Advertising Disclaimer
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me when eLetters are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrowRequest Permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Web of Science (40)
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Marciano, B. E.
Right arrow Articles by Holland, S. M.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Marciano, B. E.
Right arrow Articles by Holland, S. M.
Related Collections
Right arrow Gastrointestinal Tract
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Facebook   Add to Reddit   Add to Technorati   Add to Twitter  
What's this?
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*, 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.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Facebook Facebook   Add to Reddit Reddit   Add to Technorati Technorati   Add to Twitter Twitter    What's this?


This article has been cited by other articles:


Home page
 BloodHome page
J. D. Matute, A. A. Arias, N. A. M. Wright, I. Wrobel, C. C. M. Waterhouse, X. J. Li, C. C. Marchal, N. D. Stull, D. B. Lewis, M. Steele, et al.
A new genetic subgroup of chronic granulomatous disease with autosomal recessive mutations in p40phox and selective defects in neutrophil NADPH oxidase activity
Blood, October 8, 2009; 114(15): 3309 - 3315.
[Abstract] [Full Text] [PDF]

Home page
 J Med MicrobiolHome page
J. Roesler
Important role of corticosteroids in chronic granulomatous disease
J. Med. Microbiol., September 1, 2007; 56(9): 1253 - 1253.
[Full Text] [PDF]

Home page
 Physiol. Rev.Home page
K. Bedard and K.-H. Krause
The NOX Family of ROS-Generating NADPH Oxidases: Physiology and Pathophysiology
Physiol Rev, January 1, 2007; 87(1): 245 - 313.
[Abstract] [Full Text] [PDF]

Home page
 J Med MicrobiolHome page
Y. Arimura, A. Goto, K. Yamashita, T. Endo, H. Ikeda, K. Tanaka, H. Tsutsumi, Y. Shinomura, and K. Imai
Intractable colitis associated with chronic granulomatous disease.
J. Med. Microbiol., November 1, 2006; 55(Pt 11): 1587 - 1590.
[Abstract] [Full Text] [PDF]

Home page
 RadioGraphicsHome page
G. Khanna, S. C. Kao, P. Kirby, and Y. Sato
Imaging of Chronic Granulomatous Disease in Children
RadioGraphics, September 1, 2005; 25(5): 1183 - 1195.
[Abstract] [Full Text] [PDF]

Home page
 ASH Education BookHome page
M. C. Dinauer
Chronic Granulomatous Disease and Other Disorders of Phagocyte Function
Hematology, January 1, 2005; 2005(1): 89 - 95.
[Abstract] [Full Text] [PDF]