Triple CFTR modulator therapy in severe cystic fibrosis-associated liver disease: a case report of an adverse outcome and the role of adherence to follow-up
Background. Cystic fibrosis (CF) is a multisystem disease in which liver involvement occurs in 20-40% of patients. The introduction of CFTR modulator therapy has substantially improved the prognosis of patients with CF; however, patients with established cirrhosis have typically been excluded from clinical trials, whic...