Included in this group are FHF secondary to Wilson's disease (see Chapter 124, "Management of Wilson's Disease"), autoimmune hepatitis, Reye's syndrome, and pregnancy-related ALF from acute fatty liver of pregnancy, hemolysis, abnormal liver enzymes and low platelets(HELLP) syndrome, or hepatic rupture (see Chapter 133, "Biliary Strictures and Neoplasms"). FHF may be the first clinical manifestation of Wilson's disease. Indeed, Wilson's disease should be considered in any young patient with unexplained FHF, particularly when there is evidence of hemolysis, relatively low aminotransferases (usually less than 500), and, characteristically, a normal or even low serum alkaline phosphatase. Other findings with less diagnostic specificity include an aspartate aminotransferase/alanine aminotransferase > 4 and a low serum uric acid, the former reflecting hemolysis and the latter a Fanconi syndrome from renal tubular copper deposition. Kayser-Fleischer rings may not be present, and the serum ceruloplasmin level is often nondiagnostic in this setting as it is an acute-phase reactant. Diagnosis relies on a high index of suspicion and measurement of copper concentration in a 24-hour urine collection. Fulminant Wilson's disease usually does not respond to chelation therapy, and the prognosis without transplant is poor. Screening of family members is critically important once the diagnosis of Wilson's disease is made.
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