A patient with Wilson's disease may present with hepatobiliary disease. Hepatobiliary disease is a common presentation for children, adolescents and young adults with Wilson's disease.


Acute presentations:

(1) acute fulminant hepatitis

(2) acute hepatitis mimicing drug or viral hepatitis

(3) acute hepatitis mimicing autoimmune hepatitis

(4) acute cholelithiasis


Chronic presentations:

(1) chronic hepatitis

(2) cirrhosis with portal hypertension

(3) recurrent episodes of jaundice

(4) chronic cholelithiasis/cholecystitis associated with chronic hemolytic anemia


Reasons to consider Wilson's disease:

(1) young age at onset

(2) family history of Wilson's disease

(3) exclusion of drug, viral and autoimmune causes

(4) other manifestations of Wilson's disease (neuropsychiatric, Kayser-Fleischer rings, unexplained hemolytic anemia, other)


Needless to say it is easy to miss the diagnosis.


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