Modeling cystic fibrosis patient prognosis: Nomograms to predict lung transplantation and survival prior to highly effective modular therapy.

Treatment with the CF transmembrane-conductance regulator (CFTR) modulator ivacaftor was approved for the small cohort of patients with CFTR gating mutations in 2012 [ 2 , 3 ] which resulted in highly significant improvement in lung function, body mass index, quality of life, combined with a decreased rate of pulmonary exacerbations.

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