Recurrent Infection in Glycogenosis Type Ib: Abnormal Neutrophil Motility Related to Impaired Redistribution of Adhesion Sites

Neutrophil function was investigated in a male child with glycogenosis type Ib who demonstrated susceptibilityto staphylococcal infections and neutropenia. Random motility and directed migration of the patient's neutrophils in vitro were profoundly diminished. The patient's neutrophils stimulated in suspension with chemotactic factors (CFs) generated chemiluminescence that was comparable to or greater than that generated by neutrophils from controls, but the patient's neutrophils failed to assume a normal bipolar configuration in response to chemotactic stimuli. They also failed to demonstrate enhanced adherence after a singleexposure to CFs or decreasedadherence after sequential exposures to increasingconcentrations of CFs. Unlike neutrophils from controls, the patient's neutrophils failed to redistribute surface adhesion sites from lamellipodia (anterior pole) to uropods (tail) after sequential CF stimuli. These findings indicate a functional link between CF-induced configurational changes and altered adhesiveness of neutrophils under conditions of directed locomotion and suggest that a redistribution of surface adhesion sites is related to the mechanism of neutrophil locomotion.