A Mini Review on Clinical Aspects of Cystic Fibrosis
Subject Areas :
Nasim Eftekhari
1
*
,
Farokh Rokhbakhsh-Zamin
2
,
Amir Hossein Mohammad Bagheri
3
1 - Department of Biology, Zend Institute of Higher Education, Shiraz, Iran
2 - Department of Microbiology, Ke.C., Islamic Azad University, Kerman, Iran
3 - The Office of Vice-President for International Affair, Department of Medical Education, Faculty of Medicine, Iran University of Medical Sciences (IUMS), Tehran, Iran
Keywords: Cystic fibrosis, Inflammation, CFTR, Clinical feature, Pseudomans aeruginosa,
Abstract :
Cystic fibrosis (CF) is a rare autosomal disorder caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR). CF can lead to various health issues that affect the quality of life and can be challenging for both patients and healthcare providers. In this article, we conduct a thorough review of the clinical characteristics of cystic fibrosis in children. Classical cystic fibrosis is defined by chronic lung infections, pancreatic insufficiency, and male infertility, and may also include other conditions such as cystic fibrosis-related diabetes or liver disease. This genetic disease is diagnosed in many places through newborn screening, while in other areas, diagnosis is based on specific clinical symptoms, high sweat chloride levels, or known CFTR mutations. Management techniques, including improving mucus clearance and aggressively treating infections, have steadily increased the life expectancy of people with cystic fibrosis. Moreover, the development of new small-molecule drugs that can restore CFTR function is changing the outlook for many patients. Clinical trials are actively looking into various other approaches, which will become increasingly important as survival rates improve and the population of adults with cystic fibrosis grows. This review offers a comprehensive update on CF, covering screening as well as current and future treatments.
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