title: Diagnostic criteria for acute liver failure due to Wilson disease.
abstract: OBJECTIVE
  To describe the diagnostic criteria for acute liver failure due to Wilson disease (WD), which is an uncommon cause of acute liver failure (ALF).
  
  
  METHODS
  We compared findings of patients presenting with ALF due to WD to those with ALF of other etiologies.
  
  
  RESULTS
  Previously described criteria, such as low alkaline phosphatase activity, ratio of low alkaline phosphatase to total bilirubin or ratio of high aspartate aminotransferase (AST) to alanine aminotransferase (ALT), failed to identify patients with ALF due to WD. There were significant differences in low ALT and AST activities (53 +/- 43 vs 1982 +/- 938, P < 0.0001 and 87 +/- 44 vs 2756 +/- 2941, P = 0.037, respectively), low choline esterase activity (1.79 +/- 1.2 vs 4.30 +/- 1.2, P = 0.009), high urine copper concentrations (93.4 +/- 144.0 vs 3.5 +/- 1.8, P = 0.001) and low hemoglobin (7.0 +/- 2.2 vs 12.6 +/- 1.8, P < 0.0001) in patients with ALF caused by WD as compared with other etiologies. Interestingly, 4 of 7 patients with ALF due to WD survived without liver transplantation.
  
  
  CONCLUSIONS
  In ALF, these criteria can help establish a diagnosis of WD. Where applicable, slit-lamp examination for presence of Kayser-Fleischer rings and liver biopsy for determination of hepatic copper concentration still remain important for the diagnosis of ALF due to WD. The need for liver transplantation should be evaluated carefully as the prognosis is not necessarily fatal.
authors.affiliation: University Hospital of Heidelberg, Department of Gastroenterology and Hepatology, Im Neuenheimer Feld 410, Heidelberg D-69120, Germany. christoph_eisenbach@med.uni-heidelberg.de
authors.forename: Christoph
authors.initials: C
authors.lastname: Eisenbach
authors.forename: Olivia
authors.initials: O
authors.lastname: Sieg
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Stremmel
authors.forename: Jens
authors.initials: J
authors.lastname: Encke
authors.forename: Uta
authors.initials: U
authors.lastname: Merle
mesh_terms: D000251:Adenosine Triphosphatases / Q000235:genetics; D000293:Adolescent; D000328:Adult; D000369:Aged, 80 and over; D000410:Alanine Transaminase / Q000097:blood; D001219:Aspartate Aminotransferases / Q000097:blood; D027682:Cation Transport Proteins / Q000235:genetics; D002614:Chelating Agents / Q000627:therapeutic use; D002648:Child; D002802:Cholinesterases / Q000097:blood; D003300:Copper / Q000378:metabolism; D000073840:Copper-Transporting ATPases; D005260:Female; D006454:Hemoglobins / Q000378:metabolism; D006527:Hepatolenticular Degeneration / Q000097:blood / Q000150:complications*; D006801:Humans; D017114:Liver Failure, Acute / Q000175:diagnosis* / Q000209:etiology* / Q000628:therapy; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D009154:Mutation / Q000235:genetics; D011379:Prognosis; D012016:Reference Values; D016896:Treatment Outcome
chemical_list: D027682:Cation Transport Proteins; D002614:Chelating Agents; D006454:Hemoglobins; D003300:Copper; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase; D002802:Cholinesterases; D000251:Adenosine Triphosphatases; D000073840:Copper-Transporting ATPases