowel perforation as a potential rare but serious complication of Gleevec. He continued to have episodes of pain up to 8-10/10 in intensity and hence transferred to our facility. A repeat CT scan of the abdomen and pelvis with contrast showed evidence for extravasation of the administered rectal contrast into the perirectal region. Multiple pockets of gas were seen posterior and lateral to the distal rectum in the presacral region and in the region of the rectal wall with noted surrounding inflammatory changes but no organized fluid collection. This was followed by a colonoscopy with special care taken to avoid overinflation or advancement of the colonoscope beyond the affected region based on CT findings. However, colonoscopy did not reveal perforation. He was managed conservatively after consulting surgery and finally discharged home in improved condition. Also, he was resumed on Gleevec. One month later, he presented with recurrent lower GI bleeding. A repeat colonoscopy was done that reportedly revealed a rectosigmoid fistula into the retroperitoneal space, confirming the previous perforation. Gleevec was discontinued indefinitely at that time. He had a follow-up in 14 days with a flexible sigmoidoscopy which showed an edematous mucosa 10 cm from the entry point, and biopsy was consistent with chronic nonspecific inflammation and mild focal hyperplastic changes and reportedly negative for malignancy. Unfortunately, he again presented to the emergency department with similar symptoms of rectal pain and bleeding. CT scan at that time showed the possibility of abscess in the same area. An examination under anesthetic (EUA) along with incision and drainage of a perirectal abscess was performed along with a drain placement. He was followed up in four weeks both in hematology and surgery clinics. At this time, a follow-up CT scan of the pelvis showed that the cavity is smaller, but the track is still open which was thought to be reasonable since the drain fell out three days prior to presentation. The patient had no new accumulation of pus, no fevers, no chills, no back pain, and the perianal irritation was improving.
Aurelia Raymond	40	1983/12/2	414-998-4450x2493	[email protected]	9044 Williams Wells Suite 748	A 71-year-old female patient was presented to the emergency department after falling from her own height with a direct contusion on both hands, with the wrist and elbows in extension. She had a past medical history which was positive for arterial hypertension (treated with Captopril) and rheumatoid arthritis (treated with Leflunomide). Her fall resulted in localized pain in both shoulders, and limited range of motion leading to an inability to perform activities with her arms raised above the head. X-rays were taken on an anteroposterior (AP) bilateral view (Figure ), and she was diagnosed with a Neer III bilateral displaced proximal humerus fracture. Simple X-rays are the most commonly used diagnostic method for a proximal humerus fracture, and often two different views are necessary to analyze the pattern of a fracture. AP and lateral views should be taken. In cases of complex fractures or uncertainty based on the x-rays, a computed tomography (CT) scan can be used. After a clinical evaluation of the patient, though there was no contraindication for surgical treatment, conservative treatment was chosen to address the pathology. Two hanging casts were placed using two slings on both arms. A week later, a control x-ray was taken showing a reduction of some of the fragments (Figure ). At week three of treatment, a new x-ray was taken showing even more bone fragment reduction when compared to week one (Figure ). Clinically, the patient showed consolidation with partial radiographic consolidation, so the casts were removed. She continued to use the bilateral slings and initiated pendulum movements for the following three weeks at home. She initiated active movements at week six. At the week seven follow-up consultation, she arrived with only mild pain during any activity behind the back during active internal rotation on both arms. The bilateral ranges of motion were as follows: flexion, 100簞; extension, 15簞; internal rotation, 100簞; external rotation, 90簞; abduction, 100簞; adduction, 30簞; muscle strength, 5/5; with no alteration of the distal neurovascular status (Figure ). We obtained the Disabilities of the Arm, Shoulder and Hand (DASH) Score of 16/100 (100 representing the worst-case scenario).
Maurice Contreras	35	1995/2/7	7659168530	[email protected]	12722 Rachel Shoals	A 57-year-old woman was referred to our orthopaedics and traumatology clinic with a painful lump in the left arm. Physical examination revealed a red-colored mass on the left arm (Fig. ) and an enlarged lymph node measuring almost 5 cm in the left axillary region and 3 cm in the right axillary region. Magnetic resonance imaging (MRI) of the left arm showed a 17.7 ? 5.8 ? 7.3 cm enhancing mass in the medial aspect of the left biceps muscle and multiple left axillary lymph nodes with a maximal diameter of 4.8 ? 3.4 cm. Tru-cut biopsy of the mass in the left arm revealed infiltration of atypical centrocyte-like cells with or without clear cytoplasm with a K襤-67 proliferative index of 30 % and stained positive for CD20 and pax-5 and negative for CD5, CD10 and bcl-2, positive for CD21 in the follicular dendritic cell network, findings consistent with MZL (Figs. , ). With a final diagnosis of MALT lymphoma infiltrating the skeletal muscle, she was referred to our hematology department. The patient had no B symptoms. Her hemoglobin was 12 g/dL, white blood cell count 8420/mm3 with a lymphocyte count 2800/mm3 and platelet count was 292,000/mm3. On biochemical tests, the following were abnormal: lactate dehydrogenase 1218 U/L (240-480), CRP 40 mg/L (0??), ESR 120 mm/h (0??0) and 帣2-microglobulin level 3.52 mg/L (0.7??.8). Serum protein electrophoresis showed polyclonal gammopathy with an increased component with gamma fraction of 1.51 g/dL. Hepatitis B, C and HIV infection serology and screening for autoimmune disorders were negative. Bone marrow biopsy was negative. Cervical computed tomography (CT), and abdominopelvic CT were normal. On chest CT, there were multiple left axillary lymph nodes with a maximal diameter of 5 cm and a 3 cm right axillary lymph node. Diagnosed with stage 2 disease, R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, methylprednisolone) was initiated. After 6 cycles, mass in the medial aspect of the left biceps muscle regressed to a size of 3.2 ? 1.1 ? 1 cm and bilateral axillary lymph nodes totally disappeared. Two additional infusions of rituximab were administered after the sixth cycle of R-CHOP. Subsequently, the patient underwent radiotherapy to the left arm at a dose of 30 Gy. Physical examination 1 and 6 months and 1 year after treatment revealed no palpable residual mass, swelling or skin erythema. 6-months and 1-year after completion of treatment, MRI of the left humerus demonstrated no evidence of residual disease. Also, cervical, chest and abdominopelvic CT at 1 year showed no disease involvement.
Daniela Arellano	29	2005/3/21	(419)879-2452	[email protected]	588 Zachary Brooks	A 4-year-old girl with unexplained hypergastrinemia was referred to our department after abdominal ultrasonography detected a cystic lesion at her prepyloric antrum. When she first presented with a duodenal ulcer at 2 years of age, her serum gastrin levels had ranged between 700 and 1000 pg/ml. The imaging studies with a contrast-enhanced computed tomography (CT) scan had repeatedly revealed no tumor around the pancreas or duodenum suggestive of gastrinoma but revealed only a thickened wall of the duodenum potentially due to the initially severe duodenal ulcer. She had continuously taken a proton pump inhibitor (PPI) until the referral since the first presentation of the duodenal ulcer, which had relieved its symptoms such as epigastric pain. At the referral, the patient? physical and mental development were normal for her age. She was afebrile, and other vital signs were also within the normal ranges. A physical examination revealed no particular symptoms. Her blood tests were unremarkable except for elevated serum gastrin (877 pg/ml, normal range 37??72 pg/ml). Upper gastrointestinal series revealed a 2.5 ? 5 cm cystic lesion on the anterior wall of the antrum, communicating with the gastric lumen (Fig. ), which was also detected as a solid mass with slight enhancement on abdominal contrast-enhanced CT (Fig. ). Upper endoscopy showed a scar at the duodenal bulb, but no erosion or ulcers in the stomach or the duodenum, and we could not detect the opening of the lesion. A gastric tissue biopsy did not demonstrate Helicobacter pylori. According to these findings, we suspected gastric duplication. As we speculated that the lesion might have caused the duodenal ulcer in this patient, we proceeded with operative treatment for the lesion. The operation was performed with the umbilical approach. A 2 ? 3 cm protruded lesion was observed on the anterior wall of the gastric antrum (Fig. a) and resected with the common muscular layer with the opening to the gastric lumen (Fig. b). A pathological examination was consistent with gastric duplication with antral mucosa, which strongly stained positive for gastrin (Fig. ) and also contained heterotopic pancreas tissue. The postoperative course was uneventful, and the patient was discharged on the eleventh postoperative day (POD). Her serum gastrin level remained elevated after the operation (755 pg/ml on POD 7 and 727 pg/ml on POD 98) but started to decrease after PPI was switched to famotidine 3 months after the operation (228 pg/ml on POD 148 and 235 pg/ml on POD 291) and finally normalized after famotidine was stopped 10 months after the operation (151 pg/ml on POD 365 and 77 pg/ml on POD 461). She has remained entirely asymptomatic during a follow-up of 20 months. Enteric duplications are ectopic cystic or tubular structures composed of smooth muscle surrounding the mucosa of the gastrointestinal tract and occur most commonly along the ileum, esophagus, or colon []. Gastric duplications are relatively rare in children, accounting for only 4?? % of all enteral tract duplications []. They are usually spherical or tubular cysts in the greater curvature of the stomach and may not communicate with the gastric lumen []. Their presentations range from asymptomatic to an acute abdomen and include gastric outlet obstructions, pancreatitis, hemoptysis, gastrointestinal bleeding, and an ulcerated antral mass []; hypergastrinemia has been rarely reported [] as one of the complications associated with a gastric duplication. Normally, acid in the gastric lumen acts directly on the somatostatin cells (D cells) to stimulate the release of somatostatin, thereby preventing gastrin release from gastrin cells (G cells) by a paracrine mechanism. When gastric antral mucosa is isolated from its usual acidic environment, such as in cases of a retained gastric antrum following partial gastrectomy with Billroth II reconstruction, there is no hydrogen ion feedback inhibition of gastrin release, leading to hypergastrinemia followed by increased gastric acid production, recurrent ulceration, and bleeding []. We speculate that this same mechanism caused hypergastrinemia and the resultant peptic ulcer at the initial presentation in our case whose ectopic antral mucosa in the duplication barely communicated with her gastric lumen. If more gastric acidic juice comes into the duplication lumen through a larger opening and/or the amount of the G cells is less than that in our case, hypergastrinemia may not happen. A literature review revealed only one case of a gastric duplication [] and another of a pancreatic duplication [] presenting with hypergastrinemia. Stephen et al. [] reported a female who presented with hematemesis and melena at 2 weeks of age. The possibility of gastrinoma was excluded using a secretin stimulation test and imaging studies; however, exploratory laparotomy could not detect any pathology, and thus, antrectomy and Billroth II reconstruction were performed. The pathological findings revealed gastroduodenal erosions and an antral duplication cyst with deep glands at the intracystic mucosa that stained positive for gastrin. Siddiqui et al. [] reported the case of a 2-year-old girl who presented with failure to thrive and gastroesophageal reflux with continued low gastric pH levels. Portal and pancreatic venous sampling in that patient during the operation revealed elevated levels of gastrin to the pancreatic venous cascade, and CT demonstrated a cystic lesion in the head of the pancreas. The patient underwent local resection of the lesion, and a pathological study confirmed the duplication with gastric mucosa (immunostaining of gastrin was not performed). Although the time course of the serum gastrin level of the latter patient was not described, that of the former patient normalized promptly after the operation. In contrast, the reason why hypergastrinemia in our case did not improve until PPI and famotidine were stopped may be explained by another mechanism related to the preoperative long-term use of PPI. A modest increase in the serum gastrin level in adult patients under a long-term treatment with acid-lowering compounds, especially PPI, has been reported []. PPI causes potent suppression of gastric acid secretion and leads to persistently high intragastric pH, and long-term PPI therapy has been reported to be associated with a significant increase in the G cell numbers and the ratio of G to D cells in the gastric antrum in children []. According to previous reports [, ], we speculate that the pathophysiology of our case was as follows: (1) the antral mucosa in the duplication was isolated from its typically acidic environment, which led to the lack of hydrogen ion feedback inhibition of gastrin release, and therefore, resultant hypergastrinemia caused the duodenal ulcer; (2) PPI suppressed gastric acid secretion and improved the duodenal ulcer, but the secretion of gastrin continued from the duplication for 2 years until it was removed, with an increase in the antral G cell numbers and the G to D cell ratio caused by the long-term use of PPI, thus additionally leading to hypergastrinemia; and (3) even after the removal of the duplication, hypergastrinemia continued until PPI and famotidine were stopped by the same mechanism.
Kellan Estrada	19	1991/2/1	4798471411	[email protected]	332 Weiss Roads Suite 415	A 26-year-old gentleman presented to his primary care physician with right knee swelling after a direct fall on his knee during a Frisbee game 2 days earlier. He denied hearing or feeling a popping sensation. The swelling had developed over a few hours but started diminishing since the day before the visit. His moderate pain has been improving since the incident. He had been able to walk with minimum discomfort. His past medical, social, and family histories were unremarkable. On physical examination, he had a mild ecchymosis and abrasion in the superior aspect of his knee with mild swelling. Plain radiography demonstrated soft tissue swelling anteriorly without osseous abnormality (). He was advised to rest and use ibuprofen as needed for pain. He presented to our sports medicine clinic with continuous, painless swelling in the same region 19 days after injury. He denied mechanical symptoms and his physical examination was significant for a nontender, moderate sized swelling in the superomedial aspect of his right knee (). There was no palpable joint effusion. In-office ultrasound revealed a homogeneous, anechoic fluid collection with scattered hyperechoic substance between the superficial quadriceps fascia and subcutaneous tissue which was compressible (). After proper cleansing and local anesthesia with 1% lidocaine, using ultrasound for needle placement and an 18-gauge needle, 38 mL serosanguinous fluid was aspirated (). Patient was advised to use a compression wrap following the procedure. He presented for recurrence of his knee swelling on day 25 after injury. Another aspiration provided 35 mL of serosanguinous fluid. After the second aspiration, his symptoms were completely resolved with no reaccumulation of the fluid. At latest follow-up, 4 weeks from injury, he is asymptomatic and had returned to full, unrestricted activity.
Sawyer Simon	28	2005/2/22	001-335-498-0886x2609	[email protected]	58666 Manning Crossing Suite 538	A 41-year-old healthy male was crushed by a forklift and brought to our ER in hemorrhagic shock. He suffered a massive open injury to his left leg and pelvis (). ATLS protocol was followed, the massive transfusion protocol was activated, and a pelvic binder applied for his APC 3 pelvic injury. He was transferred to the OR for intra-abdominal bleeding identified on FAST exam and a lack of response to transfusion. A laparotomy was performed where intra-abdominal bleeding was eventually controlled and then the aorta and IVC were clamped low in the abdomen to stop the bleeding from his pelvis and left lower extremity. During this time the left leg wound was debrided and irrigated, and it was realized that a high above knee amputation was required as the leg was nonviable. A pelvic EXFIX was placed, and the patient was given a high diverting colostomy. Urology placed a suprapubic catheter and removed an avulsed devascularized left testicle. Once the vessels were ligated distally, the aorta and vena cava were released (). He was transferred to the ICU. He received 31 units of PRBC, 18 units of FFP, 25 units of platelets, and 5 units of cryoprecipitate over the first 24 hrs and stabilized hemodynamically. The patient underwent serial debridement because of ongoing and progressive tissue necrosis in the stump, treated with negative pressure wound vacs and antibiotic beads. The above-the-knee amputation was converted to a hip disarticulation but the patient still had a massive wound over his pelvis and his lower abdomen. The wound vacs were difficult to apply with the external fixator and the pelvis was still relatively unstable so the external fixator was removed and reduction of his pelvic ring injury was performed with a subcutaneous anterior internal fixator, as well as one percutaneous SI joint screw (). This presented somewhat a challenge given the extent of the patient's open wounds on the left hemipelvis leaving part of the internal fixator exposed to air on the left side. The construct stabilized the injury and the low profile of the INFIX allowed easy application of the wound vac. The wound and patient stabilized, and skin grafts were applied to the defect once good granulation tissue was apparent around the pelvic INFIX (). The patient underwent removal of his suprapubic catheter and eventual reanastomosis of the bowel once all the wounds had healed. He was fortunate to have functional return of his urethra. The traumatized area under the skin grafts has had formation of heterotopic bone which required removal on 2 occasions due to pain with prosthetic fitting. This is finally underway 2 years after the injury.
Zayne Conner	28	1983/1/26	(407)548-0677x0777	[email protected]	282 Cole Circle Suite 116	This is a 62-year-old male who was involved in a motor vehicle accident. His open injury was missed initially when he arrived in extremis and developed a massive pelvic soft tissue infection requiring debridement over the first 3 days. He had a colostomy, superpubic catheter, percutaneous reduction of his iliac wing, SI screws, and an anterior INFIX. His testicles were implanted under the medial thigh and he had extensive wound care (Figures and ). This was followed by a long ICU course and dressing changes daily. The wound granulated in and the INFIX was removed once we felt that there was adequate healing (5 months). The patient had skin grafts and still has a colostomy.
Alondra Rush	25	1989/11/18	001-267-880-2479	[email protected]	7350 Colleen Islands	A twenty-eight-year-old Caucasian female, weighing 50 kg, mentally retarded, but with no other dysfunction or any detected genetic abnormalities, had suffered from a refractory form of epilepsy for many years and had been treated in a special neurological unit with multimodal antiepileptic medication regime consisting of carbamazepin, topiramate, and clobazam and stimulatory impulses of the vagal nerve. The patient had a history of developing tonic-clonic seizures of which there were several episodes during the week before admission. Following admission to the intensive care unit (ICU), the usual antiepileptic medication was supplemented with valproate, phenytoin, and levetiracetam without successfully alleviating seizures. The patient was finally anaesthetized with propofol and remifentanil infusions and intubated to secure airway. The mean dosage of propofol infusion was 12 mg/kg/h over a period of 78 hours, but the maximal infusion rate was 84 mg/kg/t before successful burst suppression was achieved. The first symptom suspected to be PRIS manifestation was unexplained metabolic acidosis with increased lactate concentration which initially appeared 48 hours after admission to the ICU. It was decided to decrease the rate of propofol infusion. Blood pressure declined, despite norepinephrine infusion; therefore, propofol infusion was terminated. A possible alternative relying on thiopental infusion was considered, but this treatment option was not achieved. The ECG changes of ventricular extrasystole and then bradycardia with junctional rhythm occurred 7 to 8 hours after lactate acidosis had developed. Noradrenalin infusion was increased to 1 弮g/kg/min to keep MAP above 70 mmHg and adrenaline infusion was also added. It took about 100 minutes for cardiac arrest (severe bradycardia, right bundle branch block, and then asystole) to occur after propofol infusion was stopped. The patient died after 22 minutes of resuscitation.
Kaiser Burton	27	1990/3/4	226-938-4814	[email protected]	40291 Brown Glen Apt. 875	AB is a 15-year-old female student of south-Asian descent. She presented with concerns of anxiety and symptoms of hypoglycemia as well as difficulty concentrating, fatigue, headaches, asthma, and frequent urinary and vaginal infections. Her anxiety met criteria for GAD and she rated the intensity of anxiety as 8/10, with 10 being the highest level of anxiety possible. The anxiety started three months prior to the initial appointment and had worsened in the previous month. She described excessive worry that was difficult to control and impacted her daily functioning by causing her to be absent from school on several occasions. She experienced a number of somatic symptoms including heart palpitations, shakiness, discomfort in her stomach, and muscle tension. In response to the anxiety symptoms, she would eat foods like chocolate, chips, or fruit. AB was working with a counsellor to manage the anxiety symptoms and was finding some benefit. AB had experienced episodes suggestive of hypoglycemia since 12 years of age. The symptoms included muscle weakness and shaking, headaches, nausea, anxiety, and loss of concentration. Her symptoms were ameliorated by eating sweet foods. AB reported that her hypoglycemia was at its worst at 12 years of age when she had to eat a granola bar hourly in order to concentrate. A diet history revealed the following typical daily food intake:Breakfast: fruit smoothie containing fruit, fruit juice, and water. Morning snack: bagel with margarine. Lunch: pasta or white rice with vegetables. Afternoon snack: granola bar or cookies or gummy candies. After school meal: white pasta; it may include meat. Dinner: white rice or spaghetti; it may include meat. Evening snack: cookies and toast. Beverages: 2 liters of water, 1 cup of juice, 1 cup of lactose-free milk, and 1 cup of tea. Due to her difficulty concentrating, AB was prescribed dextroamphetamine 5 mg. While she found that it improved concentration, this medication caused her to lose weight. As the patient's weight was initially on the lower end of normal (weight: 115 lb., height: 5???聆? and BMI: 18.6) the dose was reduced to 2 to 3 times per week, as needed. AB lives with her mother, father, and sister. AB's sister experienced mental health concerns in the previous years which created elevated stress levels in the family home. Repeated assessments of random and fasting blood glucose and screening physical examination were within normal range. At the initial visit, the following dietary plan was prescribed:Breakfast: it includes a smoothie containing fruit, water, 1 scoop of protein powder, and 1 tablespoon of flax seeds or olive oil. Lunch and dinner: they include a serving of protein (meat, legume, and soy) and a serving of vegetables. Snacks: they include protein when possible (e.g., apple with sunflower seed butter, vegetable sticks with hummus, and pumpkin seeds). Continue to eat carbohydrate-containing snacks as needed for the management of hypoglycemia symptoms. She was asked to follow these dietary guidelines daily until the follow-up visit. While eggs, nuts, and fish would have normally been recommended as well, the patient had an anaphylactic allergy to these foods. At the first follow-up, four weeks later, AB reported that she had complied with the dietary plan since the previous visit. She reported a significant decrease in anxiety (4 to 5/10), as well as improved energy, less-frequent and less intense hypoglycemia symptoms, and fewer headaches (once per week compared to daily) in addition to improved concentration and mood. She required fewer snacks during the day and decreased her intake of granola bars, cookies, and candies (1-2 servings per day). She also reported a cessation of chronic vaginal discharge. The substantial improvement in her anxiety symptoms prompted AB to temporarily discontinue her counselling sessions. At a subsequent follow-up visit four weeks later, AB reported that she had briefly reverted back to her original diet for a period of one week and experienced a worsening of anxiety symptoms within one day. After returning to the dietary intervention prescribed, her anxiety symptoms decreased within two days.
Miriam Harper	38	1992/8/13	(763)707-4611	[email protected]	6456 Thomas Loaf	A 13-year-old girl presented with deteriorating school performance for 2 years with repetitive impulses to touch others inappropriately and believing that there is a devil living inside her body for a duration of 6 months. She has had normal developmental milestones and average school performance for 2 years back. Over the past 2 years, she had inattention, lack of interest in school work, and deteriorating school performance. During the 6 months prior to presentation, she had poor sleep, had become irritable, and had developed grandiose delusions, claiming that she had attained nirvana. She also had bizarre delusions with somatic hallucinations, believing that a devil was residing inside her and feeling this devil's movements. She had episodes of aggression and attributed these to the devil, which was consistent with a delusion of control. There were no mood symptoms. Over this period, she had obsessional impulses to perform certain acts of sexual nature. A diagnosis of schizophrenia with obsessive compulsive symptoms was made and she was commenced on risperidone 2 mg at night and escitalopram 20 mg in the morning. Over time, she showed poor response to medication and started refusing to go to school as she believed her classmates were talking about her. She started acting out on her obsessional impulses and tried to touch her classmates inappropriately. She displayed odd behaviors such as putting match sticks in her dog's mouth and sleeping on the roof. On mental state examination, she was disinhibited evident by her behavior during the interview where she made inappropriate sexual advances towards the interviewer. Her speech lacked prosody. Her mood was flat. She had persecutory delusions and ideas of reference regarding her classmates. She had obsessional impulses to touch others inappropriately, which she resisted minimally. There were no perceptual abnormalities. Her verbal fluency was reduced. Other frontal lobe functions measured by categorical fluency, proverb interpretation, Luria 3-step test, go/no-go test, and written sequencing were normal. Parietal and temporal lobe functions measured by left-right orientation, clock drawing test, and drawing reproduction and memory measured by recall of 5-item address were normal. Nonverbal IQ measured by TONI-3 (Tests of Nonverbal Intelligence-3) was normal. Physical examination revealed bradykinesia, but no rigidity, hyperreflexia, or reduced muscle power. There were no abnormal movements, cerebellar signs, or sensory deficits. Examinations of the cardiovascular, respiratory systems, and the abdomen were unremarkable. Her investigations revealed the following. Full blood count, blood picture, and erythrocyte sedimentation rate done with a view to detect any underlying inflammatory conditions including autoimmune disorders were normal. Tests for thyroid function were done to rule out psychiatric manifestations arising due to thyroid dysfunctions. Both free thyroxine and thyroid stimulating hormone were normal (free thyroxine 1.12 ng/dL and thyroid stimulating hormone 3.87 IU/L). Electroencephalography recorded in the alert state was normal for the age. An eye referral excluded the presence of Kayser-Fleischer rings. Cerebrospinal fluid lactate done to exclude mitochondrial diseases was normal. Computerized tomography brain showed an asymmetrically dilated 4th ventricle, with a cerebrospinal fluid filled cleft connected to right sigmoid sinus, bisecting cerebellar hemispheres which were poorly formed (). Appearance was consistent with a Dandy-Walker syndrome variant. Risperidone was increased to 2 mg twice daily and escitalopram continued at the same dose. Over 6 months of treatment, her psychotic symptoms, disinhibition, and obsessions showed partial response and she continued to have odd behaviors with deterioration of school performance. The option of changing the antipsychotic to olanzapine or quetiapine was discussed with the patient and her family. However, they preferred continuing the current medication due to the higher incident of side effects of weight gain and sedation associated with these medications. In addition, commencing a newer antipsychotic such as aripiprazole, which is not available in the government sector in Sri Lanka, was considered, but family was unable to afford it. Neurology opinion was sought and it was decided that no active surgical interventions were indicated at this stage.
Hayes Phillips	43	1998/5/10	+1-459-241-3430x158	[email protected]	916 Price Groves	A 56-year-old female presented with bilateral otalgia and hypoacusis gradually progressing for the past two weeks and left sided facial palsy significantly increasing within two days. She has been previously twice unsuccessfully treated with antibiotics in another hospital due to chronic otitis media. She also had a history of psoriasis and hyperthyreosis. AAV may occur with antithyroid drug therapy. However, the patient was treated only one month before the blood test; thus the possibility of drug-induced AAV is low. Otoscopic examination revealed bilaterally thickened and reddish eardrums. There was a subtotal perforation of the right tympanic membrane and an anterior perforation of the left tympanic membrane with effusion. Left facial nerve palsy was categorized as grade IV according to House-Brackmann. Nose examination was normal. The pure tone audiometry showed severe bilateral mixed hearing loss on the level of 80??00 dB with air bone. On the right the threshold is on the level of 95??00 dB with air bone gap of 50 dB. On the left the threshold gap is 70 dB. Chest X-ray revealed signs of bronchitis. Urine analysis was normal. High resolution computed tomography scans of the temporal bones showed bilateral sclerosing mastoiditis and opacification of the right tympanic cavity with an air-fluid level. No signs of bony destruction within the ossicular chain, the internal ear, or facial canal were present (). Magnetic resonance imaging (MRI) with the use of T2-weighted, T1-weighted, and contrast enhanced images was performed and showed normal appearance of cerebral structures and mastoid cells filled with fluid on both sides. Hypertrophic pachymeningitis sometimes shows in the GPA patients wit hear involvement. In this case there were no signs of pachymeningitis on MRI examination. The diagnosis of acute otitis media with peripheral facial nerve paralysis was made and intravenous antibiotics treatment was started. As there was no response to drug therapy, the patient was referred to myringoplasty and antromastoidectomy with facial nerve decompression. During surgery, granulation in the mastoid cavity was found. There was no granulation on the facial nerve canal. The facial nerve was pale and swollen during decompression. After surgery otalgia and hearing on the left side improved slightly, but facial paralysis did not show any improvement. The patient was discharged and sent home with prescribed antibiotic treatment. After two months, the patient's condition deteriorated significantly as she presented with bilateral facial paresis (House-Brackman grades IV-V), horizontal nystagmus directed to the right ear, severe hearing loss (70??0 dB), otalgia, and otorrhea. Blood tests showed WBC of 12,93 thousand/弮L (normal range: 4??0) and CRP of 147,76 mg/L (normal range: 5,0). Laboratory tests showed hyperthyreosis with positive results for antibody to thyroglobulin: A-TG of 180 U/L (normal range: 0,0??0). Renal function tests and abdominal ultrasound were normal. Intravenous antibiotic treatment was administered. Chest X-ray showed bilateral hilar mass lesions. Chest CT scans revealed mediastinal nodules and parenchymal consolidations in both lungs (). Head MRI showed no pathological changes except for complete opacification of mastoid cells and middle ears bilaterally. Ophthalmic evaluation revealed bilateral scleritis. Due to the suspicion of lungs metastatic disease, the patient was referred to thoracotomy. The histopathology tests of pulmonary lesion suggested tuberculosis, which resulted in introduction of tuberculostatic treatment. With no improvement after tuberculostatic treatment and suspicion of GPA, serological ELISA tests were performed and c-ANCA tests were positive. Subsequently the patient was transferred to Rheumatology Department, where the diagnosis of GPA was confirmed. Control laboratory tests showed WBC of 14,9 thousand/弮L (normal range: 4??0), markedly increased value of c-ANKA > 150 U/L (>8,0 U/mL positive result), and abnormal urine analysis with the presence of protein and red blood cells. Follow-up head CT showed chronic inflammatory changes with polyps in paranasal sinuses. The patient was commenced on systemic steroid therapy in the form of Solu-Medrol 2,0 g per day and cyclophosphamide (Endoxan) 600 mg per day with the protection of Uromitexan. The total dose of Endoxan was 5,4 g. In the course of treatment, the patient's condition improved concerning hearing and nystagmus. Facial nerve palsy regressed from 5th to 2nd grade bilaterally in House-Brackman scale. Unfortunately, the patient's renal function deteriorated gradually and she died 2 years after the initial otological symptoms due to progressive renal disease.
Naomi Hansen	43	2003/5/1	241-600-3717	[email protected]	04007 Miller Squares Apt. 235	A 25-year-old male patient presented with a history of fall of a large stone on chest at a construction site followed by loss of consciousness and fall on back. On initial presentation, blood pressure (BP) was 60/36 mmHg and pulse was 184 beats/min. He was immediately intubated and resuscitated with fluids and inotropes. The patient responded by stabilising BP to 110/60 mmHg. Bilateral intercostal drains were put. Contrast enhanced CT (CECT) chest showed mild pericardial fluid, fractured third rib, and bilateral hemopneumothorax. The patient also had grade 3 liver injury. The patient was shifted to the intensive care unit (ICU) for further management. On arrival at the ICU, there was a sudden drop in BP with new onset arrhythmias. Electrical alternans was noted on electrocardiogram as well. 2D echocardiogram (Fig. ) demonstrated mild to moderate pericardial fluid, regional wall motion abnormality, and low ejection fraction. Therefore, urgent pericardiocentesis was done, and BP improved marginally afterwards. The patient developed repeated episodes of atrial fibrillation (Fig. ) and ventricular tachycardia with hemodynamic instability requiring frequent defibrillations. The patient became hemodynamically stable with control of arrhythmias within 2 days. CPK MB, troponin T, and troponin I were found to be positive. Repeat echocardiography showed a decreased ejection fraction of 25 % to 30 %. On the third day after ICU stay, the patient developed cellulitis on the back and half of thigh with high grade fever episodes and new onset hemodynamic instability. Immediately, cultures (blood, urine, and tracheal) were sent and antibiotics were escalated. However, no organism could be isolated probably because antibiotics had been initiated empirically. Meanwhile, crystalloids were administered cautiously under echo guidance. However, the patient continued to deteriorate and succumbed to septic shock on the fifth day of injury. Most patients with cardiac injury have fatal outcome before reaching the hospital. Penetrating cardiac injury, requirement of inotropes, and the presence of new onset arrhythmias are associated with worse survival. However, sepsis as a worse prognostic factor has not been reported previously. Here, we present a case where sepsis complicated the blunt cardiac injury and leads to fatal outcome. Wijngaarden and associates et al. in their 10-year institutional review noted a 14 % mortality in patients with blunt cardiac trauma which increased to 58 % in patients requiring inotropic support []. The presence of arrhythmias decreases the survival to only 12 % to 13 % []. Blunt cardiac injury manifests with wide array of signs and symptoms varying from benign ectopic beats to fatal cardiac arrhythmias, shock leading to sudden collapse and death. It may also result in the rupture of the myocardium, pericardium, or valve assembly presenting with gross hemodynamic instability. The right ventricle is the most commonly to be injured portion of heart. However, subtle signs such as flail chest, ecchymosis, and sternal fractures may be the only presentation. Combined use of ECG and troponin I has been found to be one hundred percent sensitive for detection of clinically significant blunt chest trauma [] (defined as cardiogenic shock, dysrhythmias requiring treatment, or structural cardiac abnormalities) []. A normal screening ECG rules out any significant cardiac injury and predicts a benign course []. However, troponin has also been found to be elevated in patients with septic shock making it unreliable for diagnosing in septic patients []. Echocardiography is an excellent tool for diagnosing and monitoring patient with blunt cardiac injury []. However, its interpretation becomes confounded by the presence of sepsis (Table ). Sepsis added to cardiac injury may lead to worse prognosis. Myocardial depression, in the form of biventricular dilation and decreased ejection fraction, has been demonstrated in most patients with septic shock []. This further complicates the management of patients with cardiac injury by acting as a ?econd hit??to the injured heart. This depression is however reversible over 7 to 10 days in survivors. End-diastolic dysfunction and end-systolic dysfunction are also found contributory to poor prognosis [].
Charlie Holland	34	1991/10/8	001-203-606-4372x825	[email protected]	5987 Kevin Street	Case 1 (female, 20 days old, Hui nationality) was admitted to our hospital on the 21st of March 2012 due to a skin ulcer on the occiput for the previous 10 days. After birth, the parents placed a brick wrapped in a towel under the neonate? occiput because they thought her head shape was too rounded and unattractive. Ten days prior, the parents found a coin-sized skin ulcer on the occipital site and then went to the local hospital. No particular treatment was given because the neonate was too young. The skin lesion gradually formed a black, dry scab, which fell off two days prior to their admittance of our hospital, exposing the occipital bone. The neonate came from a secondary pregnancy and was a full-term, normal delivery. The examination on admission included the following: Temperature, 36 簞C; Respiration rate, 21 times/min; Pulse, 102 beats/min; Weight, 5 kg. The baby showed normal development, modest nutritional status, normal head size, and no abnormalities upon heart and lung examination. A 2.0 cm ? 2.0 cm skin lesion was located in the middle of the occipital site and deep to the occipital bone. Although the occipital periosteum disappeared, the color of the occipital bone was normal and a small quantity of purulent secretion appeared on the wound. The color of the skin surrounding the wound was normal with no swelling (Fig. ). Under the condition of necessary preoperative preparation, local rotational flap repairing was undertaken after debridement under general anesthesia on the 28th of March 2012. Routine anti-infection treatment was given, and a dense sponge-made head frame was used to restrain the head in a suspension position to avoid pressure on the occiput (Fig. ). Seven days after the surgery, the stitches were removed and the patient was discharged with primary wound healing (Fig. ).
Mariah Love	39	1997/5/6	001-740-888-8050x4818	[email protected]	0144 Gill Pass Suite 697	Case 2 (male, nine days old, Dongxiang minority nationality from Linxia Dongxiang autonomous county,Gansu Province) was admitted to our hospital on the 25th of August 2013 due to the presence of a skin sore on the occipital site and exudation for five days, as well as having a fever for one day. The parents placed a tile wrapped in a towel under the neonate? occipital site with the intention of changing his rounded occipital head shape to a flat occiput. Five days prior, the parents observed that the occipital site was pale, which was followed by the presence of a sore and exudation. Improvement was not achieved after treatment in a local clinic, and the lesion size gradually increased to exposure of the occipital bone. The neonate came from a first pregnancy with full-term normal delivery. The examination on admission included the following: Temperature, 38.5 簞C; Respiration rate, 28 times/min; Pulse, 156 beats/min; Weight, 3 kg. The neonate showed normal development, modest nutritional status, normal skin color, normal head size, and no abnormalities upon heart and lung examination. There was a 2.8 cm ? 1.8 cm size skin defect in the middle of the occipital site, where the occipital bone was exposed and the occipital periosteum was absent. The color of the occipital bone was normal with an irregular lesion edge. There was the presence of purulent secretion, tenderness was apparent, and the skin color around the lesion site was normal with no swelling (Fig. ). After systemic support, control of wound infection and preoperative preparation, occipital debridement and bilateral sliding skin flap transposition were performed to cover the exposed skull on the 30th of August 2013 (Fig. ). Routine hemostasis, infection control and further systemic support were given, and a dense sponge-made head frame was used to avoid occipital pressure. Lesion dehiscence was observed after stitches were removed nine days later (Fig. ); therefore, local suturing was undertaken on the 11th of September 2013. The stitches were removed nine days later, and the wound was healed. The patient was discharged on the 22nd of September 2013 (Fig. ).
Jeffrey Webster	45	1984/9/16	8388705445	[email protected]	372 Peterson Street	An otherwise healthy 37-year-old white man had chemorefractory classic Hodgkin lymphoma, treated with the following regimens: (1) doxorubicin, bleomycin, vinblastine, and dacarbazine; (2) ifosfamide, carboplatin, and etoposide; (3) brentuximab; (4) gemcitabine and vinorelbine; (5) bendamustine; (6) everolimus; (7) sirolimus and vorinostat; (8) lenalidomide; and (9) dexamethasone, cytarabine, and cisplatin. He then underwent an ASCT from a 10/10 matched unrelated donor, with a fludarabine and melphalan conditioning regimen. Three months after the ASCT, the disease relapsed in his abdominal and pelvic lymph nodes. He was treated with intensity-modulated RT to his para-aortic and pelvic lymph nodes, starting on day 119 after the ASCT. At that time, no evidence of GVHD was present, and the tacrolimus was tapered off. The plan was to treat his abdominal and pelvic lymph nodes with a total dose of 39.6 Gy in 22 fractions. However, after completing 28.8 Gy in 16 fractions, he experienced severe abdominal pain, nausea, vomiting, and copious watery diarrhea. An endoscopy revealed an erythematous gastrointestinal mucosa with superficial ulcers. Biopsies taken throughout his gastrointestinal tract were consistent with GVHD. In his stomach and duodenum, the mucosa showed loss of glands, dilated glands with eosinophilic and granular debris, and increased apoptotic cells (Fig. ). In his colon, increased apoptotic cells and loss of glands were identified. These findings led to a diagnosis of grade 4 GVHD of the gastrointestinal tract, experienced after 28.8 Gy. His bowel, contoured as ?owel space,??received a maximum dose of 31.8 Gy and mean dose of 14.8 Gy. The volume of bowel space that received ??0 Gy was 39 cc, ??0 Gy was 1400 cc, and ??0 Gy was 2450 cc. His stomach received a maximum dose of 25 Gy and mean dose of 2.9 Gy. The volume of stomach that received ??0 Gy was 4.3 cc and ??0 Gy was 31 cc (Fig. ). Initial treatment with tacrolimus and steroids (2 mg/kg/day) had no effect, but subsequent pentostatin and photopheresis produced a good response. At last follow-up, 6 months after RT had been stopped, his GVHD was quiescent on photopheresis and tacrolimus, with no evidence of active Hodgkin lymphoma in his abdomen or pelvis.
Kensley Wilkins	24	1983/4/4	606.480.0614x96711	[email protected]	9447 Sharon Plains	As previously reported [], an otherwise healthy 24-year-old white man with relapsed Philadelphia-positive B-cell acute lymphocytic leukemia was treated initially with cyclophosphamide, vincristine, doxorubicin, dexamethasone, cytarabine, and methotrexate (hyper-CVAD) with dasatinib. Disease relapse in his central nervous system (CNS) and bone marrow during maintenance therapy was salvaged with augmented hyper-CVAD and dasatinib, followed by a 10/10 human leukocyte antigen (HLA)-matched related ASCT from his sister, with a busulfan and clofarabine conditioning regimen. On day 82 after the ASCT, he presented with a headache; he was diagnosed as having an isolated CNS relapse and was treated with rituximab, asparaginase, dasatinib, high-dose methotrexate, and intrathecal cytarabine, followed by consolidative CSI. The CSI was with proton therapy, to a total dose of 24 cobalt Gy equivalent (CGE) in 12 fractions. His brain was treated with right and left posterior oblique beams, and his spine was treated with three posterior-anterior beams. The CSI was begun on day 197 after the ASCT, and no evidence of GVHD was present at that time. The tacrolimus dose was reduced during RT. One month after completing CSI, he developed severe dermatitis within the RT portals and conjunctivitis, keratopathy, and conjunctival ulceration. The dose delivered to his skin had been 22 CGE []. A skin biopsy showed inflammatory cell-poor interface dermatitis with vacuolar alterations of the basal keratinocytes and dyskeratotic cells, consistent with grade 2 to 3 GVHD. Treatment with tacrolimus and methylprednisolone (2 mg/kg/day) resulted in resolution of his cutaneous GVHD; however, keratoconjunctivitis sicca persisted despite prednisolone ophthalmic drops. His cutaneous GVHD returned several months later, both within and outside the RT field. This extensive chronic GVHD progressed despite steroids, tacrolimus, and photopheresis, manifesting as ulcerations, scleroderma-like changes, and chronic osteomyelitis that necessitated bilateral above-the-knee amputations. He died of aspiration pneumonia and respiratory failure 4.5 years after the ASCT, with no evidence of leukemia.
Yusuf Ramirez	22	1980/7/16	+1-758-980-1601x3458	[email protected]	387 Kenneth Flats Apt. 148	A 63-year-old male presented with a history of hepatitis B virus (HBV) infection for 20 years and no anti-HBV treatment. In mid-Jan. 2015, the patient developed progressive and dull chest pain without obvious inducement. Chest CT scan revealed multiple pulmonary nodules and enlarged mediastinal lymph nodes. The level of serum alpha-fetal protein (AFP) reached 24793 ng/mL. Due to no hepatic lesion found by contrast abdominal CT and MRI scan, F-18 positron emission tomography-computed tomography (PET/CT) was carried out. Unexpectedly, PET/CT definitely showed that multiple lesions in the thoracic and abdominal cavities, including several lung nodules in the maximum size of 1.7 cm ? 1.7 cm (Fig. a1), mediastinal lymph nodes in the maximum size of 6.4 cm ? 3.2 cm (Fig. a2), and intraperitoneal mass in the fundus of stomach, cardia, portal fissure and abdominal aortas in the size of 4.6 cm ? 2.2 cm (Fig. a3), but still no hepatic lesion (Fig. ). On Mar. 6th, 2015, the patient underwent a wedge resection of right pulmonary. The pathological morphology displayed cancer cell nest with pseudoglandular structure and focal necrosis area, typical hepatocellular carcinoma cells with polygonal shape, eosinophilic cytoplasm and big anachromasis nucleus. And immunohistochemistry showed the expressions of CK18 ++, Glypican-3 ++, Hepatocyte +, P53 + and Ki??7 60 - 70 %, which confirmed the diagnosis of primary HCC (Fig. a-f). Thus, the patient was a rare case of EHCC in an advanced stage. Following the standard therapy of HCC in National Comprehensive Cancer Network (NCCN) guidelines [], the patient began to take sorafenib 400 mg twice daily together with Chinese medicines (Jinlong capsule, Jiansheng Company, China) and immunopotentiative agents (ubenimex capsules, Yuandong Company, China) on Mar. 19th, 2015. On the tenth day (Mar. 28th, 2015), red maculopapules appeared in the face, neck, abdomen, back and legs (Fig. a and b). From the same day, the patient began to suffer from continuous high fever, with highest body temperature ranging from 39.0 簞C to 39.6 簞C each day. The patient did not decrease the dose or stop the use of sorafenib despite feeling dizzy and fatigue. There was no abnormality in blood routine or blood culture examination, no pulmonary infection or other inflammatory signs. After taking non-steroidal drugs with antipyretic and anti-allergic properties and withdrawing all drugs except sorafenib, no significant improvement was observed in high fever and maculopapules. Therefore, the patient was asked to cease the use of sorafenib from Apr. 3rd, 2015. Strikingly, the high fever and maculopapules were alleviated quickly. On the third day, the body temperature returned to the normal level, and on the fourth day, the maculopapules almost completely disappeared. Next, the patient began to take sorafenib again at the standard dose of 400 mg twice daily on Apr. 7th, 2015. As expected, the fever developed, raising the body temperature to 38.1 簞C on exactly the same day, to 38.4 簞C the next day and 38.8 簞C the third day. Also, the red maculopapules relapsed at the same time. Hence, prednisone was administered at a dosage of 30 mg/day on the fourth day. The body temperature was decreased and maculopapules relieved quickly. The dosage of prednisone was gradually reduced from 30 to 10 mg/day in one month, and then kept at 5 mg/day for another two weeks. After the patient? temperature has returned to normal for more than one month, he intermittently took ubenimex and Jinlong capsule again. There has been no recurrence of fever and maculopapule to date. The dynamic change in body temperature is reflected in Fig. . Other common side effects also need to be mentioned, such as hand-foot syndrome and rash in the scalp and ears and body (Fig. c and d). During the follow-up, the tumor response of both intrathoracic and intraperitoneal lesions reached partial response (PR) according to RECIST 1.1 criteria. The lung nodules, mediastinal lymph nodes and intraperitoneal mass shrank gradually from Mar. 19, 2015 to Apr. 12th, 2016 (Fig. b1-b3, c1-c3 and d1-d3), with the maximum size of 0.8 cm ? 0.6 cm, 3.7 cm ? 2.2 cm and 2.0 cm ? 1.9 cm at the date of Apr. 12th, 2016, respectively. There was still no lesion in liver, revealed by repeated examinations of abdominal ultrasound, CT and MRI (Fig. e and f). In addition, serum AFP gradually dropped from 24793 ng/mL to 2.19 ng/mL on Apr. 12th, 2016 following the disease control (Fig. ). Until Apr. 14th, 2016 in the last review, the patient had maintained a good condition with progression free survival (PFS) of 392 days + .
Grace Trevino	26	1995/1/5	703.701.8289	[email protected]	126 Herrera Drives Suite 310	A 12-year-old healthy girl had suffered a deep third degree scald burn at the age of 2 years. The burned area covered all of her right chest wall area. It was treated conservatively with no skin grafting. At the beginning of puberty, the right breast tissue was unable to grow because of the contracted lower pole (Fig. ). In the preoperative planning, an Integra 10 cm ? 15 cm was selected. The patient was operated under general anesthesia. First, the contracted area was excised and a gap of 7 cm? 5 cm resulted. The unmeshed Integra was putted above the exposed area and attached to the skin borders with absorbable sutures (Vicryl 4/0) (Fig. ). A tie-over dressing with Xeroform and sponge was putted above the Integra and was stapled to the skin. Operative and postoperative management were uneventful. After 4 weeks, the patient went through the second operation. Under general anesthesia, the silicone layer of the Integra was removed and a thin split skin graft (0.01 in.) taken from the anterior aspect of her thigh was put on the integra and attached to it with absorbable sutures (Vicryl 4/0) and staples. A tie-over dressing with Xeroform and sponge was putted above it. The operative and postoperative management were uneventful. After 4 days, the skin was taken and the dressing was exchanged (Fig. ). Follow-up of the patient included measurements of the suprasternal notch to nipple distance (SN-N), nipple to inframammary fold distance (N-IMF) and graft size (Table ). The patient was seen until the age of 19 (7 years post operation) with satisfying results and symmetric development of her breasts (Fig. ). Reconstruction of the post-burn breast includes excision of the contracted scar, releasing of fibrotic tissue and covering it with one of the following modalities: split thickness skin graft, expanded full thickness skin graft, musculocutaneous or fasciocutaneous flaps, or an artificial dermal matrix. Split thickness skin grafting is very common due to its ease of harvest and availability. It can be meshed up to 1:9, thus enabling a large area of coverage. Yet, it assumed to cause contraction of the reconstructed area because of secondary contraction forces of the graft. This drawback should be considered when one chooses this modality for areas of esthetic and functional importance as in our case. The full thickness skin graft is preferable because of better esthetic results and less secondary contracture of the skin. Its main drawback results from the necessity to first expand the donor site in order to get enough skin for reconstruction. This means another operation with a period of a few months of inconvenience until the expansion phase is completed. Moreover, consideration should be taken of the risks and possible complications regarding the expanders (extrusion, infection, damage to the skin, etc.). In our case, the patient was 12 years old when she first came to us. The use of an expander in her abdomen was assumed to be hard to tolerate. Musculocutaneous or fasciocutaneous flaps are other alternatives for reconstruction, with good ability of coverage and which can be matched to the defective area, thus leading to very good functional and esthetic results. The main drawbacks of these alternatives are donor site morbidity and the risks of complications and failure. The artificial dermis has become more popular in recent years for reconstruction because of its simplicity and the predictability of the reconstructive results. The biodegradable dermal replacement layer serves as a matrix for the macrophages, fibroblasts, and endothelial cells from the host tissue. It causes a new dermis to grow, thereby creating a functional tissue instead of the excised one. The only donor site that is needed is a very thin split skin graft for final closure after the dermal graft has taken. The main drawback of this reconstructive modality is its price which is very expensive. In the patient herein presented, we decided to use an artificial dermis matrix to reconstruct the defect. This method has been described in the literature for variety of reconstructions including burned breast [, ]. Palao et al. used artificial dermis in reconstruction of burned breast of 12 patients. They showed in their study that after 1 year, the host collagen completely replaces the artificial one, and elastic fibers were observed in the dermal regeneration template. They had high satisfaction rate after 1 year of follow-up (1). Two of their patients were at puberty age during the reconstruction (13 and 14 years old), though, they had no follow-up for more than 1 year, so there is no way to get knowledge from their study of the long-term development of the reconstructed breast. Our case is the first to be published that use this method for breast at the beginning of the puberty and has follow-up of 7 years until the end of the puberty period at 19 years old. By that, we showed that the burned breast developed as well as the other breast, with satisfying symmetric size and shape. The importance of this case is that it emphasizes the long-term advantage of Integra as a reconstructive tool for having a natural functional tissue.
Jaime Kelley	42	2001/12/31	+1-574-493-8210x7246	[email protected]	35652 Jeffrey Squares Suite 002	A 42-year-old previously healthy Russian man living in Estonia was hospitalised due to a one-year history of progressive cognitive decline, confusion attacks, rare hallucinations, gait disturbances and involuntary movements. At the beginning of the symptoms he was often sent on short sick leaves because of his employer? doubt about his health. He resigned 6 months after the first symptoms appeared, being unable to perform his duties at work. In addition, his spouse could not allow him to leave home due to his progressive disorientation. The patient became unable to cope with daily activities like dressing, brushing teeth and washing. He soon became dependent in most daily life activities. Therewith, he came to the neurologist for the first time. On neurological examination he had predominantly left-sided bradykinesia and rigidity and intentional tremor in his left hand and both legs. There was no weakness on motor examination. Deep tendon reflexes were brisk and more pronounced in the left but there were no extensor reflexes. His gate was cautious and wide-based. There were myoclonic jerks in his legs and left arm that were more pronounced in action (stimulus-sensitive) (Additional file ). According to the neuropsychological testing he had severe dementia with the mini mental state examination (MMSE) Score 12/30. The results were affected by severe attention deficit. He had affective symptoms like irritability, aggressive behaviour and delusions. His speech was dysarthric and dysphonic with mixed aphasia that included difficulties in word finding, impaired articulatory agility, verbal stereotypes, some paraphasias in running speech and difficulties in understanding longer sentences. He had anisocoria (the left pupil was larger), with pupils nonreactive to light, and horizontal nystagmus. Head magnetic resonance imaging (MRI) scan revealed brain atrophy (Fig. ). There was focal slowing and epileptiform discharges in the right fronto-temporal regions on the electroencephalography (EEG) (Fig. ). Blood tests were normal including hepatic function and thyroid tests, although the level of vitamin B12 was slightly decreased (124 pmol/l, reference range 141??89 pmol/l). Human immunodeficiency virus (HIV) 1 and 2 antibodies were negative but the rapid plasma reagin (RPR) test as well as the T. pallidum hemagglutination assay (TPHA) were highly positive (RPR 1:32 and TPHA 1:1520) in serum as well as in cerebrospinal fluid (CSF) (RPR 1:8 and TPHA 1:640). CSF showed predominantly lymphocytic pleocytosis (12 cells/mm3), the protein was elevated to 0.63 g/l, as well as IgG index (4.31). Based on clinical pictures and laboratory data, neurosyphilis was diagnosed and intravenous penicillin-G treatment 24 million units per day for 14 days was initiated. Thereafter intramuscular benzathine penicillin of 2.4 million units once per week was injected for 3 weeks. Complementary treatment with divalproex sodium was started as there were epileptiform discharges on the EEG that demonstrated an increased risk for developing of epilepsy, and the patient had emotional problems and agitation. On the follow-up 6 months later, he had a mild dementia (MMSE 25/30), but there were neither myoclonus nor parkinsonism. Deep tendon reflexes were still brisk but symmetrical. He still had Adie? tonic pupil in the left and very little constriction to direct light bilaterally. No clinical features of cerebellar dysfunction were detected. Both the serological markers [RPR (1:2) and TPHA] and the above mentioned CSF measures changed to negative. The patient continued treatment with divalproex sodium 300 mg bid, and enalapril with amlodipine for hypertension. On the subsequent follow-up visits (twice a year), no consistent changes have been found.
Rosalie Cortez	37	1990/12/1	2227012582	[email protected]	4361 Rebekah Pass Apt. 589	A 14-year-old female attended the Orthopedic Oncology Clinic at The First Affiliated Hospital of Nanchang University (Nanchang, China) for a follow-up. She underwent two operations successively due to pathological fractures in the left femur and tibia in a local hospital about 1 year ago. Then, she was diagnosed with FD according to the postoperative pathological examinations. Recently, she had no obvious symptoms and discomfort. Physical examinations showed nothing remarkable, but a little swelling in her left distal thigh. She had a negative family history of bone tumor. There was no fever or respiratory embarrassment accompanying the swelling. No history of weight loss or exposure to tuberculosis was mentioned. In a further examination, physical examination showed no palpable head, neck, supraclavicular, axillary, or epitrochlear lymph nodes. Inflammatory markers were within the normal limits. Plain radiographs indicated an expansile osteolytic lesion with marginal sclerosis and a matrix with a ground glass appearance and without periosteal reaction of the left distal femur, which was consistent with FD (). Then, a diagnosis of recurrence of FD in the left femur was made given the medical history of FD, symptoms, and imaging findings. Based on the exclusion of surgical contraindications, professional surgeons, who specialized in treating bone tumors, performed the surgery of curettage and grafting in the left femur. Histologic analysis of hematoxylin and eosin-stained specimens showed irregularly shaped spicules of immature bone without osteoblastic rimming and fibrous stroma with few mitotic activities (). Immunohistochemical analysis showed positive for CD68 and Vim but negative for bcl-2, CK, CD34, CD99, EMA, SMA, and S100. According to the above findings, the diagnosis of the recurrence of FD of the left femur was finally confirmed. Nevertheless, she presented to our clinic again with a chief complaint of pain and swelling in her left tibia and calcaneus 4 months later. Radiographic review showed a prominent osteolytic lesion located in the distal portion of the tibia and calcaneus with cortical destruction (). The patient underwent surgery with the removal of the tumor tissue and reconstruction with allogenous bone graft. According to the histological and immunohistochemical findings, the diagnosis of PMH secondary to FD was confirmed by an expert pathology consultant. Finally, the patient had to undergo an amputation of the left thigh. The tumor consisted of densely distributed pleomorphic cells, ranging in configuration from small spindled elements to large plump epithelioid variants with prominent pink cytoplasm. Scattered cells with vacuolated cytoplasm were also present. Prominent pleomorphic nuclei contained optically empty centers with peripheral marginalization of the chromatin, in addition to conspicuous nucleoli. Multinucleated cells were also present. Vasoformative elements, such as multi-cellular vascular channels or intracytoplasmic vacuoles, were identified (). Immunohistochemical analysis showed positive for CK, CD31 (), but negative for s-100 and CD34. According to the above findings, the diagnosis of PMH secondary to FD of the left lower limb was finally confirmed. The patient was discharged without any complications 1 week after the amputation. At the time of the 3-month follow-up, the patient reported no pain or discomfort in her left lower extremity. No evidence of recurrence or distal metastasis was noted during the 3 months after surgery. However, it is necessary to perform continuous observations of the patient because of a high rate of recurrence and metastasis. Pseudomyogenic hemangioendothelioma is a rare soft tissue tumor?istinct from epithelioid hemangioendothelioma []?riginally described as a ?ibroma-like variant of epithelioid sarcoma??[]; it has been recently concluded that the ?pithelioid sarcoma-like hemangioendothelioma??is essentially the same pathological entity []. PMH affects predominantly young males. The tumor is multifocal, involving different tissue planes and, although, it shares some histopathologic features with epithelioid sarcoma, it has a different, spindle cell morphology with common positivity for CD31, lack of CD34 reactivity, and intact INI-1 immunoreactivity [, ]. By contrast, fibrous dysplasia (FD) is a common tumor-like lesion characterized by solitary or multifocal polyostotic intramedullary lesions []. It has a frequency of 2.5 % for all bone lesions and 7 % for benign bone tumors []. While any bone can be affected by FD, the most common sites of the disease are the femur, the tibia, the ribs, the skull, the facial bones, the humerus, and the pelvis. Although many bones can be affected at once, FD is not a disease that spreads from one bone to another. Multiple affected bones are often found unilaterally []. However, malignant transformation of FD is rare and occurs in less than 1 % of the cases [, ]. The most commonly observed malignant transformations are osteosarcoma, fibrosarcoma, and chondrosarcoma []. As far as we can see, the current case is the first case reported ever of PMH secondary to FD, which is extremely rare and worthy of special remark. FD can be divided into two major types: monostotic and polyostotic []. According to the largest series, malignant changes seem more likely to occur in polyostotic than in monostotic FD [, ]. It is consistent that the current research described a malignant change in a polyostotic FD. Although it is important to recognize malignant transformation as early as possible, it may not be easy as in our case. Suspecting from malignant transformation can be extremely difficult especially in cases with monostotic disease having either subtle symptoms or none. In the reported cases up to date, the special symptoms of the malignant change were mainly pain, swelling, and late appearance of a bony mass [, ]. But given the fact that the pain is a nonspecial finding and is the most common complaint followed by pathological fracture in FD [], great care should be taken when evaluating this symptom. Pain which is rapidly becoming worse over a relatively short period without trauma should be considered alarming []. PMH is extremely difficult to diagnose because of no morphological evidence suggestive of endothelial differentiation is present to confirm a radiological pattern of vascular neoplasm (multiple well-limited purely lytic lesions) []. The tumor is composed of large spindle cells, arranged in sheets or fascicles. Tumor cells with epithelioid cytomorphology are also often present. In this case, the tumor consists of spindle cells, and round epithelioid cells exist. The cells are large and have abundant eosinophilic cytoplasm, mimicking a myoid tumor or epithelioid carcinoma cells. The tumor cells are plump but show no apparent pleomorphism. The nuclei of the cells have small nucleoli without notable atypia, and the mitotic activity is scarce. Based on previous reports in other locations, PMH has a more indolent clinical course with a small risk of metastasis []. Therefore, complete macroscopic excision is the treatment of choice. Local recurrence must be considered, even with complete, gross surgical resection; close follow-up and adjuvant therapy are warranted.
Zayn Erickson	37	1981/11/28	001-229-543-5158x9292	[email protected]	45454 Aguilar Burgs Suite 279	A 7-year-old boy was brought to the emergency room with history of crush injury by bullock cart 1 h before presentation. The boy was complaining of severe pain in the upper abdomen and had two episodes of non-bilious vomiting. On examination, pulse rate was 130/min and BP was 110/70 mmHg. The abdomen was tender over the right hypochondrium and lumbar region. The right lower limb was flexed at the hip joint, and passive extension was painful. There was no pallor. An abdominal radiograph was nonspecific and without evidence of free air. Abdominal ultrasound demonstrated minimal fluid in the pelvis and unremarkable solid organs. In view of the hemodynamic stability and unavailability of expert radiologist, the child was initially managed expectantly. The investigations revealed?emoglobin 120 g/dl, hematocrit 32 %, total leukocyte count 26 ? 103/弮l, and serum amylase 30 U/L. He had few episodes of bilious vomiting in the next 12 h. The patient complained of increased abdominal pain and had local abdominal tenderness despite adequate bowel rest. Although the child was hemodynamically stable, he had fever spikes with temperature reaching up to 38.9 簞C. A contrast-enhanced computerized tomography (CECT) of the abdomen was done, which revealed free fluid of high density in the peritoneal cavity around the hepato-renal pouch and localized free air in retroperitoneum around second part of duodenum, which was communicating with the lumen (Fig. ). An exploratory laparotomy was subsequently performed that revealed copious frank pus in the peritoneal cavity. The hepatic flexure was inflamed and revealed numerous flimsy inter-bowel adhesions. After reflecting the cecum and ascending colon medially, the duodenum was identified and was noted to be covered with bile-stained slough. After kocherization, a 2 ? 2 cm perforation was seen on the lateral wall of the second part of duodenum (Fig. a). Since the defect was less than 50 % of the circumference, duodenorrhaphy was performed by closing the perforation in a transverse fashion. A pedicled omental patch was added (Fig. b). The patient was kept nil per oral for 10 days with supplemental parenteral nutrition and had an uneventful recovery. IDP in pediatric trauma is scarcely reported. Only isolated cases of IDP following blunt abdominal trauma exists in the English literature [?. DuBose et al. in 2008 reported a series of five adult cases of IDP, which was the largest series thus far in the literature []. Concomitant injuries are more common, and overall outcome depends on the nature and the severity of these injuries. Road traffic accidents are the most common mode of blunt injury to the duodenum []. In addition to this, other peculiar mechanisms commonly encountered in children are falls, bicycle handlebar injuries, child abuse, and playground accidents [?. IDP following blunt trauma abdomen (BTA) may occur as a result of ?rush injury??or ?istraction injury?? The duodenum may get crushed between spine and other hard objects like handlebar or a steering wheel []. In our case, the duodenum got crushed between the spine and heavy bullock cart. ?istraction??injuries with perforation occur at the junction of the first and second part of the duodenum []. This usually follows sudden deceleration as after high-speed motor vehicle accidents. Presentation in IDP may be nonspecific initially. Specific signs of perforation may appear late. Symptoms may be mild at the outset and range from mild to severe upper abdominal pain with recurrent vomiting. There are reports of patients with milder symptoms having been discharged from the emergency department only to be readmitted a few hours later as the symptoms worsened [, ]. An in-depth review of the literature on IDP showed vomiting and abdominal pain, localized to the right upper abdomen, being the most prevalent symptom []. Leukocyte count has been found to be elevated in almost all cases [, , ]. Serum amylase levels may be raised but is less specific [, ]. The triad of vomiting, upper abdominal pain, and leukocytosis, though individually less specific, when present together in BTA, may suggest duodenal injury []. Our patient had all these symptoms, which gradually progressed. In addition, he also had painful flexion attitude of the right hip, which may had been related to spasm of right psoas muscle secondary to irritation from the surrounding duodenal fluid. This finding of ?soas spasm??in blunt abdominal trauma was noted in our case and has not been described in literature earlier. Therefore, if consistently present, along with the aforementioned triad, it may clinically suggest duodenal injury. Abdominal radiograph and sonography may not be useful in diagnosis of IDP due to its retroperitoneal location []. The role of early CECT abdomen with oral contrast at this point is crucial. When multi-detector CT is used, sensitivity of 88??3 % can be achieved for detecting bowel injuries in patients with blunt trauma []. However, such sensitivity and specificity data of CT in diagnosis of isolated duodenal injuries do not exist in current literature due to its uncommon occurrence []. Duodenal perforation is suggested if there is a retroperitoneal collection of contrast medium, extra-luminal gas, or a lack of continuity of the duodenal wall []. Since majority of blunt trauma cases in children with stable hemodynamics are managed expectantly, clinical correlation with mentioned CECT features can help surgeons in early decision-making. We retrospectively correlated the clinical symptoms and learned that an early CECT would have helped us in early exploration of this case. In most of the cases where the perforation is less than 50 % of the circumference, simple duodenorrhaphy is adequate []. In addition to primary repair, feeding jejunostomy or a gastrojejunostomy may be added to safeguard the repair []. For perforation sizes that preclude a primary repair, techniques like jejunal serosal patches and pedicled mucosal flap with jejunal or gastric island flap have been described in experimental setting with minimal impact in actual clinical setting []. Another technique with Roux-en-Y duodenojejunostomy has been described with encouraging results in clinical setting as well []. Irrespective of the technique used, the outcome however also depends on the timing of intervention. It has been noted that in duodenal perforation with concomitant injuries, a delay of more than 24 h has a poor outcome []. Thus, early recognition of this rare injury is necessitated for a better outcome.
Sabrina Massey	42	2005/4/3	+1-427-600-2548x9574	[email protected]	214 Amanda Plain	A 56-year-old white male, former pipefitter, 68 kg /169 cm, with no particular personal or familiar medical-surgical history, presented with a lesion of oral cavity of recent apparition. The cancer risk factors included unweaned smoking, valued at about 40 pack-years, and alcohol consumption (weaned for 3 years). Physical examination revealed an ulcerative lesion on the right anterolateral floor of oral cavity, which was adherent to the gingival fibro mucosa. Several leukoplakias were also observed on the gingival mucosa. The rest of the otorhinolaryngology examination was unremarkable. A biopsy confirmed the lesion as an invasive well- / moderately-differentiated squamous cell carcinoma. A contrasted computer tomography (CT) scan showed a tumoral process involving the muscles of the anterolateral floor of oral cavity, which extended about 3 cm in the long axis and remained lateralized to the right (Fig. ). The lesion was in contact with the mandible and furthermore developed a suspicious bone notch. No cervical lymph nodes of significant size and no other suspicious lesions on the cervical-thoracic level were present in this CT scan. A 18F-fluorodeoxyglucose positron emission tomography / computed tomography (18F-FDG PET/CT) scan found a lesion of intense hypermetabolism next to the right genio-glosse triangular muscles and lips, which seemed to repel the omohyoid muscle without infiltrating it (Fig. ). A lytic aspect of the cortical bones of the mandible body suggested a bone extension. 18F-FDG uptakes were perceptible in lymph nodes of right groups Ib and II. The patient received a panendoscopy under general anesthesia. The upper gastrointestinal endoscopy and the bronchoscopy did not find any abnormalities. The TNM staging was classified as T4N2M0 (according to American Joint Committee on Cancer 2009) [] and noninterruptive pelvimandibulectomy was validated as the primary treatment by a multidisciplinary meeting. The surgery was performed in a satisfactory manner 2 months after the first consultation. The post-operative care was performed by standard procedures without abnormalities. The pathological analysis of excised specimens confirmed the squamous cell carcinoma histology as well as the lymph node metastases (Fig. ), suggesting the definitive TNM stage as pT4aN2cM0. In addition, the margins were negative but multiple tumor nodules were found in the muscle. Immunohistochemical analysis for HPV showed the staining of p40 but no expression of p16. At the end of 6 weeks postoperative follow-up, the patient complained of a painful cervical edema as well as a trismus. A CT scan was ordered, which found regional multiple recurrences (Fig. ). A multidisciplinary meeting updated the treatment strategy including a surgical retake, followed by radiochemotherapy. However, the patient died 2 weeks later due to cancer related complications. The patient was recruited into a clinical research program (VADS - EudraCT N簞: 2010-A00586-33, approved by the regional ethic committee ?omit矇 de Protection des Personnes Est III??, which aimed to evaluate the prognostic value of CTC in HNSCC. Venous blood samples were collected at three time points (preoperative day-1, intraoperative, and postoperative day 7) for the detection of CTC. The manipulations were performed by using the CellSearch簧 system (Veridex LLC, Raritan, NJ) according to a standard protocol []. The commercially available CellSearch簧 Tumor Phenotyping Reagent Epidermal Growth Factor Receptor (EGFR) kit (Veridex LLC, Raritan, NJ, USA) was used on the fourth channel of fluorescence of the CellSearch簧 system following the manufacturer? instructions. The pre-, intra-, and post-operative enumerations of CTC are shown in Fig. . A high count of CTC was already detected at baseline (400 CTC), decreasing by 67.5 % at the intra-operative time point (150 CTC), after which it increased significantly (1400 CTC). In particular, all CTC were EGFR negative. Typical images of CTC are shown in Fig. .
Donald Reyna	18	1985/8/13	2798455257	[email protected]	3540 Perez Islands Apt. 151	A 33-year-old normotensive, non-diabetic Bangladeshi Bengali female presented with sudden onset severe retrosternal chest pain and two episodes of syncope over 4 h. Chest pain was worse on deep inspiration and associated with shortness of breath, orthopnoea and palpitations for 2 days. She had an ankle fracture and was on a cast with plaster immobilization for the preceding month, and admitted to unilateral leg pain and swelling. On admission, she was cyanosed with gasping respiration; pulse and blood pressure were non-recordable. She developed asystole soon after, and reverted to sinus rhythm following 2 min of cardio pulmonary resuscitation (CPR). After resuscitation, heart rate was 136 beats/min and blood pressure was 80/55 mmHg. SpO2 was 90 %. Respiratory rate was 35 breaths/min. She was given high flow oxygen, intravenous (IV) normal saline and dopamine infusion for hypotension. Electrocardiogram (ECG) revealed sinus tachycardia (rate 136/min), right bundle branch block (RBBB) with S1Q3T3 pattern (Fig. ). Bedside echocardiogram revealed dilated right atrium (RA) and RV (Fig. ), floating thrombus in RA (Fig. ; Additional file : Video S1); impaired RV systolic function [tricuspid annular planar systolic excursion (TAPSE) was 9 mm] with no evidence of RV hypertrophy; there was mild tricuspid regurgitation and pulmonary hypertension with pulmonary artery systolic pressure ~45 mmHg and normal left ventricular systolic function (ejection fraction 60 %). Immediate thrombolysis was done with IV streptokinase 1.5 million units over 2 h as per accelerated regimen of European Society of Cardiology (ESC) guidelines, resulting in a subsequently normal ECG (Fig. ). Subsequent CT pulmonary angiogram done immediately revealed an approximately 2 cm filling defect in the descending branch of left pulmonary artery extending up to the lateral and posterior basal segmental arteries, suggesting thrombus (Fig. ). d-dimer assay was positive. Troponin-I was 1.27 ng/mL (high risk??.11??.60). Complete blood count revealed neutrophilic leucocytosis. As she had no episodes of bleeding following thrombolysis, she was commenced on low molecular weight heparin (LMWH) at a dose of 1 mg/kg body weight for 5 days with simultaneous oral warfarin titrated to a therapeutic PT/INR of 2.0??.0. Review echo done 2 days later revealed no thrombus or pulmonary hypertension, normal RA and RV. She was discharged on warfarin 5 mg daily and was asymptomatic with therapeutic PT/INR at 3 month follow up. She denied use of the oral contraceptive pill and was advised against its use owing to its potential as a risk factor. Hypercoagulability evaluation including Protein C and S levels, antinuclear antibody, anticardiolipin antibody and serum homocysteine levels were within normal range, thus eliminating other causes of hypercoagulability as possible aetiology of thrombosis.
Luella Hines	33	1981/12/18	(702)972-4351x317	[email protected]	46862 Sellers Ramp Apt. 385	A 12-year-old Irish boy suffered a near total facial burn as a toddler when his Halloween costume caught fire. Eleven prior surgeries for correction of lip and eyelid ectropion were marginally successful. On initial exam, dense keloid scars were present over the entire face, chin, jaw, and neck (Fig. ). Central elements (the nose and upper lips) were predominantly spared. On profile, dense bands of contracting scar extended obliquely across the cervicomental angle causing substantial retrusiveness of the chin (Fig. ). The lower lip was foreshortened and evaginated with exposure of lower dentition and the dento-alveolar ridge. Multi-stage autogenous facial reconstruction was accomplished by MV free transfer of a patterned radial forearm to the neck (Fig. ) followed by bilateral sequential patterned scapular free flaps to the right and left hemiface/cheeks, respectively (Fig. ). Architectural modifications were coordinated with each of the flap transfers. A bimalar fascia lata sling was inset during neck reconstruction to elevate the lower lip (Fig. ). A Porex chin implant provided the chin thrust. Fascia lata slings from the malar arches to the lateral lip modioli were placed beneath each of the scapular flaps for lateral lip support (Fig. ). All autogenous free flaps were 100 % successful. Additional aesthetic refinements included modest debulking of the cheeks, lower lid canthoplasties, dermal plication of the nasolabial creases, and laser resurfacing of the scars. Six months after the final surgery, facial planes are restored and flaps are well incorporated into the facial geometry with seam hidden at junctions of the aesthetic units (Fig. ). On profile, neck contraction is mitigated; cervicomental angle is acute; and chin projection is restored (Fig. ).
Uriel Brock	45	2001/11/27	(800)230-3567	[email protected]	543 Wilkinson Mountain Suite 354	A 10-year-old girl sustained 80 % TBSA burns during a crib fire as an infant in Columbia. She was abandoned by her biological parents and brought to the United States for treatment where she was adopted by caring foster parents. Prior to treatment at The Mount Sinai Medical Center in NYC, she had undergone 10+ prior surgeries with limited success. On exam, the face was grotesquely deformed characterized by obliteration of facial planes, displacement of the LT ocular adnexae, nasal collapse, and microstomia (Fig. ). On profile, the chin was marked retrusive and the lower lip ectropic with exposure of the lower dentition (Fig. ). Nasal tip and bridge projection was deficient. Multi-stage autogenous reconstruction was initiated with sequential pre-patterned, sculpted MV scapular flaps to the RT and LT hemiface, respectively (Fig. ). Deep facial foundation was restored with insertion of fascial lata slings for suspension of lateral lip commissures in conjunction with each of the scapular flaps (Fig. ). Peri-ocular reconstruction was achieved by re-alignment of the medial canthal tendon by transnasal wire fixation and repositioning of the lateral canthal ligament to the lateral orbital rim. Both upper and lower lids were resurfaced with single sheet grafts to the orbital subunit with single slits opened at the ciliary apertures (Fig. ). Total nasal reconstruction included architectural enhancement of the nasal tip with conchal cartilage grafts and dorsal resurfacing with a patterned, pedicled forehead flap using partial thickness burned skin (Fig. ). The divided pedicle base was ?iggy backed??to the lower eyelid for ectropion repair prior to permanent inset. Nostril patency was restored with FTSGs wrapped around nasal stents. Additional refinements included debulking/contouring of the nasal and cheek flaps, SAL, insertion of a Porex chin implant, levator advancement OS, dermal strip grafts for upper lip augmentation, nostril thinning and repositioning, scar revisions, and laser resurfacing. Six months after the final surgery, facial planes have been restored with seams hidden at junction of aesthetic units (Fig. ). Facial components (lips, eyes, nose) are balanced, symmetrical, and complementary. Smile is symmetrical. On profile, nasal, chin, and lip projection are proportional (Fig. ).
Jada Parra	22	1995/9/7	326-534-9237x470	[email protected]	19113 Smith Common	The patient was a 28-year-old woman who was referred to our Burn Institute 1.5 h after a flame burned her head, trunk, and limbs. The patient had a distressed facial expression and was cooperative in the physical examination. She had no breathing difficulty, although obvious swelling, particularly on the head, was observed. No ashes or blisters were found on the oropharyngeal mucosa by laryngoscopy examination. Physical examination data on admission were as follows: body weight, 56 kg; blood pressure, 132/67 mmHg; pulse rate, 88 bpm; body temperature, 36.8簞C; and respiratory rate, 15 bpm. The burn wounds were distributed on the face, neck, trunk, and limbs, and the total burn area was 50% TBSA. Wounds were presented as red, or red and white in appearance, and parts of them were dry with diminished sensation. The laboratory results were as follows: blood routine examination: WBC, 18.64 ? 109/L; neutrophil granulocyte, 90.3%; Hgb, 105 g/L; RBC, 4.03 ? 1012/L; Hct, 0.329; platelet, 296 ? 109/L. The liver and kidney function indexes were all in the normal range except that the total protein level was 50 g/L and the albumin level was 24 g/L. The obstetric examination results were as follows: The patient was 35 weeks and 5 days pregnant, the uterine fundus was halfway between the umbilicus and xiphoid process, and the fetal outline was palpable. No vaginal bleeding or discharge was observed, and uterine contraction was not palpable. Fetal movements were occasionally felt. The cervix was not open, and the fetal membranes had not broken. The fetal heart rate (FHR) was 150 bpm. There was no gestational hypertension, gestational diabetes, or other combined diseases reported. The admission diagnosis was that the flame burned 50% TBSA (superficial second-degree 20% TBSA, deep partial thickness burn 30% TBSA) with a third-trimester pregnancy. During emergency treatment, lactate Ringer? solution was infused for fluid resuscitation, and 1,500 mL of lactate Ringer? solution was infused during the first 9 h after injury. The burns were covered with Vaseline gauze after simple wound cleaning. Nine hours after the injury, after consulting and discussing with the obstetrician, a cesarean section was performed under spinal epidural anesthesia, and a live baby girl was delivered. The surgery lasted approximately 1 h, and the blood pressure was stable during the surgery. The Hgb level after the surgery was 97 g/L. The baby had a body weight of 2,220 g and a body length of 46 cm with APGAR scores of 10?? 10?? and 10?? The vital signs of the patient were stable during surgery. After delivery of the baby, intravenous fluid resuscitation was continued for the patient using the Ruijin (Resuscitation) formula. Ruijin? formula involves the infusion of a crystalloid solution, which comprised primarily lactated Ringer? solution and/or colloids, usually plasma, at a rate of 1.5 mL/kg/percentage of the burned body surface area for the first 24 h and half of the amount of the actually infused crystalloid and colloid solution for the second 24 h. In addition, 2,000~3,000 mL of physical-demanding water was applied every 24 h after injury. The type and volume of the solution delivered for liquid resuscitation are summarized in Table . The hourly urine output was 50~350 mL during the first 48 h after the burn injury. The treatment for the patient included the application of silver sulfadiazine cream on the wounds when the dressing was changed every 2 days, infusion of imipenem for the antimicrobial prophylaxis of infection, and administration of oxytocin and Chinese herbal medication to promote uterine involution. No systemic infection or dysfunction of internal organs was found during the patient? stay in the hospital. The patient discharged herself without postpartum complications at 15 days after injury due to financial reasons. At discharge, the superficial second-degree and part of the deep partial thickness burn wounds that were relatively superficial were healed, and less than 10% TBSA deep partial thickness burn wounds remained on the neck, right upper limb, and both legs. No signs of difficulty of healing that could imply the connection of impaired healing and pregnancy were observed. Physical examination results were as follows: blood pressure, 119/76 mmHg; pulse rate, 90 bpm; body temperature, 36.4簞C; and respiratory rate, 17 bpm. Laboratory results were as follows: blood routine examination: WBC, 8.76 ? 109/L; neutrophil granulocyte, 71.3%; Hgb, 111 g/L; RBC, 4.12 ? 1012/L; Hct, 0.337; platelet, 359 ? 109/L; glutamic pyruvic transaminase, 19 IU/L; glutamic-oxalacetic transaminase, 23 IU/L; alkaline phosphatase, 81 IU/L; TP, 71 g/L; albumin, 36 g/L; urea nitrogen, 3.6 mmol/L; creatinine, 37 弮mol/L.
Davion Nielsen	29	1988/9/3	956.335.4102	[email protected]	419 Kelly Forge Apt. 223	A previously healthy 19-year-old male was transferred to hospital with extensive deep burns covering approximately 85 % total body surface area (TBSA). After initial assessment and debridement in the operating room, he was brought to the general systems intensive care unit (ICU) for acute renal failure likely due to myoglobulinuria. He remained in the ICU for 34 days until he was transferred to the burn unit. Several operations including multiple debridements and allograft applications were performed throughout his time in the ICU and burn unit. During his extended stay, he was treated for many complications, including multiple infections, pancreatitis, deep vein thrombosis, pleural effusion, cardiomyopathy, chronic diarrhea, depression, as well as upper airway and upper gastrointestinal tract bleeding secondary to ulcers. As well, he developed another acute kidney injury thought to be secondary to acute tubular necrosis and had multiple instances of elevated liver enzymes and abnormal liver function tests. Both of these organ insults were thought to be secondary to medications administered, as these values corrected with discontinuation of these insulting agents. In total, his acute care admission lasted approximately 15 months with 33 operations/procedures needed before he could be transferred to a rehabilitation unit. Throughout his prolonged stay, he was worked up for anemia as he required massive amounts of RBC transfusions to maintain an average hemoglobin value over his admission of 95 g/L. This value fluctuated regularly falling into the low 70s on many occasions necessitating the use of blood products. Multiple gastrointestinal bleeds presenting with melena were thought to be large contributors to this laboratory abnormality. The question of hemolysis was raised as early on in his treatment course; he occasionally had elevated lactate dehydrogenase and bilirubin values, as well as positive direct antiglobulin tests. However, his haptoglobin never fell below the normal range and multiple peripheral blood smears had minimal morphological evidence of hemolysis. His chronic anemia was managed with multiple RBC transfusions throughout his hospital admission. While in hospital, he had multiple zinc, selenium, and copper serum values in the low and normal ranges. However, he was noted to have elevated serum iron levels and an increased iron saturation index reaching 94 % (normal <60 %). His iron indices were followed throughout his stay as all values progressed to outside the normal limits (Table ). Subsequent magnetic resonance imaging (MRI) of the abdomen verified the diagnosis of hemochromatosis by showing iron deposition in the liver and spleen (Fig. ). A search for etiology revealed that in just less than 15 months, he had received 292 units of packed RBCs. He did not receive any exogenous administration of iron during his treatment course. With this information, it was thought that the increased iron saturation was due to a transfusional iron overload. Given his ethnicity and age in the presence of a clear alternative diagnosis, the clinical decision to forego genetic testing was made by the Internal Medicine and Hematology services. He had not manifested any typical signs or symptoms of secondary hemochromatosis. The Hematology service was consulted, and as per protocol, he was started on twice weekly phlebotomies []. Due to pre-syncopal episodes, he was unable to tolerate this, and thus, phlebotomy was performed weekly. There is a wide differential of potential causes to consider in patients presenting with iron overload. In this case, the MRI suggested a pattern of iron overload consistent with a secondary (i.e., non-hereditary) process. A review of secondary causes reveals that he did not receive parenteral iron during his stay, did not have a history of chronic anemia or liver disease prior, is not of African descent, and did not have any neurological dysfunction to suggest aceruloplasminemia. Based upon the quantity of RBC transfusions administered during his course, it appears this is the most likely diagnosis. This is the first reported case of transfusional iron overload resulting in secondary hemochromatosis in a burn patient. Previously, this phenomenon has been shown to occur in patients receiving chronic transfusions over an extended period of time for treatment of blood disorders [, ]. This evidence is in keeping with the notion that secondary hemochromatosis due to transfusions is mainly a concern in chronically transfused patients. It has been suggested that signs of iron overload could be present with as few as 10??0 transfusions []. This case substantiates that multiple acute transfusions may also lead to secondary hemochromatosis. Based upon this report, a screening protocol for secondary hemochromatosis may be useful in burn patients receiving acute transfusions, as has been suggested in the population receiving chronic transfusions; however, the details of such a protocol require further study []. Screening for secondary hemochromatosis requires straightforward measurement of serum iron indices, and treatment includes iron chelation and/or serial phlebotomy. There is a technique for phlebotomy described in the hereditary hemochromatosis population that could possibly be applied to transfusionally overloaded patients. The suggested regiment includes removing one unit of blood once to twice a week as serum ferritin analysis is performed periodically to ensure its decline []. Given the simplicity of screening and availability of treatment options, which reduce long-term morbidity, further research in this area is essential to document the prevalence of secondary hemochromatosis in the burn population and the potential need for such a screening protocol.
Vienna Duran	29	1982/5/8	001-881-450-8984x50001	[email protected]	309 Miller Parkway Suite 910	Further, we closely followed up the case of a 29-year-old female who was referred to our hospital for the evaluation of her cervical polyps. A diagnostic hysteroscopy with fractional curettage was performed revealing an endometrial carcinoma (grade II). The patient underwent a radical hysterectomy. Postoperative therapy included brachytherapy in afterload technique in the vagina. Approximately 12 months later after a routine clinical examination, she displayed a nodular goiter. The patient underwent a subtotal thyroidectomy. Due to unclear intestinal complaints, a colonoscopy was performed. In an endoscopic examination small polyps were found in the sigmoid colon and rectum from which a biopsy was taken (Figure ). Histopathological investigations revealed the overall picture of a mucosal ganglioneuroma. The neurological examination showed significant dysdiadochokinesia on both sides, as well as an ataxic uncertain gait pattern. Cerebral magnetic resonance imaging (MRI) was performed for further evaluation. MRI scans revealed two infra-tentorial tumorous lesions on the left side (Figure ). Due to tumor increase, a resection was performed, and the histopathological examination of the excised tumor tissue showed meningioma WHO簞 I (Figure ). Physical examination revealed macrocephaly, multiple facial papules, gingival fitrichilemmomas and acral keratoses. The family history was inconspicuous concerning CS and neurological syndromes. The patient presented clinical signs all indicative for the Cowden syndrome (Table ). Subsequently, human genetic test detected a novel germline G132F mutation in PTEN gene. This mutation occurs within exon 5, which encodes a portion of the phosphatase domain. Interestingly, the G132F missense mutation has not previously been reported in association with Cowden syndrome. Finally, we summarized 109 cases of CS from the peer-reviewed literature featured in .
Ismael Bullock	19	1980/12/30	+1-746-580-5552x0969	[email protected]	006 David Wells Apt. 375	A 66-year-old male patient began to experience vision difficulties, developed a persistent right fronto- temporal headache and memory loss for recent events. On September 3, 2013, after an evaluation, a CT of the brain was done in Panama City, Panama. The CT demonstrated a right temporal occipital tumor with significant mass effect. The patient was started on oral dexamethasone and opted to seek further management in Houston, Texas. He was evaluated at our institution by the neurosurgery team and found to have a dense left homonymous hemianopsia. A brain MRI confirmed the presence of a medial right temporo-occipital lobe mass measuring 3.8 ? 5.8 ? 4.7 cm3 with peripheral enhancement and centrally decreased T1 signal. There was increased T2 signal throughout the white matter, with effacement of the atrium and right lateral ventricle and with lateral displacement (Figure ). In addition, there was superior medial displacement of the adjacent posterior cerebral artery. In September 19, 2013, he underwent a right parieto-occipital craniotomy with maximal tumor removal (Figure ). Surgical pathology revealed a GBM WHO grade IV. Gene sequencing analysis as well as morphoproteomic analysis of the tumor was requested. Four weeks after the surgery treatment was initiated with chemo-radiotherapy. He received a total of 60 Gray (Gy) in 30 fractions with a concurrent dose of temozolomide of 75mg/m2 BSA daily for 42 days through the duration of the radiotherapy. Four weeks after completion of the chemo-radiotherapy he was started on adjuvant treatment with a combination of temozolomide and plerixafor. The temozolomide dose was 150mg/m2 BSA daily for five days the first month of treatment and subsequently escalated to 200mg/m2 BSA daily for five days monthly. Plerixafor was given subcutaneously at a dose of 0.24mg/Kg of body weight once a week. In addition he received a daily dose of lapatinib, niacinamide and metformin. At the completion of twelve months the temozolomide was stopped. He has continued the administration of the weekly plerixafor and daily niacinamide and metformin. Since completion of his concurrent chemo-radiotherapy and initiation of his adjuvant treatment there has been progressive stabilization of his clinical condition and of his brain MRI images (Figure ) with no evidence of recurrence of his tumor. His vision has improved with the assistance of special lenses and he has returned to work. Adverse events experienced during this treatment include a skin rash with diarrhea associated to the lapatinib, infrequent periods of thrombocytopenia during the temozolomide period of treatment and a right popliteal venous thrombosis treated temporarily with enoxaparin. Initially he experienced several months of depression requiring the administration of an antidepressant. His initial weight loss improved once the temozolomide was discontinued and with the assistance of a short course of anabolic steroids and physical therapy. The hematoxylin-eosin stained section of the patient's GBM revealed an infiltrating, cellular glial neoplasm comprised of an astrocyte-like and tumor cells. Mitotic index was estimated 14 per 10 high power fields. Morphoproteomic analysis [] demonstrated the following: epidermal growth factor receptor (EGFR total and EGFRvIII ) was expressed at 2-3+ (scale 0-3+) on the plasmalemmal surface of the malignant glial cells; cytoplasmic and plasmalemmal expression of protein kinase C (PKC)-alpha; activation of downstream effectors and pathways of convergence evidenced by the constitutive activation of the mammalian target of rapamycin (mTOR)/Akt pathway with predominant nuclear compartmentalization of p-mTOR (Ser 2448) and of p-Akt (Ser 473), up to 3+ signal intensity, for both, indicating dominance of the mTORC2 component of the pathway[-]; Sirt1 (silent mating type information regulation 2 homolog 1), an NAD+ histone deacetylase, was found to have variable nuclear positivity up to 2+ in tumor cells; and enhancement of Zeste homolog 2 (EZH2), an histone methyltransferase, was expressed up to 3+ in the majority of tumoral nuclei in some regions(EZH2 has the potential to block differentiation, promote proliferation, and promote tumorigenesis by methylating tumor suppressor genes. It can work collaboratively with Sirt1 in promoting tumorigenesis []). In general there was strong expression of the C-X-C chemokine receptor type 4 (CXCR4) on the patient's tumor endothelial cells including areas of tumoral angiogenesis along with occasional scattered mononuclear cells in the adjacent tissues. The tumor cells per se were negative to weakly positive (簣) but only focally. These are illustrated in Figure . Correspondingly, in the same regions of tumoral angiogenesis, there was an associated over-expression of vascular endothelial growth factor (VEGF)-A. The gene sequencing study (Foundation One, Boston, Mass.) revealed an unambiguous amplification of EGFRvIII and suggested an equivocal amplification of the AKT3 gene. The integration of the morphoproteomic and genomic findings with the pharmacogenomic and targeted therapeutic implications of temozolomide, plerixafor, lapatinib, metformin, niacinamide in this context are addressed in the pathway analysis provided by biomedical analytics (Figure ).
Winnie Galindo	24	1996/2/10	001-435-992-7369x644	[email protected]	2946 Lynn Keys	A 51-year-old male patient who underwent laparoscopic partial gastrectomy for early gastric cancer had been given intravenous cefazolin for 2 days preventively and 5 days after the surgery suddenly developed a high-grade fever (over 39 簞C) and severe diarrhea. We immediately administered oral vancomycin (VCM), Lac-B, viz. probiotics; and enough extracellular fluid because we empirically suspected that these symptoms were due to CDAC or methicillin-resistant Staphylococcus aureus-associated enteritis. A diagnosis of CDAC was rapidly made by confirming the presence of toxin A in his feces. Although these medications were initiated, 24 h after the onset the patient developed septic shock requiring vasopressor agents and MODS composed of DIC and acute renal failure (ARF). When he was transferred to the intensive care unit (ICU), his Acute Physiology and Chronic Health Evaluation (APACHE) II score [] was 22, which estimated his risk of hospital death to be 42.4 % (Table ). According to the clinical and radiological findings, he did not have any colonic perforation or toxic megacolon; thus, we avoided an invasive surgery (such as subtotal colectomy) but alternatively treated him using both PMX-HP to improve septic shock [? and rhTM to control DIC [?. In the first 6 h after starting both treatments, his systolic blood pressure (SBP) improved, requirement for the vasopressor agent decreased, and body temperature (BT) dropped by approximately two degrees. Twenty-four hours after the treatments, septic shock was dramatically improved (Fig. ). Three days after the treatments, an improvement in severe inflammation was noted according to white blood cell (WBC) count and C-reactive protein (CRP) level (Fig. ) and an improvement in DIC according to the fibrin degradation product (FDP) level and prothrombin time (PT) (Fig. ). A temporary decline in the platelet count was controllable with platelet transfusion (Fig. ). Although four cycles of continuous hemodiafiltration (CHDF) were necessary as a replacement therapy to ARF, the further progression of MODS was not observed and the APACHE II score satisfactorily decreased daily (Fig. ). Although clinically moderate diarrhea and a mild fever were observed, his general condition also improved. Consecutive toxin A tests, except the first one, were all negative. Five days after the treatments, the patient overcame fulminant CDAC through the use of the abovementioned therapies. Throughout the entire clinical course, neither endotoxemia nor bacteremia was observed. Currently, CDAC is endemic and a common nosocomial enteric disease encountered by surgeons in modern hospitals due to prophylactic or therapeutic antibiotic therapies [?. Recently, both the incidence and the severity of CDAC have been increasing, and one possible explanation for these increases is the emergence of highly toxigenic and lethal strains of CD [?. The above shows the need for surgeons to consider more serious treatment against CDAC. In fulminant CDAC, which has a higher lethal rate, it is especially necessary for surgeons to promptly decide whether or not to perform an invasive surgical treatment, such as subtotal colectomy, which means the removal of the septic sources and probable improvement of the patients??ill condition [?. In our case that suddenly developed fulminant CDAC with septic shock requiring vasopressor agents and MODS composed of DIC and ARF, prompt surgical treatment in order to remove the septic sources was recommended. However, we alternatively treated the patient with both PMX-HP and rhTM therapies. The reason for having chosen these treatments is as follows: (1) there was neither colonic perforation nor toxic megacolon, which absolutely requires surgery; (2) PMX-HP is an effective extracorporeal blood purification treatment for improving septic shock []; and (3) rhTM can effectively inhibit systemic dissemination of intravascular coagulation [?. The combination therapies produced excellent outcomes in this case, namely the dramatic improvement of septic shock and DIC, the inhibition of MODS progression, and the patient? survival. We speculate that the two below-mentioned factors corresponded to the removal of the septic source, namely as result of the surgical treatment. First, oral VCM medication could suppress CD? proliferation and the further production of CD toxins. Second, both PMX-HP and rhTM could completely inhibit the CD toxin-induced mediators??cascades. This notion is based on the following evidence. First, fulminant CDAC with MODS is caused by the shift from an inflammatory response at a local mucosal level to a general systemic inflammatory reaction in which CD toxin-induced mediators??cascades disseminate [?. Second, although PMX-HP removes circulating endotoxin by adsorption and theoretically prevents the progression of the biological cascade of sepsis, several studies and published reports have demonstrated that PMX-HP can reduce the plasma levels of cytokines and sepsis-related factors, namely TNF-帢, IL-6, IL-10, N-arachidonoylethanolamine (AEA), 2-arachidonoyl glycerol (2-AG), and high-mobility group box-1 (HMGB-1) [, , , ]. Indeed, there were case reports published which showed that PMX-HP decreases the serum levels of endogenous cannabinoids (anandamide and 2-AG) and inflammatory cytokine (IL-6) in parallel with the clinical improvement of fulminant CDAC [, ]. Third, many studies and fundamental researches have shown that rhTM also has an anti-inflammatory ability through both the activated protein C and the lectin-like domain-dependent pathway [?. In particular, the thrombin-rhTM complex demonstrates an anti-inflammatory ability through neutralizing HMGB-1 [, ], which is known to be a mediator of lethality and is released from necrotic cells or macrophages/activated dendritic cells with potent pro-inflammatory function, which in turn causes shock or MODS when being disseminated in the systemic circulation [?. Finally, septic shock and MODS in our case were not induced by endotoxemia or bacteremia, and a dramatic improvement was observed immediately after the initiation of the combination therapies.
Salvatore Webb	27	1980/10/31	327.345.1388x6781	[email protected]	076 Sarah Rapid	An 85-year-old man presented to our hospital with a 5-h history of abdominal pain and distention. He attended the outpatient department at our hospital for regular checkups but had been asymptomatic until he visited out emergency department. At age 82 years, he had undergone laparoscopy-assisted distal gastrectomy with Roux-en-Y reconstruction for early gastric cancer. An antecolic isoperistaltic gastrojejunostomy was performed using an endoscopic linear stapler. A serosubmucosal single-layer hand-sewn anastomosis (Jourdan?) was performed through a small abdominal incision for Y reconstruction. The mesentery of the Y loop was closed using nonabsorbable braided polyester (3-0) interrupted sutures, while the Petersen? defect was not closed. Pathology revealed a stage IB (T2N0M0) tumor; the resection stumps were negative. His postoperative course had been unremarkable. Computed tomography (CT) performed 1.5 months prior to admission showed no evidence of tumor recurrence and no ascites. The patient had no other relevant medical history. Physical examination on arrival showed focal tenderness and distention in the epigastrium. His body temperature was 36.4 簞C. Laboratory data were unremarkable except for a white blood cell (WBC) count of 8.7 ? 109 cells/L (normal, 4.0??.0 ? 109 cells/L) and a C-reactive protein (CRP) level of 16.2 nmol/L (normal, < 2.9 nmol/L). A contrast-enhanced abdominal CT scan (Fig. ) showed dilation of the transverse colon, severe ascites, and a whorl-like appearance of the superior mesenteric artery (SMA) and branches wrapped with adjacent mesentery and small bowel loops (the ?hirl??or ?horl??sign) [], which is characteristic of small bowel volvulus. Given these findings, we performed emergency laparotomy. Intraoperatively, a massive amount of milky fluid was observed in the peritoneal cavity, and approximately 1 L was removed (Fig. ). The greater part of the small bowel passed through the defect between the jejunal mesentery and the mesocolon (Petersen? defect). The small intestine was displaced to the left and was obstructed due to jejunal volvulus (Fig. ). The jejunum was rotated 180簞 counterclockwise around its long axis and compressed the transverse colon. The entire small bowel was edematous but viable, with no evidence of a mobile cecum, peritoneal tumor, or perforation. The volvulus and internal hernia were reduced, and the Petersen? defect was sutured closed. No bowel resection was required. Extensive lavage of the abdominal cavity was performed until the return fluid was clear, and intraperitoneal drains were placed in the rectovesical pouch and under the right hemidiaphragm. The triglyceride (TG) level of the ascitic fluid was 642 mg/dL. The cultures and cytology of the peritoneal fluid were negative for infection and malignancy, respectively. The patient? postoperative course was uneventful. The color of the ascites changed from milky to light yellow, and the TG levels decreased to 21 mg/dL on postoperative day 4. The patient was not required to eat a fat-restricted diet during hospitalization and was discharged from the hospital 16 days after surgery. He underwent a physical examination and CT scan in the outpatient department 10 months after discharge from the hospital. There was no evidence of ascites (Fig. ). He had made favorable progress by the time of the 28-month postoperative follow-up, with evidence of weight gain and no evidence of recurrent ascites (Fig. ). Chylous ascites typically develops due to congestion of the lymphatic system. Malignancy?articularly lymphoma?s a relatively common cause of chylous ascites in adults []. Metastasis of the malignancy into the lymphatic channels and the resulting occlusions can cause leakage from the dilated subserosal lymphatic system into the peritoneal cavity [, ]. However, there have been few reports of chylous ascites associated with intestinal obstruction []. In the present case, an abdominal CT scan showed a dilated colon and severe ascites with a whorl-like appearance of the SMA and mesentery, findings generally suggesting small bowel obstruction from volvulus [, ], which may be strangulated [, ]. High TG levels, typically >200 mg/dL, in the ascitic fluid are critical in defining chylous ascites []. Given that our patient had TG levels exceeding 600 mg/dL, the milky fluid was diagnosed as chylous ascites. The CT value of the ascites was 5.0 HU, not suggestive of hemoperitoneum and indistinguishable from that of water [?. Major lymphatic leaks can sometimes be visualized with MR lymphography [], aiding the diagnosis of chylous ascites. However, minor leaks may be undetectable. After surgery to repair the internal hernia, the TG levels decreased from 642 to 21 mg/dL without any ligation of lymph vessels or dietary restriction. These observations prompted us to diagnose the patient as having chylous ascites associated with small bowel obstruction. We identified eight case reports of chylous ascites with bowel obstruction in the English language literature (Table ). Chylous ascites associated with intestinal obstruction was first mentioned by Mackman in 1967, and our present case is the first case report of chylous ascites occurring with a Petersen? hernia. As in our case, all previous cases were characterized by the presence of small bowel obstruction. Detecting a cause of chylous ascites associated with bowel obstruction is difficult, because these cases are so rare. Of note, however, no previous studies [? reported the requirement for an enterectomy, and their postoperative courses were uneventful. The abdominal CT scan showed that there was an intestinal obstruction and large amounts of ascites. These findings sometimes indicate strangulated obstruction. Our case was interesting in that the physical examination and laboratory tests did not match the severity of the problem as shown on CT. It is difficult to explain this discrepancy, and it is difficult to prove accurately what caused the chylous ascites. This situation is very rare. The absence of bowel ischemia suggests that the arteriovenous system was patent while lymphatic channels were occluded in this case. This vascular patency may explain the discrepancy between the laboratory values and physical findings and the imaging findings, and the fact that no previous cases have reported the requirement for an enterectomy. The obstruction of the lymphatic system resulted in the extravasation of chylous fluid []. Pre- and intraoperative evaluation of ascites may be helpful in making decisions concerning therapeutic strategies, because bowel obstruction with chylous ascites may indicate intestinal viability.
Ariella Webster	34	1983/4/3	2622133603	[email protected]	9953 Greene Hill	A 64-year-old man presented with two weeks of progressive proximal muscle weakness causing him difficulty ambulating, combing his hair, and raising himself from a seated position. He also reported dysphagia to solids over the past week. The patient was recently admitted twice to an outside hospital for an elevated creatine phosphokinase, and diagnosed with ?habdomyolysis.??Past medical history was significant for poorly controlled diabetes mellitus. On exam, vitals were within normal limits. Cardiovascular exam revealed regular rate and rhythm with no extra heart sounds and lung exam revealed clear lungs on auscultation. On neurologic exam, patient was awake, alert, and oriented. Cranial nerves II through XII were grossly intact. There was proximal muscle weakness to 4/5 bilaterally in the shoulders and hips, but preserved strength distally. Sensation to light touch remained intact. Hemoglobin was 10.5 g/dL with a normal MCV, WBC were 3.8 K/uL, and platelets were 246 K/uL. Basic metabolic panel revealed a sodium of 141 mEq/L, a potassium of 4.8 mEq/L, and a creatine of 0.90 mg/dl. Transaminases including AST and ALT were 65 and 36 U/L, respectively. CPK was 2705 U/L. ESR and CRP were within normal limits and RF, anti-Ro/La, anti-Jo1, anti-RNP, anti-centromere, anti-Scl-70, and anti-dsDNA were all negative. An MRI of the right arm revealed prominent edema involving the deltoids and an MRI of the bilateral thighs revealed myositis with no discrete collections. An EMG showed evidence of irritative myopathy affecting the bilateral deltoids. A muscle biopsy of the right deltoid was negative for inflammation but presumed to not be representative. He was started on oral Prednisone 60mg daily with resolution of symptoms including weakness and pain and normalization of creatine kinase within one month of starting therapy.
Shawn Hester	29	1978/7/23	703-237-5267	[email protected]	029 Davis Cliffs	A previously healthy 15 year-old female presented with behavioral changes with incoherent speech, restlessness, anxiety, and expressed the feeling that ?verything is happening to me??after attending a 2-day spiritual retreat. One week before the presentation of psychiatric symptoms, she had unspecific common cold symptoms, but with no fever. On the second day of psychiatric presentation, she had a transient period of agitation and visual hallucination with spontaneous remission (she saw Jesus and her deceased grandparents). On the third day, she was brought to the emergency room (ER), for the first time, owing to an episode of agitation and speech blockade that happened at school. Concomitantly, she had somatic manifestations, such as swallowing impairment, odynophagia for water and food refusal. She showed regressive behavior, such as asking for help for basic hygiene and refusal to sleep alone. At this point, episodic speech changes, such as echolalia and verbal perseveration and intermediary insomnia were documented. Of note, there were no changes in the flow of thoughts or indication of disorganized behavior. The patient was medicated with risperidone (0.5 mg once a day) by the ER child and adolescent psychiatry attendant, and referred to the Child and Adolescent Psychiatry Outpatient Clinic. However, a few hours later, she was re-admitted in the pediatric ER with panic symptoms (tachypnea, tachycardia, shaking hands, and sweating). She was discharged and medicated with lorazepam (0.5 mg two times a day) and risperidone (1 mg once a day) with no improvement, namely, the visual hallucination persisted. At the first child and adolescent psychiatry outpatient appointment (day 7 after symptoms onset), she was oriented, and presented with unexpressive facial mimicry, provoked speech, and psychomotor retardation. No changes in the form, rhythm or flow of thoughts, or delusions were documented. The patient had anxiety with no mood changes, although she had a regressive attitude. The patient was medicated with anxiolytics (diazepam 5 mg two times a day), and risperidone (1 mg) was increased to twice daily. At the next child and adolescent psychiatry appointment (day 10 of the disease), she maintained episodes of agitation with tremor of the upper limbs, screaming, and a desperation gaze that was attributed to hallucinations, according to her family. During these episodes, the patient said that ?hey are after me (?? I am a saint (?? nobody will survive?色? On other occasions she would experience periods of elation, with tendency to sing and express grandiose delusions: ? will be the best student at my class?? During the mental state examination she was extremely sleepy, calm with provoked speech, and answered with monosyllables. Therapy was adjusted, and risperidone was replaced with olanzapine (10 mg once a day). She was recommended for hospital admission, but the patient? family refused this recommendation. On day 18 of the disease, with no signs of clinical improvement, the patient was brought to a new appointment, and admitted in a psychiatry ward. At admission she was oriented to time, place, person and situation but showed periods of negativism, mutism, echolalia, echopraxia, motor agitation, upper limb tremor (that occurred at rest, with sudden onset and remission and disappeared when she was distracted), These symptoms occurred in bursts alternated with periods of normal speech but with pervasive prostration. Also, she was unable to take care of her own hygiene. At physical examination she did not have fever or changes in blood pressure, and cardiac or respiratory frequency. Pulmonary and cardiac auscultation were unremarkable, abdomen examination was also normal, and at the examination she had no changes of the skin and upper and lower limbs. At that point, the following diagnostic hypotheses were considered: prodromal phase of an affective psychosis, anxiety disorder with conversion traits, dissociative disorder (depersonalization disorder), organic disorder, and psychosis with catatonic features. Due to the catatonic symptoms, the diagnosis of neuroleptic malignant syndrome was also raised and a possible treatment with dopamine agonist was considered. However, this specific intervention was deferred since the patient had no fever or changes in the blood pressure, cardiac or respiratory frequency or laboratory imbalance. The diagnostic hypotheses were based on the clinical presentation, physical examination, and mental state examination. We did not apply the mini-mental state examination or any other rating scale for psychosis, mood, cognitive or neurologic symptoms. Laboratory results and brain CT scan were normal. Psychiatric medication for psychosis was started. At day 5 after admission (day 23 of clinical disease), a lumbar puncture was performed with screening for antibodies for anti-NMDA receptors in the spinal fluid. Since the hypothesis of anti-NMDA receptors encephalitis was raised, it is mandatory to first rule out possible underlying neoplasm. Because the patient was female and ovarian teratoma can occur in up to 59 % of cases [], a pelvic ultrasound was performed, but no changes were found. On the same day, the patient experienced tachycardia and decreased consciousness, with the need for intensive care unit (ICU) admission. No rating scale was applied during the stay in the intensive care unit. However, antibodies for NMDA receptors were detected and diagnosis of anti-NMDA receptor encephalitis was established. At the ICU the patient experienced periods of delusion and agitation with loud screaming. She was medicated with midazolam (2 mg intravenous if needed), oral lorazepam (1 mg three times a day), and chloral hydrate (1 g every 6 h). Within the first few hours, risperidone was introduced at 0.5 mg daily and then titrated to 1 mg daily. She was subsequently treated with immunoglobulin (2 mg/kg/day), methylprednisolone (30 mg/kg/day), and plasmapheresis and rituximab (375 mg/m2/week). Regarding the maintenance psychopharmacology, the patient was on risperidone (1 mg two times a day) and lorazepam (2.5 mg if needed). Two months after specific treatment for anti-NMDA receptor encephalitis, the patient still had difficulty with verbal articulation, but there were no changes regarding form, course, rhythm or thought content, and her mood was euthymic. She reported periods of emotional lability, although less frequent than before treatment, and there were no auditory or visual hallucinations. She reported moderate verbal memory impairment, attention and concentration difficulties at school. Although a formal neuropsychological evaluation was not performed, it was known that before disease onset, the patient had no cognitive difficulties at school since her IQ was considered within the normal range, and her grades were average. She started psychotherapy, physiotherapy, and learning support at school. She had progressive clinical improvement but still experienced emotional lability, anxiety, and verbal memory impairment, attention and concentration difficulties at school that justified her enrolment in a vocational educational school. Ten months later she had a seizure episode and a new treatment cycle was started. Since she experienced depressive symptoms after the encephalitis episode, sertraline (25 mg once a day) was added to her medication regimen. As of this writing, she is medicated with oxcarbazepine (450 mg two times a day), quetiapine SR (100 mg at bedtime), sertraline (25 mg once a day) and lorazepam (1 mg if needed). Also as of this writing (almost 3 year after the disease onset), the patient? psychiatric symptoms have improved, but she still experiences verbal memory impairment, and attention and concentration difficulties.
Zendaya Humphrey	44	1995/4/23	267.816.6246x99619	[email protected]	160 Swanson Corner	The first patient was a 57-year-old woman treated for a uterine fibroid. For the induction of the sedation, the following strategy was applied: the remifentanil was kept at a constant dosage, while the propofol dosage was slowly increased to lower the breathing frequency to 5/min. Final dosages for propofol and remifentanil were 1.4 mg/kg/h and 2.5 弮g/kg/h, respectively. This stable breathing pattern was reached after <2 h.
Krew King	43	1985/3/25	001-563-989-5180x193	[email protected]	660 Smith Manors	The second patient was a 72-year-old woman with bone metastases of which one was located in the shoulder. Here, the strategy was to start with a higher dosage of remifentanil. However, the remifentanil dosage was slowly decreased during the treatment, while the propofol dose was slowly increased to induce a deeper state of sleep. A stable, low frequency (5/min) breathing pattern was reached with 1.6 mg/kg/h propofol and 0.3 弮g/kg/h remifentanil in a little under an hour.
Victoria Munoz	31	1983/3/5	688.396.2670x51907	[email protected]	60465 Kathleen Ramp Apt. 692	The third patient was 41 years old and treated for a uterine fibroid. The dosage of remifentanil was kept constant at 4.2 弮g/kg/h and the dosage of propofol was adjusted to the patient? state of sedation during the treatment and was finally 2.2 mg/kg/h. After the treatment was completed, an additional MR scan (spoiled gradient echo with echo planar imaging, EPI-factor 25, TE 15 ms, TR 39 ms, flip angle 20簞, acquisition matrix 120 ? 98, pixel size 2.34 ? 2.34 mm, slice thickness 7 mm) was performed to visualize the displacement of the diaphragm due to respiration. One hundred percent oxygen was continuously offered using a nasal prong in all three patients. End tidal carbon dioxide (ETCO2) was measured using capnography. Furthermore, breathing frequency, blood pressure values, and heart rate were monitored.
Justin Luna	26	1982/8/26	001-771-682-7701x769	[email protected]	7558 Samuel Crossing	A 64-year-old man with gross swelling in the anterior mandibular gingiva for 1 month after lower incisal teeth extraction was referred to us for a closer evaluation. Oral examination revealed a firm mass measuring 2.6 ? 2.1 cm in size arising from the extraction site (Fig. ). The surface of the mass lesion showed erosion at the site equivalent to the wounds after tooth extraction. There was no hemorrhage or paralysis in the mandibular region, and the condition was painless. For the past medical history, the patient underwent low anterior resection of a rectal malignancy diagnosed as moderately differentiated adenocarcinoma 7 years previously. There was no evidence of regional lymph node and distant metastases. Three years after the primary surgery, the patient showed bilateral lung metastases. Thus, the middle lobe of the right lung and the lower lobe of the left lung were partly resected. Four years later, multiple lung metastases and metastases to the vertebral bodies of the VI?III vertebrae were observed. The patient received 60 Gy of irradiation to control the pain from the lesion involving the vertebra. Computed tomography (CT) scan of the head and neck showed a solid mass and bony resorption in the anterior alveolar ridge of the mandible (Fig. ). There was no lymphadenopathy. Magnetic resonance imaging (MRI) revealed a heterogeneous signal on a T1-weighted image after gadolinium administration and an intense signal on a diffusion-weighted image (Fig. ). For the laboratory examination results, the serum carcinoembryonic antigen and cancer antigen 19-9 levels were 947.6 ng/ml and 1059.2 U/ml, respectively. Pathological examination by incisional biopsy of the gingival mass lesion revealed a moderately differentiated adenocarcinoma. The tumor was composed of proliferating columnar to polygonal epithelial cells with moderate atypia that showed a papillary tubular pattern (Fig. ). Immunohistological analysis demonstrated that the adenocarcinoma cells were positive for cytokeratin 20 (CK20) and negative for cytokeratin 7 (CK7) and positive for caudal type homeobox transcription factor 2 (CDX2) (Fig. ). The biopsy findings from the mandibular mass lesion were consistent with those from the resected rectal carcinoma 7 years previously. The tumor had grown gradually and an ulcer was formed on the surface, making mastication difficult. The patient received palliative radiotherapy for the mandibular mass lesion. However, once the tumor showed a tendency to decrease in size, pneumonia developed and the patient? cachexia deteriorated. Thus, the radiotherapy was discontinued, but the patient unfortunately died 2 months postradiotherapy. Oral metastatic tumors are uncommon and comprise approximately 1 % of malignant oral neoplasms []. Most cases were previously diagnosed in reference to a primary neoplasm and were treated accordingly. Therefore, the primary site is usually assumed based on the past clinical history. However, 23 % of oral metastases were shown to be the first sign of the metastatic process []. In the present case, the origin of the metastatic lesion was clarified based on the past history of rectal adenocarcinoma resection and the subsequent lung metastases. The most common primary site in women is the breast followed by the female genital tract, kidney, and colon-rectum. In men, the most common primary site is the lung followed by the kidney, liver, and prostate []. In the oral site where metastatic tumors arise, most tumor lesions are observed in the jawbones, and only 16 % are found in the soft tissues such as the gingiva []. The metastatic rate in the posterior body of the mandible is higher than that in the symphyseal region []. The gingivae (55 %) followed by the tongue (30 %) are the most common soft tissue sites affected by metastatic malignancy []. In the present case, the metastatic lesion has been presumed to develop in the gingival tissue of the symphysis. This lesion was an exophytic growing mass, and the radiological appearance of the mandibular bone invasion was not demonstrated apparently. Likewise, some case reports have shown that the metastatic site was the gingiva for the primary lesion of colonic and rectal adenocarcinomas [?. Adenocarcinoma of the colon and rectum commonly spreads to the regional lymph nodes, liver, and lungs, but less commonly to the peritoneum, bone, adrenal glands, brain, kidney, thyroid, pancreas, ovaries, and skin. Clausen and Poulsen proposed the following three criteria for metastatic oral tumor: firstly, the metastatic tumor is pathologically similar to the primary tumor; secondly, the oral tumor is considered to be a metastasis clinically and pathologically; and thirdly, the oral tumor is atypical compared with common oral primary tumors []. The most common primary tumor arising from the oral mucosa is squamous cell carcinoma. Therefore, there may be a need to differentiate metastatic adenocarcinoma from adenocarcinoma derived from the salivary gland, although the frequency of occurrence is very low. Three clinical courses may be considered to underlie the metastasis of colonic adenocarcinoma to the oral cavity as follows: (1) direct metastasis with identification of the primary tumor, (2) secondary metastasis derived from metastasis in another site after treatment of the primary tumor, and (3) occult metastasis without identification of the primary tumor. The present case follows the second clinical course in which the secondary metastasis in the jawbone was derived from multiple lung metastatic lesions. The primary rectal tumor has already been resected completely. The present case conformed to Clausen? criteria. Histopathologically, approximately 70 % of the metastatic lesion in the oral region is reportedly adenocarcinoma, followed with much less cases by clear cell carcinoma from the kidney and squamous cell carcinoma from the lungs []. Also in the present case, the hematoxylin and eosin-stained section showed features of adenocarcinoma arising from the rectum. Furthermore, the histological diagnosis was supported by the immunohistochemical analysis. The metastatic tumor cells demonstrated a CK20-positive and a CK7-negative phenotype. In the evaluation of gastrointestinal tract carcinomas, the great majority of well-differentiated or moderately differentiated large intestinal adenocarcinomas reportedly showed a CK7-negative/CK20-positive phenotype. Adenocarcinomas of the upper gastrointestinal tract were also reported to be positive for both CK7 and CK20 in 78 % of cases []. Thus, an assessment of the CK7 and CK20 phenotype may help in determining the primary site of a metastatic tumor []. In addition to the CK7 and CK20 immunoprofiles, the tumor cells showed CDX2 immunoreactivity. CDX2 is a sensitive and specific maker for adenocarcinoma with a colorectal origin []. CDX2 expression has also been shown in a subset of adenocarcinomas arising from the colon, rectum, stomach, esophagus, and ovary []. Accordingly, CK7, CK20, and CDX2 may be useful for the diagnosis of metastatic tumors of gastrointestinal origin. In the case of oral metastatic tumors, they usually have a poor prognosis because often they have already spread to other sites. Therefore, the appropriate palliative treatment has to be carefully selected. If the tumor is widely disseminated, palliative radiotherapy is recommended []. However, when possible, surgical resection may be recommended mostly for metastatic lesions in the soft oral tissue []. In the present case, as the oral metastatic lesion was rapidly growing with accompanying multiple lung metastases, resection was not possible under general anesthesia. Accordingly, we performed palliative radiotherapy for the mandibular mass. Alternatively, chemotherapy with the FOLFIRI regimen (5-FU, leucovorin, irinotecan) is usually performed for advanced metastatic colorectal carcinoma []. Although the IRIS regimen (irinotecan, S-1) plus bevacizumab (monoclonal antibody targeted against vascular endothelial growth factor) was planned when the lung metastasis occurred to play the role of another second-line chemotherapeutic regimen, the patient showed no response to the chemotherapy. Patients with distant metastasis of colorectal adenocarcinoma usually have a poor prognosis. In particular, if a secondary metastatic oral tumor is identified, the disease state may be commonly progressive and the prognosis is extremely poor. In cases involving metastasis to the oral cavity, the mean survival period from the time of mass appearance to the time of death of a patient has been reported to be about 7 months []. Regarding metastasis to the gingiva, some cases have been documented showing that the duration of survival from the indication of gingival mass development was only within 1 year [, , ]. The present case also showed a short survival period of 4 months.
Journey Parks	34	1992/6/30	+1-849-770-5390x06499	[email protected]	804 Lee Falls	The first patient is a 43-year-old male, who consulted our center for night sweats and weight loss in the last 3 weeks. He had no previous history or records in our center. The physical examination was normal except for a slight splenomegaly and the blood test was compatible with hyperleukocytosis, anemia and thrombocytopenia, (leukocytes 136.00 繚 109/L, hemoglobin 11.70 g/dl, platelet count 39.70 繚 109/L) and 93 % of small-medium sized lymphoblast in the Grunwald-Giemsa blood smear. The bone marrow aspirate examination had an hypercellular infiltration of 90 % which was confirmed by immunophenotype (CD19+, CD10+, CD34+, TdT+, DR+, CD79 alfa+, CD20?? cytoplasm IgM negative). 80 % of the blast cells also expressed CD33+. A fresh sample from the bone marrow aspirate was collected for molecular and cytogenetic analysis. The assay revealed the presence of the following karyotype:46,XY,t(9;22)(q34;q11) [2] /46,XY,del(9)(p22)t(9;22)(q34;q11) [4] /46,XY,del(9)(p22)t(9;22)(q34;q11),del(20)(q13) [5] /46,XY [9]. FISH analysis confirmed the presence of BCR-ABL1 fusion gene (Fig. ). The patient? RNA was isolated from the peripheral blood and subjected to a two round multiplex reverse transcription and polymerase chain reaction (RT-PCR). In order to avoid RNA quality and/or handling errors, we included an internal positive control in where a 690-bp segment of the ubiquitously expressed transcription factor E2A mRNA was amplified. The primers and PCR conditions used in the first and second round of the nested PCR reaction are described by Pallisgaard et al. []. We identified an atypical amplification band of approximately 100 bp, much smaller than the p190 (e1a2) fragment. In order to confirm the presence of a BCR-ABL transcript this band was extracted from the agarose gel, purified and then analyzed by DNA sequencing. cDNA sequence revealed the presence of the e1a3 variant (Fig. ). The patient started induction treatment with ALL-type polychemotherapy and Imatinib, achieving complete response by morphology and immunophenotype. However, the BCR/ABL was still detected by RT-PCR at the end of the induction phase. He is ongoing consolidation with allogenic stem cell transplantation (SCT) from an HLA-identical sibling donor, 7 months after diagnosis.
Gianni Schaefer	36	1997/10/27	+1-346-897-7899x773	[email protected]	8912 Guzman Mountain	The second patient is a 65-year-old male who consulted for pain in the right leg and a history of recurrent periodontal infections in the last month. An eco-doppler confirmed a thrombophlebitis and the blood test found hyperleukocytosis and anemia (leukocytes 223.00 繚 109/L, Hemoglobin 11.60 g/dl, platelet count 118 繚 109/L) and the presence of 80 % lymphoblasts in the Grunwald-Giemsa blood smear. The bone marrow aspirate found an infiltration of 25 % lymphoblast which was confirmed by immunophenotype (CD19+, CD34+, DR+, CD10+, CD22+, low intensity CD20?? TdT+, cytoplasmatic IgM negative, CD33+). The karyotype was 46,XY,t(9;22)(q34;q11) [20]. FISH analysis confirmed the presence of BCR-ABL1 fusion gene (Fig. ). The patient? RNA was isolated from the peripheral blood. We identified an atypical e1a3 variant after a two round PCR amplification by DNA sequencing (Fig. ). The patient started induction treatment and Imatinib. The bone marrow aspirate on day 34 confirmed progressive disease with 30 % blast and persistence of BCR/ABL. After a second line of intensive ALL-type induction therapy the patient finally died due to toxicity (gastrointestinal bleeding). No SCT had been planned due to age and unfit status. It is noteworthy that the blood counts in a regular check-up 4 months before the diagnosis had a slight leukocytosis that was not studied.
Mavis McMillan	27	1989/8/4	566.572.9131	[email protected]	4844 Taylor Spring	A 72 years old female complaining of hemoptysis, dyspnea, fatigability and weight loss was referred to the surgery clinic. A history of 7 abortions was obtained. 33 years ago she self-induces an abortion using a knitting needle without extraction of the foreign body. Clinical exam showed a decreased mobility of the right hemidiaphragm but no scars. Hemoglobin and hematocrit were borderline low (HGB 11.6 and HCT 38.4), with RBC indices suggestive for anemic status (MCV 67.5, MCH 20.4). Pulmonary function tests showed decreased forced vital capacity (FEV) and Forced expiratory volume in 1 s (FEV1). Initial radiological workup included a chest x-ray which showed a linear metallic foreign body extending from mid-thorax into the abdomen (Fig. ). Two more x-rays were performed (Fig. and ), one focused on the lesion and another one in lateral view, establishing its caudal end at the level of the liver and its size at 18 cm. In retrospect a focal increased density lesion is viewed in the posterior-basal segment of right inferior lobe. Following clinical meeting a CT was ordered showing the foreign body extending from right liver lobe (segment V and VIII), transdiaphragmatic, to the right upper lobe, where it ends in a thick walled cavity of 3 cm (Figs. and ). No traces of migration have been described in the abdomen or pelvis. A 3.9 cm consolidation in the posterior-basal segment of RLL was also described with associated mediastinal adenopathy. No abdominal or bone lesions were detected. Right posterolateral thoracothomy was performed. Intraoperative examination detected pulmonary right lower lobe to diaphragm adhesions and fibrous tissue delimiting a thoracoabdominal transdiaphragmatic fistula due to a foreign body (Fig. ). An 18 cm metallic foreign body producing a hepatic fistula of 4 cm and RLL fistula of 8 cm is extracted (Fig. ). Wedge resection of RLL and diaphragmatic mesh suture with infradiaphragmatic drainage were performed. The postoperative results were good, with the patient discharge 8 days later. Only one follow-up was available; at 3 months??control the patient was in good status with no complains.
Rocky Curtis	24	1984/11/29	+1-932-771-3442x234	[email protected]	860 Nicole Well	A 56-year-old woman began to suffer from an occipital headache for 7 days before admission to our hospital, and dizziness appeared 5 days later. On admission, she complained of dizziness and a mild occipital headache. She showed nystagmus and the right nose-finger-nose test was positive. In peripheral blood cell counts and biochemical studies, elevations in white blood cells (9,200/弮l) and low-density lipoprotein cholesterol (215 mg/dl) were observed. Coagulation studies and immunological studies were normal. Head computed tomography (CT) showed low-density areas in the right cerebellar hemisphere, diffusion weighted imaging (DWI) of magnetic resonance imaging (MRI) displayed high-intensity signal areas in the cerebellar vermis and the right cerebellar hemisphere (Fig. ). In addition, a high-intensity signal area was observed just adjacent to the right VA on T1-VISTA (Fig. ), whereas no such high intensity was detected on conventional T1 image. Conventional MRA did not depict the right PICA or any abnormal findings of the right VA (Fig. ). Echocardiography and 24-hour electrocardiography showed no findings suggestive of cardioembolic causes. According to these findings, ischemic stroke of the cerebellum induced by the right PICA-D was diagnosed, and after confirming that she had neither hypertension nor aneurysmal formation, combined therapy with clopidogrel and unfractionated heparin administration was started. T1-VISTA showed a slight expansion of the high-intensity area at 3 days after admission, but still no lesions on T1 images. At 9 days after admission, cerebral angiography revealed the ?earl and string sign??at the proximal portion of the right PICA (Fig. ). At 12 days after admission, the patient was discharged with single antiplatelet therapy (clopidogrel). On T1 images, a pale high-intensity area was documented at the dissected lesion at 19 days after admission. Thereafter, the right PICA was gradually depicted on MRA and completely visualized at 5 months after the onset. In parallel with that, the high-intensity signal area on T1-VISTA gradually decreased in size and disappeared by 5 months after the onset (Fig. ). Clopidogrel administration was then stopped.
Alexis Harrington	36	1997/2/27	001-989-411-8724x51206	[email protected]	642 Paul Lights	A 52-year-old woman suddenly experienced vertigo and vomited while cleaning her bathtub at home and was subsequently transferred to our hospital by an ambulance. Head CT showed no abnormal findings, thus she was admitted under the suspicion of acute drug poisoning by a detergent. Although no abnormalities on a physical examination or in laboratory data were observed, her nausea and dizziness persisted. Neurologically, she presented only mild dizziness. MRI was then performed at 4 days after the onset, which showed an acute infarction in the left cerebellar hemisphere (Fig. ). The left VA was poorly visualized on MRA as compared to the right VA (Fig. ). In addition, the external diameter of the left VA on basiparallel anatomic scanning (BPAS) was narrowed, suggesting hypoplasia. The proximal portion of the left PICA was not visualized, and the adjacent distal portion appeared to be dilated on MRA (Fig. ). T1-VISTA showed a high-intensity signal area at the dilated portion (Fig. ). Conventional T1 image was obtained on admission and at 24 days after admission, but no high-intensity area was observed at both time points. Cardioembolic causes were excluded by echocardiography and 24-hour electrocardiography. According to these findings, Ischemic stroke of the cerebellum caused by dissection of the left PICA was diagnosed, and the administration of unfractionated heparin was started. Three days later, the dilated portion was enlarged on MRA, therefore, we discontinued unfractionated heparin administration. Since her blood pressure was not high, we even didn? use antihypertensive treatment. At 8 days after the onset, cerebral angiography demonstrated the ?earl and string sign??at the proximal portion of the left PICA (Fig. ). Dilatation gradually normalized on MRA and the high-intensity signal area on T1-VISTA gradually reduced and disappeared at 4 months after the onset (Fig. ).
Omari Duke	20	1983/8/23	(969)588-1021	[email protected]	69222 Kristen Divide Apt. 784	A 26-year-old Polish male was diagnosed with GD at the age of 3 years. His past medical history was significant for splenomegaly, identified at the age of 12 months, and severe pancytopenia. Gaucher cells were not found in aspiration biopsies of the bone marrow. He was splenectomized at the age of 3 years due to massive splenomegaly, and the diagnosis of GD was established by the presence of low activity of glucocerebrosidase in peripheral blood leukocytes. Further direct DNA sequencing revealed the homozygous mutation c.1448T > C in the GBA1 gene (i.e. mutated alleles L444P/L444P), which suggested GD3. At nearly 6 years of age, he developed avascular necrosis of the left femoral head. The patient started intravenous ERT with macrophage-targeted recombinant glucocerebrosidase at the age of 8 years, immediately after ERT became available in Poland. At the age of 20, his ERT was discontinued for 4 months during the unintentional world-wide imiglucerase (Cerezyme簧, Genzyme Corporation, Cambridge, MA, USA) supply shortage (). This was followed by a rapid increase in plasma chitotriosidase activity (), pathological bilateral forearm fractures and fractures in the left brachial bone and both fibulae. The fractures healed slowly, caused chronic pain, and created difficulties in walking for the patient. Currently, the patient is receiving infusions of imiglucerase at a dose of 56 units/kg of body weight, administered every other week. The patient had annual ophthalmologic follow-up examinations from diagnosis. His eye fundus examinations were normal until lesions were first noted at the age of 18 (i.e. 15 years from his diagnosis of GD and 10 years from the start of ERT). The fundus lesions were described as white spots located peripherally and in the posterior pole. In January 2012, when the patient was 23 years old, his eye changes were documented in photographs ( and ). At that time, the lesions were located on the surface of the retina and in the preretinal part of the vitreous body. OCT confirmed the presence of discrete lesions located preretinally, intraretinally in the nerve fiber layer, and in the vitreous body (). Electroretinography (ERG) did not show any pathological pattern, thereby indicating normal retinal function. A recent ophthalmologic examination (performed in December 2014) showed a best-corrected visual acuity of 20/20 bilaterally and an intraocular pressure of 19 mmHg (normal values: 11??1 mmHg). Ocular motility examination demonstrated limitations in abduction of both eyes. Dilated fundoscopy revealed optic discs with well-defined borders and pink color in both eyes. The maculae were normal. Further examinations showed yellowish-white punctuate deposits in the posterior pole and the periphery of the fundus bilaterally. A 3-year follow-up OCT examination was performed in January 2015, and no signs of progression of the fundus lesions were evident.
Melani Wise	34	1987/10/11	328.357.3936x628	[email protected]	318 John Manor	The patient was a 10-month-old Chinese female who was first diagnosed with HLH when she showed various symptoms, such as persistent high fever of unknown origin, seizures, jaundice, pancytopenia, coagulopathy, and hepatosplenomegaly. Physical examination also revealed lethargy, pallor, cervical lymph node swelling, and hepatosplenomegaly. Laboratory evaluations showed Hg b at 9.7 g/dl, H ct at 19.7 %, platelet at 60 K/弮L, WBC at 2.67 K/弮L, ferritin at 622 ng/mL, triglyceride at 532 mg/dl, ALT at 4068 U/L, AST at 9162 U/L, ALP at 1016 U/L, lactate dehydrogenase (LDH) at 12,108 U/L, total bilirubin at 100.9 umol/L, direct bilirubin at 51.5 umol/L, fibrinogen at 390 mg/dl, PT/PTT at 20.5/65.4 s, thrombin time of 46.7 s, and D-dimers at 851 ng/L. In summary, the patient fulfilled 5 out of 8 HLH-2004 criteria []. The cerebrospinal fluid examination showed protein at 3.5 g/L and pleocytosis at 20 ? 109/L. A magnetic resonance imaging (MRI) of the brain revealed no abnormality. Polymerase chain reaction (PCR) analysis for the EBV EBNA-1 was positive in peripheral blood. The EBV-DNA in blood was 6.25 ? 104 IU/mL. Bone marrow examination showed increased hemophagocytic histiocytes (HS) (Fig. ), which is consistent with HLH. Cytogenetic analysis was normal. A hereditary defect predisposing to HLH was not excluded. However, the long recurrence-free period argues against an hereditary background. The patient received chemotherapy according to the HLH-2004 protocol, which includes etoposide, steroids, intrathecal methotrexate, and cyclosporin. The patient achieved complete remission of the disease at the end of the initial treatment period of 16 weeks. The patient did not achieved full remission of the disease until the end of the initial treatment period of 8 weeks. Treatment was thus continued until week 16 and was then stopped asl the parents declined further therapy. In total, the patient? cumulative dose of etoposide was 2100 mg/m2 during the 16-week period. After 23 months, the patient was readmitted with fever, leukocytosis, anemia, diffuse lymphadenopathy, and hepato-splenomegaly of 5 and 4 cm, which is palpable below the costal margins. LDH was elevated at 1788 U/L. Bone marrow examination revealed 89 % monoblasts, without evidence of HS (Fig. ). Flow cytometry studies indicated that 86.4 % of leukocytes were positive for CD33, CD7, CD11b, CD13, CD15, CD34, CD38, CD64, CD117, myeloperoxidase (MPO), and cytoplasmic CD68. Chromosome analysis of 48 h culture from bone marrow aspiration revealed complex karyotype aberrations. The karyotype was interpreted as 46, XX, d e r (11) t (11; 19) (q23; p13) inv (11) (q23 p15), and d e r (19) t (11; 19). The fusion gene was MLL/ELL. The diagnosis of AML (M5b) was made,. Induction chemotherapy with idarubicin and Ara-C (DA) was given, and the patient achieved complete remission in bone marrow morphology after two courses. The patient is currently on chemotherapy for AML, and her bone marrow revealed no residual or recurrent leukemia cell after the initial complete remission.
Frederick Macias	45	2003/5/12	650.469.4086x37873	[email protected]	0525 Robert Expressway	A 15-year-old girl presented to her primary care provider with episodes of altered behavior. She reported a 30-minute period of fatigue, confusion, poor concentration, irritability, and ?taring off??which was noted by parents while the patient was getting ready for school. The patient herself did not recall the event. Her parents had witnessed several similar events lasting up to 30 min, at least once per week, in the morning, for the past 5 months. Their daughter? symptoms would resolve after eating breakfast. They had not sought medical care previously, assuming her behavior was related to early morning drowsiness. When the frequency of these episodes increased to several times per week, they presented to their primary care physician. Following her primary care visit, the patient was scheduled for an outpatient EEG after BMP, thyroid studies, and UDS which were normal. The patient became unresponsive during the EEG study and was referred to the local hospital with the following laboratory data when she was symptomatic: point-of-care glucose 36 mg/dL (65??10), insulin 36.8 uIU/mL (2.6??4.9), C-peptide 4.6 ng/mL (1.1??.4), and betahydroxybutyrate 0.6 mg/dL (0.2??.8). Her mental status immediately improved with intravenous dextrose. In search of an insulin-producing mass, she underwent abdominal CT with contrast which was negative per the local radiologist? report. Her EEG was interpreted as abnormal prompting the initiation of valproic acid therapy for potential seizure activity. She was discharged home from the emergency room with a glucometer and planned neurology follow-up. An outpatient endoscopic ultrasound was pursued as another attempt to localize any potential insulin producing mass, and this too was unremarkable. She was subsequently referred for subspecialty consultation with a pediatric endocrinologist. Prior to her scheduled endocrinology appointment, she was admitted to a tertiary care pediatric center due to near daily episodes of hypoglycemia associated with altered mental status. The patient became hypoglycemic overnight while fasting in less than 6 h (Fig. ). A critical sample was obtained and confirmed hyperinsulinemic hypoglycemia (Table ). Testing for sulfonylurea ingestion was performed and the result was negative. An MRI of the abdomen revealed a 0.9 ? 1.1 ? 1.3 cm pancreatic mass (Fig. ). Valproic acid was stopped and her hypoglycemia medically managed successfully with diazoxide 150 mg orally 3 times per day (8 mg/kg/day) and two tablespoons of cornstarch at bedtime. She ultimately underwent laparoscopic distal pancreatectomy with pathologic examination of the mass confirming a benign insulinoma (Fig. ). She had mild hyperglycemia post-operatively which resolved within 48 h without insulin therapy and was discharged home on post-operative day three. Gene sequencing by using DNA from a peripheral blood sample for multiple endocrine neoplasia was negative.. The patient remained euglycemic, asymptomatic, and lost 15 kg in the 10 months following tumor resection which dropped her BMI from the 87th to 45th percentile. A follow up EEG was performed and was completely normal. She is now considered cured. The time from onset of symptoms to correct diagnosis, tumor localization, and surgical management was approximately 8 months.
Adley Simmons	23	1991/3/12	001-956-696-6630x03462	[email protected]	770 Ashlee Parkway	A 30-year old male truck driver presented in our center with a 10-day history of abdominal crash injury followed by secondary lower back pain, lower extremity pitting edema, scrotal edema, dyspnea and a temperature of 38簞C. On examination, he was hemodynamically stable and his abdomen was soft with mild right upper quadrant abdominal tenderness. Hematological analysis confirmed no anemia and the lactate dehydrogenase level was normal and the C-reactive protein was markedly elevated at 190 mg/L. All other hematological, coagulation, thrombophilic tests and biochemical analyses were normal. Bilateral lower limb arterial examination was normal. Plain X-rays of the chest, abdomen and lumbar spine were normal. Laboratory results also showed urea (23 mg/dl), creatinine (1.2 mg/dl), sodium (136 mmol/L), and potassium (3.6 mmol/L) that were normal. Urinalysis, using a urine dipstick, revealed three pluses of proteinuria, which equates to ??g urinary protein per day. Lab examination for the measurement of serum albumin revealed hypoalbuminemia. Doppler ultrasound of the femoral veins demonstrated marked expansion of both vessels without intra-luminal thrombus. A trans-thoracic echocardiography revealed a free floating mass in the IVC entrance extended to the right atrium ( and ). Poor view abdominal sonography revealed a probable clot in the supra hepatic IVC but the IVC and bilateral iliac veins were distended and no tumor was found in liver or retroperitoneal spaces. The patient was scheduled for emergency open heart surgery. Median sternotomy and vertical pericardiotomy were performed. Cardiopulmonary bypass (CPB) was established with aortocaval cannulation. The pulmonary artery was dissected and clamped. After cooling to 20簞C, cardioplegic arrest was achieved with cold blood cardioplegia. Total circulatory arrest was established. The right atrium (RA) was opened parallel to the atrio-ventricular groove. The thrombus was extracted. The IVC was thoroughly irrigated with normal saline. The circulation was temporarily restarted to flush out any small remnant of the thrombus in the IVC. The venous cannula was advanced into the SVC and the SVC was snuggled. The main pulmonary artery was opened and the left and right pulmonary artery suctioned and a large clot was found in the left pulmonary artery. Circulation was restarted and the RA was closed. CPB was weaned off after re-warming. The perioperative course was uneventful. The CPB and aortic cross-clamp times were 55 and 21 minutes, respectively. Total circulatory arrest time was 5 minutes. With continuation of lower extremity edema and signs of nephrotic syndrome, an IVC venogram via the femoral vein in the post-operative period demonstrated patency of the IVC inferior to the right atrium and patent renal veins and hepatic veins ( and ). Anticoagulation with warfarin was started on the day following surgery and continued for 6 weeks. At the four month follow up, there was no evidence of recurrent thrombosis in the hepatic vein, IVC or RA or proteinuria. It was felt on balance that treatment should be directed towards the thrombus. His bilateral lower limb pain and edema resolved at an early stage and the patient remained well two months later with regular vascular and hematological clinical review.
Harrison Maynard	30	1982/4/13	536-296-8672	[email protected]	57523 Christina Views Suite 377	A 59-year-old woman with follicular lymphoma was initially treated with six cycles of chemotherapy and prednisolone. After 2 months, the patient had received a total of 1350 mg of prednisolone and was on her fourth cycle of chemotherapy. She fell from bed and sustained a blow to her left shoulder. She complained of pain in her left shoulder and was examined on the same day. The shoulder was swollen with tenderness over the ACJ. Radiographs showed an ACJ dislocation with a distal clavicle fracture and CP deformity (). Three-dimensional computed tomography (3D-CT) reconstruction clearly showed a small CP bone fragment at the CCL insertion site (). We diagnosed the patient with a CP avulsion fracture. She was treated nonoperatively in a sling for 3 weeks, followed by progressive range-of-motion exercises. One month after her injury, the patient's shoulder functioned normally without pain.
Carolyn Brock	25	2004/10/24	(981)337-4675	[email protected]	43876 Mcdowell Ports Suite 351	A 28-year-old lady, gravida 3 para 2 live 2 with previous one LSCS, married for 8 years arrived in gynecological emergency of the institute with two months of amenorrhea, excruciating pain abdomen ?1 day, and 5-6 episodes of vomiting ?1 day. She had not been using any contraception. Her previous menstrual cycles were regular of thirty days with bleeding period of 3 days. Upon missing her periods and a positive UPT at home, she took MTP pill as advised by a local doctor for unwanted pregnancy. However in event of RPOCs, she underwent a dilatation and curettage at a private hospital in the vicinity of her residence. Following the same, pain abdomen and spotting per vaginum still persisted. When abdominal pain became unbearable she was brought to hospital. Her past medical and family history were not significant. Her general condition on admission was guarded with maternal tachycardia (pulse 110 per minute), hypotension (BP = 70/40 mmHg), RR = 20/min, and pallor. Her abdomen was tender on palpation with positive rebound tenderness and guarding and absent bowel sounds. Pelvic bimanual examination revealed a bulky uterus with cervical excitation and bilateral adnexal fullness and tenderness that was more marked in the right fornix. A transvaginal ultrasound scan showed an empty uterus with thickened endometrium and a right adnexal mass that was suggestive of a right tubal pregnancy with a moderate amount of free fluid in the pouch of Douglas. Culdocentesis done was positive. Her investigations revealed Hb 7.6 gm%, TLC 12,200, DLC N88 L12, platelets 3.46 Lac, PT, PTTK normal, blood urea 24, creatinine 0.4, Na 131, and K 3.9. Ultrasound revealed ruptured left tubal pregnancy with significant free fluid in the abdomen and pelvis. There was no obvious ultrasonic evidence of right tubal disease. She was resuscitated and taken up in emergency for exploratory laparotomy and proceed in view of deteriorating vitals, after written informed consent. Consent for tubal ligation was also taken because she was multiparous. Preoperatively 2 litres of hemoperitoneum was drained. There was a ruptured left sided ampullary ectopic pregnancy ~6 ? 3 cm; right tube revealed ?? ? 3 cm organized hematoma seen attached to fimbrial end. Uterus and bilateral ovaries are normal (). A bilateral salpingectomy was hence performed. She was transfused three units of packed red blood cells intraoperatively. She had an unremarkable postoperative recovery and was discharged on the third postoperative day. Histopathological examination showing chorionic villi and trophoblasts in both tubes confirmed the diagnosis of bilateral tubal ectopicpregnancy with evidence of rupture on one side (Figures and ).
Julio Logan	38	1986/5/7	402.482.3173x706	[email protected]	605 Carr Glens	A 49-year-old Chinese female was admitted to the hospital with gait disturbance, dysarthria, and involuntary movement of limbs for 10 days, with a history of edema in both lower extremities for 10 years, hypertension for almost 8 years, and diabetes mellitus for 2 years. Seven years ago when her serum creatinine level reached 2300 弮mol/L, she began taking regular hemodialysis treatments twice a week and then three years later this increased to three times a week due to her restless legs syndrome (RLS) secondary to chronic renal failure. Besides, she had polycystic kidney disease and hyperparathyroidism. There was no previous history of movement disorders and family history of this kind. Clinical examination revealed her highest blood pressure to be 200/100 mmHg. Generalized chorea was noted mainly on the extremities. Laboratory results showed elevated concentrations of blood urea nitrogen (BUN) of 33.6 mg/dL and creatinine (Cr) of 5.4 mg/dL, serum potassium of 6.1 mmol/L, and parathyroid hormone of 1457 pg/mL. Axial brain MRI revealed symmetric edema in the bilateral basal ganglia, which exhibited hypointensity on T1-weighted images () and hyperintensity on both T2-weighted images () and fluid-attenuated inversion recovery (FLAIR) images (). Diffusion-weighted imaging (DWI) demonstrated slightly higher inhomogeneous signals in the involved regions (). Concurrently, increased apparent diffusion coefficient (ADC) values were demonstrated in the periphery compared with normal brain tissue (), indicating vasogenic edema rather than cytotoxic edema. Follow-up MRI of brain was performed 3 weeks later, which showed lesions worsened and extended in both T1WI () and T2WI (). Simultaneously, MR imaging revealed demyelination around bilateral ventricles and lacunar infarctions in FLAIR (). MR angiography showed atherosclerotic alterations of small brain vessels. Proton (1H) MR spectroscopy (MRS) (Figures and ) with region of interest pinpointed on the left basal ganglia lesion displayed a decrease of N-acetylaspartate (NAA) peak and lactate (Lac) doublet. 3D-ASL results () reflected the local perfusion condition, and the increased regional cerebral blood flow (rCBF) suggested hyperperfusion in the corresponding lesion area. Some dotted low signals were noted in the susceptibility weighted imaging (SWI) findings (). After two months, we were surprised to find that the abnormal signals of the lesions totally disappeared when we scanned her again, and this demonstrated that the acute bilateral basal ganglia lesions are reversible. The reports have been approved by the Institutional Review Board with patient informed consent.
Kora Fuller	19	1982/6/16	001-939-983-8753	[email protected]	105 Shelley Square	A 20-year-old right-handed woman presented with pain on her left elbow radiating along the radial side of the forearm since the age of 14. At that time, our young patient had undergone surgical decompression of ulnar nerve at the cubital tunnel for the first time due to ulnar neuritis confirmed by nerve conduction studies. One year later, medial epicondylectomy was performed additionally. Because of poor results, nerve conduction studies were repeated and concluded that there was no ulnar nerve lesion. The young patient presented at our department four years later and she was methodically examined to investigate her chronic pain. At first, a thorough history was taken. Apart from two surgical decompression procedures of the ulnar nerve, she reported no previous elbow trauma and no previous medical history. Physical examination was carried out and revealed full range of motion of the elbow. She had negative Tinel's sign and Phalen's test and no tenderness at the epicondyles. Since there were no significant clinical findings; a radiographic examination of her left elbow was undertaken. Radiographs showed an area of dense mineralization along with surrounding reactive sclerosis at the bicipital tuberosity of the radius (). To confirm the diagnosis, further imaging with a three-phase bone scan () and an MRI control of the region was made. The bone scan showed a high tracer uptake during all phases in the proximal end of the radius. The MRI scan revealed an intraosseous lesion that had no expansion at the surrounding tissues. Surgical removal of the tumor was decided. The lesion was excised en bloc, under general anesthesia and tourniquet application. We used demineralized bone matrix graft to fill in the gap of the radius. There was no need to transpose the bicipital insertion in the radius. Postoperatively, no evidence of elbow instability was observed. The diagnosis of the osteoid osteoma was confirmed histologically. Since then, our patient has been pain-free and reports no need for painkillers. There was no need for physiotherapy to obtain full motion of the elbow. The normal follow-up visits for the patient were one after 6 weeks and a second after one year. Eight years after the operation, the patient visited again our outpatient department, had a full range of motion, and reported not consuming any painkillers for her elbow. She was fully satisfied by the operation and the final outcome ().
Andre Vance	44	1992/4/20	+1-879-637-7264x005	[email protected]	97819 Rios Glens	A 62-year-old otherwise healthy Caucasian male complained of some minor nonspecific respiratory symptoms for a few days. These complaints evolved into a more worrisome pulmonary disease characterized by cough and progressive respiratory distress 2 days prior to the hospitalization. Chest CT scan on admission to an outside hospital showed diffuse bilateral alveolar and interstitial lung infiltrates. A diagnosis of atypical pneumonia and possible myocarditis was made and the patient was given antibiotic treatment and was discharged two weeks later. However, dyspnea persisted and he was admitted to our hospital for further workup. On admission, patient was found to have fever, acute hypoxic respiratory failure, pleural effusion, pericardial effusion, and irregular heartbeats with bouts of ventricular fibrillation. Upon hospitalization, additional diagnoses were made including acute renal failure, as well as possible liver disease marked by an elevation of liver function tests. A poorly defined pancreatic mass was noted on the CT scan. Head CT without contrast was performed which showed minimal mucosal thickening in the left maxillary sinus. There was opacification of several left ethmoid air cells with fluid levels in the left sphenoid and left maxillary sinuses. A nasoenteric tube was in place in nasal cavity. There was no mass lesion, erosion, or bone destruction. These finding were most consistent with mild inflammatory changes (). Sepsis was suspected and he was given antibiotics without improvement of his clinical condition. His condition rapidly deteriorated with progressive dyspnea and pulmonary infiltrates which by X-ray examination involved all five lobes (). Extensive microbiological studies were performed with no positive bacteriologic, viral, or fungal isolates. The only positive test was a mildly increased DNA load of Epstein-Barr virus (EBV) in blood with 0.5 copies per microliter detected by a quantitative PCR assay. EBV capsid IgG and nuclear antigen/antibody were positive. Peripheral blood smear showed mild normocytic anemia with moderate thrombocytopenia. White cells count was within the normal range with absolute lymphopenia and no atypical lymphocytes or lymphoma cells. As his condition deteriorated, he developed signs of cardiac tamponade. Pericardial effusion was drained and sent to the pathology laboratory. He developed uncontrollable ventricular fibrillation with subsequent cardiac arrest, which could not be reversed by resuscitation. At the same time, patient hemoglobin and platelet count decreased to 6.9 mg/dL and 22,000/弮L. His fibrinogen was decreased (178 mg/dL), ferritin was markedly elevated (1763 ng/Ml), and lactate dehydrogenase was elevated (628 U/L). Neutrophil count and triglyceride were within normal range. Hemophagocytic lymphohistiocytosis (HLH) was suspected. However, the final diagnosis of HLH was never made since the patient's condition deteriorated quickly and he died in a cardiopulmonary failure. The pericardial fluid sent for cytological analysis one day before his death contained numerous markedly atypical lymphoid cells () which were positive for Epstein-Barr virus (EBV) as detected by in situ hybridization (). Immunohistochemical stains performed on the cell block showed that the atypical cells were positive for CD45, CD2, and CD3 () and negative for CD4, CD8, CD56 (), CD138, pan-cytokeratin, MOC-31, Ber-EP4, chromogranin, synaptophysin, calretinin, S-100, and HHV8. Ki-67 (MIB-1) showed an increased proliferation index of approximately 20% reflecting the number of EBV-positive atypical cells. Flow cytometry confirmed that the lymphoma cells were CD2 positive. There was cytoplasmic CD3 positivity, but there was no surface CD3 expression. Bone marrow aspiration and core biopsy revealed a normocellular marrow for age (30??0% cellularity) with active trilineage hematopoiesis and left shifted granulopoiesis but no evidence of lymphoma. Cytogenetic analysis did not reveal any clonal chromosomal abnormalities. At autopsy, the decedent was found to have cardiomegaly (weight 650 grams) () with four-chamber dilatation and serous pericardial effusion (100 mL). Patchy whitish discoloration was found on the surface of epicardium and endocardium, and the myocardium had a mottled appearance on cross section (). The right and left lungs were heavy and weighed 1000 and 940 grams, respectively. The pleural surfaces were smooth, glistening, and unremarkable and the cut surface revealed patchy consolidation of pulmonary parenchyma with red and grey discoloration. There was 500 mL of straw colored pleural fluid in each of the pleural cavities. The pancreas was enlarged and the cut surface showed scattered areas of fat necrosis with hemorrhage. There was 500 mL of opaque fluid present in the abdominal cavity. The spleen was enlarged, weighed 330 grams, and had a smooth intact capsule covering red-purple semiautolytic parenchyma. The splenic white pulp was grossly unremarkable. Other organs were grossly unremarkable. Under microscopic examination, the myocardial tissue was found to be diffusely infiltrated by atypical lymphoid cells (). These cells had abundant pale cytoplasm, irregular nuclear contours, granular chromatin, and inconspicuous nucleoli (). In situ hybridization performed on the cardiac tissue showed that the atypical lymphoid cells were strongly positive for EBV. Immunohistochemical stains performed on the same tissue showed that the lymphoid cells were positive for CD3 (cytoplasmic stain) () and granzyme B () and negative for CD5, CD10, CD20, CD30, CD34, CD43, and CD56. Cells infiltrating the lung parenchyma in all five lobes (Figures and ) had the same immunohistochemical features. Diffuse infiltrates of identical neoplastic cells were found in the pancreatic () and gastric tissue ().
Maxine Valentine	28	1981/7/19	551-579-4215	[email protected]	4895 Heather Keys	Month 0: A 44 year-old man, 12 years of education, started complaining acutely of slowness and sustained attention difficulties. He had neither clinical comorbidities nor relevant familial history. Within three weeks after the first complaints, his status rapidly worsened and the patient needed help for every daily life activities. He was hospitalized, and presented significant cognitive impairment and walking difficulties related to marked parkinsonian syndrome. Depression with suicidal ideation was reported. No cerebellar features were observed. Neuropsychological assessment showed marked cognitive slowness and impairment of anterograde memory, mental calculation, verbal fluency, praxies and mental flexibility (Table ). Electroencephalogram (EEG) showed slow cerebral activity without any epileptic or pseudo periodic abnormalities. Tianeptine and ropinirole treatments were introduced.Month 2: His condition worsened, and he was hospitalized in the Department of Neurology, in University Hospital, Toulouse, France. In the absence of sustained stimulation, the patient remained motionless and speechless, due to severe apathy. Falls occurred frequently (about once a day), due to altered postural reflexes. After marked stimulation, it was noticed that the patient was depressed but not melancholic or suicidal. Marked symmetric and axial parkinsonian syndrome did not improve after L-dopa challenge. Slight reflex myoclonus was noticed in the upper limbs. Ocular saccades were slow but not limited. Apraxia and executive dysfunctions had worsened. Detection of 14???? protein performed from cerebrospinal fluid (CSF) sample by western-blot was positive, whereas cytology and biochemistry were normal. Structural cerebral MRI revealed fronto-temporal atrophy on visual assessment of T1 sequence, with no abnormality on diffusion weighted imaging (DWI). Ropinirole, and tianeptine treatments were replaced by citalopram, oxazepam and zopliclone. Month 6: Daily risperidone treatment was introduced due to delusions of persecution and rare visual hallucinations. Month 7: Parkinsonism did not deteriorate with the addition of neuroleptic agents. Delusions and hallucinations remained present, although less prominent, and cognitive functions kept worsening. Structural MRI was unchanged, and 18F-FDG-PET visual assessment showed severe hypometabolism in parietal regions. Month 10: The patient only experienced few hallucinations without delusions. Risperidone treatment was stopped but parkinsonian symptoms had worsened: he had severe dysarthria and was not able to walk without assistance because of his instability. The patient had to be institutionalized 12 months after symptoms onset.Month 20: Axial parkinsonian symptoms had worsened: he had difficulties swallowing, severe dysarthria, freezing in narrow place and total loss of postural reflexes. He was bed-ridden most of the day. CSF 14???? protein remained positive, while total-tau (238 pg/mL) and A帣42 (511 pg/ml) levels were within the normal ranges (norm < 450 pg/mL and > 500 pg/mL, respectively). MRI showed moderate striatal, brainstem, and bilateral fronto-temporo-parietal atrophy on visual assessment.Month 43: Parkinsonian symptoms were very severe, and myoclonus more pronounced. Important perseverations, imitations, and grasping were reported. Speech was unintelligible. Detection of 14???? protein in CSF was negative. The patient died one year later, aged 49, after a total duration of 55 months. Cerebral histopathological examination was performed. Grey matter density was assessed in our patient from his first T1 MRI sequence (Fig. ), and compared to the grey matter density of a group of 30 age-matched healthy controls (age = 44.9 簣 3.8) from the online database OASIS (), using a whole brain z-score map. The 18F-FDG-PET scan from our patient (Fig. ) was also compared to another group of 23 age-matched healthy controls (age = 44.3 簣 9.4) using z-scores map (for details on the control population and imaging processing procedures, see Additional file ). All z-scores below ?? or above 2 were considered as significant. The patient showed global bilateral decrease of grey matter density affecting all cortical regions as well as subcortical structures and cerebellum (Fig. and Additional file ). Negative z-scores below ?? were found in parietal, temporal, but also frontal and insular cortices. Left hippocampus and bilateral thalami were spared. No z-score > 2 was found. Regarding 18F-FDG-PET, the patient showed widespread significant bilateral hypometabolism, encompassing all cerebral lobes, most predominant in the parietal lobe (Fig. and Additional file ). Negative z-scores below ?? were found in parietal and medial temporal regions. No difference compared to controls was found in the thalamus, orbitofrontal regions, or the posterior cingulate. Hypermetabolism was found in the putamen. Preliminary examination was carried out on hematoxylin-eosin stained paraffin sections, completed by immunohistochemistry with the following antibodies: anti-tau AT8 (Thermo Fisher Scientific, Illkirch, France), anti-amyloid-beta A4 (Dako, Trappes, France), anti-alpha-synuclein (Leica Biosystems, Newcastle, UK), and anti-prion protein 12 F10 (Spibio, Montigny Le Bretonneux, France). Macroscopic brain examination showed a marked palor of the substantia nigra, while anti-alpha-synuclein immunohistochemistry revealed numerous Lewy bodies and Lewy neurites in the frontal and temporal cortices, hippocampus, substantia nigra, locus c?ruleus, as well as in the dorsal motor nucleus of the vagus, occipital and entorhinal cortices, and more rarely in the thalamus, caudate nucleus, putamen, and pallidum (Fig. ). Spongiosis was absent and anti-prion protein immunohistochemistry was negative. A few AT8-positive neurofibrillary tangles were noticed in the hippocampus and the entorhinal cortex, whereas anti-amyloid-beta 4 staining revealed neither neocortical deposits nor amyloid angiopathy. SNCA gene (encoding the alpha-synuclein) mutations were investigated using bidirectional Sanger sequencing on an ABI 3730 automated sequencer (Applied Biosystem) and SeqScape v2.6 software (Applied Biosystems), and multiplex ligation-dependent probe amplification (MLPA, MRC-Holland). PRNP gene (encoding the prion protein) mutations were investigated using a previously described protocol []. Neither missense mutations nor multiplications were found in the SNCA gene (alpha-synuclein), and no mutation was identified in the PRNP gene (prion protein) either. The polymorphism at codon 129 was methionine-methionine.
Demetrius Marsh	30	2003/8/9	639-486-7774x047	[email protected]	0378 Hill Circle Apt. 815	The proband is a 17-year-old white female with agenesis of the uterus and vagina who had a peripheral blood karyotype revealing a de novo balanced translocation 46,XX,t(3;16)(p22.3;p13.3). She has no associated renal, skeletal, or hearing anomalies. She has two unaffected sisters and two brothers (Fig. ). Both parents and her unaffected sister II-5 have normal karyotypes, and all three nieces (III-1, III-2, and III-3) have no known mullerian, renal, or skeletal defects.
Adelina Espinoza	35	1979/6/28	(355)443-5855	[email protected]	7303 Carol Junctions Suite 671	A 31-year-old Caucasian woman was diagnosed with lupus in 2011. She initially presented with arthritis, serositis (pleuritis), autoimmune hemolytic anemia, idiopathic thrombocytopenia (ITP), positive antinuclear antibody (ANA), Smith and RNP antibodies, and low complement levels. She also has a history of autoimmune hepatitis and diabetes mellitus type 1. She was initially treated with intravenous immunoglobulin (IVIG), rituximab, and splenectomy. Her disease was relatively stable on hydroxychloroquine 200 mg BID and mycophenolate mofetil (MMF) 500 mg twice a day. In June 2013, she presented with complaints of dyspnea, lower extremity swelling, fevers, anorexia, and jaundice. Initial workup revealed worsening of chronic anemia with hemoglobin of 5.2 g/dL (her baseline, 8??0 g/dL, and normal, 11.2??5.7 g/dL), hyperbilirubinemia with total bilirubin of 9.2 mg/dL (normal, 0.2??.2 mg/dL), direct bilirubin of 6.4 mg/dL (normal, 0.0??.3 mg/dL), alkaline phosphatase of 250 弮/L (normal, 35??04 弮/L), normal ALT and AST, and a right-sided pleural effusion on chest X-ray. She was treated for a flare of autoimmune hemolytic anemia with IVIG, blood transfusions, intravenous pulse doses of methylprednisolone, empiric treatment with broad spectrum antibiotics for pleural effusion, and continuation of the home dose of hydroxychloroquine 200 mg daily and MMF 500 mg twice a day. Antibiotics were stopped after pleural fluid and blood cultures were found to be negative. Liver biopsy showed iron overload in hepatocytes and no significant inflammation or fibrosis. Her creatinine increased from a baseline of 0.8 to 1.8 mg/dL and peaked at 2.81 mg/dL (normal, 0.5??.10 mg/dL) on the fifth day of hospitalization. Urinalysis showed large protein and blood, and urine microscopy showed many red blood cells (RBCs) with few dysmorphic RBCs and many granular casts, fatty casts, and white blood cell casts but no RBC casts. Urine random protein/creatinine ratio was elevated at 2.49 (normal < 0.15). Renal biopsy () showed glomeruli with cellular and fibrocellular crescents, focal segmental necrosis, and mild interstitial and tubular injury. IF showed only trace C3, IgM, and fibrinogen deposits. Electron microscopy (EM) showed endothelial and podocyte injury with glomerular basement membrane (GBM) remodeling; no electron dense immune deposits were seen. The findings were consistent with pauci-immune crescentic glomerulonephritis with focal acute activity and mild chronic changes. Additional laboratory testing revealed a positive ANA with a titer of 1 : 640 (homogenous pattern), elevated anti-Smith antibody of >8.0 (normal, 0.0??.9 AI), RNP antibody of 7.2 (normal, 0.0??.9 AI), p-ANCA at >1 : 640 (normal, <1 : 20), anti-myeloperoxidase (MPO) antibodies at 86.0 U/mL (normal, 0.0??.0 U/mL), anticardiolipin IgM at 27 U/mL (normal, 0??2 U/mL), and positive lupus anticoagulant. She had a low C3 level of 56 mg/dL (normal, 90??80 mg/dL) and normal C4 level. Anti-double stranded DNA antibodies (dsDNA), c-ANCA, anti-proteinase 3 (PR-3) antibodies, and beta-2 glycoprotein antibodies were negative. Based on biopsy results, MMF was switched to cyclophosphamide 175 milligrams daily orally. Creatinine returned to normal and her anemia improved. She was tapered off of cyclophosphamide and switched to azathioprine for maintenance therapy.
Dallas Monroe	41	2003/12/29	(791)866-1612x6224	[email protected]	18958 Stephen Harbor	A 58-year-old African American woman was diagnosed with mixed connective tissue disease (MCTD) in 2000. She initially presented with Raynaud's phenomenon, myalgia, synovitis, esophageal dysmotility, and positive anti-RNP antibody. She also had interstitial lung disease with a nonspecific interstitial pneumonitis (NSIP) pattern and was treated with cyclophosphamide. She has no sclerodactyly or skin findings suggestive of scleroderma. She was hospitalized at our institution in September 2009 for acute renal failure with a creatinine level of 5.52 mg/dL (normal, 0.5??.10 mg/dL); her baseline was 1.12 mg/dL. Other than increased nocturia she had no other symptoms. Urinalysis showed 2+ blood, 2+ protein, and RBC casts. Urine random protein/creatinine ratio was elevated at 1.28 (normal < 0.15). Renal biopsy was performed for further evaluation. Renal biopsy () showed predominantly sclerotic glomeruli with occasional fibrous and fibrocellular crescents and focal segmental necrosis. Marked background tubular atrophy and interstitial fibrosis were also noted. IFE revealed weak, nonspecific reactivity for C3 and fibrinogen. Electron microscopy showed glomerular damage and rare electron dense C3 deposits. The findings were consistent with pauci-immune crescentic glomerulonephritis with focal acute activity and marked chronic changes. Additional laboratory workup revealed that ANA was high at 64 by EIA (normal, 0??0 U/mL). C3 and C4 were within normal limits. Anti-double stranded DNA antibody, anti-centromere antibody, anti-topoisomerase antibody, anti-JO 1 antibody, anti-ribosomal P protein antibody, anti-SSA/SSB antibody, anti-Smith antibody, anti-MPO antibody, anti-PR-3 antibody, c-ANCA, p-ANCA, and atypical ANCAs were negative. She received a pulse dose of IV methylprednisolone along with five monthly infusions of cyclophosphamide IV and was transitioned to MMF and low dose oral prednisone. Her renal function continued to worsen, and she was started on hemodialysis and underwent renal transplant in 2014. She remains on MMF, tacrolimus, and prednisone; she has had no recent flares of her MCTD. Her transplanted kidney continues to function well, as demonstrated by a stable creatinine of 1.11??.17 mg/dL.
Carly Castro	45	2001/4/17	5419485465	[email protected]	89817 Michael Mill Apt. 732	A 24-year-old African American female with past medical history of asthma and eczema presented to our emergency room with nausea, vomiting, myalgia, and fever in May 2015. Her mother has rheumatoid arthritis. Physical exam was unremarkable. Initial workup revealed creatinine of 4.25 mg/dL (normal, 0.5??.10 mg/dL) and WBC count of 20 10e3/弮L (normal, 4.0??0.0 10e3/弮L). Urinalysis showed 2+ blood, 2+ protein, and WBC and granular casts. Urine random protein/creatinine ratio was elevated at 1.90 (normal < 0.15). Ultrasound of the kidneys showed enlarged and edematous kidneys. Further workup revealed elevated ANA of >8 (normal < 0.9 AI), anti-dsDNA antibody at 32 (normal < 5 IU/mL), and anti-chromatin antibody of 7.3 (normal, 0.0??.9 AI). Anti-MPO, anti-PR-3, anti-Smith, anti-RNP, and anti-SSA/SSB antibodies, C-ANCA, P-ANCA, lupus anticoagulant, and anti-cardiolipin antibody were negative. C3 and C4 were within normal limits. Kidney biopsy was performed for further evaluation. Renal biopsy () showed cellular crescent formation, focal segmental necrosis, and mild interstitial fibrosis. IFE showed sparse positivity for C3 and strong reactivity for fibrinogen within necrotic foci. EM was unremarkable. The findings were consistent with pauci-immune crescentic glomerulonephritis with acute activity and mild chronic changes. She was treated with pulse dose IV methylprednisolone 1000 mg/day for 3 days followed by oral prednisone at 1 mg/kg/day. She received rituximab 375 mg/m2 every week for a total of 4 doses. Her kidney function improved and creatinine returned to normal. She is on rituximab infusions for maintenance therapy and continues to do well.
Jasper Torres	44	1978/12/6	-7155	[email protected]	683 Eddie Ridge Suite 323	A 56-year-old male Caucasian, 60-pack-year smoker with a past medical history of sleep apnea presented as an outpatient with gradual abdominal distention. During the last 2 months he reported painless swelling of the right testis. Physical examination revealed ascites and right scrotal hard mass with enlarged testis. Complete blood count and biochemistry were normal, while serum CA 125 was increased (319 弮/mL). In November of 2015 he was admitted to the Oncology Department for further investigation. Computed tomography of the thorax and abdomen revealed a minimal pleural effusion of the left hemithorax, diffuse peritoneal fluid in the abdomen, and peritoneal implants (). Since no solid literature data exist (apart from the sensitivity of PET/CT scan in hidden primaries mainly of head neck) no PET/CT scan was requested in our case. Upper and lower GI endoscopy revealed no abnormalities. Patient had a scrotal ultrasound imaging that revealed an enlarged right epididymis with small amount of fluid in the right side of the scrotum. Abdominal paracentesis revealed exudative fluid with neoplastic signet-ring cells indicative of metastatic adenocarcinoma. Gross evaluation of the tissue specimen revealed several poorly defined, whitish, and hard in consistency foci throughout the testicular parenchyma, the epididymis, and the spermatic cord. The tunicae surrounding the testis were thickened. Microscopical examination of multiple tissue sections taken from the grossly described foci showed the presence of a poorly differentiated carcinoma composed of signet-ring cells (). Perineural and neural invasion as well as vascular invasion were observed. By histochemical stains (PAS, Alcian Blue) a large amount of mucin was demonstrated in the cytoplasm of tumor cells (). Immunohistochemically, the neoplastic cells were diffusely positive for cytokeratin 20 and EMA, focally positive for cytokeratin 7, CEA, and c-kit (CD117), and negative for PLAP, a-fetoprotein, CD30, inhibin, calretinin, PSA, p504S (AMACR), TTF-1, and Melan A (). The pathological diagnosis was in favor of a metastatic adenocarcinoma, probably of gastrointestinal origin. Tissue HER2 was negative. Taking into consideration the aforementioned findings, the primary site could not be established and the case was classified as CUP. In November 2015 he started on systemic therapy consisting of Capecitabine and Oxaliplatin. Up till January 2015, he has received three cycles of the above regimen with good partial remission of his ascites and excellent drug toleration. However, just before the fourth cycle he developed right pleural effusion with accompanying moderate dyspnea (). Pleural fluid cytology was positive for metastatic adenocarcinoma with signet-ring cells. A second-line regimen consisting of Doxorubicin, Cyclophosphamide, and Fluorouracil (DCF) was administered. Up till now, the patient has received six cycles of DCF with complete response of the disease on abdominal and thoracic CT scans as well as normalisation of serum CA 125 (6 弮/mL).
Violet Curry	18	1992/6/1	415.508.5518x8914	[email protected]	310 Timothy Dam	A 53-year-old female presented to our outpatient clinic with rather acute chronic severe left-sided knee pain. The pain was not trauma related and had lasted for a few days. There were symptoms of locking. The knee joint has not been operated on before. Clinical findings confirmed a stable left knee joint with limited motion for full flexion as well as extension (110-5-0). Lachman, anterior, and posterior drawer were negative. The collateral ligaments were stable in full extension as well as in 30簞 of flexion. The patellofemoral alignment was normal and there was no apprehension sign. Clinical test for the menisci was negative (partly false positive for the anterior part of the medial meniscus). There was mild effusion and clear sharp pain at the medial femoral condyle. Conventional X-rays confirmed no significant pathology, no signs of advanced arthritis, and a straight mechanical axis (not shown). Subsequent magnetic resonance imaging (MRI) confirmed a large area of freshly appearing bone marrow edema (BME) at the dorsomedial femoral condyle with overlying highly irregular cartilage (). The remaining joint appeared normal on MRI. After discussing the case with the patient we indicated to approach with knee joint arthroscopy first in order to inspect the medial condyle and debride the lesion plus potential antegrade drilling for relieve of the BME. During arthroscopy there appeared a large just recently separated pure chondral fragment at the dorsomedial femoral condyle with healthy appearing surrounding and opposing cartilage (). The medial meniscus appeared intact. The remaining joint structures appeared intact. With regard to a recent separation and healthy appearing surroundings we decided to proceed with arthrotomy in order to repair the cartilage defect by mincing the healthy appearing cartilage piece. Following arthrotomy the large fragment could be retrieved easily. It was purely chondral. A refixation was deemed not promising. Consequently the large fragment with healthy appearing cartilage was minced into multiple small cartilage chips (<1 ? 1 ? 1 mm) using a scalpel at the back table. In parallel the defect was debrided to create a stable and healthy cartilage rim. The subchondral bone was intact. Defect dimensions after debridement were 2.5 ? 1.5 cm and ICRS grade 3b. Yet, with regard to the BME seen on MRI, we frequently drilled into the subchondral bone at different locations and in different angles using a constantly water cooled 1.4 K-wire in antegrade fashion. Hereafter, the autologous chips were placed into the debrided lesion and fixed using fibrin glue. The chips had more than enough quantity to cover the lesion. After dehydration the joint was put through multiple full range of motion procedures. The repair tissue remained in place. Subsequently, the joint was closed in layers. Rehabilitation was performed as previously reported []. Following an uneventful postoperative course the patient presented without pain or locking sensations at our outpatient department at 6 weeks, 12 weeks, and 6 months postoperatively. Albeit no full muscular function, swimming and biking were already possible at last follow-up. Six-month MRI was in display of almost full regression of the BME and satisfying novel cartilage surface with good integration into the surrounding cartilage and subchondral bone. The transplant signal appeared almost isointense to the neighbouring cartilage (). The calculated MOCART [] score was 85 points. Lysholm score was 80 points. The patient was subjectively very satisfied with the procedure and would undergo it every time again.
Briggs Fields	32	1998/2/27	879.218.6470	[email protected]	090 Brewer Drive Suite 360	An 80-year-old woman was admitted with reported fever that started 12 days before with no associated symptoms. According to her medical history, she was diagnosed with a myelodysplastic syndrome (MDS) 8 years ago, which was effectively managed with erythropoietin. Seven years earlier, she was diagnosed with ulcerative colitis, treated with mesalazine 800 mg twice daily. Finally, a tuberculosis (TB) infection was reported more than 40 years before. A former urine examination had revealed a urinary tract infection (urinary culture with the presence of E. coli > 105 CFU susceptible to almost all antibiotics). She received amoxicillin/clavulanic acid for a week and subsequently ciprofloxacin, one week prior to her presentation to the emergency department, but fever failed to settle. At presentation, her body temperature was 38.5簞C and she was hemodynamically stable. She reported no cough, dyspnea, abdominal pain, diarrhea, or dysuria. She had no clinical signs indicating a respiratory, urinary, or abdominal infection while a systolic aortic valve murmur was present. Her ECG revealed a 1st-degree AV block and her chest X-ray showed a cavity at the right upper lung field. Her tests revealed anemia (HCT = 23%, HGB = 7.2 mg/dL), a low WBC count, an elevated ESR (>100 mm/h), and CRP = 71.4 mg/L (0.0??.0 mg/L); thus, she received 1 RBC unit for treating anemia. Fever was initially considered as a symptom of a urinary tract infection, which did not respond to the antibiotic treatment per os, and therefore she was treated with intravenous meropenem. At the same time, the patient received multiple blood transfusions, due to the fact that she was not responding to erythropoietin. Since fever was persisting even after antibiotic treatment, simultaneously further investigation was deemed necessary. The patient had a CT scanning of the chest and the abdomen. The chest scanning revealed a cavity at the right upper lung field, which was consistent with the history of the old TB infection and the chest X-ray findings. The abdominal scan did not reveal any significant findings apart from the presence of edema at the rectum (). Subsequently, the patient had a colonoscopy, which revealed a thickened fold of rectum mucosa and diverticulosis of the sigmoid with redness of the surrounding mucosa. The patient was treated for 12 days with meropenem before the colonoscopy and diverticulosis was excluded as the cause of pyrexia since her fever did not settle. However, the coexistence of diverticulitis in remission could not be excluded. A few days later, the biopsies from colonoscopy revealed a mild chronic, unspecific inflammation of the sigmoid and the rectum. Awaiting the results of the biopsies, the patient had a gastroscopy, which only revealed a mild gastritis. The transthoracic cardiac ultrasound confirmed a mild aortic valve stenosis with no other findings indicating endocarditis; thus, we did not proceed to a transoesophageal echocardiogram (multiple blood cultures were all negative). A myelogramconfirmed the MDS, RARS type. Moreover, a full laboratory examination of the immune system was conducted (ANA, anti-dsDNA, anti-ENA, anti-RNP, anti-Ro, anti-La, anti-sm, C-ANCA, P-ANCA, anti-MPO, anti-PR3, and anti-CCP) as well as serum protein electrophoresis, right temporal artery biopsy, and investigation for possible relapse of the old TB infection (Mantoux, urine, gastric fluid, sputum and bone marrow Ziehl-Neelsen stain, and culture for 帣-Koch). Pending the results, the patient was discharged with prescription for paracetamol to treat the fever. A few days later, the patient was readmitted for a blood transfusion and she was still febrile. The results of the immunologic tests were inconclusive {ANA (??, anti-ENA (+), and anti-RNP (+)}; the serum protein electrophoresis and the temporal artery biopsy were negative and so were the tests for the reactivation of the old TB infection. The patient was evaluated by a rheumatologist who recommended treatment with 20 mg of prednisone daily. After 21 days, the patient remained febrile and thus underwent a gallium-67 scintigraphy searching for a hidden focus of inflammation. The gallium-67 scintigraphy revealed persistent diffuse concentration of the gallium in the ascending colon () at 24-hour imaging, in discordance with the colonoscopy (no signs of active inflammation at the ascending colon). The gallium-67 diffuse uptake of the ascending colon remained stable at the repeated 48- and 72-hour imaging without any topographic change after the use of laxatives (). Considering the results of gallium-67 scintigraphy, colonoscopy, biopsies of rectum and sigmoid, and the absence of another diagnosis, the patient was started on 30 mg of prednisone daily with mesalazine, as treatment for active ulcerative colitis. At the same time, she was receiving isoniazid and rifampicin as prophylaxis for the old TB infection. Subsequently, the patient's condition improved markedly, the fever retreated, and the need for blood transfusions also substantially decreased. The patient was discharged. One month later, she was reevaluated with a gallium-67 scintigraphy, which was notably different, with total absence of gallium-67 concentration in the ascending colon (). The cortisone dosage was decreased and close monitoring of the patient continued on an outpatient basis.
Annie Cline	33	1995/3/28	8708375137	[email protected]	7443 Stephanie Passage	Our patient is a 75-year-old African-American woman with a history of diabetes mellitus, hypertension, hyperlipidemia, and four prior cerebrovascular accidents with mild right sided residual weakness and ventral hernia status after surgical repair. She has no prior history of smoking, alcohol use, illicit drug use, or family history of premature coronary artery disease. She initially presented with shortness of breath, rhinorrhea, and cough productive of yellowish sputum and tested positive for influenza A for which she was started on oseltamivir. On her second day of hospitalization she reported mild retrosternal chest pain accompanied with a Troponin I elevation up to 0.3 ng/mL with an unchanged EKG showing right bundle branch block (RBBB) (). She received Aspirin, Clopidogrel, and Heparin drip in addition to Diltiazem and Atorvastatin as treatment for a NSTEMI. On the following day, she underwent a coronary angiogram revealing only minor luminal irregularities and no significant CAD (). Two days later, she underwent nasopharyngolaryngoscopy for progressive dysphonia that showed inflammatory changes of true and false vocal folds, mild granulation changes of the subglottis, and pachydermia. This was deemed secondary to her upper respiratory tract infection and the resulting acute tracheobronchitis. Subsequently, she was started on intramuscular dexamethasone 10 mg Q8 hours. One day later, she complained of severe retrosternal chest pain with a blood pressure of 90/65 mmHg, heart rate of 95 bpm, and oxygen saturation of 95% on room air. Cardiovascular exam was unremarkable revealing regular heart rate and normal S1 and S2 with no appreciated murmurs, rubs, or gallop. She had no JVD and clear lungs on auscultation. Distal pulses were well felt without appreciated lower extremity edema. The EKG however showed ST elevation in the anterior leads, V2 to V5 (). Bedside echocardiogram revealed a left ventricular ejection fraction (EF) of 30??5% and regional wall motion in the form of apical dyskinesis and severe hypokinesis in the mid to apical anteroseptal, anterior, apical inferior, inferoseptal, and lateral segments. Of note, the pre-MI echocardiogram showed a normal EF with no appreciated regional wall motion abnormalities. The Troponin I level was 0.1 ng/mL (cutoff value of 0.2 ng/mL). She was immediately transferred to the catheterization lab where the angiogram surprisingly revealed a filling defect, likely a thrombus, occluding the midsegment of the LAD (). Subsequently, a 2.5 ? 18 mm drug eluting stent was deployed with pre- and postballoon dilation resulting in TIMI III flow (). Eptifibatide was started in addition to Ticagrelor, Aspirin, Deltiazem, and Atorvastatin. The chest pain resolved shortly after the intervention and the EKG returned to baseline (). Blood work revealed a platelet count of 461 k/cumm, hemoglobin of 10.5 mg/dL, and white blood cell count of 9 k/cumm. Homocysteine level was mildly elevated (15.5 micromoles/L, normal range 3.2??0.7). High sensitivity CRP was elevated at 40.9 mg/L. Factor VIII activity (325%, normal range: 63??50), factor XI activity (211%, normal range: 71??24), and thrombin antithrombin complex (13.7 ng/mL, normal range: 0.7??.2) were all increased while factor VII activity was normal. Fibrinogen level and protein C and S activity were normal. Cardiolipin antibody, JAK2 V617F mutation, and Factor V Leiden were all negative.
Cullen Harvey	30	1989/6/9	(892)905-3447x04439	[email protected]	055 Kristen Ports Suite 927	A 48-year-old Chinese woman with a history of a right parasagittal meningioma with surgery and gamma knife excision done, was first put on valproate but then stepped up to topiramate 26 months later for uncontrolled seizures. The initial dose was topiramate 25 mg twice a day, which was eventually stepped up to 100 mg twice a day for adequate seizure control. She was referred to our ophthalmology clinic for dry eyes before the use of topiramate, with an examination showing a baseline visual acuity of 1.2 in both eyes with unremarkable anterior segment and fundoscopy examination. There was no family history of retinal diseases. She complained of blurring of vision in both eyes after using topiramate for 9 months. She was not on other medications when her visual symptoms developed. On examination, her visual acuity was 0.7 in both eyes. Her pupils were equal with no relative afferent papillary defect. The intraocular pressure, Ishihara test, and anterior segment examination were within normal limits. A fundus examination revealed there was bilateral diffuse pigmentary retinopathy (Fig. ). Automated perimetry showed bilateral peripheral constrictions (Fig. ) while microperimetry showed normal macula sensitivity (Fig. ). Autofluorescence fundus pictures showed loss of autofluorescence at the periphery, which is compatible with areas of pigmentary retinopathy (Fig. ). A fundus fluorescein angiogram also showed blocked fluorescence in the areas of pigmentation (Fig. ). Optical coherence tomography was unremarkable. A full field electroretinogram (ERG) according to International Society for Clinical Electrophysiology of Vision (ISCEV) standard was performed at 16 months after onset of symptoms (25 months since topiramate was first given). Both photopic and scotopic responses were found to be within normal limits. Administration of topiramate was immediately ceased due to suspected correlation with her eye signs and symptoms. She was switched to levetiracetam monotherapy. There was no more seizure recurrence after this treatment change. All investigations were repeated 1 year after discontinuation of topiramate. Bilateral diffuse pigmentary retinopathy was still present, with visual field test showing similar peripheral constriction, although her visual acuity improved back to 1.0 in both eyes and she reported subjective improvement in vision.
Nicole Garrett	29	1997/3/5	716.876.5024	[email protected]	9020 Carter Ramp	A 7-year-old male child was referred to the Department of Oral and Maxillofacial Surgery, Dr ZA Dental College, Aligarh Muslim University, Aligarh from primary health care center, Aligarh. The chief complain of the patient was wide mouth and compromised feeding. Child looked thin and was underweight as compare to the children of same age group. He was born prematurely at six months of gestation by cesarean operation. The obstetric history of mother was G3P2Ao (Gravida 3, Para 2, Abortion O). Child had a history of blood transfusion but the parents of the child neither could furnish any documented evidence nor tell the disease for which of blood transfusion was done. The extraoral examination revealed that the child had a wide mouth. The angles of the mouth were extending posteriorly on either side but not beyond the anterior border of the masseter. The cleft is not beyond the anterior border of masseter was identified by asking the patient to clinch his teeth. Since the muscle is close to skin, it can be easily palpated. When it contracts on clenching, its anterior border stands clearly. Bilaterally, the clefts were lined with skin externally and with buccal mucosa internally. The demarcation between end of the lips and beginning of the defect was noticeable on close observation (). Intraoral examination revealed mixed dentition, with normal eruption time and sequence. Orthopantomogram, revealed mild hyperplasia of the mandible. On further local and systemic physical examination no other abnormalities or congenital anomalies were found.
Kairo Ponce	39	2002/9/10	(986)537-5188	[email protected]	6373 James Fords Apt. 098	A 16-year-old Indian female patient was referred for endodontic treatment of mandibular left first molar with chief complaint of spontaneous pain. The tooth was percussion sensitive, cold and heat sensitive, although there was no referred pain. Radiographical examination revealed an additional distolingual root (). Tooth was anesthetized and isolated with rubber dam. The pulp chamber was opened, and one distal and two mesial canal orifices were located using an endodontic explorer (DG-16 Endodontic Explorer, Ash Instruments, Dentsply, Gloucester, United Kingdom). Upon visual inspection with a surgical loupe (Neitz BLS-3, Japan), a dark line was observed between the distal canal orifice and the distolingual corner of the pulp chamber floor. At this corner, overlying dentin was removed with a diamond bur with a noncutting tip (Diamendo, Dentsply Maillefer) and a second distal canal orifice was detected. The root canals were explored with a K-file #15 (Dentsply Maillefer, Ballaigues, Switzerland). The radiographical length measurement was performed with the Rinn set (Dentsply Rinn, Elgin, IL, USA) () and confirmed with an electronic apex locator (Raypex 5, VDW GmbH, Munchen, Germany). The root canals were shaped with protaper rotary instruments (Dentsply Maillefer) up to the F2-Protaper. During preparation EDTA (Glyde File Prep, Dentsply Maillefer) was used as a lubricant and the root canals were disinfected with a sodium hypochlorite solution (2.5%). The canals were obturated with protaper gutta-percha F2 () and AH Plus sealer (Dentsply Maillefer). The pulp chamber was sealed with Ketac Fil glass ionomer cement (ESPE, Seefeld, Germany) and tooth was restored with silver amalgam.
Aileen Serrano	37	1989/7/23	418-856-4675x7555	[email protected]	646 Lee Gateway Suite 483	A male child aged 4 years was brought to the hospital with a complaint of swelling on the upper anterior teeth region since 15 months. Upon analyzing the history, the father in-formed that, the right upper central incisor was not erupted till the age of 2 and the child was taken to the dental clinic. Dental records stated that opperculectomy was done in 51 region following which the tooth erupted. 6 months later the patient developed a small swelling in the gum region in relation to 51. Swelling was gradually increasing in size with the displacement of incisor teeth and associated with pain. On examination, a single well-defined sessile growth was evident on the anterior maxillary region encircling the crown of right upper central incisor (). The size was approximately 4 ? 3 cm anteroposteriorly and mediolaterally protruding between the lips (). Mouth closure was incomplete. Mucosa overlying the growth was erythematous. Palpatory findings include bony hard consistency, fixation to the deep structures, tenderness and bleeding. 51 and 61 were not mobile and no response was elicited on using electronic pulp vitality tester. No evidence of paresthesia on lip and para-oral structures. Above features made us to think, bony sarcoma and odontogenic tumor as possible differential diagnosis. Maxillary occlusal view in relation to 51 and 61 region showed well-defined radiopaque mass with numerous specks producing ground - glass appearance between widely displaced 51 and 61. The opaque mass was surrounded by unilocular radiolucency. No evidence of root resorption in relation to 51 and 61. Tooth buds of permanent central incisors were found above the radiopaque mass (). CT maxillary view demonstrated expanded cortex in the anterior alveolar region with ill-defined mixed mass surrounded by areas of corrugated margins (). Microscopic analysis of incisional biopsy specimen demonstrated nonencapsulated rich cellular connective tissue stroma composed of proliferative plumps of fibroblasts along with collagen fibers (). Round to ovoid areas of osteoid and slender trabeculae of woven bone were interspersed in the connective tissue stroma (). Few giant cells were present. No evidence of mitotic figures. Clinical, radiographic and histopathological corelation suggested the final diagnosis of juvenile ossifying fibroma.
Milan Phillips	26	1980/10/22	976-343-2491x1391	[email protected]	9462 Alexandra Trail	A 9-year-old female came to the pediatric dental clinic for dental treatment. The medical and family history was noncontributory. Clinical examination disclosed carious lesions in primary and permanent molars, and a prominent cusp-like structure on the palatal surface of the maxillary left lateral incisor. It was pyramidal in shape and extended from CEJ half-way to the incisal edge causing minimal occlusal interference (). Clinically, the tooth was asymptomatic and responded normally to pulp vitality tests. The grooves at the junction of the cusp and palatal surface were stained and contained dental plaque. Maxillary right lateral incisor displayed a deep lingual pit suggesting type I invagination. Further, prominent bifid cingulae with deep stained fissures were apparent on the palatal aspects of both maxillary right and left central incisors. White spot lesions were visible in the invagination and around the tubercle (). A periapical radiograph showed a ???shaped radiopaque structure superimposed on the affected crowns, with the ???pointing towards the incisal edge. In addition, periapi-cal radiograph revealed an invagination lined by enamel, adjacent to the tubercle indicating probable Oehler? type I invagination in 22. Radiographically no evidence of associated pathosis was found (). The treatment plan consisted of oral hygiene instructions, restoration of carious teeth, and prophylactic restorative procedures in the maxillary central incisors and lateral incisors. The prophylactic treatment consisted of selective grinding of extra cusp with flare-shaped diamond bur under water coolant using high-speed hand piece at 3-month intervals. Each grinding session was followed by the application of a desensitizing/remineralizing agent with 0.2% fluoride (GC Tooth mousse plus, Recaldent, GC Co. Japan), to the surfaces of reduced cusp, after polishing, to reduce dentin sensitivity. The treatment lasted 12 months, during which preventive measures to caries were followed regularly. Invasive sealing of the cusp-tooth intersection in incisors was planned. After prophylaxis of affected teeth, ameloplasty was performed on the grooves/fissures near the bifid cingulum using carbide bur in a high-speed hand piece and were then sealed with an acid-etch flowable composite resin to avoid penetration of irritants and microorganisms into the invagination (). After 18-month follow-up period, the tooth was still asymptomatic and responded normally to vitality tests. Patient is under regular clinical and radiographical re-evaluation to assess the occlusion and the progress of pulp recession.
Naomi Hogan	40	1994/2/15	001-779-430-5125x30113	[email protected]	4458 Solis Terrace	A 6-year-old female presented for a routine dental examination. Her medical history was noncontributory and extraoral examination did not reveal any abnormalities. On intraoral examination, the left maxillary deciduous lateral incisor (62) was slightly mobile and revealed an extra cusp which extended from CEJ halfway to incisal edge, at right angles to the mesiodistal surface of the tooth crown on the lingual surface resembling an eagle? talon, suggesting dens evaginatus. The cusp measured 2 to 2.5 mm in length (inciso-cervically), 1 to 1.5 mm in width (mesiodistally) and about 2 to 2.5 mm in thickness (labiolingually) approximately (). She was born out of consanguineous marriage and family history was free of similar dental abnormalities. A periapical radiograph of 62 revealed a radiopaque structure superimposed on the crown. Further, the unerupted left maxillary permanent lateral incisor presented an invagina-tion like appearance lined by enamel with possible aspect of Oehler? type I dens invaginatus. Additionally, an unerupted supernumerary lateral incisor (supplemental type) was noticed mesial to 62. No evidence of any periapical pathosis was evident radiographically (). There were neither esthetic concerns nor clinical signs of any problems associated with deciduous maxillary lateral incisor. So no immediate dental intervention was advised and the patient is under regular follow-up.
Sonny Villalobos	39	1985/6/11	001-406-801-8702	[email protected]	843 Huffman Fords Suite 714	A 28 years old male, the first kid/sib of healthy parents with consanguineous marriage admitted to Hasheminejad hospital with hypertension, lower extremities edema, and high serum creatinine level. Past medical history was remarkable for hyperuricemia and gout. On admission the blood pressure was 160/90 and pitting edema of both lower limbs was found, other organs were otherwise normal. On sonographic examination right kidney was 114 mm and contained a thick wall cyst measured 23 ? 24 mm and left kidney was 90 mm, with increased cortical echogenicity in the cortex of both kidneys. Doppler sonographic findings were RI=0.65 in right and 0.7 in left kidney. In the family history, his father and mother were cousins, his elder brother was diagnosed with gout and renal failure at age 33, and other positive findings were hyperuricemia and renal stone in his two cousins. Laboratory examination was as follow: Due to renal failure and normal kidney size, a renal biopsy was done, which revealed advanced glomerulosclerosis in focal and segmental pattern, severe tubular atrophy and proportional fibrosis mostly secondary and mild to moderate arterionephrosclerosis (). We proposed Familial juvenile hyperuricemic nephropathy as the most possible diagnosis for this patient with respect to clinical manifestations, paraclinical findings, and family history, which led us to perform genetic study to confirm the diagnosis. Sequencing analysis showed that the patient is homozygous for a novel nonsense mutation, c.589G>T; p.E197X, in exon 3 of the UMOD gene (). This confirms and interprets his involvement in familial juvenile hyperuricemic nephropathy.
Zoya Cano	21	2001/12/2	(202)312-4187	[email protected]	469 Dunlap Rapid	An 86-year-old caucasian woman presented with Stage-IV colorectal cancer. The patient agreed to participate and was explained the nature and objectives of this study, and informed consent was formally obtained. No reference to the patient's identity was made at any stage during data analysis or in the report. The patient had a large, undifferentiated tumor at the splenic flexure along with liver metastasis. She underwent resection of the primary tumor due to an impending obstructing tumor. Because of her advanced age, it was decided to proceed with monotherapy consisting of capecitabine only. A pre-treatment genetic testing for capecitabine consisting of thymidine synthase (TYMS) and dihydropyrimidine dehydrogenase (DPD/DPYD) analysis was performed that showed intermediate risk for 5-FU and related agents' toxicity.Therefore, capecitabine was initiated at a low dose (500 mg BID) and then gradually increased to a maximum dose of 1500 mg/m2/day for two-weeks-on and one-week-off schedule days = 1 cycle). She tolerated it well except mild intermittent diarrhea (Grade 1) and Grade 1 HFS manifested as erythroderma on the palms of both hands and later some dry desquamation of limited duration which promptly resolved when capecitabine was held. Her restaging imaging including a PET-CT scan showed a good response in the liver metastasis. All of her symptoms resolved when capecitabine was discontinued. After a few months of treatment with capecitabine, she noticed 'dry skin with itching' had developed for which she saw a dermatologist. Subsequently, she also developed a progressive cough and shortness of breath, hoarseness, mild hypertension, and symptoms of gastroesophageal reflux disease (GERD) manifested as heartburn with reflux symptoms. During that period, she was evaluated by a multi-disciplinary team comprising of a pulmonologist, rheumatologist, ear-nose-throat (ENT), and gastroenterology in addition to her oncologist. Pulmonary fibrosis was noticed on the CT scan of the chest. In addition, she also developed a new onset renal dysfunction during that period. Anti-nuclear, anti-centromere, antibodies to Smith antigen, antibodies to ribonucleoprotein, and anti-scl 70 antibodies were all negative. However, complement fixing antinuclear antibodies (C-ANA) on HEP-2 cells showed higher titer (320) with speckled pattern and C-ANA/ANA titers ratio of 320/40. A C-ANA with such a pattern and C-ANA/ANA titers ratio is suggestive of systemic sclerosis or one of the other diseases in group A of systemic connective tissue diseases. Moreover, a skin biopsy was performed. A biopsy with surface dimensions of 2.5 mm x 2 mm was reviewed from the upper right wrist skin lesion. For the C+DIF test, a fresh source of complement was added to the biopsy sections followed by fluorescein-labeled antibodies to the C3 component of the complement. This biopsy included skin of the epidermis and dermis with no structural deformities. Direct immunofluorescent studies of the skin biopsy specimen showed granular focal deposits of fibrin at the dermal-epidermal junction and deposits of IgG, IgA, IgM, fibrin, and trace C3 in the connective tissue fibers to the epidermal nuclei. These positive C+DIF reactions and serum results are consistent with or at least suggestive of systemic scleroderma or another 'group A' systemic connective tissue disease. She was placed on nifedipine, aspirin, lisinopril, and omeprazole. She was also started on cyclophosphamide treatment at the starting dose of 500 mg and then dose escalated to 700 mg and finally to 900 mg as tolerated per her Rheumatologist's recommendation []. She did not tolerate the 900 mg of the third cycle; therefore, the dose was decreased back down to 700 mg, and she completed six cycles of treatment. Cyclophosphamide was stopped and replaced with mycophenolate mofetil (MMF) at a dose of 0.5 g per day to a maximum of 2 g per day []. Her dyspnea resolved with treatment with mycophenolate. Her lung function also improved and eventually normalized. She was tapered off of mycophenolate after 23 months of therapy. Six months after cessation of mycophenolate, her lung function remained stable.
Terry Acosta	25	1980/8/20	909.683.0990	[email protected]	6671 Braun Mills	A 73-year-old woman presented with nausea, increasing confusion, ataxia, and mechanical falls. MRI imaging revealed a 6.4 x 4.3 x 3.8 cm multilobulated fluid collection in the supracerebellar cistern and quadrigeminal cistern consistent with a complex arachnoid cyst (Figure ). The patient agreed to participate and was explained the nature and objectives of this study, and informed consent was formally obtained. No reference to the patient's identity was made at any stage during data analysis or in the report. Because she had symptomatic hydrocephalus due to the mass effect of the arachnoid cyst, an external ventricular drain was placed into the right lateral ventricle at the time of admission. She was then taken for definitive surgical treatment with decompression and fenestration of the cyst through a superior cerebellar approach as described in detail below. She tolerated the procedure well and post-operatively recovered in the intensive care unit. Her symptoms resolved, and after a cisternogram confirmed that the cerebral aqueduct was patent with good fenestration of the arachnoid cyst (Figure ), the external ventricular drain was removed on postoperative day two. The patient did well, symptoms resolved, and on postoperative day eight she was discharged in good condition.
Kaia Stein	34	2004/1/25	(959)410-9710x7235	[email protected]	66581 Christopher Brook	The patient was a 41-year-old widowed male at the time of screening who started drinking at age eight and progressed to become a regular drinker by age 16. At screening, he reported heavy drinking for the last ten years and had a positive family history of alcoholism. He reported excessive drinking (one-two pints of hard liquor daily, [8.5 - 17 drinks]) after a major life event occurred as the death of his spouse (approximately eight months prior to admission). His BMI was 22; he reported heavy smoking (one pack daily), and recent involvement in unsafe lifestyle practice. He showed confirmatory signs of alcohol use disorder with withdrawal symptoms during the psychiatric evaluation. Patient also reported that he was never tested positive for viral infection or taken medication for its treatment during intake. This patient was diagnosed with positive HCV and HIV viral tests during the screening. HCV RNA IU quantification was at 8,920,000 with genotype 1A. HIV infection was detected based on both the criteria for confirmation for HIV infection as developed by the Center for Disease Control; with HIV-1 Ab detection, and western blot assay at viral load of >50. His sCD4 count was 210, which put him in the category 2 according to the CD4+ T-Lymphocytic classification. The test was reconfirmed with western blot band with second positive test at bands p24, gp41, and gp120/160. There was no other remarkable systemic observation and he was not diagnosed with any other relevant medical condition, other than the discussed drug abuse and viral infection. This patient presented asymptomatic conditions of HIV infection (resembling criteria for ?linical category A??. This patient presented with a remarkable liver injury profile (), with all the liver injury markers showing above the normal values. In co-infected HIV+HCV cases, even with severe liver fibrosis and cirrhosis, 10% to 25% patients have shown normal alanine aminotransferase (ALT) levels []. Therefore, this case seems to show added severity in liver injury due to heavy alcohol drinking. Also, clinically relevant complete blood count (with differential) was identified at low hemoglobin (13.3, normal: 13.7 - 17.5 g/dL), hematocrit (37.2, normal: 40.1% - 51.0%) and MCV (78.6, normal: 79.0 - 92.2 fL) levels showing suppression of blood cell production this state of the infection. Ferritin was identified as high, 408 (26 - 388) as is generally observed in infections. Abnormal levels of leucocytes (WBC: 146/mcl; normal 0 - 25) were present and specific subtypes namely monocytes and lymphocytes [] as shown in . In this patient, fatty acid panel tested with blood (plasma) specimen showed that linoleic acid was the only PUFA that was abnormally high at 3957 nmol/mL (normal range: 2270 - 3850) that has shown important role in liver injury []. Both HCV and HIV cause dysregulation of lipid metabolism, especially in polyunsaturated fatty acids (PUFAs) []. We also evaluated the ?-6/ ?-3 ratio, which was 18 (Total ?-3 was 0.3; and Total ?-6 was 5.4, units mmol/L) in this patient supporting significant increase in the pro-inflammatory shift []. Triene Tetraene ratio was at the lower margin at 0.01 (normal range: 0.010 - 0.038). No other clinically significant change was observed in the FAs involved in inflammation; or in the lipid panel. Immunoglobulin panel at this point was not abnormal. Patient received adequate medical management for alcohol withdrawal during the screening process [] and was referred for infectious disease specialty.
Creed Alexander	31	2000/4/27	967.496.5647	[email protected]	0484 Bryan Branch Apt. 604	A 30-year-old otherwise healthy female presented with the sudden onset of headache and subsequent obtundation. Upon arrival to our emergency department, her airway was secured with endotracheal intubation. Initial vital signs revealed bradycardia. The Glasgow coma scale score on admission was a 4T, as the patient had decerebrate posturing. Her eyes deviated downward with non-reactive, pinpoint pupils. Informed consent was waived due to the patient's neurological presentation. A CT scan of the head revealed a calcified lesion in the right cerebellar cortex with an acute intralesional hemorrhage and adjacent subdural hematoma. Effacement of the fourth ventricle with associated obstructive hydrocephalus was also noted (Figure ). An emergent right frontal external ventricular drain was placed in the emergency department as a temporizing measure. The opening pressure was over 30 cm H2O (Figure ). A CT angiogram of the head and neck to rule out underlying vascular lesions was unremarkable. The patient was then taken to the operating room for surgical decompression. A paramedian suboccipital craniotomy was performed on the right side. The dura was opened and a subdural hematoma was quickly encountered and evacuated. An intra-axial cerebellar tumor rapidly presented as the dissection proceeded and a significant amount of intratumoral hemorrhage was noted. A gross total resection was undertaken (Figure ). A very small amount of tumor was purposefully left on the brainstem in an effort to avoid significant morbidity from an injury to the lower cranial nerves and the vertebral artery branches that were involved within the tumor. Final pathology of the tumor revealed a pilocytic astrocytoma (Figures -). The patient was following commands on the first postoperative day and was extubated a few days later. Postoperative neurological assessment revealed bilateral sixth cranial nerve palsies, right sensorineural hearing loss, and gait ataxia. The external ventricular drain was weaned and removed without any need for permanent CSF diversion. She was transferred for acute rehabilitation and was eventually discharged home with significant clinical improvement. At her one-year follow-up, she had minimal residual brainstem and cerebellar deficits. Adjuvant radiation therapy was considered, but the radiation oncologist felt that the risks of radiation therapy were not justified. She was able to walk independently and eventually return to work. Three years after her initial presentation, the patient got married and had a child via Caesarean section. During the postoperative period and during her pregnancy, her residual tumor has remained stable and no further episodes of hemorrhage or growth have been noted. Some of her symptoms, such as ataxia, got worse when she was pregnant but improved after delivery of the child.
Lyla Lee	42	1982/2/20	+1-910-541-9064x95705	[email protected]	95010 Brewer Cliff Suite 444	A 45-year-old woman presented with a 13-month-history of pain and swelling in the anterior aspect of the right elbow. There was no history of trauma or any other exacerbating factor. She had been taking analgesics for last nine months due to intermittent pain which affected her ability to use the dominant elbow. Local examination revealed a 3 cm x 3 cm soft tissue swelling on the anterolateral aspect of the elbow, which was tender on palpation. The swelling was mobile in all directions. Elbow range of movement was from zero degrees to 125 degrees. There was no associated erythema around the elbow and over the swelling. The MRI showed a multiseptate fluid collection measuring 1.73 x 1.32 cm on the lateral aspect of cubital fossa anterior to the radio-capitellar joint space possibly communicating with it showing T1 hypo and T2/STIR hyperintense signals. Anteriorly it was burrowed into the brachioradialis muscle (Figure , ). This symptomatic swelling was excised through an anterolateral approach to the elbow joint [], after making a plane between brachialis and brachioradialis. A cystic swelling of about 2 cm x 2 cm in size was found (Figure ). It was carefully isolated from the surrounding soft tissues and excised en-masse. The stalk of the lesion was found to be originating from the radio-capitellar joint (Figure ). The rent in the capsule was then closed with an absorbable Vicryl 3-0 suture (Figure ). The excised lesion was sent for histopathological examination, which confirmed the diagnosis of a ganglion cyst. Written consent was obtained from the human subject who participated in this case study. Institutional Review Board of Indraprastha Apollo Hospital, India approved the research for this case.
Jack Huang	38	1981/1/22	7419814785	[email protected]	9064 Debra Port Suite 056	An 18-year-old female patient was presented with a slowly growing, painless swelling at the lateral aspect of left ankle since four months. There was no other significant contributing history. Local examination revealed a well-defined bony hard swelling (7 cm x 4 cm) which was nonadherent to the overlying skin. The ankle and subtalar joints had full range of painless movements. Plain radiographs revealed a multi-loculated, expansile and well-defined lesion involving the lower end of fibula mimicking a soap bubble like appearance (Figure ). The radiographic findings were suggestive of a GCT. A Computed Tomogram (CT) confirmed the presence of a large lesion in the distal fibula with no cortical breach with preserved ankle mortise (Figure ). An extensive curettage of the tumour along with chemical cauterization was done using phenol (Figure ). The bone defect was reconstructed using a tricortical iliac crest autograft (Figure ) and fixation was done with a 3.5 mm (8 holes) reconstruction plate. Two screws were passed through the syndesmosis to augment it. The lateral fibular wall containing the tumour was excised, preserving the medial wall (with syndesmosis) and lateral malleolar articular surface. She was mobilized nonweight bearing, with crutches, with a below knee cast for 3 months, until the bone graft got incorporated. The histopathological features showed multinucleated giant cells (Figure ) which were suggestive of a diagnosis of GCT. There were regular and uniform distribution of stromal cells and giant cells. The stromal cells are mononuclear, resembling macrophages while giant cells are large, multinucleated (10-50 nuclei) with similar nuclei as stromal cells, resembling osteoclasts. At 18 months follow-up, there was no evidence of local recurrence, with a full range of pain-free movements of ankle and subtalar joints (Figure ).
Francesca Collier	39	1980/3/30	431-494-3589	[email protected]	24719 Reyes Gardens	Patient A, a 5-day-old infant female, 2.2 kg, born prematurely at 35 weeks gestational age, was transported to an outside hospital emergency room via emergency medical services following an apneic event at home that resolved after the administration of a few rescue breaths by the mother. Upon presentation to the emergency room, the patient demonstrated stable vital signs and was subsequently transferred to our pediatric intensive care unit (PICU) for respiratory monitoring and diagnostic workup for the cause of the apneic event. Upon arrival to the PICU, the patient was vigorous, crying, and tachypneic, but, otherwise, with relatively normal vitals signs (HR 145, BP 77/44, 97% on 1 LPM NC O2). Given the patient? age and history of apnea, a workup to rule out serious bacterial infection was initiated. Basic labs were sent, and a lumbar puncture was completed showing 20 WBC/弮L (2,030 RBC/弮L) with 33% neutrophils. Broad-spectrum antimicrobials were initiated including ceftriaxone (50 mg/kg/dose prior to arrival to PICU), acyclovir (60 mg/kg/day), and ampicillin (400 mg/kg/day). Initial labs were significant for thrombocytopenia (platelets 39,000/弮L) but without any other cytopenias (Hgb 17.1 g/dL, WBC 8,760/弮L). At hour 6 after admission, the patient rapidly decompensated and developed lethargy, respiratory failure due to apnea, and was intubated and mechanically ventilated. At hour 10 after admission, additional laboratory testing revealed very elevated liver transaminases (ALT 2,214, AST > 7,500), severe coagulopathy (PT > 120, INR > 16.6, PTT > 200) and a ferritin of 241,000 ng/mL (result verified with duplication). The patient? triglyceride level was 41 mg/dL, and serum lactate level at this time was significantly elevated at 14 mmol/L. At hour 18 after admission, the HSV PCR from CSF returned positive for HSV-2, and the C-reactive protein (CRP) was elevated (2.09 mg/dL). We made the preliminary diagnosis of HSV-associated hemophagocytic lymphohistiocytosis (HLH) based on a similar case report (). The patient? respiratory and hemodynamic profile continued to deteriorate (Figure A) in spite of escalating vasopressor support (Figure B), and the patient needed repeated fluid boluses of colloid (5% human serum albumin in NS) and blood product administration (Figure C) to maintain mean arterial pressure (MAP) of at least 40 mmHg. At this time, 30 mg/kg methylprednisolone was administered to ameliorate the inflammatory process and, with the addition of norepinephrine, the patient stabilized transiently, and we were able to wean the epinephrine support slightly (Figure B). At hour 30 after admission, the patient again became unstable and required repeated volume resuscitation in the form of colloid fluid boluses to maintain the MAP goal of 40 mmHg, and then, even this MAP goal became unobtainable. After long discussion with the parents, it was decided that recovery was unlikely and technological support was withdrawn. The parents agreed to an autopsy, and the report stated that ?he presence of hemophagocytosis was demonstrated with the bone marrow and spleen exhibiting large cells containing particles consistent with ingested, disintegrating blood cells. Given that diagnostic guidelines for HLH designate bone marrow, lymph node, and spleen as the organs, which most reliably show the histologic features, and secondary HLH can be triggered by systemic infection, as this patient had, the findings post-mortem supported a diagnosis of HLH.??,[[5.0
Edison Arroyo	32	1986/2/10	4726007373	[email protected]	95438 Ramsey Path	Patient A, a 37-year-old healthy male, underwent open reduction and internal fixation (ORIF) of an open fracture of his right distal tibia and fibula 22 years ago. This was complicated by posttraumatic osteomyelitis and resulted in multiple re-operations with debridements of the bone, removal of most hardware and free flap coverage of a soft tissue defect. He was referred to our hospital with a persistent clinical infection around his right distal tibia and a near wound breakdown of the scar. Medical imaging was requested (a) to confirm the diagnosis of osteomyelitis and (b) to determine the anatomical location of the suspected osteomyelitis. First, according to the diagnostic imaging protocol in our hospital, a three-phase bone scan was performed since the fracture and surgery were >2 years ago. All three phases of the bone scan were positive, only the late phase (Fig. , g: anterior view, h: lateral view) is presented here. This increased osteoblastic uptake can be the result of an infection, but also due to a healing fracture or recent surgery. For further differentiation, the patient underwent a WBC scan (Fig. , a?: images after 4 h, e?: images after 24 h). This showed increased uptake in both intensity and size over time, suspect for an infection. To localize this accumulation of leukocytes a SPECT-CT was performed (Fig. ) which revealed that the uptake was located outside the bone, in the soft tissue. Final diagnosis was a soft tissue infection.
Kyra Brewer	25	1996/3/14	521.803.9232x491	[email protected]	36722 Jay Islands	Patient B, a 46-year-old schizophrenic but otherwise healthy male sustained an open and comminuted talar neck fracture after a fall from height. This was initially treated with multiple soft tissue debridements and an external fixation, later augmented with screw and K-wire fixation of the fracture. The soft tissue defect was closed with a local myocutaneous flap. Two months after this last procedure, the patient presented with a draining sinus in the scar on the lateral side of the ankle joint. Medical imaging was requested to (a) assess the viability of the talus and (b) to determine the anatomic location of the suspected osteomyelitis. The X-ray is shown in Fig. (left image). To answer question (a) a three phase bone scan was performed. The first phase (flow phase) is shown in Fig. (upper row images): positive flow at the talar region of the left foot. Obviously also the second phase (blood pool phase, middle image lower row) and the late phase (combined with CT image, right image lower row) are positive. This means the bone is viable. However, differentiation between infection or healing fracture is not possible. Therefore, WBC scintigraphy was performed (Fig. , upper row: images after 4 h, lower row: images after 24 h). The uptake decreased in time, meaning that the leukocyte accumulation is the result of a healing fracture and not of an osteomyelitis. After proper wound care further healing was uneventful.
Cruz Cox	34	1996/9/4	001-531-735-2603	[email protected]	2480 Taylor Estate Suite 218	Patient C, a 33-year-old healthy male was referred to our hospital because of a suspected posttraumatic osteomyelitis in combination with a malunion of his left tibia. He sustained a gunshot wound to his left lower leg in the middle east conflict 2 years prior to this presentation which was treated with a prolonged immobilization in an external fixator combined with several wound debridements. The last operation was only a few months prior to presentation. On examination, apart from the obvious malalignment of his left lower leg, we noted a closed but unstable scar on the medial side of his left tibia. Medical imaging was requested to (a) confirm the diagnosis and (b) to determine the anatomic location of the suspected osteomyelitis. Since his last surgery was <6 months ago, immediately a WBC scan was performed (Fig. , left image: anterior view after 4 h, right image: anterior view after 24 h): uptake is visible at three locations. When calculating the ratios (uptake focus-to-contralateral side) the uptake at the most proximal and most distal focus decreases in time. This means these uptakes are due to regeneration of bone marrow. However, the uptake at the middle focus increases in time, which is suspect for an infection. The SPECT-CT (Fig. ) shows the uptake in the bone and a small fistula to the bone marrow. Indeed, surgery revealed an infection at this location.
Sadie Mullins	36	1983/8/17	6117760677	[email protected]	1678 Mark Course Apt. 775	A 56-year-old female presented to the emergency room with a three-day history of right hip pain. She reported decreased range of motion and difficulty ambulating. She denied constitutional symptoms or fever. She had undergone intra-articular steroid injection of her right hip three days prior. Review of systems was otherwise unremarkable. Her past medical history was relevant for hereditary spherocytosis with splenectomy at age 14 and chronic right hip osteoarthritis. Her only medication was celecoxib as needed. She denied any drug allergy. She was unaware of her immunization history. She worked as a flight attendant, denied smoking or recreational drug use, and had not recently travelled outside of Canada. On initial examination she was afebrile and her vital signs were within normal limits. On examination of her right hip she had limitation of internal rotation with reproducible pain, but her physical exam otherwise was unremarkable. Her initial investigations showed elevation in C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) at 22.9 mg/L and 25 mm/hr, respectively. Her white blood cell count was not elevated. An X-ray of her right hip showed severe joint space narrowing with osteophytosis consistent with severe osteoarthritis. Arthrocentesis was performed and synovial fluid samples were directly inoculated onto solid culture media, including blood agar and chocolate agar. Two of three samples also underwent cytospin centrifugation and direct Gram stain. Gram-negative bacilli were observed in one sample though heavy neutrophils were observed in both. Synovial fluid analysis for cell count and chemistry was not performed due to insufficient sample. She was admitted to hospital for 24 hours of observation and was subsequently discharged with instructions to return to hospital if her symptoms worsened or if cultures subsequently grew a pathogenic organism. Three days later she was seen in a follow-up clinic. In the interim she had developed a fever (38.3簞C), had chills, and had worsening right hip pain. Repeat physical examination showed deterioration in range of motion at her right hip with significant pain. Cardiovascular examination was unremarkable with no appreciable murmur. Synovial fluid cultures from the initial arthrocentesis were now growing Haemophilus parainfluenzae with colonies observed on solid media within 24 hours of inoculation. She received one dose of ceftriaxone and subsequently underwent right hip arthrotomy with synovectomy and irrigation. The Infectious Disease service was consulted the following day. The organism was susceptible to ceftriaxone and cefuroxime and resistant to ampicillin and ciprofloxacin (). Blood cultures and intraoperative tissue cultures did not demonstrate growth, likely due to the administration of antibiotics prior to collection. The patient was treated with intravenous ceftriaxone and was subsequently discharged with outpatient follow-up for home intravenous antibiotics. Ongoing pain, difficulty ambulating, and persistently elevated inflammatory markers necessitated a prolonged, nine-week course of antimicrobial therapy (). At the time of treatment discontinuation, she reported pain and her functional ability had not yet returned to baseline. She is currently awaiting evaluation for total hip arthroplasty.
Allen Kirby	23	2001/9/20	(381)301-4994	[email protected]	30186 Miller Grove Apt. 377	A 58-year-old male presented in the summertime with fever, generalized weakness, and encephalopathy after being found unconscious. Upon arrival to the hospital, he was disoriented to place and time, dysarthric, and amnestic to details of the event. Review of systems was positive for one week of progressive gait instability but negative for any other preceding or ongoing infectious or neurologic symptoms. He had no recent travel or exposure to sick contacts. Seven months prior to his presentation, the patient had been diagnosed with a poorly differentiated neuroendocrine carcinoma, which presented primarily in his parotid gland. He had completed seven cycles of chemotherapy, with the last cycle given two weeks previously. Two months earlier, he had developed numerous asymptomatic brain metastases. A whole-brain radiation therapy was recommended for him; however, he declined. Other chronic medical problems included a history of prostate cancer in remission, dyslipidemia, and hypertension. He also had a history of ?isual seizures??for which he was taking daily antiepileptic medication. On presentation, the patient was somnolent but arousable. His vital signs revealed that he was febrile (temperature of 102.4 F) and tachycardic (heart rate of 126), blood pressure was 150/105 mmHg, respiratory rate was 14, and oxygen saturation was 95% on 2 liters per minute nasal cannula. Cardiopulmonary and gastrointestinal examinations were otherwise unremarkable. On neurological examination, the patient exhibited a resting tremor in both upper extremities, which his family reported was new within the past few days. There was no evidence of tongue biting, urinary incontinence, photophobia, or nuchal rigidity. Muscle strength, tone, and deep tendon reflexes were normal and symmetric. Babinski signs were absent. Sensory examination was normal. Laboratory evaluation was significant only for mild hyponatremia (132 mmol/L) and elevated creatinine kinase (236 mmol/L). Tests for liver function, complete blood count, and urinalysis were unremarkable. Blood cultures and urine cultures were sent upon presentation and eventually revealed no growth. Urine drug screen was positive for cannabinoids only. Chest radiograph revealed no acute abnormalities. A noncontrasted head computed tomography was unremarkable with poor visualization of his known intracranial metastases. Initially, there was suspicion for an infectious etiology or progression of previously known brain metastases; therefore, the patient was started on empiric broad-spectrum antibiotics and systemic glucocorticoids for potential vasogenic edema until further diagnostic evaluation could be performed. His antiseizure medication was also continued. One day after presentation, the patient's mental status was remarkably improved, although not completely to baseline. The transient nature of his encephalopathy raised suspicion of a generalized seizure as the inciting event with resultant temporary postictal state. Given this, an electroencephalogram (EEG) was obtained, revealing mild-to-moderate generalized background slowing, indicating diffuse encephalopathy, without any epileptogenic foci identified. Two days after presentation, the patient remained febrile and tremulous; however, his cognition continued to improve. Because no definitive diagnosis had been reached and he continued to have unexplained fevers, a lumbar puncture was performed for further evaluation. Cerebrospinal fluid (CSF) studies revealed lymphocytic inflammation with an elevated glucose (84 mg/dL), elevated protein (89 mg/dL), and elevated total nucleated cell count (95/cumm), with a differential of neutrophils 8%, lymphocytes 53%, plasma cells 25%, and monocytes 14%. CSF Gram stain was negative, and cytology was negative for malignant cells. Herpes Simplex Virus-1 (HSV-1) and HSV-2 reverse transcription-polymerase chain reaction (RT-PCR) tests were negative, as was Cryptococcus neoformans antigen. CSF and serologic testing for West Nile Virus (WNV) IgM, IgG, and PCR was also ordered. Approximately one week after presentation, a final diagnosis of acute WNV encephalitis was made. Although CSF IgG and PCR for WNV were negative, the patient's serum and CSF WNV IgM were qualitatively positive, establishing the diagnosis of WNV neuroinvasive disease [, ]. Empiric antibiotics and steroids were discontinued. Brain magnetic resonance imaging was obtained, which revealed stable intracranial metastases compared to one month earlier. Over a period of several days, his mental status improved completely to baseline and his fever and tremors subsided. He did, however, continue to have significant diffuse weakness, presumably secondary to prolonged hospital stay and systemic glucocorticoids. The patient was eventually discharged to a subacute rehabilitation facility.
Skyla Brewer	28	1988/4/19	(267)624-8590x64931	[email protected]	430 Stephanie Wall Suite 020	We present a 58-year-old female who presented to the emergency department with violent behavior and agitation. The patient had a history of bipolar affective disorder as per DSM-5/ICD-10 classification. According to medical records and collateral history from the patient's legal guardian, the patient was diagnosed with this mood disorder over 20 years ago due to repeated manic episodes which include irritability, labile effect, increased energy, and erratic behavior. Past medical history includes cellulitis, hypertension, hypothyroidism, and repeated urinary tract infections. The patient has a history of developmental delay with a diagnosis of intellectual disability. There was an absence of psychiatric illness in her family. During evaluation by the psychiatrist in the emergency room on admission, patient was clearly distressed, with her hair not groomed, and was slightly disheveled in her appearance. The patient was loud, yelling at the psychiatry and medical staff in the hospital, and was routinely uncooperative and belligerent. Patient was also clearly agitated which appeared in behaviors such as kicking and hitting the unit staff. There were no signs of psychosis, as the patient denied hallucinations in all sensory modalities and no delusions were elicited, and there was no evidence of disorganized thinking or paranoia. Her mood overall was angry and irritable, with a labile effect. At the time of examination, she was alert and oriented to person, place, and time. Due to her intellectual disability, she lacked judgment and had poor insight regarding her mental illness. Patient was admitted with acute mania to the inpatient psychiatry unit and was continued on her home dose of valproic acid (VPA) at 1000 mg PO twice daily as well as continuing her other home medications (atorvastatin 10 mg PO at night, levothyroxine 175 micrograms PO once daily, and metoprolol 12.5 mg orally PO twice daily). It should be noted that the patient has been on VPA for over 10 years for treatment of her bipolar affective disorder, and her therapeutic levels and dosages are monitored by her outpatient psychiatrist. The high dose of her valproic acid is due to many years of outpatient psychiatry evaluation and titration of the dose to a level that allows for the patient to attain mood stability. Admission laboratory tests were all within reference range (CBC, BMP, and LFTs) and she had VPA level of 58 弮g/mL (therapeutic range: 50 to 100 弮g/mL). EKG showed slight sinus tachycardia, HR: 108. During her admission, patient had episodes of severe agitation and her behavior was very unpredictable but was eventually controlled through psychotherapy and group therapy while being an inpatient. Patient was also encouraged to be compliant with her pharmacotherapy, particularly her mood stabilizer medication valproic acid. According to medical records, patient had a history of high ammonia levels on previous admissions, and therefore VPA levels and ammonia were monitored during the course of her hospital stay (Tables and ). In the morning of day 51 of admission, patient was examined on the daily ward round. She was alert and oriented to person, place, and time and consciousness indicated that she was very much awake and aware of her surroundings, with no signs of altered consciousness. Patient was calm and cooperative during the psychiatric interview, with her appearance being well groomed. Her speech was of normal rate, rhythm, tone, and volume. There was no evidence of psychosis, and the patient appeared to be relatively euthymic with a full and congruent affect. Thought process was linear and thoughts were logical. Her judgment was still relatively poor and she had poor insight, but this was attributed in part to cognitive deficits. She had no neurologic deficits peripherally, and all cranial nerves were grossly intact. Lower extremity deconditioning secondary to lack of use had been an ongoing issue for over one year. Gait could not be assessed as she is nonambulatory but no abnormalities or deficits were present in her upper body chronically, and the patient had a Glasgow Coma Scale Score of 15 (Eyes: 4, Verbal: 5, and Motor: 6). Conclusively, patient elicited no clinical concerns from a neurological standpoint and therefore no clinical signs of encephalopathy or delirium. Laboratory testing revealed an ammonia level of 225 弮mol/L (reference range: 10??0 弮mol/L) and VPA level of 117 (reference range: 50??25 弮g/mL). According to routine laboratory protocol, 0.5 mL of blood had been taken from the patient and transferred into a heparinized vacutainer and placed immediately on ice (for ammonia level). The specimen was transferred to the lab within 15 minutes of collection from the patient. Based on the Naranjo score of 9, it is reasonable to conclude that the elevated level of ammonia was secondary to VPA therapy []. Liver functions tests were within normal limits, congruent with her baseline. It was decided to treat the hyperammonemia with lactulose 20 grams orally twice a day as per standardized protocol, as she had 2 previous episodes of high levels of ammonia during her admission that resolved with the same dosing of lactulose (20 grams PO twice daily). Levocarnitine 990 grams PO three times daily was also started. More importantly, valproic acid was not discontinued since the patient reported no symptoms of valproic acid toxicity or hyperammonemia, as she was cognitively and neurologically intact. After dual treatment with carnitine and lactulose, the patient's ammonia levels soon began to decline, from 225 弮mol/L to 163 弮mol/L to 27 弮mol/L within 2 weeks. The patient's dose of VPA did not change and she continued to receive this medication. During the course of these events, the patient did not develop any clinical signs or symptoms of hyperammonemia and remained stable neurologically and psychiatrically.
Cruz Walls	32	1993/7/17	(410)672-0376x885	[email protected]	63095 Murray Tunnel	A 35-year-old African American female with no prior history of coronary artery disease and no significant family history presented with sudden onset of exertional chest discomfort with radiation to the back. The patient became unresponsive shortly after arrival to the emergency department and was subsequently found to be in ventricular fibrillation-cardiac arrest (V-fib). The patient was in V-fib for 6 minutes, with conversion after electrical cardioversion and subsequent development of PEA-arrest for a total of 4 minutes. Repeat EKG after return of spontaneous circulation demonstrated inferolateral STEMI (). The patient received tenecteplase and heparin prior to urgent transfer to the catheterization laboratory. Left heart catheterization showed 99% thrombotic occlusion of mid-distal LAD (Figures and ). Two overlapping drug eluting stents (Xience RX 2.5 mm and Xience RX 2.75 mm) were placed in the distal LAD, and TIMI III flow was achieved (). Due to severely decreased ventricular function with ejection fraction of 10??5%, the patient received ventricular support with an Impella, with discontinuation soon after secondary to hemolysis. Further evaluation revealed positive toxicology screen for amphetamines. Pertinent laboratory results for possible autoimmune causes of the acute myocardial infarction were negative, including antinuclear antibody (ANA), ribonucleoprotein antibody (RNP), RA latex turbid, anti-chromatin IgG antibodies, SSA (Ro) and SSB (La) antibodies, and double stranded DNA antibody (dsDNA). The patient's cardiac function recovered with medical management. Subsequent transthoracic echocardiograms revealed improved ejection fraction to 60??5% after 11 days and a new finding of left ventricular apical thrombus. The patient received anticoagulation with intravenous heparin, as well as continuous treatment with dual antiplatelet therapy with aspirin and clopidogrel. The patient remained in the coronary care unit (CCU) for a total of 17 days. CCU course was further complicated by development of pulmonary edema with diffuse alveolar hemorrhage and developing MRSA and pseudomonas pneumonia. Due to the need for prolonged mechanical ventilation, the patient received a tracheostomy and continued to improve in terms of her pulmonary function while treated with antibiotics for ventilator-associated pneumonia. Her neurological status improved significantly, and on interview, she denied any use of Adderall, amphetamines, or illicit drugs that could have precipitated this event. She reported that recently she had increased her level of physical activity in order to lose weight and was supplementing such efforts with the addition of a natural weight loss dietary supplement.
Lilianna Vasquez	38	1979/7/8	703-433-5199x803	[email protected]	2418 Daniel Canyon	A 43-year-old man with a past medical history of HIV, bipolar disorder, and epilepsy was admitted to the neurology service for a video electroencephalogram (vEEG) to characterize syncopal episodes that were felt to be epileptic in origin. These episodes started about ten years priorly and were associated with a preceding aura followed by loss of consciousness. He described this aura as a numbness that spread from head to toe and left him unresponsive for several minutes. He denied posturing, incontinence, eye deviation, tongue biting, or clonic activity during the events. He reported postevent disorientation and lethargy lasting from several hours to a full day. He had been on multiple antiepileptic medications without resolution of his episodes. On examination, the patient was an anxious appearing well-built gentleman with normal vital signs and normal cardiac and neurologic exams. During the vEEG, he experienced symptoms of his typical aura. Concurrently, his vEEG activity was normal, but his cardiac telemetry demonstrated pauses of up to six seconds (), reflecting a transient symptomatic high degree AV block. He was transferred to the cardiac care unit for close monitoring and temporary pacemaker placement. An MRI of the brain and neck showed a mass in the left posterior carotid space at the skull base (). The differential of this mass included a left vagal schwannoma or a lymph node in proximity to the vagus nerve. Neurosurgery declined surgical intervention, recommending Lamotrigine as seizure prophylaxis and serial MRIs to examine progression of the mass. The patient underwent permanent dual chamber MRI-compatible pacemaker placement for his heart block during the admission and was discharged with plans for outpatient follow-up. Follow-up MRI after 4 months revealed a stable left carotid space mass. The patient denied further syncopal episodes since pacemaker implantation. He did, however, endorse episodes of numbness, decreased vision, and change in alertness occurring nearly daily, for which he was advised to consult his cardiologist.
Rowan Boyle	39	1997/12/3	540.766.6680x1856	[email protected]	6882 Gutierrez Green	The patient was a 22 year old woman who was referred to the surgery ward early in the February 2014 from the orthopedic ward for multidisciplinary surgery. Patient had a mass in anterior thorax and clavicle, discovered 6 months earlier. It had grown steadily and was not painful at first but the patient complained of pain at the time of referral. She had no history of alcohol or drugs, nor did she have a history of smoking. She had no family history of known diseases. In examination, a large mass with the dimensions of 106 centimeters was discovered, taking up two thirds of the clavicle. Head, neck, heart, lungs, organs and the pulse were normal in physical examination. Needle biopsy The patient underwent core needle biopsy and was diagnosed as grade II chondrosarcoma. CT scan and MRI In CT scan and MRI, a 9157*59 millimeters mass was observed, proximal. There was no metastasis (). Due to the tumor? situation and its proximity to neurovascular network, the patient was sent to Shariati Hospital's surgery ward to be operated on by a multidisciplinary team consisting of thoracic, vascular, orthopedic surgeons. Decision on surgical resection of the mass was made after a review meeting between the mentioned surgical team and oncologists. Pathology report Pathologic stage is definitely important in prognosis, grade I chondrosarcoma has low malignant nature while grade II and grade III chondrosarcoma are high malignant. Five year survival in grade I is 90%, it falls to 60% for grades II and III. In the aforementioned patient the chondrosarcoma was grade II ( and ).
Aliya Sandoval	36	1992/6/25	879-974-0959	[email protected]	1129 Paul Locks	A 74-year-old woman underwent emergency replacement of the ascending aorta because of Stanford type A acute aortic dissection at another hospital. The entry of the Debakey type I aortic dissection was located in the middle of the ascending aorta, and extended distally to the distal abdominal aorta. The brachiocephalic artery and left common iliac artery were also dissected. Graft replacement of the ascending aorta was done, which were used by Teflon felt strip and GRF glue to reinforce the anastomosis of the dissection aortic wall. The post-operative course was unremarkable except for leg paresis that might have been due to spinal infarction caused by the dissection. She was diagnosed with a small cerebral infarction, jaundice and anemia 10 years later and admitted to our hospital. Blood tests revealed severe hemolytic anemia. The laboratory results were Hb, 7.3 g/dl; T. BIL, 4.06 mg/dl; and LDH, 3020 IU/l. Microscopy revealed fragmented red blood cells. No stigmata were found that could be associated with TTP or ITP. CT revealed severe stenosis at the proximal anastomosis of the inner felt strip and aortic intimal flap (Fig. ), which was one of two layers of Teflon felt strips positioned using the sandwich technique to reinforce the dissecting aortic wall. The pressure gradient at the site was not measured because it was impossible to insert a catheter. Transthoracic echocardiography (TTE) showed mosaic pattern in the felt flap of the ascending aorta although pressure gradient could not be measured (Fig. ). We thus considered that the cause of the hemolytic anemia was mechanical injury caused by disrupted blood flow and repaired the proximal anastomosis. The patient underwent a second operation. ECC was established via a median sternotomy with a cannula at the distal site of the ascending aortic graft and a two-staged venous cannula in the right atrium. A thrill was felt at the ascending aorta, and we cross-clamped the distal site-of the ascending aorta. We opened the prosthetic graft to explore the proximal anastomosis and found that the inner felt strip was turned up and that it had reduced the diameter of the inner lumen (Fig. ). We also identified local dissection and intimal flap of the proximal site of ascending aorta at non cusupid valve site (Fig. ). The proximal site of the graft, local dissection of the ascending aorta and the Teflon strips were removed, and the ascending aortic was replaced once again with a 22-mm graft that was directly sutured to the previous graft at the distal site of the ascending aorta. The patient was quite easily weaned from ECC and hemostasis achieved without difficulty. The postoperative course was uneventful, and no fragmented red blood cells or progression of anemia was observed. The serum LDH and T.BIL levels gradually returned to normal.
Brantley Wilcox	29	1978/3/20	6974403953	[email protected]	2530 Williams Parkways	A 17-year-old Asian male adolescent was admitted to our hospital with a chief complaint of sudden-onset fever and altered consciousness. He had a 6-month history of DM complicated by HLH controlled with corticosteroids (60 mg daily) and no obvious family, psychosocial, and genetic history. At the time of diagnosis (6 months prior to admission), heliotrope eruption was seen. A blood examination showed no evidence of myositis-specific antibody (anti-aminoacyl antibody). A muscle biopsy revealed lesions that were consistent with myositis; he met the diagnostic criteria of DM. A positron emission tomography-computed tomography (PET-CT) examination (Fig. ), bone marrow examination, and liver biopsy had shown no malignancy; therefore, his corticosteroid dose was gradually reduced to 8 mg daily. However, he experienced intermittent leg pain, and laboratory data showed a gradual elevation of creatine kinase (CK) levels and progression of leukopenia over the preceding 6 months; therefore, his steroid therapy was increased to 30 mg daily. On the day of admission, he was irritable, but there were no remarkable findings on physical examination. Laboratory data showed increased levels of CK (1869 U/L), soluble interleukin-2 receptor (4120 U/mL), and ferritin (967.4 ng/mL) and decreased counts of white blood cells (3910/弮L) and platelets (124,000/弮L). C-reactive proteins were hardly detected (0.01 mg/dL). We suspected exacerbation of DM and HLH. Active hemophagocytosis and no malignancy were observed on bone marrow examination. Brain magnetic resonance imaging (MRI) revealed multiple bilateral subcortical lesions (Fig. ). Systemic computed tomography (CT) did not show any evidence of interstitial pneumonia or malignancy. Cerebrospinal fluid (CSF) aspiration revealed the presence of 12 cells (nine neutrophils and 3three lymphocytes), glucose levels of 57 mg/dL, protein levels of 44 mg/dL, adenosine deaminase levels of 3.9 (>1) U/L, and interleukin-6 levels of 91.9 (>4.3) pg/mL. Polymerase chain reaction (PCR) analyses of his CSF showed no evidence of a recent infection with the Epstein?arr virus, cytomegalovirus, varicella zoster virus, human herpes virus 6, and herpes simplex virus. We could not perform a PET-CT examination on admission because of his altered consciousness. On the basis of the MRI findings, elevation of CSF interleukin-6 levels, peripheral blood cell count, and biochemistry results, and the hemophagocytosis observed in the bone marrow, we diagnosed the CNS lesions as leukoencephalopathy secondary to DM and HLH. The clinical course of our patient is presented in Fig. . Dexamethasone (DEX) pulse therapy (20 mg daily for 3 days) and intravenous immunoglobulin therapy (400 mg/kg/day for 5 days) were initiated on the day of admission to treat his CNS lesions. Methylprednisolone (mPSL; 60 mg daily) was administered post-treatment. His CK levels improved rapidly (from 1869 U/L on the day of admission to 287 U/L on day 14); however, his bicytopenia (leukopenia and thrombopenia) remained unchanged, and his CNS lesions were still observed. Therefore, cyclophosphamide pulse therapy was added on day 8. On day 24, a second bone marrow examination revealed that his hemophagocytosis remained, for which cyclosporine was started the next day. On day 28, the 60 mg daily mPSL was changed to 20 mg daily DEX because DEX diffuses to the CSF more easily than does mPSL. In addition, as his hemophagocytosis persisted, etoposide (120 mg/body) was started on day 38 and was administered once per week between days 38 to 89 (eight times in total). On day 47, tacrolimus was administered instead of cyclosporine to suppress hemophagocytosis because both drugs have the same effects and because of a severe liver function disorder due to cyclosporine (aspartate transaminase and alanine transaminase levels increased to 318 and 917 IU/mL, respectively). Subsequently, his bicytopenia (leukopenia and thrombopenia) recovered to within normal ranges, and his CNS lesions disappeared. He was administered sedative drugs to produce a state of calmness, to help him sleep, and to prevent acts of violence caused by his altered state of consciousness that lasted for 5 months. The medications were eventually discontinued because altered consciousness was no longer observed. However, disturbances of his higher cerebral functions remained. Five months after his initial admission, he could speak and walk like a very young child. Corticosteroid therapy was successfully tapered without a relapse of his symptoms (including fever and altered consciousness), and without changes in his laboratory and MRI findings. Eight months after admission, a follow-up brain MRI revealed a recurrence of the CNS lesions (Fig. ), although these recurrent lesions were different from the previous lesions, considering that there was no fever and no altered consciousness. Moreover, his laboratory findings were normal. We hypothesized that the pathology of the new CNS lesions differed from the pathology of the previous lesions and presumed that they were caused by progressive multifocal leukoencephalopathy or drug-induced (tacrolimus) leukoencephalopathy. However, a PCR analysis for the John Cunningham virus in his CSF (to diagnose progressive multifocal leukoencephalopathy) was negative, and no improvement in CNS lesions was observed when tacrolimus was discontinued. One month after the recurrence of the CNS lesions, his abilities to walk and speak deteriorated. A MRI revealed a progression of the CNS lesions; therefore, a brain biopsy of his right frontal lobe was performed on day 297. On day 325, a diagnosis of peripheral T-cell lymphoma (PTCL) was made based on the T-cell receptor rearrangement seen in the tumor cells of his brain specimen. Therefore, on day 330, chemotherapy combined with high-dose methotrexate and cytarabine was initiated. A follow-up MRI on day 345 revealed that his CNS lesions had increased in size, suggesting that the chemotherapy regimen was ineffective, and he died of sepsis on day 348. An autopsy revealed yellowish to brownish extended geographic lesions with softening, atrophy, and cavitation in the white matter of the frontal, temporal, and occipital lobes. Hepatomegaly (1910 g) and splenomegaly (205 g) were also observed. Microscopy of the cerebral white matter showed bilateral extended multiple liquefaction necroses (Fig. ). Infiltration of predominantly CD8(+) T cells undergoing dyskaryosis or mitosis was observed in the Virchow?obin space (Fig. ), bone marrow, liver, and spleen. The rearrangements of the TCR gene were confirmed by PCR of a brain specimen. Hemophagocytosis was observed in the spleen and bone marrow. On immunohistochemistry, no cells were positive for herpes simplex virus or John Cunningham virus, and Epstein?arr virus-encoded ribonucleic acid in situ hybridization was negative.

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