Undetected carrier state in chronic granulomatous disease

Abstract

A male child who previously had polydactylitis due to Serratia marcescens was found to have chronic granulomatous disease. The motility and phagocytic activity of his polymorphonuclear leukocytes and the opsonic and chemotactic activities of his serum were normal, but nitroblue tetrazolium reduction after phagocytosis was reduced greatly. The killing of a H 2 O 2 -producing bacteria, Streptococcus viridans , by his blood leukocytes was normal, but the killing of non-H 2 O 2 -producing bacteria, Serratia marcescens and Staphylococcus aureus , was reduced. The activities of NADH and NADPH oxidase, glucose-6-phosphate dehydrogenase, 6-phosphogluconate dehydrogenase, galactose-6-phosphate dehydrogenase, and glutathione peroxidase and reductase in the blood leukocytes were normal, and myeloperoxidase was detected cytochemically. In contrast to most other reports, a carrier of chronic granulomatous disease could not be demonstrated by either nitroblue tetrazolium reduction or bactericidal tests.