Outpatient management with oral corticosteroid therapy for obstructive conditions in chronic granulomatous disease

Danziger, Roger N.; Goren, Avner; Becker, Jack M.; Greene, Jeremy A.; Douglas, Steven D.

doi:10.1016/s0022-3476(06)80138-1

Cited by 45 publications

(18 citation statements)

References 8 publications

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“…When upper endoscopy was performed for symptoms of nausea and vomiting, there was no evidence of gastric outlet obstruction, unlike previous reports 22,23 . On our endoscopies, obstructive lesions such as ulcers, masses or pyloric stenosis, were not found.…”

Section: Discussionmentioning

confidence: 58%

Gastrointestinal Features of Chronic Granulomatous Disease Found During Endoscopy

Khangura

Kamal

et al. 2016

Clinical Gastroenterology and Hepatology

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Background & Aims Chronic granulomatous disease (CGD) is an inherited disorder of the NADPH oxidase complex within phagocytic cells that predisposes people to bacterial and fungal infections. Approximately 40% of patients with CGD have gastrointestinal involvement. We aimed to characterize the endoscopic features of gastrointestinal CGD and define the role of endoscopy in patients. Methods We created a database of all patients with CGD seen at the National Institutes of Health (NIH) from 1990 through 2010. We identified patients who had an endoscopy, and collected information from those with CGD-associated inflammatory bowel disease (CGD-IBD). We analyzed clinical data (demographic information and symptoms), endoscopic data (indication, preparation quality, degree of inflammation, mucosal findings, and complications), and pathologic data. Results A total of 211 endoscopies (96 esophagogastroduodenoscopies, 82 colonoscopies. and 33 flexible sigmoidoscopies) were performed at the NIH on 78 patients with CGD. Esophageal, gastric, and duodenal inflammation were detected in 21%, 74%, and 37% of patients, respectively. Esophageal dysmotility and structural abnormalities were noted in 26%. Of the patients who had colonic CGD-IBD, 74% had skip lesions and 93% had ano-rectal disease. Enteric fistulae were found in 18% of patients; 73% of these were perianal. Colonic strictures were observed in 24% of patients; 80% were in the ano-rectal area. Conclusions Based on an analysis of clinical and endoscopic data from 78 patients, CGD-IBD is a distinct entity, primarily involving the anus and rectum, with skip lesions in the remaining bowel. Bowel strictures and fistulae are present in a significant number of patients. Upper gastrointestinal tract inflammatory disease is common, though typically not as severe as colonic disease. Upper and lower endoscopies are important in characterizing the gastrointestinal features of CGD.

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Section: Discussionmentioning

confidence: 58%

Gastrointestinal Features of Chronic Granulomatous Disease Found During Endoscopy

Khangura

Kamal

et al. 2016

Clinical Gastroenterology and Hepatology

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“…The strongest association between phenotype and genotype was identified in patients with chronic GI or GU complications of CGD. These endpoints are characterized by an abnormal granulomatous or inflammatory process of the GI or GU mucosal surface, resulting in a chronic, debilitating condition (6)(7)(8)(9)(10)(11). Variant alleles of MPO, Fc ␥ RIIa, and Fc ␥ RIIIb each are associated with one or more of these outcomes (Table I) and in combination may contribute additively to susceptibility to one or more of these endpoints (Table II).…”

Section: Resultsmentioning

confidence: 99%

“…Nearly half of all CGD patients also develop significant chronic complications. These include gastric or urinary tract obstruction secondary to granuloma formation, granulomatous ileocolitis, perirectal abscesses, and rheumatologic disorders (6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17). The pathophysiologic processes contributing to these complications of CGD are poorly understood, but most certainly are mediated by NADPH oxidase-independent inflammatory pathways.…”

Section: Introductionmentioning

confidence: 99%

Host defense molecule polymorphisms influence the risk for immune-mediated complications in chronic granulomatous disease.

Foster¹,

Lehrnbecher²,

Mol³

et al. 1998

J. Clin. Invest.

224

132

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Chronic granulomatous disease (CGD) is an inherited disorder of phagocyte function in which defective superoxide production results in deficient microbicidal activity. CGD patients suffer from recurrent, life-threatening infections, and nearly half develop chronic gastrointestinal (GI) complications (colitis, gastric outlet obstruction, or perirectal abscess) and/or autoimmune/rheumatologic disorders (AIDs). To identify genetic modifiers of disease severity, we studied a cohort of 129 CGD patients, in whom seven candidate genes (

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“…A second feature of CGD in humans and mice is the frequent development of inflammatory granulomas in lung, skin, liver, and the lining of gastrointestinal and genitourinary tracts (27). Although incomplete resolution of active infection has been suggested as a possible reason for granuloma formation, the rapid response to systemic steroid therapy suggests a noninfectious etiology (28). Potential mechanisms for the in vivo occurrence of inflammatory granulomas have not been clearly defined.…”

mentioning

confidence: 99%

Exuberant Inflammation in Nicotinamide Adenine Dinucleotide Phosphate-Oxidase-Deficient Mice After Allogeneic Marrow Transplantation

Yang

Panoskaltsis‐Mortari

Shukla

et al. 2002

The Journal of Immunology

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We have shown that NO and superoxide ()contribute to donor T cell-dependent lung dysfunction after bone marrow transplantation (BMT) in mice. We hypothesized that inhibiting production during inducible NO synthase induction would suppress oxidative/nitrative stress and result in less severe lung injury. Irradiated mice lacking the phagocytic NADPH-oxidase (phox−/−), a contributor to generation, were conditioned with cyclophosphamide and given donor bone marrow in the presence or absence of inflammation-inducing allogeneic spleen T cells. On day 7 after allogeneic BMT, survival, weight loss, and indices of lung injury between phox−/− and wild-type mice were not different. However, the majority of macrophages/monocytes from phox−/− mice given donor T cells produced fewer oxidants and contained less nitrotyrosine than cells obtained from T cell-recipient wild-type mice. Importantly, suppressed oxidative stress was associated with marked infiltration of the lungs with inflammatory cells and was accompanied by increased bronchoalveolar lavage fluid levels of the chemoattractants monocyte chemoattractant protein-1 and macrophage-inflammatory protein-1α and impaired clearance of recombinant mouse macrophage-inflammatory protein-1β from the circulation. Furthermore, cultured macrophages/monocytes from NADPH-deficient mice produced 3-fold more TNF-α compared with equal number of cells from NADPH-sufficient mice. The high NO production was not modified during NADPH-oxidase deficiency. We conclude that phox−/− mice exhibit enhanced pulmonary influx of inflammatory cells after BMT. Although NO may contribute to increased production of TNF-α in phox−/− mice, the data suggest that NADPH-oxidase-derived oxidants have a role in limiting inflammation and preventing lung cellular infiltration after allogeneic transplantation.

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Outpatient management with oral corticosteroid therapy for obstructive conditions in chronic granulomatous disease

Cited by 45 publications

References 8 publications

Gastrointestinal Features of Chronic Granulomatous Disease Found During Endoscopy

Gastrointestinal Features of Chronic Granulomatous Disease Found During Endoscopy

Host defense molecule polymorphisms influence the risk for immune-mediated complications in chronic granulomatous disease.

Exuberant Inflammation in Nicotinamide Adenine Dinucleotide Phosphate-Oxidase-Deficient Mice After Allogeneic Marrow Transplantation

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