Elexacaftor/Tezacaftor/Ivacaftor use in Pediatric Cystic Fibrosis Patients with Advanced Liver Disease

Cystic fibrosis (CF) is an autosomal recessive genetic disease that affects about 30,000 individuals in the United States [1,2]. Advances in the treatment of CF, including cystic fibrosis transmembrane conductance regulator (CFTR) modulators, have increased the life expectancy, lung function, and nutritional status in people with CF (pwCF). With increased longevity, extrapulmonary manifestations, such as CF-related liver disease (CFLD), have become increasingly relevant in the management and care of pwCF [3].
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Original Article Source Type: research