Chronic inflammatory bowel disease in glycogen storage disease type 1B.
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.
Sanderson
I R
IR
Hospital for Sick Children, London, UK.
Bisset
W M
WM
Milla
P J
PJ
Leonard
J V
JV
D002648:Child; D002908:Chronic Disease; D003424:Crohn Disease / Q000209:etiology; D005953:Glycogen Storage Disease Type I / Q000150:complications* / Q000473:pathology; D006801:Humans; D015212:Inflammatory Bowel Diseases / Q000178:diet therapy / Q000209:etiology* / Q000473:pathology; D008297:Male; D009504:Neutrophils / Q000473:pathology