WILSON´S DISEASE IN CHILDREN, REPORT OF 25 CASES
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| Title | WILSON´S DISEASE IN CHILDREN, REPORT OF 25 CASES |
| Authors | |
| 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. |
| Publisher | Tehran University of Medical Sciences |
| Date | 2012-10-20 |
| Source | Acta Medica Iranica Vol 26, No 1-4 (1984) |