Brief Report: Treatment of Chronic Inflammatory Bowel Disease in Glycogen Storage Disease Type Ib with Colony-Stimulating Factors

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Abstract
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