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.
Bibliographical noteFunding Information:
From the Departments of Pediatrics and Cell Biology, Baylor College of Medicine and Howard Hughes Medical Institute; and the Department of Physiology, Biochemistry and Pharmacology, New York State College of Veterinary Medicine, Cornell University. Supported by United States Public Health Service Grants RR-O0188, AM-19625, and GM-25397," and by the United States Department of Agriculture support for the Children's Nutrition and Research Center Texas Children's Hospital. *Reprint address: Department of Pediatrics, Baylor College of Medicine, Houston, Texas 77030.