Cystic fibrosis and survival to 40 years: a study of cystic fibrosis transmembrane conductance regulator function.

doi:10.1183/09031936.00079010

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Article

Cystic fibrosis and survival to 40 years: a study of cystic fibrosis transmembrane conductance regulator function.

The European respiratory journal - 1 May 2011

Simmonds N J, D'Souza L, Roughton M, Alton E W F W, Davies J C, Hodson M E

Abstract excerpt

Significant survival heterogeneity exists in cystic fibrosis. Our aim was to determine whether residual function of the cystic fibrosis transmembrane conductance regulator (CFTR) is present in long-term survivors with severe mutations. Nasal potential difference (PD) and sweat chloride were measured in 34 long-term survivors (aged ≥ 40 yrs) and compared with young patients (18-23 yrs) with severe (n = 30) and...

Topics

Adolescent
Adult
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator
Female
Humans
Male
Membrane Potentials
Middle Aged
Mutation
Nasal Mucosa

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Prospective Studies

Respiratory Function Tests

Severity of Illness Index

Survivors

Sweat

Young Adult

Source

Open in PubMed

Identifiers

PMID: 20847077
DOI: 10.1183/09031936.00079010

Genes

CFTR

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 31 Aug 2026.

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