The Changing Epidemiology of Cystic Fibrosis The Implications for Adult Care
The Changing Epidemiology of Cystic Fibrosis The Implications for Adult Care
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DOI：
10.1016/j.chest.2022.07.004
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发表时间：
2023-01-09
期刊：
影响因子：
9.6
通讯作者：
Martin, Clemence
中科院分区：
文献类型：
--
作者：
Burgel, Pierre-Regis;Burnet, Esperie;Martin, Clemence
囊性纤维化（CF）是一种遗传性疾病，其中编码CF跨膜传导调节蛋白的基因突变导致以消化和呼吸表现为主的多系统疾病。在世纪中期，CF在生命的最初几年内导致死亡。在过去的几十年里，疾病管理的进步，包括系统的新生儿筛查，多学科症状CF护理，肺移植，以及最近的高效CF跨膜传导调节调节剂，已经显着改变了CF患者的预后。在大多数拥有完善CF护理的国家，成年人现在超过了儿童，预期寿命预计将进一步增加，缩小与一般人群的生存差距。然而，根据专业CF护理提供网络的存在和质量，CF的预后不仅在高收入、低收入和中等收入国家之间存在显著差异，而且在高收入国家之间也存在显著差异。目前的证据表明，患者临床状态和生存率的差异不仅可归因于内在的疾病严重程度，而且还可归因于获得高质量专业护理的差异。由于CF通常是一种进行性疾病，患有CF的成人通常表现出肺部严重程度和并发症增加以及合并症的发生率增加，这突出了对专业成人CF中心的需求。本文旨在描述CF人口在过去几十年的演变，预测未来的趋势，并预测未来提供成人CF护理。胸部2023; 163（1）：89-99
Cystic fibrosis (CF) is a genetic disease in which mutations in the gene encoding for the CF transmembrane conductance regulator protein result in a multisystem disease dominated by digestive and respiratory manifestations. In the mid-20th century, CF caused death within the first years of life. Over the past decades, advances in disease management, which includes systematic neonatal screening, multidisciplinary symptomatic CF care, lung transplantation and, more recently, highly effective CF transmembrane conductance regulator modulators, have transformed the prognosis of people with CF markedly. In most countries with well -established CF care, adults now outnumber children, and life expectancy is expected to in-crease further, narrowing the survival gap with the general population. However, marked dif-ferences in the prognosis of CF exist not only among high-, low-, and middle-income countries but also among high-income countries, based on the presence and quality of a specialized CF care provision network. Current evidence suggests that differences in patient clinical status and survival could be attributable not only to intrinsic disease severity but also to disparities in access to high-quality specialized care. Because CF is generally a progressive disease, adults with CF often show increased pulmonary severity and complications and increased occurrence of comorbidities, which highlights the need for specialized adult CF centers. This article seeks to describe the evolution of CF demography over the past decades, predict future trends, and anticipate the future provision of adult CF care. CHEST 2023; 163(1):89-99