Prevalence and Characteristics of Liver Involvement in Cystic Fibrosis Patients- An Initial Experience From India

Abstract

Background: Cystic fibrosis (CF) is an uncommon autosomal recessive disorder that results due to dysfunction of epithelial chloride transport channel caused by defects in the CF trans membrane regulation (CFTR) gene. This leads to production of abnormally thick secretions that cause alterations in multiple organ systems including lungs, pancreas, liver, intestine, and reproductive tract. The liver involvement in cystic fibrosis described as Cystic fibrosis-associated liver disease (CFLD) affects about 26-45% of patients and is considered the third common cause of death after lung disease and transplantation complications respectively.