Article
Prevalence of CFTR variants in primary immunodeficiency patients with bronchiectasis is an important modifying cofactor.
The Journal of allergy and clinical immunology - 1 Jul 2023
Lawless Dylan, Allen Hana Lango, Thaventhiran James E D, Goddard Sarah, Burren Oliver S, Robson Evie, Peckham Daniel, Smith Kenneth G C, Savic Sinisa
Abstract excerpt
BACKGROUND: Cystic fibrosis (CF) is one of the most common life-limiting autosomal-recessive disorders and is caused by genetic defects in the CF transmembrane conductance regulator (CFTR) gene. Some of the features of this multisystem disease can be present in primary immunodeficiency (PID). OBJECTIVE: We hypothesized that a carrier CFTR status might be associated with worse outcome regarding structural lung...
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