WILSON'S DISEASE IN CHILDREN, REPORT OF 25 CASES

Abstract

We conducted a retrospective study of 25 cases (15 boys and 12 girls) of Wilson's disease(Hepatolenticular degeneration)spanning a period of 8 years. Age at the time of diagnosis ranged between 3-17 years. The f ollowing clinical forms were observed:
Hepatic-13, neurologic-4, mixed (hepatic & neurologic)-S, --a s ympt oma t i c - 3 , Kayser-Fleischer corneal ring s were obs erved in 12 pat ients .
Diagnosis wa s confi rmed by l ow serum cer ulop lasmi n , low ser um c oppe r, Increased uri nar y copper and abno rmal aminoaciduria. Of different treatment s chedul e s (l ow copper di ets, D'. Penicillamine, metro"nidazole, K sulfide 1 T.E.T., 2Cl . and BAL) the diatary management plus D. Penicillamine was the most effective.Mortality was 8(32%)due to either fulminent hemorrhage or hepatic failure.