Short Communication. Glycogenosis Ib: Neutrophil Microbicidal Defects Due to Impaired Hexose Monophosphate Shunt
作者:
REINHARD SEGER,
BEAT STEINMANN,
LOUIS TIEFENAUER,
TEIICHI MATSUNAGA,
RICHARD GITZELMANN,
期刊:
Pediatric Research
(OVID Available online 1984)
卷期:
Volume 18,
issue 3
页码: 297-299
ISSN:0031-3998
年代: 1984
出版商: OVID
数据来源: OVID
摘要:
SummaryWe studied neutrophil microbicidal function and oxidative metabolic activity in a patient with glycogenosis Ib. The intracellular killing defect and the respiratory burst abnormality in gycogenosis Ib neutrophils were confirmed. The impaired oxygen-dependent microbicidal activity was shown to result from impaired hexose monophosphate shunt activity (impaired endogenous NADPH synthesis) and could be corrected by homogenization of the cells, followed by the addition of exogenous NADPH. Our data are thus consistent with a possible role for glucose-6-phosphate transport in neutrophil microbicidal function. We recommend a continuous prophylaxis with co-trimoxazole in patients with glycogen storage disease Ib.
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