Abstract
Cystic fibrosis is a hereditary disease caused by mutation in cystic fibrosis transmembrane conductance regulator (CFTR) gene, which controls CFTR proteins. Many are or have been threatened by this rare but life-threating disease, therefore, more attention should be paid to better solve this public health issue. Here we provide a thorough review about cystic fibrosis in the aspects of disease pathogenesis, clinical manifestation as well as clinical therapeutics to achieve a deeper understanding of this disease. Among the demand of innovative clinical strategies, appearance of the combination drug trikafta is a promising sign of better dealing with cystic fibrosis.
Highlights
Respiration is an essential activity to maintain vital signs
We present a thorough review about cystic fibrosis in the aspects of disease pathogenesis, clinical manifestation as well as clinical therapeutics
The mucus outside of the cell would have a greater viscosity. Both of these imbalances prompt mucus to stuck in lungs, generate difficulties in breath and initiate cystic fibrosis
Summary
Respiration is an essential activity to maintain vital signs. various respiratory diseases still persecute a large population in their health and daily lives. According to U.S National Library of Medicine, cystic fibrosis affects about 1 in 2,500 to 3,500 white newborns, 1 in 17,000 African Americans, and 1 in 31,000 Asian Americans It seems to be a rare disease, which there are over 70,000 patients around the world, it can be life-threatening. Improvement of medical care such as airway mucus clearance and controlling lung infection has greatly changed the epidemiology and demographics performances of cystic fibrosis [1, 2]. This disease is currently predominant in adult instead of children [3]. We present a thorough review about cystic fibrosis in the aspects of disease pathogenesis, clinical manifestation as well as clinical therapeutics
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