GASTROINTESTINAL COMPLICATIONS OF CHRONIC GRANULOMATOUS-DISEASE - CASE-REPORT AND LITERATURE-REVIEW

Chronic granulomatous disease (CGD), an inherited disorder of phagocyt ic leukocyte function, is characterized by recurrent infections with c atalase-positive organisms. Gastrointestinal (GI) tract involvement, p resent in the majority of affected individuals, may be present initial ly and recurrently, mimics other entities such as inflammatory bowel d isease, and causes substantive morbidity and mortality. Disorders of m otility, ulceration, obstruction, and infection (e.g., abscesses) occu r from the mouth to the anus and stereotypically manifest with vomitin g, diarrhea, abdominal pain, weight loss, and Fever. Careful physical examination, in concert with appropriate diagnostic studies, is necess ary to delineate intraabdominal pathologic processes. Abdominal radiog raphs, ultrasonography, computerized tomography, and endoscopy are use ful ancillary diagnostic procedures. Drainage of accessible abscesses, antimicrobial therapy based on organisms cultured from blood and tiss ue, and interferon gamma may lead to suppression or eradication of inf ections and resolution of symptoms. Corticosteroids are useful for gas tric outlet obstruction and sulfasalazine and cyclosporine for large b owel disease. Gallbladder dysfunction may be ameliorated, as in our pa tient, with administration of cholestyramine.