Liver disease related to cystic fibrosis: pathogenesis, diagnosis, and therapeutic approaches

Abstract

Objective: To examine the prevalence, pathophysiological mechanisms, and management approaches of liver disease in cystic fibrosis patients, focusing on identifying factors that influence disease progression and the effectiveness of current therapeutic interventions. Methods: This is a integrative literature review study using PubMed as the database. The search terms included "cystic fibrosis," "liver disease," and related terms. A total of 11 articles were selected and analyzed. Results: The prevalence of liver disease in cystic fibrosis patients was found to be significant, being identified as the leading non-pulmonary cause of mortality. Regarding therapeutic options, it was observed that, although not preventive, therapies with CFTR protein modulators significantly reduce complications associated with liver disease, such as cirrhosis. Final considerations: This study contributes to a deeper understanding of the disease mechanisms and the improvement of treatment, diagnosis, and prognosis strategies for liver disease in cystic fibrosis patients. However, it highlights the need for future and in-depth research proportional to the complexity of the topic addressed.