Akutes Leberversagen bei abdominellem Morbus Wilson im frühen Erwachsenenalter^*

Abstract

The acute abdominal form of Wilsons's disease was observed in three female patients aged 17, 18 and 30 years. Clinically uncharacteristic general symptoms and jaundice were dominant. Chemical pathology showed high bilirubin values with predominance of direct bilirubin, signs of hepatic and renal insufficiency and haemolytic anaemia. There were no neurologic symptoms and the Kayser-Fleischer ring was absent. Coeruloplasmin in blood was normal, copper concentration in blood and urine was clearly increased. Diagnosis of Wilsons's disease could only be established at post mortem by quantitative copper estimation in liver tissue. The patients succumbed 1--4 weeks after onset of symptoms through hepatic failure.