Journal
INFLAMMATORY BOWEL DISEASES
Volume 7, Issue 2, Pages 128-132Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/00054725-200105000-00008
Keywords
inflammatory bowel disease; glycogen storage disease type Ib; granulocyte-colony stimulating factor
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Chronic inflammatory bowel disease (IBD)-like colitis is occasionally associated with glycogen storage disease-type 1b (GSD- Ib). We describe a 17-year old boy with GSD-1b who developed an IBD-like colitis. Roentgenography and colonoscopy showed the lead-pipe appearance of the colon and circumferential ulcers. Histopathologic examination revealed nonspecific inflammation without granulomatous lesions. High-dose granulocyte-colony stimulating factor (G-CSF) and sulfasalazine led to the resolution of the colitis, although neutropenia continued. Besides this cast, 10 published cases of GSD-lb and IBD-like colitis were reviewed. All cases had severe neutropenia and/or neutrophil dysfunction. The mean onset of bowel; disease was 12.3 years of age. Seven cases required surgical treatment. All five patients with G-CSF/GM-CSF therapy showed clinical remission. These findings suggest that IBD-like colitis is a grave complication of GSD-lb and that recurrent enteric infections due to neutrophil deficiency may contribute to the development of this bowel disease.
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