Maintenance zinc therapy after initial penicillamine chelation to treat symptomatic hepatic Wilson’s disease in resource constrained setting
| Autor: | Mehul Choksi, Jeyamani Ramachandran, Grace Rebekah, Ashish Goel, George Chandy, George Kurian, Uday Zachariah, K G Sajith, Chundamannil E. Eapen, Piyush Gupta |
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| Rok vydání: | 2018 |
| Předmět: |
Adult
Male medicine.medical_specialty Time Factors Adolescent medicine.medical_treatment Liver transplantation Severity of Illness Index Gastroenterology Young Adult 03 medical and health sciences Liver disease 0302 clinical medicine Hepatolenticular Degeneration Cost Savings Internal medicine medicine Humans Chelation therapy Child Adverse effect Chelating Agents Retrospective Studies medicine.diagnostic_test Drug Substitution business.industry Penicillamine Hepatology medicine.disease Zinc Sulfate Wilson's disease Treatment Outcome Child Preschool Female 030211 gastroenterology & hepatology Liver function tests business Copper 030217 neurology & neurosurgery Follow-Up Studies medicine.drug |
| Zdroj: | Indian Journal of Gastroenterology. 37:31-38 |
| ISSN: | 0975-0711 0254-8860 |
| Popis: | Experience with zinc in treating symptomatic hepatic Wilson’s disease (WD) is limited. To study the efficacy of Penicillamine followed by zinc in treating symptomatic hepatic Wilson’s disease. We retrospectively analyzed case records of 31 symptomatic hepatic WD patients for whom disease severity scores (Child’s, model for end-stage liver disease (MELD), Nazer’s, and New Wilson Index (NWI) score) and 24-h urinary copper were compared at 3-time points—baseline at presentation, at transition from penicillamine to zinc and at end of follow up. Thirty-one patients (median age 11 [5–24] years) with symptomatic hepatic WD were studied; ten had associated neuropsychiatric manifestations of WD. Penicillamine was changed to zinc sulfate either due to financial constraints (28 patients) or due to adverse effects of penicillamine (3 patients). At presentation (baseline), six patients belonged to Child’s class A, five to Child’s B, and 17 to Child’s C. Duration of initial penicillamine chelation therapy was 134 (2–320) weeks, and of subsequent zinc therapy was 363 (35–728) weeks. There was a significant improvement in liver function tests and disease severity scores (Child’s, MELD, Nazer’s, and NWI score) at the transition from penicillamine to zinc compared to baseline. This improvement was maintained until the end of study period with 90% survival at 10 (2–20) years. Fifteen of the 17 Child’s C cirrhotic patients showed significant improvement in disease severity scores from baseline until end of follow up. Penicillamine followed by zinc may be a safe and effective treatment in resource-constrained setting for symptomatic hepatic WD patients in all grades of baseline disease severity. Some patients with decompensated cirrhosis due to WD may be managed with medical treatment, avoiding liver transplantation. |
| Databáze: | OpenAIRE |
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