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PEDIATRICS Vol. 114 No. 2 August 2004, pp. 462-468

Gastrointestinal Involvement in Chronic Granulomatous Disease

Beatriz E. Marciano, MD*, Sergio D. Rosenzweig, MD*, David E. Kleiner, MD{ddagger}, Victoria L. Anderson, MSN, CRNP*, Dirk N. Darnell, RN, MSN*, Sandra Anaya-O'Brien, RN, MSN*, Dianne M. Hilligoss, MSN, CRNP*, Harry L. Malech, MD*, John I. Gallin, MD* and Steven M. Holland, MD*

* Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Department of Health and Human Services, Bethesda, Maryland
{ddagger} Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Bethesda, Maryland

Objective. Chronic granulomatous disease (CGD) is a rare disorder of phagocyte oxidative metabolism. In addition to infectious complications, granulomatous lesions often involve hollow viscera, especially the gastrointestinal (GI) tract. The objective of this study was to evaluate the clinical presentation, prevalence, and consequences of GI involvement in patients with CGD.

Methods. The medical records of 140 patients with CGD (67% X-linked) followed at the National Institutes of Health were reviewed and abstracted for GI manifestations. All available GI pathology was reviewed.

Results. GI involvement was recorded in 46 (32.8%) of 140 patients with CGD, 89% of whom had X-linked inheritance. The median age at the time of initial GI manifestations was 5 years (range: 0.8–30 years); 70% of the affected patients presented with GI involvement in the first decade of life. Abdominal pain was the most frequent symptom (100%), and hypoalbuminemia was the most frequent sign (70%). Prednisone controlled symptoms and signs in the majority of affected patients, but relapse of symptoms occurred in 71%. GI involvement had no effect on mortality and was unassociated with interferon-{gamma} use.

Conclusion. GI involvement is a common and recurring problem in CGD, especially in those with X-linked inheritance. Currently, there is no clear evidence for an infectious cause. The frequency of GI involvement is unaffected by the use of interferon-{gamma} and does not affect mortality. GI involvement should be sought in patients who have CGD with abdominal pain, growth delay, or hypoalbuminemia.

Key Words: chronic granulomatous disease • inflammatory bowel disease • interferon-{gamma} • colitis • obstruction • steroids

Abbreviations: CGD, chronic granulomatous disease • GI, gastrointestinal • NIH, National Institutes of Health • CI, confidence interval • IFN-{gamma}, interferon-{gamma} • OR, odds ratio

Received for publication Aug 7, 2003; Accepted Dec 2, 2003.


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