CFTR genotype as a predictor of prognosis in cystic fibrosis.

CFTR genotype as a predictor of prognosis in cystic fibrosis. Research Abstract Details 

Research Abstract Table of Contents

Jump to the:

CFTR genotype as a predictor of prognosis in cystic fibrosis. Abstract Text:

Edward F McKone,Christopher H Goss,Moira L Aitken,

STUDY RATIONALE: Certain CFTR genotypes are associated with reduced mortality. The accuracy of using CFTR genotype as a predictor of survival and the mechanisms through which CFTR genotype influences survival are unknown. PARTICIPANTS: All patients with cystic fibrosis (CF) enrolled in the US Cystic Fibrosis Foundation national registry between 1993 and 2002. DESIGN: We examined the prognostic value of CFTR genotype, grouped into "high-risk" and "low-risk" categories based on the effect of their CFTR genotype on phenotype and protein production. MEASUREMENTS AND RESULTS: Clinical and genetic data were available from 15,651 patients with CF. Patients with a high-risk CFTR genotype had a greater than twofold increased risk of death compared to patients with a low-risk CFTR genotype (relative risk, 2.25; 95% confidence interval [CI], 1.77 to 2.84; p < 0.001). This association was partly explained by lung function, nutritional status, pancreatic insufficiency, and Pseudomonas aeruginosa colonization. Of the 1,672 patients who died, median age at death for the high-risk CFTR genotype was 24.2 years (interquartile range, 18.4 to 32.0 years) and for the low-risk CFTR genotype was 37.6 years (interquartile range, 28.8 to 47.9 years; p < 0.001). The positive predictive value of this classification method as a test to identify patients who died before or after their 30th birthday was 69% (95% CI, 67 to 72%) with a negative predictive value of 71% (95% CI, 60 to 80%). CONCLUSIONS: Grouping patients into high-risk and low-risk CFTR genotype categories is associated with significant differences in survival and median age at death. These differences are not fully explained by lung function, nutritional measures, pancreatic insufficiency, or P aeruginosa colonization. Modest reassurance about the likelihood of a milder than average course can be provided for CF patients with a low-risk CFTR genotype, although it should be acknowledged that substantial phenotypic variability exists.

CFTR genotype as a predictor of prognosis in cystic fibrosis. Publishing Authors By Initials

EF McKone,CH Goss,ML Aitken,

For similar investigative techniques: epidemiologic methods: data collection: vital statistics: mortality: survival rate research abstracts see: investigative techniques: epidemiologic methods: data collection: vital statistics: mortality: survival rate research

PUBMED ID PMID:

MEDLINE DATE:

CFTR genotype as a predictor of prognosis in cystic fibrosis. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: Chest

VOLUME: 130

Page Numbers: 1441-7

Journal Abbreviation: Chest

ISSN: 0012-3692

DAY: 3

MONTH: Nov

YEAR: 2006

CFTR genotype as a predictor of prognosis in cystic fibrosis. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 231335

CFTR genotype as a predictor of prognosis in cystic fibrosis. Keywords Mesh Terms:

KEYWORDS: Survival Rate

MESH TERMS: pathogenicity

Chemical & Substance for Abstract: CFTR genotype as a predictor of prognosis in cystic fibrosis. Information

Substance Name: Cystic Fibrosis Transmembrane Conductanc

Registry Number: 126880-72-6

Grant and Affiliation Information for CFTR genotype as a predictor of prognosis in cystic fibrosis.

AFFILIATION: Division of Pulmonary and Critical Care Medicine and Adult Cystic Fibrosis Center, University of Washington Medical Center, Seattle, WA 98195-6522, USA. emckone@u.washington.edu

Country: United States

AGENCY: United States NHLBI

GRANT: K23 HL/70849-01

ACRONYM: HL

MEDLINETA: Chest

REFSOURCE:

DATABASENAME:

ACCESSION NUMBER:

Number Hits: 0

CFTR genotype as a predictor of prognosis in cystic fibrosis Related Publications

• A survey of modern therapeutics.
• A survey of modern therapeutics. The newer antibiotics.
• A survey of modern therapeutics; new drugs on the market.
• Adaptive gradients and isolation-by-distance with postglacial migration in Picea sitchensis.
• Analysis of chaperone proteins associated with human spermatozoa during capacitation.
• Anorectic drugs.
• CFTR genotype as a predictor of prognosis in cystic fibrosis.
• Deformity of the elbow joint as a sequel to Erb's obstetrical paralysis.
• Drug dependence.
• Effect of peripheral connexions on the maturation of regenerating nerves.
• Ensuring the health and safety of civilian disaster medical assistance teams.
• Evaluation of the BD ProbeTec ET system for direct detection of Mycobacterium bovis in veterinary specimens.
• Improving patient services by alternative prescribing: experience with radioiodine treatment.
• Isolation by phage display of recombinant antibodies able to block adherence of Escherichia coli mediated by the K99 colonisation factor.
• Isoniazid overdose : a case series, literature review and survey of antidote availability.
• Let's take a look at the metric system.
• Lithium; its effect on human spermatozoa, rat testicular tissue and upon rats in vivo.
• Measuring children and monitoring childhood obesity.
• Modern therapeutics drugs in modern usage.
• On the Wishart distribution in statistics.
• Posture.
• Pregnancy in cystic fibrosis.
• Q fever in the United Kingdom and Ireland.
• Radiographic changes in acute exacerbations of cystic fibrosis in adults: a pilot study.
• Simple calibrated phonocardiography.
• Stable-isotope probing with multiple growth substrates to determine substrate specificity of uncultivated bacteria.
• The effect of peripheral connexions on the maturation of regenerating nerve fibres.
• The effect of timing of laparoscopic insemination in superovulated ewes, with or without sedation, on the recovery of embryos, their stage of develpoment and subsequent viability.
• The estimation of calcium requirement: a re-examination.
• The training of physicians.