WILSON'S DISEASE IN CHILDREN, REPORT OF 25 CASES
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 &a...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
Tehran University of Medical Sciences
1984-12-01
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| Series: | Acta Medica Iranica |
| Online Access: | https://acta.tums.ac.ir/index.php/acta/article/view/177 |
| Summary: | 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.
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| ISSN: | 0044-6025 1735-9694 |