Abstract
Chronic granulomatous disease (CGD) is a diverse disorder characterized by several clinical presentations and a multitude of metabolic defects. The authors summarize the clinical features and current management of CGD, offer a definition of the disease based on molecular defects of the respiratory burst, and present their experience with trimethoprim/sulfamethoxazole prophylaxis.
Publication types
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Adolescent
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Adult
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Bone Marrow Transplantation
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Child
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Child, Preschool
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Drug Combinations / pharmacokinetics
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Drug Combinations / therapeutic use
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Drug Therapy, Combination
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Female
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Granulomatous Disease, Chronic / immunology
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Granulomatous Disease, Chronic / metabolism
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Granulomatous Disease, Chronic / therapy*
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Humans
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Infant
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Infection Control
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Infections / epidemiology
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Infections / etiology
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Male
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NADH, NADPH Oxidoreductases / deficiency
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NADH, NADPH Oxidoreductases / metabolism
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NADPH Oxidases
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Oxygen Consumption
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Phagocytes / enzymology
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Phagocytes / immunology
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Phagocytes / metabolism
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Sulfamethoxazole / pharmacokinetics
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Sulfamethoxazole / therapeutic use
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Superoxides / metabolism
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Trimethoprim / pharmacokinetics
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Trimethoprim / therapeutic use
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Trimethoprim, Sulfamethoxazole Drug Combination
Substances
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Drug Combinations
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Superoxides
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Trimethoprim, Sulfamethoxazole Drug Combination
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Trimethoprim
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NADH, NADPH Oxidoreductases
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NADPH Oxidases
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Sulfamethoxazole