PEDIATRICS Vol. 48 No. 5 November 1971, pp. 730-739
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CHRONIC GRANULOMATOUS DISEASE: CORRELATION BETWEEN PATHOGENESIS AND CLINICAL FINDINGS

Richard B. Johnston Jr. M.D.1 and Robert L. Baehner M.D.1

1 Department of Pediatrics, University of Alabama Medical Center, Birmingham; and the Division of Research Hematology, Department of Medicine, Children's Hospital Medical Center, and the Department of Pediatrics, Harvard Medical School, Boston

Chronic granulomatous disease (CGD) results from a failure of patients' phagocytic cells to kill ingested bacteria. The inability to kill intraphagocytic microorganisms leads to granuloma formation and abscesses throughout the reticuloendothelial system. Thus, the clinical picture of CGD is characterized by suppurative lymphadenitis, hepatosplenomegaly, pneumonia, and hematologic evidence of chronic infection. The bactericidal defect which characterizes CGD phagocytes appears to be due to their inability to generate hydrogen peroxide (H2O2). The organisms which infect patients are those which require phagocytic production of H2O2 for death–staphylococci and gram-negative enterics. There are simple means of confirming a clinical diagnosis of CGD, and prolonged therapy with specific antibiotics may therefore suppress the frequency of such serious infections.

Submitted on January 8, 1970
Accepted on January 22, 1971




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