Abstract

Cystic fibrosis related liver disease (CFLD) affects a small proportion of people with CF (PWCF) but is associated with a significant increase in morbidity and mortality. The development of cystic fibrosis transmembrane conductance regulator (CFTR) modulators has led to dramatic improvements in life expectancy for PWCF. Consequently, there has been a significant emergence of liver dysfunction complicating the clinical course of the disease. The FIB-4 index has been shown to be an accurate, non invasive method for assessing liver fibrosis in non CF liver disease. We aimed to assess whether the FIB-4 index could reliably exclude significant fibrosis in an adult population of PWCF.

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