Body mass index and nutritional intake following Elexacaftor/Tezacaftor/Ivacaftor modulator therapy in adults with cystic fibrosis

Cystic fibrosis (CF) is a life-limiting autosomal recessive disease [1]. It is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene which encodes the CFTR protein, an anion channel expressed throughout the body [1]. Aberrant production and function of CFTR protein in the lungs leads to dehydrated airways, viscous mucus, recurrent infections and inflammation [1]. The CFTR protein is also expressed in the gastrointestinal (GI) tract resulting in exocrine pancreatic insufficiency (PI), inflammation, dysmotility, dysbiosis and GI symptoms [2].

Source: Journal of Cystic Fibrosis - July 6, 2023 Category: Respiratory Medicine Authors: L.R. Caley, H.H. Jarosz-Griffiths, L. Smith, L. Gale, J. Barrett, L. Kinsey, V. Davey, M. Nash, A.M. Jones, J.L. Whitehouse, D. Shimmin, R.A. Floto, H. White, D.G. Peckham Tags: Original Article Source Type: research