Literature DB >> 17101573

Homozygosity for the CARD15 frameshift mutation 1007fs is predictive of early onset of Crohn's disease with ileal stenosis, entero-enteral fistulas, and frequent need for surgical intervention with high risk of re-stenosis.

Julia Seiderer1, Fabian Schnitzler, Stephan Brand, Tanja Staudinger, Simone Pfennig, Karin Herrmann, Katrin Hofbauer, Julia Dambacher, Cornelia Tillack, Michael Sackmann, Burkhard Göke, Peter Lohse, Thomas Ochsenkühn.   

Abstract

OBJECTIVE: The identification of CARD15 as a susceptibility gene for Crohn's disease (CD) offers new possibilities for patient classification and risk assessment. The purpose of this study was to carry out a CARD15 sequence analysis in a large single-center IBD cohort and to investigate the impact of different genotypes on disease phenotypes.
MATERIAL AND METHODS: A total of 445 unrelated patients with IBD (68.1% CD, 28.5% ulcerative colitis (UC), 3.4% indeterminate colitis (IC)) were included in the study. Clinical data were recorded by detailed questionnaire and analysis of the charts. CARD15 variants (R702W, G908R, 1007fs (frameshift)) were identified by DNA sequence analysis.
RESULTS: CARD15 variants were found in 142 inflammatory bowel disease (IBD) patients (31.9%) including 120 CD patients (39.6%). In CD, the presence of two CARD15 variants was associated with ileal disease (p=0.008 versus wild-type (wt); OR 4.04; 95% CI 1.36-11.96) and a fibrostenotic phenotype (p=0.002 versus wt; OR 5.47; 95% CI 1.61-18.58). Subgroup analysis of 19 patients (4.3%) homozygous for the CARD15 variant 1007fs (3020ins C) revealed an association with onset of CD at an early age (p=0.014 versus wt), ileal involvement (p=0.001), and intestinal stenoses in all patients (p=0.001) frequently requiring surgery (73.7%; p=0.093). Of these patients 78.6% developed re-stenoses after surgical resection; 52.6% of the homozygotes were diagnosed as having entero-enteral fistulas.
CONCLUSIONS: Patients homozygous for the 1007fs mutation had an early disease onset with long-segment ileal stenoses and entero-enteral fistulas. They frequently needed surgical intervention and had a high risk of re-stenosis. Genotyping therefore appears to be an important diagnostic tool in identifying severely affected patients requiring individualized treatment strategies at an early stage of the disease.

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Year:  2006        PMID: 17101573     DOI: 10.1080/00365520600703900

Source DB:  PubMed          Journal:  Scand J Gastroenterol        ISSN: 0036-5521            Impact factor:   2.423


  29 in total

1.  The presence of fistulas and NOD2 homozygosity strongly predict intestinal stenosis in Crohn's disease independent of the IL23R genotype.

Authors:  Matthias Jürgens; Stephan Brand; Rüdiger P Laubender; Julia Seiderer; Jürgen Glas; Martin Wetzke; Johanna Wagner; Simone Pfennig; Cornelia Tillack; Florian Beigel; Maria Weidinger; Fabian Schnitzler; Martin E Kreis; Burkhard Göke; Peter Lohse; Karin Herrmann; Thomas Ochsenkühn
Journal:  J Gastroenterol       Date:  2010-04-29       Impact factor: 7.527

2.  NOD2 gene mutations in ulcerative colitis: useless or misunderstood?

Authors:  Paulo Freire; Ricardo Cardoso; Pedro Figueiredo; Maria M Donato; Manuela Ferreira; Sofia Mendes; Ana Margarida Ferreira; Helena Vasconcelos; Francisco Portela; Carlos Sofia
Journal:  Int J Colorectal Dis       Date:  2014-03-22       Impact factor: 2.571

Review 3.  Paneth cells, antimicrobial peptides and maintenance of intestinal homeostasis.

Authors:  Charles L Bevins; Nita H Salzman
Journal:  Nat Rev Microbiol       Date:  2011-03-22       Impact factor: 60.633

Review 4.  New insights into Nod-like receptors (NLRs) in liver diseases.

Authors:  Tao Xu; Yan Du; Xiu-Bin Fang; Hao Chen; Dan-Dan Zhou; Yang Wang; Lei Zhang
Journal:  Int J Physiol Pathophysiol Pharmacol       Date:  2018-03-10

Review 5.  Paneth cell defensins and the regulation of the microbiome: détente at mucosal surfaces.

Authors:  Nita H Salzman
Journal:  Gut Microbes       Date:  2010 Nov-Dec

6.  Prediction of Crohn's disease aggression through NOD2/CARD15 gene sequencing in an Australian cohort.

Authors:  Maneesha Bhullar; Finlay Macrae; Gregor Brown; Margie Smith; Ken Sharpe
Journal:  World J Gastroenterol       Date:  2014-05-07       Impact factor: 5.742

7.  Evidence for STAT4 as a common autoimmune gene: rs7574865 is associated with colonic Crohn's disease and early disease onset.

Authors:  Jürgen Glas; Julia Seiderer; Melinda Nagy; Christoph Fries; Florian Beigel; Maria Weidinger; Simone Pfennig; Wolfram Klein; Jörg T Epplen; Peter Lohse; Matthias Folwaczny; Burkhard Göke; Thomas Ochsenkühn; Julia Diegelmann; Bertram Müller-Myhsok; Darina Roeske; Stephan Brand
Journal:  PLoS One       Date:  2010-04-29       Impact factor: 3.240

8.  The cannabinoid 1 receptor (CNR1) 1359 G/A polymorphism modulates susceptibility to ulcerative colitis and the phenotype in Crohn's disease.

Authors:  Martin Storr; Dominik Emmerdinger; Julia Diegelmann; Simone Pfennig; Thomas Ochsenkühn; Burkhard Göke; Peter Lohse; Stephan Brand
Journal:  PLoS One       Date:  2010-02-26       Impact factor: 3.240

Review 9.  Role of Nod2 in the development of Crohn's disease.

Authors:  Soichiro Yamamoto; Xiaojing Ma
Journal:  Microbes Infect       Date:  2009-06-30       Impact factor: 2.700

10.  Genotype/phenotype analyses for 53 Crohn's disease associated genetic polymorphisms.

Authors:  Camille Jung; Jean-Frédéric Colombel; Marc Lemann; Laurent Beaugerie; Matthieu Allez; Jacques Cosnes; Gwenola Vernier-Massouille; Jean-Marc Gornet; Jean-Pierre Gendre; Jean-Pierre Cezard; Frank M Ruemmele; Dominique Turck; Françoise Merlin; Habib Zouali; Christian Libersa; Philippe Dieudé; Nadem Soufir; Gilles Thomas; Jean-Pierre Hugot
Journal:  PLoS One       Date:  2012-12-27       Impact factor: 3.240

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