CROHNS-LIKE COLITIS IN GLYCOGEN-STORAGE-DISEASE-IB - A CASE-REPORT

Association between glycogen storage disease type Ib and Crohn's like disease is rare. We present one such case. A young woman with glycogen storage disease type Ib underwent a right hemicolectomy for stenosis and a blind fistulous tract. The histological picture of the resected bowel was consistent with the diagnosis of Crohn's like disease. A lea k in the enterocolostomy occurred during medical treatment with steroi ds, sulfasalazine, and methronidazole. Partial ileal and colonic resec tion and a new anastomosis were then performed. Recombinant human colo ny-stimulating factor (300 mcg/die subcutaneously) was then started. T he neutrophils promptly increased to a normal range, and the neutrophi l function tests improved. The postoperative course was uneventful and the patient did not have a relapse of the bowel disease.