Advertisement
Research Article Free access | 10.1172/JCI105568
Department of Pediatrics, University of Minnesota Medical School, Minneapolis, Minn.
Department of Microbiology, University of Minnesota Medical School, Minneapolis, Minn.
†John and Mary R. Markle Scholar in Academic Medicine. Recipient of U. S. Public Health Service Career Development Award (5-K3-AI-10, 109-05).
Address requests for reprints to Dr. P. G. Quie, Dept. of Pediatrics, University of Minnesota Medical School, Minneapolis, Minn. 55455.
‡American Legion Memorial Heart Research Professor of Pediatrics and Microbiology.
*Submitted for publication October 31, 1966; accepted December 30, 1966.
Supported in part by National Foundation, American Heart Association, and U. S. Public Health Service (AI-06931, HE-02085, AI-00798, and NB-02042) grants and the Commission on Streptococcal and Staphylococcal Diseases, Armed Forces Epidemiological Board, Office of the Surgeon General, Dept. of the Army, Washington, D. C.
Presented in part at the 58th Annual Meeting of the American Society for Clinical Investigation, Atlantic City, N. J., May 2, 1966.
Find articles by Quie, P. in: JCI | PubMed | Google Scholar
Department of Pediatrics, University of Minnesota Medical School, Minneapolis, Minn.
Department of Microbiology, University of Minnesota Medical School, Minneapolis, Minn.
†John and Mary R. Markle Scholar in Academic Medicine. Recipient of U. S. Public Health Service Career Development Award (5-K3-AI-10, 109-05).
Address requests for reprints to Dr. P. G. Quie, Dept. of Pediatrics, University of Minnesota Medical School, Minneapolis, Minn. 55455.
‡American Legion Memorial Heart Research Professor of Pediatrics and Microbiology.
*Submitted for publication October 31, 1966; accepted December 30, 1966.
Supported in part by National Foundation, American Heart Association, and U. S. Public Health Service (AI-06931, HE-02085, AI-00798, and NB-02042) grants and the Commission on Streptococcal and Staphylococcal Diseases, Armed Forces Epidemiological Board, Office of the Surgeon General, Dept. of the Army, Washington, D. C.
Presented in part at the 58th Annual Meeting of the American Society for Clinical Investigation, Atlantic City, N. J., May 2, 1966.
Find articles by White, J. in: JCI | PubMed | Google Scholar
Department of Pediatrics, University of Minnesota Medical School, Minneapolis, Minn.
Department of Microbiology, University of Minnesota Medical School, Minneapolis, Minn.
†John and Mary R. Markle Scholar in Academic Medicine. Recipient of U. S. Public Health Service Career Development Award (5-K3-AI-10, 109-05).
Address requests for reprints to Dr. P. G. Quie, Dept. of Pediatrics, University of Minnesota Medical School, Minneapolis, Minn. 55455.
‡American Legion Memorial Heart Research Professor of Pediatrics and Microbiology.
*Submitted for publication October 31, 1966; accepted December 30, 1966.
Supported in part by National Foundation, American Heart Association, and U. S. Public Health Service (AI-06931, HE-02085, AI-00798, and NB-02042) grants and the Commission on Streptococcal and Staphylococcal Diseases, Armed Forces Epidemiological Board, Office of the Surgeon General, Dept. of the Army, Washington, D. C.
Presented in part at the 58th Annual Meeting of the American Society for Clinical Investigation, Atlantic City, N. J., May 2, 1966.
Find articles by Holmes, B. in: JCI | PubMed | Google Scholar
Department of Pediatrics, University of Minnesota Medical School, Minneapolis, Minn.
Department of Microbiology, University of Minnesota Medical School, Minneapolis, Minn.
†John and Mary R. Markle Scholar in Academic Medicine. Recipient of U. S. Public Health Service Career Development Award (5-K3-AI-10, 109-05).
Address requests for reprints to Dr. P. G. Quie, Dept. of Pediatrics, University of Minnesota Medical School, Minneapolis, Minn. 55455.
‡American Legion Memorial Heart Research Professor of Pediatrics and Microbiology.
*Submitted for publication October 31, 1966; accepted December 30, 1966.
Supported in part by National Foundation, American Heart Association, and U. S. Public Health Service (AI-06931, HE-02085, AI-00798, and NB-02042) grants and the Commission on Streptococcal and Staphylococcal Diseases, Armed Forces Epidemiological Board, Office of the Surgeon General, Dept. of the Army, Washington, D. C.
Presented in part at the 58th Annual Meeting of the American Society for Clinical Investigation, Atlantic City, N. J., May 2, 1966.
Find articles by Good, R. in: JCI | PubMed | Google Scholar
Published April 1, 1967 - More info
Diminished bactericidal capacity was found to be characteristic of polymorphonuclear leukocytes (PMN) from five children with the clinical syndrome of granulomatous disease of childhood. The PMN from these children demonstrated nearly normal phagocytic capacity, and the majority of viable bacteria, after 2 hours of incubation in the phagocytosis system, were found associated with leukocytes.
The morphology of the unstimulated polymorphonuclear leukocytes from patients with chronic granulomatous disease was similar to those from normal persons of similar ages by light and electron microscopy. In addition, the total lysozyme and phagocytin activity of leukocyte extracts from these patients was similar to those from equal numbers of leukocytes from controls.
A striking difference in the cytoplasmic response after phagocytosis characterized the PMN of the patients with granulomatous disease. Whereas degranulation, vacuole formation, and rapid bacterial digestion were the rule in the PMN from controls, little degranulation and persistence of intact bacteria in the cytoplasm characterized disease.
The deficiency of bactericidal capacity and the minimal degranulation after active phagocytosis by the PMN of these children with an inherited syndrome suggest that separate metabolic processes are involved in phagocytosis and in intracellular digestion. Continuing study of the metabolic function of leukocytes from these children should provide an opportunity for increased understanding of the metabolic basis for degranulation and intracellular digestion in phagocytic cells.
Images.