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 on chromosome 7. The dysfunction produces abnormally thick secretions that cause changes throughout multiple organ systems, such as the lungs, pancreas, liver, intestine, and reproductive tract. The liver involvement in cystic fibrosis described as Cystic fibrosis-associated liver disease (CFLD) aff ects about 26-45% of patients and is considered the third common cause of death after lung disease and transplantation complications respectively.