WILSON'S DISEASE IN CHILDREN, REPORT OF 25 CASES

• Main Authors: Y. AGHIGHI, R. AFTENDELIANS
• Format: Article
• Language: English
• Published: Tehran University of Medical Sciences 1984-05-01
• Series: Acta Medica Iranica
• Online Access: http://journals.tums.ac.ir/PdfMed.aspx?pdf_med=/upload_files/pdf/5048.pdf&manuscript_id=5048

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:"nHepatic-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 ."nDiagnosis 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.