Clinical feature: 

Definition: Bronchiectasis with or without elevated sweat chloride are autosomal recessive disorders caused by mutations of genes encoding the sodium channel ENaC (SCNN1A, SCNN1B, SCNN1G,). Mutations not only account for a systic fibrosis-like disease but also typical cystic fibrosis can be aggravated.

Literature: 

Casals T et al. (2004) Bronchiectasis in adult patients: an expression of heterozygosity for CFTR gene mutations?
Danielson GK et al. (1967) Middle lobe bronchiectasis. Report of an unusual familial occurrence.
Davis PB et al. (1983) Familial bronchiectasis.
Fajac I et al. (2008) Could a defective epithelial sodium channel lead to bronchiectasis.
Hoo JJ et al. (1979) Familial middle lobe bronchiectasis.
Mutesa L et al. (2009) Genetic analysis of Rwandan patients with cystic fibrosis-like symptoms: identification of novel cystic fibrosis transmembrane conductance regulator and epithelial sodium channel gene variants.
Sheridan MB et al. (2005) Mutations in the beta-subunit of the epithelial Na+ channel in patients with a cystic fibrosis-like syndrome.
Viel M et al. (2008) ENaCbeta and gamma genes as modifier genes in cystic fibrosis.