Natural history of chronic granulomatous disease.

Abstract

An analysis of the patient characteristics, clinical course, and therapy of 52 patients with chronic granulomatous disease (CGD) was carried out through a multinstitutional questionnaire survey in order to further define the natural history of this disorder of phagocyte microbicidal function. The results suggest that CGD is characterized by significant heterogeneity in clinical course and prognosis. The age at diagnosis, severity of disease, or early death in an affected sibling does not seem to be of value in predicting the prognosis for individual patients with CGD. A more detailed, large-scale multicenter study and the establishment of a patient registry is recommended.