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Clinico-Pathological Conference
Case Study

CPC #1: Tuesday, September 08, 2009
Hurd Hall, The Johns Hopkins Hospital
Clinicopathological Conference at 12:00 PM

Clinical Discussant: Luis Diaz, MD, PhD
Radiologist: Ihab Kamel, MD
Pathology Resident: Aatur Singhi, MD PhD
Pathologist: Pedram Argani, MD
Moderator: Charles Wiener, MD
Chief Complaint

Right upper and lower quadrant pain, decreased urination and jaundice.


History of Present Illness

The patient is a 62-year-old Caucasian male with history of hypertension, chronic obstructive pulmonary disease (COPD), diabetes mellitus type II, and Hepatitis B who presents with right upper and lower quadrant abdominal pain, jaundice, and decreased urine output.

The patient reports for the last 4 weeks, he has felt increasingly fatigued with darkening urine and light/clay colored stools. He has also been markedly nauseated and unable to keep more than "a piece of food" down daily per his partner. He has had an intermittent RUQ "grabbing pain" and associated back pain, which over the last several days increased during defecation. He reports the pain as a 10/10 in intensity. The patient also reports that over the past 3 days, he has noticed his skin and eyes turning yellow. He denied any recent alcohol or acetaminophen use.

His past medical history is notable for a 30 year history of chronic Hepatitis B presumably incurred after an orthopedic surgical procedure (that required blood transfusion) when he was in his 20s. He never received treatment and is known to have chronically positive hepatitis B surface antigen. He had a liver biopsy in 1998 that showed mild fibrosis. He has never had an upper GI bleed, ascites, or other sequelae of chronic liver disease. He has had periodic liver ultrasounds, most recently 4 months ago that showed a mildly nodular liver with no evidence of liver mass.

He has no history of myocardial infarction, stroke, or acute renal failure. He does not recreationally hunt mushrooms nor eat raw seafood. He has no known contact with cleaning chemicals.


Past Medical History

Chronic Hepatitis B
Hypertension
COPD
Diabetes mellitus type II- diet controlled


Family History

The patient reports his mother died in her late 50s with history of myocardial infarction, ovarian carcinoma, cerebrovascular accident and leukemia. His father died at a young age due to a motor vehicle accident. Per patient, his aunt has gallbladder problems. The patient has two daughters that are otherwise in good health.


Social History

The patient formerly was a fiberglass worker who reports previous heavy abuse of alcohol; however , he quit 10 years ago. He admits to smoking cigarettes for 45 years (1 pack per day = 45 pack years); however, he also quit 3 years ago. He denies any illicit drug use. The patient currently lives with his girlfriend in East Baltimore. They have an 8 year old dog. His hobbies include Keno and Civil War history.


Medications

Albuterol inhaler as needed
Tiotropium inhaler daily
Amlodipine 10 mg daily
Fosinopril 10 mg / Hydrochlorothiazide 12.5 mg - 2 tablets daily
Aspirin 650 mg Q4 to relieve pain for the past 2 weeks (has been instructed to avoid acetominophin)

Allergies

No known drug allergies.


Review of Systems

Constitutional: Poor appetite.
Neuro: Weakness (lower greater than upper extremities) for the last 1-2 weeks.
HEENT: Requires glasses.
CV: Orthopnea. Chest pain for years with heavy exercise.
Respiratory: Yellow sputum and chronic cough. Dyspnea on exertion
GI: Nausea with abdominal pain and jaundice over last 2-4 weeks
GU: Decreased secondary to low PO intake.
Skin/Skeletal: Myalgias. No edema.


Physical Exam on Admission

* T: 36.6 HR: 112 BP: 77/29 RR: 18-20 SaO2: 98-99% on 2 liters nasal cannula
* General: Obese Caucasian male who is slightly slow to answer questions.
* HEENT: Normocephalic/atraumatic. Pupils equal round and reactive to light. Extraocular muscles are intact. Sclera are icteric. Poor dentition. Dry mucous membranes.
* Neck: Supple with no masses or lymphadenopathy. No carotid bruits.
* CV: Regular rate and rhythm with I/VI systolic murmur at upper sternal border.
* Lungs: Diminished breath sounds at the bases. Crackles at the left lower lobe.
* Abdomen: Distended with decreased bowel sounds and tympany to percussion. Tenderness at the right upper quadrant. No palpable masses. Liver edge palpable and firm. No splenomegaly. No fluid wave or shifting dullness.
* Extremities: No cyanosis or clubbing. Trace bilateral petal edema. Pulses 2+.
* Rectal: Normal rectal tone. Hemoccult is positive.
* Neuro: Alert and oriented, slightly somnolent. 5/5 strength in all extremities. Sensation and cranial nerves intact. Reflexes and strength appear symmetric.


Laboratory Values on Transfer

* Na 127; K 4.0; Cl 87; BUN 63; Cr 4.0; Glucose 116; Calcium 9.0; Total protein: 6.9; Albumin: 3.1; Total bilirubin: 17.6; Direct bilirubin: 11.4; ALT: 151; AST: 161; Alkaline phosph: 330; Anion gap: 28; Lactic acid: 3.6. Hepatitis B Surface antigen- positive, Hepatitis B Surface antibody- negative, Hepatitis e-antigen positive, Hepatitis A, B, D, E negative, HIV negative, EBV negative, leptospira antibody negative.
* WBC 19,320 (ANC 14,683); Hemoglobin 13.5; Hct 36.6; MCV 85.5; Platelets 120,000
* Urine analysis: Cloudy brown, positive for bilirubin and trace protein; negative for glucose, ketones, leukocyte esterase and nitrite; 46 RBCs.
* ABG - pH: 7.36; pCO2: 24 mm Hg, pO2: 102 mm Hg; calculated HCO3 13 mmol/L
* PT 12.8; INR 1.3; aPTT 30.4
* CEA 2.1 ng/ml, Carbohydrate antigen 19-9 787 U/ml, PSA 4.2 ng/ml, AFP 10 ng/ml


EKG

Normal sinus rhythm with low voltage QRS complex and prolonged QT interval or TU fusion.


Radiologic Studies

Abdominal computed tomography (CT) revealed consolidation and nodularity at the periphery of the lingula and the left lower lobe of the lung, multiple enlarged upper abdominal and retroperitoneal lymph nodes, and cholelithiasis within the gallbladder.

The liver (Image 1) appears cirrhotic with nodular contour. There is intrahepatic ductal dilatation of both lobes (yellow arrows) In addition, there is a low attenuation poorly defined central mass (green arrow)

Abdominal ultrasound reveals normal liver echogenicity with biliary duct dilatation. The liver is noted to be at the upper limits of normal in size. No ascites is noted. The gallbladder is contracted with multiple stones, however no evidence of cholecystitis. The right kidney is at the upper limits of normal in size and otherwise unremarkable.


Clinical Course

The patient was admitted to the ICU with shock. He developed worsening liver function, lactic acidosis, and acute renal failure over the first 5 days (AST: 3834, ALT: 1425; Alkaline phosphatase: 359; Albumin: 1.7; Total bilirubin: 13.2, INR 2.2). There was no evidence of acute myocardial infarction or pulmonary embolism. Despite vasopressors and antibiotics, the patient continued to have worsening lactic acidosis and hypotension. He eventually developed worsening lactic acidosis, refractory shock requiring the addition of vasopressin, and mental status changes requiring intubation for airway protection. Continuous Veno-Venous Hemodialysis (CVVHD) was initiated for acute renal failure and metabolic acidosis. Blood and urine cultures remained negative. Despite aggressive therapy, his renal, hepatic, respiratory, and metabolic dysfunction worsened. On Hospital Day 5 he began having acute upper GI bleeding that was not controlled with fresh plasma and platelet transfusion.

The family expressed wishes for comfort care from the start of the hospitalization, as soon as the clinical picture was consistent with a worsening condition and a poor prognosis with little chance of recovery. The family was informed of the grim prognosis and the patient was immediately made DNR and full withdrawal of care was initiated. The patient expired within 2 hours.


Questions

1.What are the likely causes of liver failure in patients with hepatitis B?
2.What are the common causes of a liver mass in patients with cirrhosis?
3.What is the most likely cause of his acute condition leading to death?


Images Click on an image below to enlarge.

Image 1

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