Comprehensive diagnosis and management of cystic fibrosis: A narrative review


Review Article

Author Details : Vignesh Murugan*

Volume : 9, Issue : 3, Year : 2024

Article Page : 90-97

https://doi.org/10.18231/j.ijirm.2024.019



Suggest article by email

Get Permission

Abstract

Cystic fibrosis (CF) is a genetic disorder caused by mutations in the CFTR gene, leading to the malfunction of the CFTR protein, a chloride channel present on various epithelial surfaces. This narrative review examines the current state of CF diagnosis and treatment. CF results in a range of complications, including chronic lung infections, pancreatic insufficiency, liver disease, and gastrointestinal issues. Recent advancements in CFTR modulators like ivacaftor, lumacaftor, and tezacaftor have significantly improved the management of CF, expanding treatment eligibility to around 90% of patients. These therapies correct the defective protein function, enhancing chloride ion transport and improving clinical outcomes. Gene therapy has also shown potential, though it faces challenges such as transient gene expression and immunogenicity. Surgical interventions, including lung and liver transplantation, remain crucial for patients with advanced disease. Pulmonary rehabilitation, combining exercise training, airway clearance techniques, and psychological support, is essential for maintaining lung function and quality of life. Despite these advancements, CF continues to be a complex, multisystem disease requiring comprehensive and individualized care strategies. This paper aims to address the current diagnostic methods and treatment approaches in CF management to further improve patient outcomes and extend survival.
 

Keywords: Cystic Fibrosis, CFTR modulators, Gene therapy, Pulmonary rehabilitation, Lung transplantation, Screening


How to cite : Murugan V, Comprehensive diagnosis and management of cystic fibrosis: A narrative review. IP Indian J Immunol Respir Med 2024;9(3):90-97


This is an Open Access (OA) journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.







Article History

Received : 05-07-2024

Accepted : 05-09-2024


View Article

PDF File   Full Text Article


Copyright permission

Get article permission for commercial use

Downlaod

PDF File   XML File   ePub File


Digital Object Identifier (DOI)

Article DOI

https://doi.org/10.18231/j.ijirm.2024.019


Article Metrics






Article Access statistics

Viewed: 196

PDF Downloaded: 32