Cystic Fibrosis: Review

Authors

  • I Gusti Agung Ari Kusuma Yana Magister Farmasi Fakultas Farmasi Universitas Surabaya, Surabaya Author

Keywords:

Cystic fibrosis, Mokus, Saluran Pernafasan, Mutasi Gen

Abstract

Cystic fibrosis (CF) merupakan penyakit genetik autosomal yang mematikan yang diturunkan terutama oleh populasi ras putih atau kaukasia. Kondisi ini disebabkan oleh mutasi gen CFTR terutama CFTR Phe508del. Jika terjadi kerusakan atau kesalahan CFTR maka fungsi normal akan terganggu dan berdampak klinis pada organ yang lain atau multiorgan. Komplikasi seperti pada pankreas, hati, saluran pencernaan, tulang, genital, serta saluran pernafasan yang terutama dapat menyebabkan kematian paling banyak pada penderita cystic fibrosis. Kondisi malabsorpsi, inflamasi, infeksi dan obstruksi paru-paru kronis merupakan tanda-tanda dari cystic fibrosis. Terapi untuk cystic fibrosis sebatas pada pembersihan mokus saluran pernafasan, pencegahan infeksi yang presisten dengan antibiotik, meningkatkan kesehatan fisik dan gizi pasien

References

Doull I. Cystic fibrosis 2019: Year in review. Paediatr Respir Rev 2020:125. https://doi.org/10.1016/j.prrv.2020.04.001.

Kristensen M, Prevaes SMPJ, Kalkman G, Tramper-Stranders GA, Hasrat R, de Winter-de Groot KM, et al. Development of the gut microbiota in early life: The impact of cystic fibrosis and antibiotic treatment. J Cyst Fibros 2020;19:55361. https://doi.org/10.1016/j.jcf.2020.04.007.

Thornton T, Pillai A. Encephalopathy in cystic fibrosis. Paediatr Respir Rev 2020:310. https://doi.org/10.1016/j.prrv.2020.03.003.

Sakiani S, Kleiner DE, Heller T, Koh C. Hepatic Manifestations of Cystic Fibrosis. Clin Liver Dis 2019;23:26377. https://doi.org/10.1016/j.cld.2018.12.008.

Bragonzi A, Horati H, Kerrigan L, Ivan N, ScholteBJ, Weldon S. In fl ammation and host-pathogen interaction: Cause and consequence in cystic fi brosis lung disease. J Cyst Fibros 2017. https://doi.org/10.1016/j.jcf.2017.10.004.

Hough NE, Chapman SJ, Flight WG. Gastrointestinal malignancy in cystic fibrosis. Paediatr Respir Rev 2020. https://doi.org/10.1016/j.prrv.2020.03.002.

Garcia AM, Dorsey J. 52 -Nonpulmonary Manifestationsof Cystic Fibrosis. Ninth Edit. Elsevier Inc.; 2019. https://doi.org/10.1016/B978-0-323-44887-1.00052-3.

Anabtawi A, Le T,Putman M, Tangpricha V, BianchiML. Cystic fibrosis bone disease: Pathophysiology, assessment and prognostic implications. J Cyst Fibros 2019;18:S4855. https://doi.org/10.1016/j.jcf.2019.08.018.

Yoon JC, Casella JL, Litvin M, Dobs AS. Male reproductive health in cystic fibrosis. J Cyst Fibros 2019;18:S10510. https://doi.org/10.1016/j.jcf.2019.08.007.

Hughan KS, Daley T, Rayas MS, Kelly A, Roe A. Female reproductive health in cystic fibrosis. J Cyst Fibros 2019;18:S95104. https://doi.org/10.1016/j.jcf.2019.08.024.

Edmondson C, Davies JC. Current and future treatment options for cystic fibrosis lung disease: Latest evidence and clinical implications. Ther Adv Chronic Dis 2016;7:17083. https://doi.org/10.1177/2040622316641352.

Downloads

Published

2024-11-14

Issue

Vol. 3 No. 1 (2021): J. Sains Kes.

Section

Review

How to Cite

Cystic Fibrosis: Review. (2024). Jurnal Sains Dan Kesehatan, 3(1), 79-87. https://jsk.ff.unmul.ac.id/index.php/JSK/article/view/189