Clinical features and current management of chronic granulomatous disease.
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.
Forrest
C B
CB
Children's Hospital of Pennsylvania, Philadelphia.
Forehand
J R
JR
Axtell
R A
RA
Roberts
R L
RL
Johnston
R B
RB
D000293:Adolescent; D000328:Adult; D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D004338:Drug Combinations / Q000493:pharmacokinetics / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000276:immunology / Q000378:metabolism / Q000628:therapy*; D006801:Humans; D007223:Infant; D017053:Infection Control; D007239:Infections / Q000453:epidemiology / Q000209:etiology; D008297:Male; D009247:NADH, NADPH Oxidoreductases / Q000172:deficiency / Q000378:metabolism; D019255:NADPH Oxidases; D010101:Oxygen Consumption; D010586:Phagocytes / Q000201:enzymology / Q000276:immunology / Q000378:metabolism; D013420:Sulfamethoxazole / Q000493:pharmacokinetics / Q000627:therapeutic use; D013481:Superoxides / Q000378:metabolism; D014295:Trimethoprim / Q000493:pharmacokinetics / Q000627:therapeutic use; D015662:Trimethoprim, Sulfamethoxazole Drug Combination
D004338:Drug Combinations; D013481:Superoxides; D015662:Trimethoprim, Sulfamethoxazole Drug Combination; D014295:Trimethoprim; D009247:NADH, NADPH Oxidoreductases; D019255:NADPH Oxidases; D013420:Sulfamethoxazole