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:"nHepatic-13, neurologic-4, mixe...
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| Format: | Article |
| Language: | English |
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Tehran University of Medical Sciences
1984-05-01
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| Series: | Acta Medica Iranica |
| Online Access: | http://journals.tums.ac.ir/PdfMed.aspx?pdf_med=/upload_files/pdf/5048.pdf&manuscript_id=5048 |
| _version_ | 1811245901903036416 |
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| author | Y. AGHIGHI R. AFTENDELIANS |
| author_facet | Y. AGHIGHI R. AFTENDELIANS |
| author_sort | Y. AGHIGHI |
| collection | DOAJ |
| description | 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.   |
| first_indexed | 2024-04-12T14:46:04Z |
| format | Article |
| id | doaj.art-609919c1584246e0ba29a9609590dfc4 |
| institution | Directory Open Access Journal |
| issn | 0044-6025 |
| language | English |
| last_indexed | 2024-04-12T14:46:04Z |
| publishDate | 1984-05-01 |
| publisher | Tehran University of Medical Sciences |
| record_format | Article |
| series | Acta Medica Iranica |
| spelling | doaj.art-609919c1584246e0ba29a9609590dfc42022-12-22T03:28:38ZengTehran University of Medical SciencesActa Medica Iranica0044-60251984-05-01261-44556WILSON'S DISEASE IN CHILDREN, REPORT OF 25 CASESY. AGHIGHIR. AFTENDELIANSWe 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.  http://journals.tums.ac.ir/PdfMed.aspx?pdf_med=/upload_files/pdf/5048.pdf&manuscript_id=5048 |
| spellingShingle | Y. AGHIGHI R. AFTENDELIANS WILSON'S DISEASE IN CHILDREN, REPORT OF 25 CASES Acta Medica Iranica |
| title | WILSON'S DISEASE IN CHILDREN, REPORT OF 25 CASES |
| title_full | WILSON'S DISEASE IN CHILDREN, REPORT OF 25 CASES |
| title_fullStr | WILSON'S DISEASE IN CHILDREN, REPORT OF 25 CASES |
| title_full_unstemmed | WILSON'S DISEASE IN CHILDREN, REPORT OF 25 CASES |
| title_short | WILSON'S DISEASE IN CHILDREN, REPORT OF 25 CASES |
| title_sort | wilson apos s disease in children report of 25 cases |
| url | http://journals.tums.ac.ir/PdfMed.aspx?pdf_med=/upload_files/pdf/5048.pdf&manuscript_id=5048 |
| work_keys_str_mv | AT yaghighi wilsonapossdiseaseinchildrenreportof25cases AT raftendelians wilsonapossdiseaseinchildrenreportof25cases |