Crohn's-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 leak in the entero-colostomy occurred during medical treatment with steroids, sulfasalazine, and methronidazole. Partial ileal and colonic resection and a new anastomosis were then performed. Recombinant human colony-stimulating factor (300 mcg/die subcutaneously) was then started. The neutrophils promptly increased to a normal range, and the neutrophil function tests improved. The postoperative course was uneventful and the patient did not have a relapse of the bowel disease.