Impaired metabolic function of polymorphonuclear leukocytes in glycogen storage disease Ib.

Abstract:

:To elucidate the basis for the recurrent infections in patients with glycogen storage disease (GSD) Ib we tested polymorphonuclear leukocyte (PMN) function in one patient. Bactericidal capacity and phagocytosis-induced O2 consumption were reduced. Also, phorbol myristate acetate-stimulated superoxide production and glucose oxidation through the hexose monophosphate shunt were diminished compared to control subjects. Therefore it could be speculated that in PMN of patients with GSD Ib, glucose-6-phosphate has no access to the enzymes of the hexose monophosphate shunt due to a transport-related defect as shown for glucogenesis in hepatocytes.

journal_name

Eur J Pediatr

authors

Gahr M,Heyne K

doi

10.1007/BF00442674

subject

Has Abstract

pub_date

1983-09-01 00:00:00

pages

329-30

issue

4

eissn

0340-6199

issn

1432-1076

journal_volume

140

pub_type

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