Medicine and Pediatrics, Case Western Reserve University School of Medicine, LeRoy W. Matthews Cystic Fibrosis Center, University Hospitals Rainbow Babies & Children's, 11100 Euclid Avenue, RBC 3001, Cleveland, OH 44106, USA.
Center for Community Health Integration, Case Western Reserve University School of Medicine, 10900 Euclid Avenue, Suite T-402, Cleveland, OH 44106-4922, USA.
Clin Chest Med. 2022 Dec;43(4):579-590. doi: 10.1016/j.ccm.2022.06.002.
The incidence of cystic fibrosis remains constant in North America and Western Europe is 1 in 3500 live births, but survival and quality of life have improved. The cystic fibrosis population has shifted toward the adult age range with a concomitant shift in the spectrum of complications. Survival increased because of aggressive symptomatic therapy, earlier diagnosis by newborn screening, and the introduction of modulators of the cystic fibrosis transmembrane conductance regulator, so that predicted median survival age is now about 50 years. In the United States, members of low socioeconomic status populations or members of racial or ethnic minorities have benefitted less from these advances.
囊性纤维化的发病率在北美和西欧保持不变，每 3500 例活产中就有 1 例，但生存和生活质量都有所提高。囊性纤维化人群的年龄范围已经向成年转移，并发症的范围也随之改变。由于积极的对症治疗、通过新生儿筛查更早的诊断，以及囊性纤维化跨膜电导调节体调节剂的引入，生存率提高了，因此现在预测的中位生存年龄约为 50 岁。在美国，社会经济地位较低的人群或少数族裔成员从这些进展中获益较少。
