| Autor: |
Anna, Sabaté Rotés, Mireia, Del Toro Riera, Dimpna C, Albert Brotons, José Antonio, Arranz Amo, Antonio, Carrascosa Lezcano, Josep, Girona Comas |
| Rok vydání: |
2011 |
| Předmět: |
|
| Zdroj: |
Medicina clinica. 137(13) |
| ISSN: |
1578-8989 |
| Popis: |
Cardiomyopathy in childhood is a rare entity. Inborn errors of metabolism can cause myocardial involvement by several mechanisms.Patients under 16 years diagnosed with cardiomyopathy and EIM in a period of 11 years (1998-2009) were included.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.Patients with heart failure at onset are diagnosed earlier (before 3 months of life). Echocardiography helps in the diagnosis and monitoring of metabolic disease. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
|
