RESUMO

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.

Assuntos

Agranulocitose/etiologia , Quimiotaxia de Leucócito , Doença de Depósito de Glicogênio Tipo I/sangue , Neutropenia/etiologia , Glucose-6-Fosfatase/análise , Doença de Depósito de Glicogênio Tipo I/complicações , Humanos , Lactente , Fígado/análise , Masculino , Neutrófilos