Case 1:

Chief Complaint Three days of dyspnea and increased edema.

History of Present Illness The patient was a 47-year-old woman with a past medical history significant for cirrhosis, diabetes, and chronic pancreatitis. She never received a liver biopsy, but because of a long history of alcohol abuse was presumed to have alcoholic cirrhosis. In 2/2004, she was first admitted to The Johns Hopkins Hospital for esophageal varices and ascites. She underwent successful transjugular intrahepatic portal shunt (TIPS) placement. During this admission, she developed acute renal failure and an episode of bradycardic arrest. In 3/2004, the patient had an echocardiography, which showed an ejection fraction of 60%. In 7/2007, she was diagnosed with hepatocellular carcinoma. Shortly thereafter, she was admitted to The Johns Hopkins Hospital and underwent successful hepatic chemoembolization. The patient continued to have worsening liver function, and she was placed on the transplant recipient list. On 2/21/2008, she was admitted to The Johns Hopkins Hospital and underwent successful orthotopic liver transplantation. During this admission, echocardiography identified decreased systolic function with an ejection fraction of 25%. Her explanted liver showed advanced micronodular cirrhosis with marked hepatocellular and bile duct hemosiderin. At the chemoembolization site there was focal necrosis and fibrosis, no evidence of malignancy. Her post-operative course was unremarkable and she recovered well and was discharged from Hopkins. On follow- up in clinic, she developed hyperkalemic renal insufficiency and edema. She was readmitted to the Johns Hopkins Hospital in March and also in April of 2008 for failure to thrive, renal insufficiency, and leg edema. Echocardiography at that time identified distal septal and apical hypokinesis with an ejection fraction of 35%. On 6/16/2008, the patient was transferred to The Johns Hopkins Hospital from an outside institution following three days of dyspnea and increased edema. In the outside hospital, she was hypotensive with systolic pressures in the 80s and diastolic pressures in the 50s. She was also hypothermic with temperatures as low as 33C with hypoglycemia.

Past Medical History 1. ESLD s/p TIPS in 2/08, history of variceal bleeding, hepatic encephalopathy, hepatic hydrothorax s/p R pleurodesis 2. HCC diagnosed 2007 s/p RFA 3. Orthotopic liver transplant on 2/21/2008 (recipient and donor CMV negative). 4. Post-transplant course complicated by acute renal failure in 3/2008 (Cr in the 2 range) due to Prograf, Klebsiella oxytoca bacteremia 4/12/2008, 5. Cardiomyopathy-recent diagnosis 6. Hypothyroidism- >5 years 7. Diabetes- Type II; >5 years

Family History There is no prior known history of liver disease or Cardiomyopathy. Her father had diabetes and hypertension.

Social History She smokes 1/2 pack per day but says she is trying to quit. She was a heavy alcohol drinker up until 02/04. She has stopped completely ever since. She had tried pot at a younger age but has not used illicits otherwise. She worked for a restaurant up until her hospitalization in 02/04. She is a widow and she has 2 healthy children. She lived with her children in Jessup, but now lives with her sister who is her primary caretaker and aide. She has no pets and has not travelled recently. Medications (6/22) Levothyroxine Pantoprazole Hydrocortisone DRIPS: Levophed (2mcg/Kg/min) Vasopressin (0.04 - stable) Dobutamine (100 mcg/Kg/min) ANTIBIOTICS: Ambisome 300 mg q24 (5mg/Kg/q24) 6/20 - now Vancomycin dosed by levels now 6/16 - now Cefepime 500 mg q12 6/21 - now (On admission the patient was on prophylactic fluconazole, dapsone and valcyte) IMMUNOSUPPRESSION: Sirolimus 0.5 mg daily

Allergies Codeine

Review of Systems Could not be obtained

ANTIBIOTICS: Ambisome 300 mg q24 (5mg/Kg/q24) 6/20 - now Vancomycin dosed by levels now 6/16 - now Cefepime 500 mg q12 6/21 - now (On admission the patient was on prophylactic fluconazole, dapsone and valcyte) IMMUNOSUPPRESSION: Sirolimus 0.5 mg daily

Allergies Codeine

Review of Systems Could not be obtained

Physical Exam (6/22) General: intubated, alert, follows commands, no acute distress Vitals: Tc 35.6, Tmax 35.6, BP 80s/60s, HR 100-120s, RR 24, O2 sat 97%, Weight 58 Kg (has gained 9 lbs since admission) HEENT: ETT in place, icteric, pale conjuctiva, no sinus tenderness, L IJ CVC clean, supple neck, moist mucous membranes Pulmonary: coarse breath sounds bilaterally, no wheezing, no rales Cardiac: tachycardic, regular, normal S1 and S2, systolic murmur in apex, S3 present Abdomen: soft, distended, nontender, bowel sounds decreased, abdominal wall edema, no Murphy sign Extremities: anasarca, extremities cool with thread pulses, distal fingers/toes slightly blue Lymph nodes: no lymphadenopathy

Laboratory Values (6/22) WBC: 7.7 (85% N) Hgb: 7.8 PLTs: 18,000 Fibrinogen: 139 PT/PTT/INR: 35.1/44/4 ABG: 7.32/31/126 BuN/Cr: 31/1.2 (Cr peaked at 2.8 - was 2.1 on admission) CO2: 15 Anion gap: 22 AST: 7141 ALT: 1236 ALP: 409 TBili: 2.2 Alb: 1.9 Ammonia: 65 Lactic acid: 9.6 AfP < 1 CK: 432 Troponin: 1 TSH: 0.7 CMV PCR- negative DRUG LEVELS: Rapamycin: 8.1 MICRO LABS: all cultures negative

EKG SINUS TACHYCARDIA RIGHT BUNDLE BRANCH BLOCK LEFT POSTERIOR FASICULAR BLOCK BIFASCICULAR BLOCK

Clinical Course After admission to the Johns Hopkins Hospital Surgical ICU, she was given antibiotics for possible sepsis, and then was transferred to the floor. All cultures remained negative. However, her blood pressure remained low. On 06/19/08, a cardiology consult did a bedside echo showing a large left pleural effusion and an ejection fraction of 10%. She was then transferred to the CCU for hypotension and cardiogenic shock. A PA catheter demonstrated low cardiac output and high right atrial pressure. A pulmonary capillary wedge pressure could not be obtained. During the rest of her hospitalization, she was routinely hypotensive requiring pressor support. On 6/21/08 she had an episode of cardiac arrest due to PEA/V-tach. She was resuscitated and intubated. Following this episode, she developed severe renal failure and was started on continuous venovenous hemodialysis. Despite these measures, her condition rapidly deteriorated. On 6/27/2008, she had a second PEA cardiac arrest. Cardiopulmonary resuscitative efforts this time were unsuccessful, and she expired at 1641.

LEFT POSTERIOR FASICULAR BLOCK BIFASCICULAR BLOCK

Clinical Course After admission to the Johns Hopkins Hospital Surgical ICU, she was given antibiotics for possible sepsis, and then was transferred to the floor. All cultures remained negative. However, her blood pressure remained low. On 06/19/08, a cardiology consult did a bedside echo showing a large left pleural effusion and an ejection fraction of 10%. She was then transferred to the CCU for hypotension and cardiogenic shock. A PA catheter demonstrated low cardiac output and high right atrial pressure. A pulmonary capillary wedge pressure could not be obtained. During the rest of her hospitalization, she was routinely hypotensive requiring pressor support. On 6/21/08 she had an episode of cardiac arrest due to PEA/V-tach. She was resuscitated and intubated. Following this episode, she developed severe renal failure and was started on continuous venovenous hemodialysis. Despite these measures, her condition rapidly deteriorated. On 6/27/2008, she had a second PEA cardiac arrest. Cardiopulmonary resuscitative efforts this time were unsuccessful, and she expired at 1641.

case 2:

Chief Complaint Bilateral lower quadrant abdominal pain History of Present Illness The patient is a previously healthy 30-year-old male who presented to his physician with a chief complaint of stabbing bilateral lower quadrant abdominal pain. The pain began somewhat abruptly five days before he sought medical attention and had not significantly changed over that time period. The patient described the pain as worse with sudden movements, such as while riding in the car over uneven pavement. He rated the pain as a 6 to 7 out of 10 at baseline, increasing to an 8 or 9 out of 10 with motion. Though he felt the pain was somewhat similar to severe constipation, he described having regular bowel movements throughout. Oxycodone was the only medication to relieve the pain. He denied any vomiting, diarrhea, change in stool frequency or bloody stools. He also denied any urinary frequency, urgency or dysuria. In addition to his abdominal pain, the patient complained of about two weeks of right leg pain and numbness. Around the time his leg pain began, the patient had an acute episode of left scrotal swelling which was felt to be due to infection. He was prescribed an antibiotic but never filled the prescription, and the swelling resolved spontaneously. He denied any pain or numbness in his left leg and experienced no swelling of the right scrotum. Because of findings on an abdominal CT scan, the patient was referred to The Johns Hopkins Hospital for evaluation. Past Medical & Surgical History Appendectomy for acute appendicitis (1990) Family History Both parents and all siblings alive and well. Maternal aunt with ovarian cancer. Social History The patient is employed as a construction supervisor. He is married and has one son who is 5 years old and healthy. The patient occasionally drinks alcoholic beverages socially and has recently attempted to quit smoking using nicotine patches. Prior to this attempt, he had smoked 1 to 1 packs of cigarettes per day for 9 years. Medications Oxycodone p.o. and nicotine patch. Allergies No known drug allergies. Review of Systems Few night sweats and mild generalized pruritus. Slightly decreased appetite. Some easy bruising but no excessive bleeding. Physical Exam on Admission Weight: 201 lbs Height: 6 feet T: 97.5 BP: 108/70 P: 80 General: Caucasian male consistent with stated age. Appears mildly uncomfortable. HEENT: Sclera anicteric. EOM intact. No lymphadenopathy. Normal thyroid. CV: Regular rate and rhythm without murmur. No carotid bruits. Lungs: Clear to auscultation bilaterally. No rales or rhonchi. Abdomen: Diffusely tender nondistended abdomen, most tender in the left lower quadrant. No palpable mass, though examination limited by patient discomfort. Normoactive bowel sounds. No rebound tenderness. No inguinal hernias or inguinal lymphadenopathy. Genitalia: Left testicle slightly larger than the right with some tenderness to palpation on the left. Extremities: No pedal edema. Laboratory Values Na 139; K 4.0; Cl 103; BUN 11; Cr 0.7; Glucose 94; Calcium 8.3; TP 5.9; Albumin 3.4; TBili 0.2; AST 29; ALT 44; Alk Phos 93 WBC 11,490; RBC 4.72; Hemoglobin 15.2; Hct 42.9; MCV 90.9; Platelet 293 PT 10.1; APTT 27.9 LDH 332; AFP 416; HCG < 2 Radiologic Studies (Images 1-3) Plain film of abdomen: Vague suggestion of mass in left mid abdomen. Psoas shadow not obliterated. CT abdomen & pelvis : Left retroperitoneal soft tissue mass (9.4 x 4.4 cm) extending to involve a prominent left paraaortic lymph node (2.4 x 2.5 cm). Normal liver, spleen, pancreas, gallbladder, adrenals and kidneys. Normal caliber bowel loops. Minimal pelvic free fluid. No bulky pelvic lymphadenopathy. Scrotal ultrasound: Left varicocele and small bilateral hydroceles.

case3: Chief Complaint Subacute, progressive neurological decline History of Present Illness The patient is a previously healthy 39-year-old male with past medical history significant for remote traumatic brain injury (1978) and well-controlled seizure disorder (2002) who recently presented to an outpatient neurology clinic following two months of a progressive neurological decline. His decline began following an unusual headache associated with diplopia, blurred vision, generalized weakness and slurred speech. These symptoms resolved after an hour; however, they were followed by a subacute decline with progressive inability to perform daily activities. Since onset of the headache, the patient experienced personality changes including social withdrawal and depression, and worsening falls, dysarthria, and dysphagia. In the clinic, he was noted to have a slow, shuffling gait, retropulsion, and marked hypophonia bordering on mutism. Following an unrevealing MRI, EEG, and LP, he was treated for possible early-onset Parkinson's disease with ropinirole and returned home. A short time later, however, the patient presented to The Johns Hopkins Hospital with a dramatic oculomotor disorder characterized by decreased and intrusive eye movements and dramatic bifrontal hypofunction including emotional incontinence, utilization behavior and perseveration. At the time of his admission, he continued to have bradykinesia, anarthria, and dysphagia. Further, he reported intermittent fevers (to 102F) and diarrhea over the past few weeks. Past Medical & Surgical History Status post traumatic left frontal brain injury following a motor vehicle accident with ensuing coma (4 days) and resultant

case3: Chief Complaint Subacute, progressive neurological decline History of Present Illness The patient is a previously healthy 39-year-old male with past medical history significant for remote traumatic brain injury (1978) and well-controlled seizure disorder (2002) who recently presented to an outpatient neurology clinic following two months of a progressive neurological decline. His decline began following an unusual headache associated with diplopia, blurred vision, generalized weakness and slurred speech. These symptoms resolved after an hour; however, they were followed by a subacute decline with progressive inability to perform daily activities. Since onset of the headache, the patient experienced personality changes including social withdrawal and depression, and worsening falls, dysarthria, and dysphagia. In the clinic, he was noted to have a slow, shuffling gait, retropulsion, and marked hypophonia bordering on mutism. Following an unrevealing MRI, EEG, and LP, he was treated for possible early-onset Parkinson's disease with ropinirole and returned home. A short time later, however, the patient presented to The Johns Hopkins Hospital with a dramatic oculomotor disorder characterized by decreased and intrusive eye movements and dramatic bifrontal hypofunction including emotional incontinence, utilization behavior and perseveration. At the time of his admission, he continued to have bradykinesia, anarthria, and dysphagia. Further, he reported intermittent fevers (to 102F) and diarrhea over the past few weeks. Past Medical & Surgical History Status post traumatic left frontal brain injury following a motor vehicle accident with ensuing coma (4 days) and resultant encephalomalacia, but no clear residual functional deficit (1978, age 13). Seizure disorder characterized by generalized tonic-clonic seizures, well-controlled with medications (2002). Family History Both parents and his brother and sister are alive and well. Both parents and both siblings have Crohns disease. In recent years, his father has experienced memory loss of unclear etiology, possibly due to Alzheimer's disease or vascular disease. His brother suffers from depression. Social History The patient is a college graduate, earning a Masters degree in Economics. Prior to the recent onset of his symptoms, he was a self-employed consultant and was a physically active marathon runner and cyclist. Although a functional member of society, his social interactions were reportedly awkward and possibly deviant, leading to job instability in the 1990s. Over the past two months, he experienced job instability related to his personality changes and neurologic decline. He denies use of alcohol, tobacco, and illicit drugs. Medications Valproic Acid 500 mg po BID, Ropinerole 0.25 mg po TID, Zaleplon 5 mg po qhs Allergies No known drug allergies. Review of Systems Intermittent mild to moderate fever. Diarrhea. Physical Exam Weight: 99 lbs Height: 5'2" T: 99.7 RR: 16 BP: 128/83 P: 100-110 General: Thin, well-developed Caucasian male consistent with stated age. Anxious piercing gaze, despite a bland emotionless face. HEENT: Sclera anicteric. Clear oropharynx. No lymphadenopathy. Normal thyroid. CV: Regular rate and rhythm without murmur. No carotid bruits. Lungs: Clear to auscultation bilaterally. No rales or rhonchi. Abdomen: Soft, and nontender to palpation. Normoactive bowel sounds. No inguinal hernias or inguinal lymphadenopathy. Genitalia: Descended, non-tender testes, without palpable masses. Normal digital rectal exam. Extremities: No clubbing, cyanosis or edema. Skin: No rashes Neurologic Examination: Mental State: Alert and oriented to person, date, and location. Affect and behavior: Disinhibited, with emotional incontinence, perseveration, utilization behavior and a positive clapping sign. Communication: Near mute, but able to communication at a sophisticated level via slow typing on a computer or hand motions (thumbs up - yes, thumbs down - no). Comprehension: Able to follow multi-part commands and type sentences and paragraphs. Able to perform serial seven calculations. Cranial Nerves II-XII: Static eyelid retraction and fluttering exacerbated by attempted eye movements. Reflex blepharospasm. Pupils equal and reactive to light. Incomplete mixed vertical and horizontal supranuclear gaze palsy with frequent intrusions. Intrusions included both back-to-back, low-amplitude saccades (multi-vectorial [vertical/torsional > horizontal]) and slow intrusions (mostly torsional). There were unusual nystagmoid movements on attempted convergence. Visual fields full to confrontation. Symmetric face. Midline tongue and uvula. No palatal myoclonus or adventitious jaw movements. Dysphagia including difficulty swallowing. Motor Exam: Strength - 5/5 bilaterally. Tone - rigid without cogwheeling, most pronounced in the upper extremities. Coordination - largely intact but bradykinetic. Difficulty with rapid alternating movements. Gait Small, slow, shuffling steps with a tendency to retropulse when standing with feet together (positive Romberg). Bulk, sensation and reflexes - within normal limits. Laboratory Values Na 132; K 4.1; Cl 94; BUN 18; Cr 0.8; Glucose 87; Calcium 10.0; TP 7.8; Albumin 5.0; TBili 0.5; AST 21; ALT 36; Alk Phos 42 WBC 8,750; RBC 4.58; Hemoglobin 14.8; Hct 41.0; MCV 89.5; Platelet 267 PT 11.2; INR 1.0; APTT 28.9 Vitamin B12 435; Vitamin B1 111; RBC folate 447; TSH 1.78; pyruvate 0.03; lactic acid 0.7, Ammonia 25, Magnesium 1.8 RPR non-reactive, HIV negative, Lyme disease antibody negative SPEP and UPEP without evidence of a monoclonal gammopathy CSF (Lumbar Puncture): WBC 2 (mononuclear cells); RBC 520/0; glucose 56; protein 60; 14-3-3 protein <2.0; cryptoccocus antigen negative; PCR negative for Tropheryma Whipplei, EBV, CMV, VZV, HSV, and JC; culture negative for bacteria and fungi; PSA 0.2; AFP 15, CEA 4.1, hCG negative ESR 3; CRP 18.8; Negative ANA, anti-DNAse, Anti-Ro, Anti-La, and Rheumatoid Factor Heavy metal urine screen (arsenic, lead, and mercury) negative Radiologic Studies CT, head: Left greater than right encephalomalacia, consistent with prior trauma. Increased right frontal hypodensity compared to prior scan (two years prior). MRI, brain (Image 1): Serial axial fluid-attenuated inversion recovery (FLAIR) images show (A) bifrontal encephalomalacia indicating previous traumatic injury. In addition, there is increased signal intensity in the (B) bilateral globus palladi, (C) right greater than left substantia nigra, (D) hypothalamic regions adjacent to the inferior third ventricles, and a hyperintense lesion in the left temporal white matter. No associated mass effect or enhancement. CT, chest, abdomen and pelvis: Negative for occult tumor and lymphadenopathy. FDG-PET brain and body: No metabolic abnormality. Biopsy Results Duodenal biopsy: Duodenal mucosa with non-diagnostic findings. Negative for T. whipplei by routine H&E stain, PAS stain and immunohistochemistry. Right Brain Biopsy: Histologically unremarkable dura and deep white matter. Cortical brain with vascular changes, including thickened vessels negative for amyloid on Congo red stain. No evidence of fungi (GMS), bacteria (Brown and Hopps) and acid fast organisms (AFB) by special stains. No evidence of prion protein, Herpes virus, adenovirus, papova virus, or T. whipplei by immunohistochemical stains.

cryptoccocus antigen negative; PCR negative for Tropheryma Whipplei, EBV, CMV, VZV, HSV, and JC; culture negative for bacteria and fungi; PSA 0.2; AFP 15, CEA 4.1, hCG negative ESR 3; CRP 18.8; Negative ANA, anti-DNAse, Anti-Ro, Anti-La, and Rheumatoid Factor Heavy metal urine screen (arsenic, lead, and mercury) negative Radiologic Studies CT, head: Left greater than right encephalomalacia, consistent with prior trauma. Increased right frontal hypodensity compared to prior scan (two years prior). MRI, brain (Image 1): Serial axial fluid-attenuated inversion recovery (FLAIR) images show (A) bifrontal encephalomalacia indicating previous traumatic injury. In addition, there is increased signal intensity in the (B) bilateral globus palladi, (C) right greater than left substantia nigra, (D) hypothalamic regions adjacent to the inferior third ventricles, and a hyperintense lesion in the left temporal white matter. No associated mass effect or enhancement. CT, chest, abdomen and pelvis: Negative for occult tumor and lymphadenopathy. FDG-PET brain and body: No metabolic abnormality. Biopsy Results Duodenal biopsy: Duodenal mucosa with non-diagnostic findings. Negative for T. whipplei by routine H&E stain, PAS stain and immunohistochemistry. Right Brain Biopsy: Histologically unremarkable dura and deep white matter. Cortical brain with vascular changes, including thickened vessels negative for amyloid on Congo red stain. No evidence of fungi (GMS), bacteria (Brown and Hopps) and acid fast organisms (AFB) by special stains. No evidence of prion protein, Herpes virus, adenovirus, papova virus, or T. whipplei by immunohistochemical stains. Neurological Studies Electromyogram: Within normal limits. EEG: Posterior basic rhythm slowing, no seizure discharge or localizing signs. Fundoscopic exam with slit lamp biomicroscopy: No evidence of uveitis or vitreitis. Clinical Course Despite a thorough work up for metabolic, autoimmune, neoplastic, and infectious etiologies, a definitive cause of the patient's symptoms was not identified. The patient began empiric treatment with intravenous Ceftriaxone and experienced a notable improvement of his eye movements with a decrease in intrusive eye movements, an improvement in his swallowing difficulties, and an improvement in his social interactions. His gait and speech, however, remained unchanged. Overall, his symptoms waxed and waned, and his response to Ceftriaxone was considered equivocal.

case4:

Chief Complaint Several days of increased lethargy

History of Present Illness The patient is a 54 year old African-American female with a 2-3 year history of cirrhosis with diuretic refractory ascites requiring intermittent large volume paracentesis admitted to Johns Hopkins Hospital from home with 2 days of increasing lethargy and confusion after a recent paracentesis. The patient was diagnosed with idiopathic cirrhosis approximately 3 years ago at an outside hospital after presenting with increasing abdominal girth and easy bruising. She had no evidence of infectious or autoimmune hepatitis by laboratory markers. Iron saturation and transferrin were not elevated. There was no history of alcohol consumption, drug ingestion, or toxin exposure. The patient is a Jehovah's Witness and refused liver biopsy or transplant evaluation because of the potential need for blood transfusion. Over the last 2 years she had paracentesis approximately monthly. There was no history of acute variceal bleeding. She has had a few episodes of worsening encephalopathy that were treated with protein restriction and increased lactulose. Her other past medical history is notable for obesity and >20 years Type 2 diabetes. She failed weight loss strategies and oral diabetes treatment and has been on insulin for more than 15 years. Her family reports that she is often non-compliant with her insulin. She has had numerous admissions to an outside hospital for episodes of hypoglycemia and hyperglycemia. Over the past 5 years, she's been followed for chronic kidney disease with a baseline creatinine of 1.4. Three days prior to admission, she had a routine paracentesis at a local surgical center. The next day her family noted her to have increased lethargy and some confusion. They gave her more lactulose but her symptoms progressed. While visiting a nephew in Fells Point she was barely arousable and confused so her family brought her to Johns Hopkins Hospital.

Past Medical History 1. Cirrhosis with ascites and encephalopathy 2. Type 2 diabetic, currently on insulin 3. Chronic kidney disease with a baseline creatinine of 1.4 4. Obesity 5. Possible portal pulmonary hypertension with an RVSP of 56 by echocardiogram 6. History of neck abscess, status post incision and drainage a few years ago 7. History of C-section in 1970, 1973 8. History of ingrown toenail removal when she was 9 years old.

Family History Mother had diabetes and kidney complications secondary to diabetes. She also had an amputation secondary to diabetes and died in her late 50's. Her father had hypertension and he also died in his late 50's. She is divorced for 25 years and is not sexually active. She has 2 Children who are healthy

Social History The patient denies smoking, alcohol, illicit drug use. She is a Jehovahs Witness who is a leader in her local church. Prior to her liver disease, she was active in a variety of church and leadership activities. Until 10 years ago, she worked as a shift supervisor at the Comcast call center. She has no travel history outside of the Mid-Atlantic, lives in the suburbs of Howard County and has no pets.

Medications 1. Furosemide 40 mg by mouth twice a day. 2. Lactulose 30 mg every 6 hours, titrated to bowel movements 2- 3 times a day. 3. Metronidazole 250 mg by mouth every 8 hours. 4. Pantoprazole 40 mg by mouth daily.

Family History Mother had diabetes and kidney complications secondary to diabetes. She also had an amputation secondary to diabetes and died in her late 50's. Her father had hypertension and he also died in his late 50's. She is divorced for 25 years and is not sexually active. She has 2 Children who are healthy

Social History The patient denies smoking, alcohol, illicit drug use. She is a Jehovahs Witness who is a leader in her local church. Prior to her liver disease, she was active in a variety of church and leadership activities. Until 10 years ago, she worked as a shift supervisor at the Comcast call center. She has no travel history outside of the Mid-Atlantic, lives in the suburbs of Howard County and has no pets.

Medications 1. Furosemide 40 mg by mouth twice a day. 2. Lactulose 30 mg every 6 hours, titrated to bowel movements 2- 3 times a day. 3. Metronidazole 250 mg by mouth every 8 hours. 4. Pantoprazole 40 mg by mouth daily. 5. Propranolol 10 mg by mouth twice day. 6. Spironolactone 100 mg by mouth daily. 7. Darbepoietin injection 40 mcg subcutaneously every 7 days.

Allergies No known drug allergies

Physical Exam on Admission (2/26/2009) VITAL SIGNS: T 97.8, Pulse 112, RR 26, BP 80/46, Oxygen saturation 94% RA; Weight 115 kg, Dexi- stick 250 mg/dl GENERAL: Weak appearing female in mild distress who appears older than her stated age. Difficult to arouse. HEENT: PERRLA, scleral icterus, no cervical lymphadenopathy, normocephalic, atraumatic CARDIAC: JVP flat, regular rate and rhythm, normal S1 and S2. 3/6 murmur at the left upper sternal border RESPIRATORY: Clear to auscultation bilaterally GI/ABDOMINAL: She has ascites and her abdomen is tense. She has hypoactive bowel sounds. Abdomen nontender to palpation. Unable to palpate liver or spleen. NEUROLOGIC: Somnolent, oriented to person not place or date. Positive asterixis. Mild diffuse muscle wasting with slightly decreased strength. Reflexes normal. Sensation normal. SKIN: Hyperpigmented areas are noted. No ecchymoses EXTREMITIES: Extremities are remarkable for 2+ pitting edema to the knee.

Laboratory Values on Admission White blood cells: 16,070 (80% neutrophil, 12% band, 8% lymphocyte) Hematocrit: 34 % (Baseline 28%) Platelets: 71, 000 Sodium: 134, Potassium: 6.5, Chloride: 101, CO2: 13, BUN: 51, Glucose: 110, Creatinine: 3.1 aPTT 34.6 s, INR 2.1 Albumin 2.1 g/dL (reference range 3.5-5.3 g/dL) Total bilirubin 6.0 (reference range 0.1-1.2 mg/dL) ALT 43 U/L (reference range 0-40U/L) AST 169 U/L (reference range 0-37 U/L) Alkaline Phosphatase 251 U/L (reference range 30-120) Ammonia 39 mcmol/L (reference range 0-32 mcmol/L) Arterial blood gas on 2 L O2: 7.24/30/110

EKG Sinus Bradycardia; Left Axis Deviation, Delayed Precordial R Wave development

Radiologic Studies CT of the chest showed hypoinflation of lungs and increased density in the abdomen suggesting ascites (Image 1). Axial CT of the abdomen with oral contrast showed a nodular liver and large amount of ascites (Image 2). Coronal CT of the abdomen with oral contrast showed ascites and "wet bowel" pattern related to liver disease and hypoproteinemia (Image 3).

Clinical Course The patient was admitted to the medical intensive care unit for fluid resuscitation and broad spectrum antibiotics. Serial hematocrit studies showed no significant change. After receiving 5L of intravenous fluids, her blood pressure remained low and vasopressors were initiated. Bladder pressure was monitored to rule out abdominal compartment syndrome. She remained somnolent and confused. Her coagulopathy, thrombocytopenia, acute renal failure, and metabolic acidosis worsened. Her family reiterated her desire to receive no blood or plasma products. She was intubated and placed on mechanical ventilation for airway protection and hyperventilation. Despite maximal therapy, the patient had refractory hypotension and coagulopathy over the next 24 hours. After a family meeting vasopressors and mechanical ventilation were discontinued and the patient expired approximately 30 hours after admission.

case 5:Chief Complaint Shortness of breath and abdominal distention

History of Present Illness The patient is a 38 year-old African American male with a history of hypertension who was admitted to a nearby hospital during July, 2008 due to nausea, anorexia and jaundice. He was subsequently diagnosed with HIV/AIDS (CD4 ct. 34, viral load >150,000) and hepatitis B. The patient underwent paracentesis, at which time 2 liters of ascites fluid were removed. The

hematocrit studies showed no significant change. After receiving 5L of intravenous fluids, her blood pressure remained low and vasopressors were initiated. Bladder pressure was monitored to rule out abdominal compartment syndrome. She remained somnolent and confused. Her coagulopathy, thrombocytopenia, acute renal failure, and metabolic acidosis worsened. Her family reiterated her desire to receive no blood or plasma products. She was intubated and placed on mechanical ventilation for airway protection and hyperventilation. Despite maximal therapy, the patient had refractory hypotension and coagulopathy over the next 24 hours. After a family meeting vasopressors and mechanical ventilation were discontinued and the patient expired approximately 30 hours after admission.

case 5:Chief Complaint Shortness of breath and abdominal distention

History of Present Illness The patient is a 38 year-old African American male with a history of hypertension who was admitted to a nearby hospital during July, 2008 due to nausea, anorexia and jaundice. He was subsequently diagnosed with HIV/AIDS (CD4 ct. 34, viral load >150,000) and hepatitis B. The patient underwent paracentesis, at which time 2 liters of ascites fluid were removed. The ascitic fluid had a WBC= 100, Glucose= 70, Protein= 0.2, cultures negative. The patient was discharged two weeks later. Despite compliance with his medications, the patient re-developed abdominal distention with worsening icterus and abdominal pain. On August 16, 2008 the patient presented to The Johns Hopkins emergency department complaining of shortness of breath and abdominal distention.

Past Medical History 1. HIV/AIDS- diagnosed 7/2008 2. Hepatitis B- diagnosed 7/2008 3. Hypertension- >5 years

Family History Father- deceased, had cirrhosis due to alcoholic liver disease Mother- deceased, had rheumatic heart disease Siblings- 3 all without known medical problems Children- none

Social History Patient denies smoking, illicit drug and alcohol use. Lives with his sister in a suburban setting and is unemployed since July. Worked as a systems analyst for a home security company. Presumed to contract HIV/Hepatitis B due to homosexual contacts during vacation travel to Asia (Thailand, Malaysia, Taiwan) and Mexico over the last 10 years. Last steady sexual partner > 5 years ago, is healthy. Has 2 cats, no dogs. Hobbies include chess and Super Smash Brothers Brawl on his Wii.

Medications (8/16/2008) Pantoprazole PO Nystatin PO Hydromorphone HCL PO Azithromycin PO Spironolactone PO Bactrim PO Lasix PO Lactulose PO

Allergies NKDA

Physical Exam on Admission (8/16/2008) VITAL SIGNS: T 37.2 Pulse 110 RR 24 BP 142/74 O2 sat 95% on Room Air GENERAL: Ill-appearing, thin, icteric in mild distress. HEENT: Pupils are equally normal and reactive to light, extraocular muscles are intact. Marked scleral icterus. Marked thrush throughout orophraynx. Mouth and throat with dry mucus membranes. Fundoscopic examination shows no evidence of retinitis. CARDIAC: Tachycardic, regular rhythm, S1 and S2 normal. No gallops, rubs, or murmurs. VASCULAR: Normal 2+ pulses bilaterally. Normal capillary refill. No clubbing or cyanosis. 2+ edema bilaterally. RESPIRATORY: Tachypneic, decreased breath sounds in the left base with bilateral basilar crackles. GI/ABDOMINAL: Positive bowel sounds, abdomen distended with protruding umbilicus, positive fluid wave, tender to palpation throughout. Unable to palpate liver or spleen due to abdominal distension NEUROLOGIC: Awake and alert. No asterixus. Mild diffuse muscle wasting with slightly decreased strength. Reflexes normal. Sensation normal. SKIN: no telangectasia, rash, or lesions.

Laboratory Values on Transfer (8/16/2008) WBC: 12,080 /cu mm Hct: 29.3 % Hgb: 3.41 M/cu mm PLTs: 81 K/cu mm Na 125 K 4.5 Cl 93 Glucose 111 Cr 1.8 PT 20.7 (reference range 9.6-11.5 seconds) INR 2.1 (reference range 0.9-1.1) Albumin 2.1 g/dL (reference range 3.5-5.3 g/dL) Total bilirubin 23.5 (reference range 0.1-1.2 mg/dL) Direct bilirubin 10.0 mg/dL (reference range 0-0.4 mg/dL) ALT 164 U/L (reference range 0-40U/L) AST 333 U/L (reference range 0-37 U/L) Alk Phos 258 U/L (reference range 30-120)

SKIN: no telangectasia, rash, or lesions.

Laboratory Values on Transfer (8/16/2008) WBC: 12,080 /cu mm Hct: 29.3 % Hgb: 3.41 M/cu mm PLTs: 81 K/cu mm Na 125 K 4.5 Cl 93 Glucose 111 Cr 1.8 PT 20.7 (reference range 9.6-11.5 seconds) INR 2.1 (reference range 0.9-1.1) Albumin 2.1 g/dL (reference range 3.5-5.3 g/dL) Total bilirubin 23.5 (reference range 0.1-1.2 mg/dL) Direct bilirubin 10.0 mg/dL (reference range 0-0.4 mg/dL) ALT 164 U/L (reference range 0-40U/L) AST 333 U/L (reference range 0-37 U/L) Alk Phos 258 U/L (reference range 30-120) Ammonia 50 mcmol/L (reference range 0-32 mcmol/L) Microbiology: Cultures of blood and peritoneal fluid were negative CMV IgG- positive CMV PCR- 3340 copies/ml CD4-25 Hepatitis B: positive surface antigen, negative surface antibody, positive e- antigen HSV- IgG positive for HSV1 and HSV2 Toxoplasma- IgG negative

EKG Sinus tachycardia, low voltage QRS, left posterior fasicular block Radiologic Studies Chest Radiograph (8/16/2008): Moderate-sized left-sided pleural effusion. Discoid atelectasis right lung base. No evidence of infiltrate. Cardiothoracic ratio 13/28.6 CT of the Abdomen/Pelvis (8/17/2008): Changes compatible with cirrhosis with portal hypertension seen. Changes suspicious for gastritis and hypoproteinemia involving small bowel also noted. Abdominal Ultrasound (8/17/2008): Moderate ascites. Distended gallbladder containing sludge with thickened wall, likely related to hepatitis and ascites.

Clinical Course On hospital day #2, the patient developed hematemesis resulting in an abrupt 10% decrease in his hematocrit, and was admitted to the MICU on August 17, 2008. Prior to upper endoscopy (EGD) the patient was intubated and placed on mechanical ventilation. EGD showed "active hematemesis at the time of exam. An ulcer was found in the mid esophagus. The ulcer measured approximately 5-6 cm in length. It was not bleeding. There were 2-3 small associated esophageal varices. 2 bands were initially placed. A 1.5 cm bluish, 'bean-like' lesion was seen at GE junction. This appeared to be a vermiform lesion rather than a visible vessel. A 2 cm polyp was seen near the angularis. This lesion was not biopsied. With a limited examination, no other lesions were visualized in the remainder of stomach and proximal duodenum with exception of blood pool in gastric fundus." The patient was treated with an octreotide drip, IV proton pump inhibitor, and replacement of RBCs and fresh frozen plasma. Tests for CMV, including immunoglobulin G and quantitative PCR, were positive. There was no evidence of CMV infection by immunohistochemical staining of the esophagus. Repeat paracentesis showed no evidence of spontaneous bacterial peritonitis. The patient was treated with ganciclovir, fluconazole, vitamin K. Amazingly, the patient did not receive vancomycin or piperacillin/tazobactam. The patient's hospital course was complicated by oliguric renal failure requiring hemodialysis. On the night of August 20, 2008 he developed recurrent active hematemesis with profound hypotension requiring vasopressor support. An EGD was attempted, but active bleeding precluded visualization of the esophagus and stomach. An abdominal arteriogram showed no evidence of active bleeding from branches of the celiac artery, including the left gastric and gastroduodenal arteries (see Images). Surgery did not feel that he would survive laparotomy. His condition continued to deteriorate, and despite maximum supportive care, he expired on 08/20/2008 at 2010.

case 6:Chief Complaint 89 year old female presents with altered mental status and abdominal pain. History of Present Illness The patient was brought to the Emergency Department by her family reporting 1-2 days of worsening disorientation, dysarthria, headache, and abdominal pain. The patient was unable to provide any history. The patient's family reported that approximately 6 weeks prior to this admission she had been admitted to an outside hospital with possible pneumonia. She was treated with an unknown antibiotic and discharged home after 3 days to complete a 10 day course of antibiotics. Shortly after leaving the hospital she developed crampy abdominal pain, loose brown stools, nausea, and vomiting. There was no GI bleeding or purulent stools. She denied fevers or chills. These GI symptoms persisted after completing her course of antibiotics and she presented to Johns Hopkins Hospital approximately one week after completing the antibiotics. She reported no unusual ingestion of foods or recent travel. Her physical examination was notable for normal vital signs, a non-reducible ventral hernia, and otherwise normal abdominal examination. Labs were notable for a normal WBC, BUN= 68, creatinine= 1.3, normal UA, normal lipase and amylase. An abdominal CT was performed. Stool was positive for C. difficile antigen and toxin. She was discharged on po Flagyl to complete a 14 day course approximately 3 weeks prior to this admission. Family reports that her GI symptoms improved after leaving JHH last time. She had been weak but improving until the two days prior to this admission. She'd had no fevers, chills, sweats, chest pain, cough, or dysuria. She had had a diminished appetite since the episode of colitis, but was taking some fluids and food. Two days prior to admission, she developed diffuse malaise and weakness. On the day of admission, she had worsening abdominal pain, dysarthria, and headache. Past Medical History Other than described above, she'd been in good health and had not been hospitalized recently. The ventral hernia was longstanding and asymptomatic. She had been counseled about obesity. Family History Noncontributory. Social History She lives with her son in Baltimore, who is her caretaker. They have a pet cat and a healthy parrot. She does not smoke, drink, or use any illicit drugs. She was formerly employed at Bethlehem Steel as an administrator. She eats hamburger and eggs regularly. She does not drink unpasteurized milk and hates sushi, oysters, and all other forms of raw or undercooked food. Medications

brown stools, nausea, and vomiting. There was no GI bleeding or purulent stools. She denied fevers or chills. These GI symptoms persisted after completing her course of antibiotics and she presented to Johns Hopkins Hospital approximately one week after completing the antibiotics. She reported no unusual ingestion of foods or recent travel. Her physical examination was notable for normal vital signs, a non-reducible ventral hernia, and otherwise normal abdominal examination. Labs were notable for a normal WBC, BUN= 68, creatinine= 1.3, normal UA, normal lipase and amylase. An abdominal CT was performed. Stool was positive for C. difficile antigen and toxin. She was discharged on po Flagyl to complete a 14 day course approximately 3 weeks prior to this admission. Family reports that her GI symptoms improved after leaving JHH last time. She had been weak but improving until the two days prior to this admission. She'd had no fevers, chills, sweats, chest pain, cough, or dysuria. She had had a diminished appetite since the episode of colitis, but was taking some fluids and food. Two days prior to admission, she developed diffuse malaise and weakness. On the day of admission, she had worsening abdominal pain, dysarthria, and headache. Past Medical History Other than described above, she'd been in good health and had not been hospitalized recently. The ventral hernia was longstanding and asymptomatic. She had been counseled about obesity. Family History Noncontributory. Social History She lives with her son in Baltimore, who is her caretaker. They have a pet cat and a healthy parrot. She does not smoke, drink, or use any illicit drugs. She was formerly employed at Bethlehem Steel as an administrator. She eats hamburger and eggs regularly. She does not drink unpasteurized milk and hates sushi, oysters, and all other forms of raw or undercooked food. Medications Calcium carbonate 500 mg po tid Lasix 20 mg po prn edema No herbals or alternative medications Allergies No known drug allergies. Review of Systems Her son reports that the patient has no history of heartburn, chronic abdominal pain, liver disease, constipation, or loose stools (other than this illness). She does not use antacids at home. Her routine health maintenance screening is up to date. Physical Exam on Admission T: 96 (oral) BP: 82/46 HR: 120 RR: 20. General: Obese woman with slurred speech HEENT: No facial droop. CV: Regular rate and rhythm, normal S1 and S2. Lungs: Coarse rales bilaterally. Abdomen: Diffusely tender without rebound, bowel sounds hypoactive, no masses. Ventral hernia. Rectal: Brown stool, heme-positive. Extremities: 3+ lower extremity edema bilaterally. Neurologic: Unable to follow commands, disoriented; dysarthric, no focal findings.

Laboratory Values on Transfer Na 145; K 1.7; Cl 107; CO2 18; BUN 57; Cr 2.4; Glucose 7; Calcium 7.2; Total Protein 5.4; Albumin 1.8; TBili 0.8, ALT 29; AST 28; Alk Phos 77 WBC 6600 (Bands 13%, Neutrophils 77%, Lymphocyte 4%); Hct 32.7; MCV 94.2; Platelet 122 ABG pH= 7.21, PCO2= 47, PO2=101 on nasal canula 2 L/M Lactate 9.8; Serum myoglobin 1572.1

Radiologic Studies PA chest radiograph on admission: Normal left ventricle. Moderate obesity causing hypoinflation of lungs and moderate right hemidiaphragm elevation. Moderate arthritis shoulder joins with probable aseptic necrosis right humeral head. Brain CT without contrast on admission: Minimal cerebral atrophy. No evidence of bleed, hematoma, midline shift, or sulcal effacement. Ventricles are normal in size. Normal gray-white differentiation. No acute abnormality. case7:-

Chief Complaint A 71-year-old man with a 4-month history of worsening shortness of breath was transferred to The Johns Hopkins Hospital from an outside hospital. History of Present Illness The patient was a 71 year old Caucasian male with a 100-pack-year history of tobacco abuse, hypertension, coronary artery disease, congestive heart failure, and chronic obstructive lung disease who presented to an outpatient clinic at an outside hospital with a four-month history of worsening symptoms of congestive heart failure. He noted increased dependent edema in both of his legs and progressively worsening shortness of breath with orthopnea and paroxysmal nocturnal dyspnea. He specifically denied a history of previous myocardial infarction or angina. On that day, the patient refused to be admitted to the hospital for treatment of his symptoms; however, he relented the following day and was admitted to the outside hospital with a presumptive diagnosis of biventricular congestive heart failure. On a two-dimensional echocardiogram, a 5.2 x 1.9 cm mass, presumed to be an atrial myxoma, was found occupying 80% of the left atrium. The patient was transferred to The Johns Hopkins Hospital for resection of this tumor. Past Medical History Hypertension Coronary artery disease Congestive heart failure Chronic obstructive pulmonary disease Past Surgical History appendectomy (20 years ago) hernia repair (2002) Medications (at home prior to admission to JHH) diltiazem, metoprolol Social History Quit smoking 20 years ago after 100-pack-years of tobacco use. Family History Non-contributory Allergies NKDA Physical Exam on Admission General: 84.5 kg, alert, affable, no acute distress Vital Signs: 138/66, HR 86, RR 24, O2 sat 98% 3L NC HEENT: PERRL, EOMI, oropharynx clear, small oral aperture Neck: JVP to angle of jaw, 2+ carotid pulses, full range of motion Cardiac: RRR, normal S1 S2, distant heart sounds Chest: I:E 1:4, diffuse end-expiratory wheeze, decreased breath sounds right base Abdomen: bowel sounds present, firm, nontender, nondistended, no hepatosplenomegaly GU: redness and fungal looking rash on penis and scrotum

Chronic obstructive pulmonary disease Past Surgical History appendectomy (20 years ago) hernia repair (2002) Medications (at home prior to admission to JHH) diltiazem, metoprolol Social History Quit smoking 20 years ago after 100-pack-years of tobacco use. Family History Non-contributory Allergies NKDA Physical Exam on Admission General: 84.5 kg, alert, affable, no acute distress Vital Signs: 138/66, HR 86, RR 24, O2 sat 98% 3L NC HEENT: PERRL, EOMI, oropharynx clear, small oral aperture Neck: JVP to angle of jaw, 2+ carotid pulses, full range of motion Cardiac: RRR, normal S1 S2, distant heart sounds Chest: I:E 1:4, diffuse end-expiratory wheeze, decreased breath sounds right base Abdomen: bowel sounds present, firm, nontender, nondistended, no hepatosplenomegaly GU: redness and fungal looking rash on penis and scrotum Skin: epidermolysis of extremities, erythematous and edematous to shins; nonpalpable pulses in feet but warm; moves without pain Neurological: CNII-XI grossly intact, strength intact throughout Laboratory Values on admission Na 135, Cl 85, K 4.6, HCO3 41, BUN 15, Cr 0.8, Glu 88, Ca 9.1 pH 7.38, pCO2 81, pO2 43, calculated HCO3 48 (room air) TP 5.6, Alb 3.3, Bili 0.5, LFTs nl WBC 9090, Hgb 13.9, Hct 46.6, Plt 324, MCV 75.4, RDW 20.5 Coags nl, UA nl Radiology Echocardiogram- 5.2 x 1.9 cm mass occupying 80% of the left atrium CXR- right pleural effusion (Image 1) Chest CT- The positive findings include right pleural effusion, right lower lobe atelectasis, right paratracheal lymphadenopathy, subcarinal mass, and small left pleural effusion (Image 2 and 3). Thoracic computed tomography image 5 cm caudal to Image 3 reveals occlusion of bronchus intermedius, collapsed right lower lung, and a filling defect in the left atrium (Image 4).