Neutropenia and impaired neutrophil migration in type IB glycogen storage disease.

AL Beaudet, DC Anderson, VV Michels… - The Journal of …, 1980 - europepmc.org
AL Beaudet, DC Anderson, VV Michels, WJ Arion, AJ Lange
The Journal of pediatrics, 1980europepmc.org
The association of neutropenia with type IB glycogen storage disease was observed in
siblings. Biochemical studies of liver demonstrated a defect in glucose-6-phosphate
transport. Neutrophil mobilization in vivo was impaired but the bone marrow was normal
histologically. In vitro studies of neutrophils indicated a defect in random and directed cell
migration. The studies suggest that a role for glucose-6-phosphate transport in neutrophils
should be considered.
The association of neutropenia with type IB glycogen storage disease was observed in siblings. Biochemical studies of liver demonstrated a defect in glucose-6-phosphate transport. Neutrophil mobilization in vivo was impaired but the bone marrow was normal histologically. In vitro studies of neutrophils indicated a defect in random and directed cell migration. The studies suggest that a role for glucose-6-phosphate transport in neutrophils should be considered.
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