Acute haemolytic crisis with hepatic failure as the first manifestation of morbus Wilson in children
Abstract
The authors demonstrate four cases of confirmed Wilson's disease (MW) in girls aged 14, 15, 10 and 16 years with a very similar clinical and laboratory finding. The disease was manifested by sudden intensive jaundice with marked hyperbilirubinaemia, only slight or mild increase of aminotransferases in all four girls (maximal AST values 7.0, 4.7, 7.3 and 3.2 ukat. resp.), extreme haemolytic anaemia with the lowest haemoglobin values of about 60 g/1, a negative Coombs test and rapidly progressing hepatic failure.
Only in one case the ceruloplasmin level was markedly reduced, in the remainder it was also below the lower range of normal values, but the values were marginal. At the onset of the disease all patients were admitted to the infectious departments, as hepatitis was suspected.
Rapid progression of acute hepatic failure was fatal in three patients. On necropsy micronodular cirrhosis was found, the copper content of the liver was 952, 983 and 985 ug/1 resp. per g of dry matter.
In one patient an urgent transplantation of the liver was performed successfully and her condition is satisfactory. The authors draw attention to the fact that MW can be manifested already in childhood by an atypical course.
An acute haemolytic crisis with hepatic failure in Wilson's disease is nowadays an indication for urgent transplantation of the liver.