Chronic inflammatory bowel disease in glycogen storage disease type 1B
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Two children with glycogen storage disease type 1B developed chronic inflammatory bowel disease. The first, a 7-year-old boy, had ileitis and later developed perianal disease. The second developed colitis by the age of 9 years; in both the features were consistent with Crohn disease. The children had neutropenia and neutrophil mobility defects characteristic of GSD-1B. It is suggested that these neutrophil abnormalities are important in the pathogenesis of the bowel inflammation.
KeywordsCrohn Disease Glycogen Storage Disease Chronic Inflammatory Bowel Disease Glycogen Storage Disease Type Hexose Monophosphate
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- Fine, R. N., Kogut, M. D. and Donnell, G. N. Intestinal absorption in type 1 glycogen storage disease.J. Pediatr. 75 (1969) 631–635Google Scholar
- Hers, H.-G., Van Hoof, F. and de Barsy, T. Glycogen storage disease. In Scriver, C. R., Beaudet, A. L., Sly, W. S. and Valle, D. (eds.)The Metabolic Basis of Inherited Disease, 6th edn., McGraw-Hill, New York, 1989, pp. 425–452Google Scholar
- Sacher, D. B., Aulander, M. O. and Walfish, J. S. Aetiological theories of inflammatory bowel disease.Clin. Gastroenterol. 9 (1980) 231–257Google Scholar