Literature DB >> 11025834

Mutation in the gene responsible for cystic fibrosis and predisposition to chronic rhinosinusitis in the general population.

X Wang1, B Moylan, D A Leopold, J Kim, R C Rubenstein, A Togias, D Proud, P L Zeitlin, G R Cutting.   

Abstract

CONTEXT: Chronic rhinosinusitis (CRS) is a common condition in the US general population, yet little is known about its underlying molecular cause. Chronic rhinosinusitis is a consistent feature of the autosomal recessive disorder cystic fibrosis (CF).
OBJECTIVE: To determine whether mutations in the cystic fibrosis transmembrane regulator (CFTR) gene, which is responsible for CF, predispose to CRS.
DESIGN: Case-control study conducted from 1996 to 1999 in which the DNA of CRS patients and controls was typed for 16 mutations that account for 85% of CF alleles in the general population. Chronic rhinosinusitis patients with 1 CF mutation were evaluated for a CF diagnosis by sweat chloride testing, nasal potential difference measurement, and DNA analysis for additional mutations.
SETTING: Otolaryngology-head and neck clinic of a US teaching hospital. PARTICIPANTS: One hundred forty-seven consecutive adult white patients who met stringent diagnostic criteria for CRS and 123 CRS-free white control volunteers of similar age range, geographic region, and socioeconomic status. MAIN OUTCOME MEASURES: Presence of CF mutations by DNA analysis among CRS patients vs controls.
RESULTS: Eleven CRS patients were found to have a CF mutation (DeltaF508, n = 9; G542X, n = 1; and N1303K, n = 1). Diagnostic testing excluded CF in 10 of these patients and led to CF diagnosis in 1. Excluding this patient from the analyses, the proportion of CRS patients who were found to have a CF mutation (7%) was significantly higher than in the control group (n = 2 [2%]; P =.04, both having DeltaF508 mutations). Furthermore, 9 of the 10 CF carriers had the polymorphism M470V, and M470V homozygotes were overrepresented in the remaining 136 CRS patients (P =.03).
CONCLUSION: These data indicate that mutations in the gene responsible for CF may be associated with the development of CRS in the general population. JAMA. 2000;284:1814-1819.

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Year:  2000        PMID: 11025834     DOI: 10.1001/jama.284.14.1814

Source DB:  PubMed          Journal:  JAMA        ISSN: 0098-7484            Impact factor:   56.272


  64 in total

1.  Proposal of a CT scoring system of the paranasal sinuses in diagnosing cystic fibrosis.

Authors:  H B Eggesbø; S Søvik; S Dølvik; K Eiklid; F Kolmannskog
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2.  Medical reversal of chronic sinusitis in a cystic fibrosis patient with ivacaftor.

Authors:  Eugene H Chang; Xiao Xiao Tang; Viral S Shah; Janice L Launspach; Sarah E Ernst; Brieanna Hilkin; Philip H Karp; Mahmoud H Abou Alaiwa; Scott M Graham; Douglas B Hornick; Michael J Welsh; David A Stoltz; Joseph Zabner
Journal:  Int Forum Allergy Rhinol       Date:  2014-10-31       Impact factor: 3.858

Review 3.  Severe rhinosinusitis.

Authors:  Rosemary Hallett; Stanley M Naguwa
Journal:  Clin Rev Allergy Immunol       Date:  2003-10       Impact factor: 8.667

4.  Clan genomics and the complex architecture of human disease.

Authors:  James R Lupski; John W Belmont; Eric Boerwinkle; Richard A Gibbs
Journal:  Cell       Date:  2011-09-30       Impact factor: 41.582

5.  Analysis of most common CFTR mutations in patients affected by nasal polyps.

Authors:  Marzena Kostuch; Janusz Klatka; Andrzej Semczuk; Jacek Wojcierowski; Lucas Kulczycki; Jan Oleszczuk
Journal:  Eur Arch Otorhinolaryngol       Date:  2005-06-17       Impact factor: 2.503

6.  A haplotype framework for cystic fibrosis mutations in Iran.

Authors:  Elahe Elahi; Ahmad Khodadad; Ilya Kupershmidt; Fereshteh Ghasemi; Babak Alinasab; Ramin Naghizadeh; Robert G Eason; Mahshid Amini; Mehran Esmaili; Mohammad R Esmaeili Dooki; Mohammad H Sanati; Ronald W Davis; Mostafa Ronaghi; Yvonne R Thorstenson
Journal:  J Mol Diagn       Date:  2006-02       Impact factor: 5.568

7.  Identification of susceptibility genes for complex diseases using pooling-based genome-wide association scans.

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8.  Best practice guidelines for molecular genetic diagnosis of cystic fibrosis and CFTR-related disorders--updated European recommendations.

Authors:  Els Dequeker; Manfred Stuhrmann; Michael A Morris; Teresa Casals; Carlo Castellani; Mireille Claustres; Harry Cuppens; Marie des Georges; Claude Ferec; Milan Macek; Pier-Franco Pignatti; Hans Scheffer; Marianne Schwartz; Michal Witt; Martin Schwarz; Emmanuelle Girodon
Journal:  Eur J Hum Genet       Date:  2008-08-06       Impact factor: 4.246

9.  Cystic fibrosis transmembrane conductance regulator function, not TAS2R38 gene haplotypes, predict sinus surgery in children and young adults with cystic fibrosis.

Authors:  Nicholas M Dalesio; Melis A Aksit; Kwangmi Ahn; Karen S Raraigh; Joseph M Collaco; Sharon McGrath-Morrow; Pamela L Zeitlin; Steven S An; Garry R Cutting
Journal:  Int Forum Allergy Rhinol       Date:  2020-04-13       Impact factor: 3.858

Review 10.  Consensus on the use and interpretation of cystic fibrosis mutation analysis in clinical practice.

Authors:  C Castellani; H Cuppens; M Macek; J J Cassiman; E Kerem; P Durie; E Tullis; B M Assael; C Bombieri; A Brown; T Casals; M Claustres; G R Cutting; E Dequeker; J Dodge; I Doull; P Farrell; C Ferec; E Girodon; M Johannesson; B Kerem; M Knowles; A Munck; P F Pignatti; D Radojkovic; P Rizzotti; M Schwarz; M Stuhrmann; M Tzetis; J Zielenski; J S Elborn
Journal:  J Cyst Fibros       Date:  2008-05       Impact factor: 5.482

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