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Chronic inflammatory bowel disease in glycogen storage disease type 1B
Author(s) -
Sanderson I. R.,
Bisset W. M.,
Milla P. J.,
Leonard J. V.
Publication year - 1991
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf01799948
Subject(s) - inflammatory bowel disease , medicine , glycogen storage disease , ileitis , disease , pathogenesis , gastroenterology , inflammation , colitis , immunology , crohn's disease
Summary 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.