[Acute liver failure and hemolysis in a 16-year-old woman. First manifestation of Wilson's disease].
METHODS
A 16-year-old previously healthy female patient was admitted with progressive weakness and jaundice. There was no history of journeys to far-away countries. The patient was on oral contraceptives but no other medication, there were no signs of drug abuse. Apart from scleral and skin jaundice all physical findings were normal.


METHODS
Diagnostic ultrasound showed an enlarged and hyperdense liver. Laboratory tests revealed a markedly increased serum bilirubin, while aminotransferases were only slightly elevated and alkaline phosphatase was unexpectedly low. Prothrombin and antithrombin III levels were low. There was a Coombs-negative hemolytic anemia. Ceruloplasmin was lower than normal. Based on these findings, acute Wilson's disease was suspected.


METHODS
The patient was immediately transferred to a hepatologic center with transplantation facilities. There, urinary copper excretion and copper concentration in the liver tissue were found to be elevated. Liver histology showed fibrosis but no cirrhosis. Under conservative therapy with trientine liver function recovered, and the patient is well 3 years after the onset of symptoms.


CONCLUSIONS
Acute liver insufficiency should always suggest the possibility of Wilson's disease, particularly when hemolysis is also present.
Christl
Stefan U
SU
II. Medizinische Abteilung, Allgemeines Krankenhaus Harburg, Hamburg. stefan.christl@ak-harburg.lbk-hh.de
Flieger
Dimitri
D
Keller
Ralf
R
Stremmel
Wolfgang
W
Fischbach
Wolfgang
W
D000208:Acute Disease; D000293:Adolescent; D000743:Anemia, Hemolytic / Q000175:diagnosis / Q000209:etiology*; D001707:Biopsy, Needle; D002614:Chelating Agents / Q000627:therapeutic use; D003300:Copper / Q000378:metabolism / Q000652:urine; D005260:Female; D005500:Follow-Up Studies; D006527:Hepatolenticular Degeneration / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008099:Liver / Q000378:metabolism / Q000473:pathology; D017114:Liver Failure, Acute / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D013997:Time Factors; D016896:Treatment Outcome; D014266:Trientine / Q000627:therapeutic use; D014463:Ultrasonography
D002614:Chelating Agents; D003300:Copper; D014266:Trientine