Diversion of the Fecal Stream Resolves Ulcerative Colitis Complicating Chronic Granulomatous Disease in an Adult Patient

Villanacci, Vincenzo; Falchetti, Diego; Liserre, Barbara; Soresina, Anna Rosa; Plebani, Alessandro; Ekema, George; Bassotti, Gabrio

doi:10.1097/01.mcg.0000212638.44735.78

Cited by 4 publications

(2 citation statements)

References 17 publications

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“…In terms of treatment, there is some evidence to support therapeutic benefits of steroids and 5-aminosalicylate drugs, 77 and there have been occasional reports of the efficacy of ciclosporin, 78 infliximab, 73,74 G-CSF, 79 thalidomide, 74 interferon-␥, 74 intestinal diversion, 80 bowel resection, and bone marrow transplantation. 73 There have been no studies that are informative on maintaining disease remission.…”

Section: Chronic Granulomatous Diseasementioning

confidence: 97%

Phagocyte dysfunction and inflammatory bowel disease

Rahman

Marks

Hayee

et al. 2008

Inflammatory Bowel Diseases

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Inflammatory bowel diseases are common chronic inflammatory disorders. The majority are idiopathic and can be broadly divided into Crohn's disease and ulcerative colitis. Their cause is unknown, but most hypotheses focus on a primary role for T-cell dysfunction. Conversely, there is a collection of congenital disorders of phagocyte function that result not only in immunodeficiency but also in noninfectious inflammatory bowel disease. In all cases, the latter is strikingly reminiscent of the clinical and pathological features of Crohn's disease. This coincides with recent work demonstrating that despite previous emphasis on adaptive immune dysfunction, patients with Crohn's disease actually possess an unusually weak acute innate inflammatory response. This review consolidates the literature on inflammatory bowel disease in congenital immunodeficiencies and considers the role of phagocyte dysfunction in Crohn's disease. Concepts about pathogenesis and treatment that can be carried across these disorders are also discussed.

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Section: Chronic Granulomatous Diseasementioning

confidence: 97%

Phagocyte dysfunction and inflammatory bowel disease

Rahman

Marks

Hayee

et al. 2008

Inflammatory Bowel Diseases

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“…In animal models of colitis, inflammation is prevented by germ-free conditions [88,89]. Surgical diversion of the faecal stream is followed by improvement of inflammation in many patients with recurrence after restoration of the intestinal faecal stream [90,91]. Several probiotics (such as Escherichia coli Nissle 1917) have shown efficacy in the treatment or the prophylaxis of flares in ulcerative colitis [92][93][94].…”

Section: The Important Role Of the Intestinal Microbiotamentioning

confidence: 99%

New insights into the pathophysiology of inflammatory bowel disease: microbiota, epigenetics and common signalling pathways

2018

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The exact pathophysiology of inflammatory bowel disease (IBD) is still unknown. However, over the years important insights allowed the development of novel therapeutic approaches that are at the threshold of introduction into clinical practice, or at least in clinical trials. After being first described by Burrill B. Crohn, Crohn's disease, one of the two major forms of IBD, was perceived as an infectious disease. When the concept of autoimmune diseases was formulated, Crohn's disease and ulcerative colitis were thought to be members of this disease group. T cells certainly contribute to the chronification of the intestinal inflammation and targeting T cell migration has been introduced some years ago as a successful therapeutic approach in IBD. Despite the development of successful therapy based on this pathophysiological concept, IBD is no longer seen as a typical autoimmune disease. After the millennium, genome wide association studies on genetic variants and risk factors in these polygenetic diseases have told us a lot about pathogenetic pathways. However, genetic susceptibility explains only up to one third of the cases. Environmental factors also must play a role. Those environmental factors may "transfer" their disease-promoting potential into pathophysiological pathways with the intestinal microbiota as mediator. Hence, the intestinal microbiota has gained much attention as an important factor in disease development. Microbial factors, as well as other direct environmental influences, have been shown to affect epigenetic signatures, intestinal epithelial cells and the innate immune system, providing another important concept on how these diseases originate and can cause repeated flares at the same gut segments even after years of remission and after intermediate complete mucosal healing. Current pathophysiological concepts of IBD not only help us to better understand these diseases and develop new therapies. They also illustrate the evolution of basic scientific concepts over time and that sometimes partially or even largely abandoned concepts persistently influence out current thinking/clinical practice.

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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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Diversion of the Fecal Stream Resolves Ulcerative Colitis Complicating Chronic Granulomatous Disease in an Adult Patient

Cited by 4 publications

References 17 publications

Phagocyte dysfunction and inflammatory bowel disease

Phagocyte dysfunction and inflammatory bowel disease

New insights into the pathophysiology of inflammatory bowel disease: microbiota, epigenetics and common signalling pathways

Gastrointestinal Features of Chronic Granulomatous Disease Found During Endoscopy

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