Gastrointestinal Abnormalities among Patients with Chronic Granulomatous Disease
|Arnon Broides1,2, Reem Mohammed2, Brenda Reid2, Chaim M. Roifman2 and Eyal Grunebaum2*|
|1 Pediatric Immunology Clinic, Division of Pediatrics, Soroka University Medical Center, Ben-Gurion University, Beer Sheva, Israel|
|2 Division of Immunology and Allergy, Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, Ontario|
|Corresponding Author :||Eyal Grunebaum MD
Head, Division of Immunology and Allergy
Hospital for Sick Children, 555 University Avenue
Toronto, Ontario M5G 1X8, Canada
E-mail: [email protected]
|Received December 15, 2013; Accepted March 31, 2014; Published April 07, 2014|
|Citation: Broides A, Mohammed R, Reid B, Roifman CM, Grunebaum E (2014) Gastrointestinal Abnormalities among Patients with Chronic Granulomatous Disease. J Clin Cell Immunol 5:204. doi: 10.4172/2155-9899.1000204|
|Copyright: © 2014 Broides A, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.|
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Objective: Chronic granulomatous disease (CGD) is characterized by increased susceptibility to infections and inflammation that may lead to various gastrointestinal (GI) abnormalities. Our objective was to better characterize the GI manifestations among patients suffering from CGD as well as the effects of different treatments.
Methods: We analyzed 11 patients with CGD managed by the immunology service at the Hospital for Sick Children, Toronto, Ontario between 2000 and 2012.
Results: All patients had one or more GI abnormality including colitis (72.7%), peri-anal fissure/abscess (36.3%) or oral aphthous ulcers (36.3%). Failure to thrive occurred in 5 patients (45.4%), all with associated colitis. Bone marrow transplantations (BMT) using HLA-identical sibling donors were performed in 4 patients, with 3 patients surviving. In these 3 patients, the inflammation-mediated GI manifestations present before BMT resolved during the follow-up period of 3.2-4.6 years. In contrast, 6 of 7 patients who did not receive BMT (p=0.033) continued to suffer from GI disease resulting in failure to thrive, GI bleeding and life threatening small bowel perforation and often required immune suppressive medications.
Conclusions: Inflammatory GI manifestations, particularly colitis, are very common in CGD and are often associated with significant morbidity. Allogeneic BMT, particularly if an HLA-matched sibling donor is available should be considered in patients with CGD who suffer from significant GI involvement.