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Wiskott-Aldrich syndrome

1969; American Medical Association; Volume: 99; Issue: 3 Linguagem: Inglês

10.1001/archderm.99.3.372

1538-3652

I. Mulhim,

Cellular Mechanics and Interactions

block but is common with associated malformations and symptomatic patients. In asymptomatic patients, prolonged QTc may herald the onset of symptoms(9). The risk of sudden death is twice great in patients who have a prolonged QTc interval. When all the high risk factors are present in a patient, prognosis remains grave. However, prompt treatment with ionotropic agents, temporary pacing and decongestive measures may save some babies, if diagnosed in utero. On the other hand, congenital heart block due to pure electric discontinuity remains asymptomatic and such subjects do fairly well. There is an approximately 95% twenty year survival of patients without anatomic heart defect(7).