| Journal: | Annals of hepatology |
| Database: | PERIÓDICA |
| System number: | 000417658 |
| ISSN: | 1665-2681 |
| Authors: | Rodríguez, Beatriz1 Burguera, Juan1 Berenguer, Marina |
| Institutions: | 1Hospital Universitari i Politecnic La Fe, Valencia. España |
| Year: | 2012 |
| Season: | Nov-Dic |
| Volumen: | 11 |
| Number: | 6 |
| Pages: | 907-914 |
| Country: | México |
| Language: | Inglés |
| Document type: | Artículo |
| Approach: | Aplicado, analítico |
| English abstract | There are certain areas of uncertainty regarding the best therapeutic approach in patients diagnosed with Wilson Disease (WD). Our aim was to assess treatment response to different therapies in a cohort of WD patients followed in a single center. Material and methods. This is an observational, descriptive study in which clinical, laboratory and imaging data are reviewed in a series of 20 WD patients with a median follow-up of 14 years. Type of presentation, treatment used, biochemical and copper homeostasis parameters were elicited. Results. Median age at diagnosis was 22 years. The most frequent form of presentation was hepatic (n = 10, 50%; mean age: 21.5 years), followed by neurological (25%; mean age: 34.5 years) and mixed (15%). The initial treatment in both symptomatic and asymptomatic patients at diagnosis was d-penicillamine in 90% and Zinc (Zn) in 10%, respectively. Patients who were maintained on d-penicillamine for the whole period had complete biochemical normalization (baseline ALT: 220 IU/l; last follow up 38 IU/l). In contrast, patients in whom d-penicillamine was switched to Zn, irrespective of the cause, did not show a complete biochemical remission (baseline ALT: 100 IU/l vs. 66 IU/l at last follow-up). Conclusions. Treatment was found to be effective in most cases regardless of the drug used. However, side effects were common in those treated with d-penicillamine agents, and required switching to zinc. Therapy with zinc was well tolerated and appeared to have a good efficacy. However, in 33%, a complete normalization of liver enzymes was never reached |
| Disciplines: | Medicina |
| Keyword: | Gastroenterología, Terapéutica y rehabilitación, Metabolismo y nutrición, D-penicilamina, Zinc, Cobre, Ceruloplasmina, Errores innatos del metabolismo, Eficacia |
| Keyword: | Gastroenterology, Therapeutics and rehabilitation, Metabolism and nutrition, D-penicillamine, Zinc, Copper, Ceruloplasmin, Inborn metabolic errors, Efficacy |
| Full text: | Texto completo (Ver PDF) |
