Comprehensive diagnosis and management of cystic fibrosis: A narrative review

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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

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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

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