BRIEF REPORT - TREATMENT OF CHRONIC INFLAMMATORY BOWEL-DISEASE IN GLYCOGEN-STORAGE-DISEASE TYPE-IB WITH COLONY-STIMULATING FACTORS

GLYCOGEN storage disease Type Ib is a rare hereditary metabolic disorder characterized by growth failure, hepatomegaly, hypoglycemia, lactic acidosis, and neutrophil deficiency.1 , 2 Except for the neutrophil abnormalities, which are unexplained, all these features appear to be due to the absence of a microsomal transport system for glucose-6-phosphate within hepatocytes.3 Oral lesions and perianal abscesses are common in this disorder4 and are attributable to neutropenia and to impaired neutrophil chemotaxis and metabolism.5 6 7 8 9 10 In 1986 we reported the association of glycogen storage disease Type Ib and chronic enteritis, indistinguishable from Crohn's disease, in two adolescent boys.11 This association was subsequently confirmed by… © 1992, Massachusetts Medical Society. All rights reserved.