Colitis in Childhood

Russo, Pierre; Brown, Kenneth N.; Baldassano, Robert N.

doi:10.1007/978-1-4419-9066-2_5

Cited by 1 publication

(1 citation statement)

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“…In CD, noncaseating granulomas are found in about 50% of the mucosal biopsies of the GI tract (85,86). These sarcoid-like granulomas are composed of epithelioid cells and multinucleated giant cells and can be well formed (38,87,88). Necrosis is usually absent (or limited to a small central area).…”

Section: Granulomas In the Gi Tractmentioning

confidence: 99%

Overlap, Common Features, and Essential Differences in Pediatric Granulomatous Inflammatory Bowel Disease

Damen

Krieken

Hoppenreijs

et al. 2010

J. pediatr. gastroenterol. nutr.

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Overlap in the clinical presentation of pediatric granulomatous inflammatory bowel disease may be substantial, depending on the mode of presentation. Chronic granulomatous disease (CGD) may present with granulomatous colitis, perianal abscesses, hepatic abscesses or granulomas, failure to thrive, and obstruction of the gastrointestinal tract (including esophageal strictures and dysmotility, delayed gastric emptying, and small bowel obstruction). Anemia, thrombocytosis, elevated C-reactive protein and erythrocyte sedimentation rate, and hypoalbuminemia are nonspecific and may occur in any of the granulomatous inflammatory bowel diseases. In histology, macrophages with cytoplasmic inclusions will be rather specific for CGD. Sarcoidosis may present with abdominal pain or discomfort, diarrhea, weight loss, growth failure, delayed puberty, erythema nodosum, arthritis, uveitis, and hepatic granulomata. Only in 55% of the patients will angiotensin-converting enzyme be elevated. The noncaseating epithelioid granulomata will be unspecific. Bronchoalveolar lymphocytosis and abnormalities in pulmonary function are reported in sarcoidosis and in Crohn disease (CD) and CGD. Importantly, patients with CD may present with granulomatous lung disease, fibrosing alveolitis, and drug-induced pneumonitis. Sarcoidosis and concomitant gastrointestinal CD have been reported in patients, as well as coexistence of CD and sarcoidosis in siblings. Common susceptibility loci have been identified in CD and sarcoidosis. CD and CGD share defects in the defense mechanisms against different microbes. In the present review, common features and essential differences are discussed in clinical presentation and diagnostics--including histology--in CGD, sarcoidosis, and CD, together with 2 other granulomatous inflammatory bowel diseases, namely abdominal tuberculosis and Hermansky-Pudlak syndrome. Instructions for specific diagnosis and respective treatments are provided.

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