Case Reports: Tuesday, October 25, 2011 |

Acute Liver Failure in a Young Adult - Not Always the Usual Suspects FREE TO VIEW

Vipul Kumar, MD; Shashank Jain, MD; Anthony Donato, MD
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The Reading Hospital and Medical Center, West Reading, PA

Chest. 2011;140(4_MeetingAbstracts):101A. doi:10.1378/chest.1077538
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INTRODUCTION: Young adults presenting with acute liver failure (ALF) are suspected to have toxic ingestion or viral hepatitis. Overlooking other causes of ALF can delay treatment which can be fatal. We report a case of Wilson’s disease (WD) presenting with ALF without any neurological, psychiatric or ocular findings.

CASE PRESENTATION: A 25 year old healthy male developed a red, itchy rash with fevers and headache. He self-prescribed Acetaminophen 650 mg. two tablets four times a day for two weeks. Ten days into his illness, he noticed yellow discoloration of his eyes and had occasional vomiting. Three days subsequently, he presented to our hospital with yellow skin, dark stools and abdominal swelling. History was negative for alcohol, recreational drug or herbal medication use, recent travel, high risk behavior or known family history of liver diseases. On examination, he had diffuse jaundice, icteric sclera, and mild abdominal distention. No residue of the skin rash was present. Mentation and central nervous system exam was normal. Initial suspicion was for toxic ingestion of Acetaminophen and N-acetylcysteine therapy was started. Acetaminophen (8.7 mg/L) level was within normal range. Viral hepatitis and encephalopathy were ruled out. Urine drug screen was positive only for marijuana. Laboratory data confirmed cholestasis (total bilirubin 32.4 mg/dL, direct bilirubin 12.3 mg/dL) and coagulopathy (INR 5.70, PT 52.5 seconds, PTT 100 seconds). There was a modest elevation in aminotransferases (ALT 134 IU/L, AST 34 IU/L) and a low alkaline phosphatase (34 IU/L). Hemolytic anemia (Hemoglobin 6.2 g/dL, Reticulocytes 7.7 % and positive urine hemoglobin) and acute renal failure (Creatinine 2.7 mg/dL with estimated GFR 29 mL/minute/1.73 m2) was present. Ultrasound and CT imaging confirmed moderate ascites, a normal sized, nodular heterogeneous, cirrhotic liver and splenomegaly (17.5 cm). Ascitic fluid was transudative and negative for spontaneous bacterial peritonitis.

DISCUSSION: Patient soon developed multi-organ failure requiring ventilation, ionotropic support and dialysis. His coagulopathy was unresponsive to oral Vitamin K. A detailed work-up for acute liver failure was initiated. 24 hour urinary copper excretion of 5980 micrograms/day and serum ceruloplasmin level 19 mg/dL triggered a liver biopsy which confirmed Wilson’s disease (liver copper content 745 microgram/gram dry weight). Retrospectively, Kayser-Fleischer rings were absent on formal ophthalmologic slit lamp assessment. He was referred for and treated with liver transplantation thereafter.

CONCLUSIONS: Kinnear Wilson described WD as a lethal neurological disease accompanied by chronic liver disease leading to cirrhosis. Acute liver failure as an initial presentation of Wilson’s disease usually follows a characteristic pattern of clinical illness. A combination of low level of alkaline phosphatase with minimally elevated aminotransferases is seen (both less than 2000 IU/L). Other findings include hemolytic anemia, coagulopathy unresponsive to phylloquinone and rapid progression to renal failure. Recognition of this constellation can lead to early diagnosis and treatment of a potentially fatal condition.

Reference #1 Roberts, EA, Schilsky, ML. A practice guideline on Wilson disease. Hepatology 2003; 37:1475

Reference #2 Wilson's disease in patients presenting with liver disease: a diagnostic challenge. Steindl P, Ferenci P, et al.Gastroenterology. 1997;113(1):212-8.

Reference #3 Clinical differentiation of fulminant Wilsonian hepatitis from other causes of hepatic failure. Berman DH, Leventhal RI, Gavaler JS, Cadoff EM, Van Thiel DH. Gastroenterology. 1991;100(4):1129-34.

DISCLOSURE: The following authors have nothing to disclose: Vipul Kumar, Shashank Jain, Anthony Donato

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