[Cardiomyopathy and inborn errors of metabolism in children. Study of 12 cases].
| Autor: | Sabaté Rotés A; Servicio de Pediatría, Hospital Universitari Vall d'Hebron, Barcelona, España. annasabaterotes@gmail.com, Del Toro Riera M, Albert Brotons DC, Arranz Amo JA, Carrascosa Lezcano A, Girona Comas J |
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| Jazyk: | Spanish; Castilian |
| Zdroj: | Medicina clinica [Med Clin (Barc)] 2011 Nov 19; Vol. 137 (13), pp. 596-9. Date of Electronic Publication: 2011 Sep 28. |
| DOI: | 10.1016/j.medcli.2011.06.019 |
| Abstrakt: | Background and Objective: Cardiomyopathy in childhood is a rare entity. Inborn errors of metabolism can cause myocardial involvement by several mechanisms. Patients and Methods: Patients under 16 years diagnosed with cardiomyopathy and EIM in a period of 11 years (1998-2009) were included. Results: A total of 12 patients were studied (8% of all cardiomyopathies), 9 boys and three girls, with a median age at diagnosis of 6 months (range: birth-8.8 years). Fifty percent had an onset with cardiac symptoms and heart failure was associated with an earlier diagnosis of the disease (P<.05). On ultrasound 10 patients had ventricular hypertrophy, which was associated with mitochondrial and lysosomal disease; only 2 patients had ventricular dilatation, which was associated with altered fatty acid metabolism (P<.05). The median survival was 5 months (range: 2-11 months). No variable was significantly associated with the likelihood of death. Conclusions: Patients with heart failure at onset are diagnosed earlier (before 3 months of life). Echocardiography helps in the diagnosis and monitoring of metabolic disease. (Copyright © 2011 Elsevier España, S.L. All rights reserved.) |
| Databáze: | MEDLINE |
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