CareLit Fachartikel

Non-CF bronchiectasis: a spectrum of CFTR (and ENaC) dysfunction?

FC Ringshausen · Pneumologie · 2016 · Heft 10

Dokument
1146221
CareLit-ID
Jahr
2016
Publikation
PDF
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Metadaten
DOI
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Bibliografische Angaben

Zeitschrift
Pneumologie
Autor:innen
FC Ringshausen
Ausgabe
Heft 10 / 2016
Seiten
–
Erschienen: 2016-10-11 00:00:00
ISSN
0934-8387
DOI
10.1055/s-0036-1592248

Zusammenfassung

Disorders of the respiratory epithelium's ion conductance result in abnormal airway surface liquid hydration, defective mucociliary clearance and impaired host defense and may contribute to the pathogenesis of bronchiectasis. This may affect either the amiloride-sensitive epithelial sodium channel (ENaC) or the chloride channel cystic fibrosis transmembrane conductance regulator (CFTR). The most prominent channelopathy associated with bronchiectasis is cystic fibrosis (CF), an autosomal recessive disease caused by mutations in the CFTR gene, which encodes the chloride CFTR channel. The diagnosis of a defective i…

Schlagworte

Bronchiectasis Diagnosis Chloride Cystic Fibrosis Transmembrane Conductance Regulator Amiloride Affect Pneumologie