HEMOLYTIC CRISIS WITH LIVER-FAILURE AS INITIAL MANIFESTATION OF WILSONS-DISEASE

An 18-year-old woman developed an acute haemolytic anaemia, acute tran sient renal failure and progressive hepatic failure. Coeruloplasmin an d serum copper concentration were normal; a Kayser-Fleischer ring and any neurological symptoms were absent initially. Liver biopsy was cont raindicated because of increased bleeding tendency. Wilson's disease w as diagnosed only after the acute renal failure had regressed, on the basis of the urinary copper excretion (2890 mu g/d, rising to 7330 mu g/d after D-penicillamine administration). Progressive liver failure r equired transplantation. After it the patient quickly recovered and is now, two years later, free of disease. - This case demonstrates that Wilson's disease may be difficult to diagnose at the time of initial a cute manifestation. But it can be recognized early from the pathognomo nic low alkaline phosphatase and by calculation of free serum copper.