Adult Cystic Fibrosis Centre, Department of Respiratory Medicine, Cork University Hospital, University College Cork, Cork, Ireland.
Division of Pulmonary and Critical Care Medicine, University of Washington, Seattle, WA, USA.
Lancet Respir Med. 2013 Apr;1(2):164-74. doi: 10.1016/S2213-2600(13)70025-0. Epub 2013 Mar 19.
Several key advances have been made in the treatment and management of people with cystic fibrosis in the past two decades. Substantial improvements in survival have resulted from the introduction of key drugs, coordinated care packages, improved nutritional support, and the intensive use of antibiotics. The age profile of people with cystic fibrosis has changed greatly during this time-some countries now have more adult than paediatric patients with the disease. With their increasing age and more advanced lung disease, several important sequelae (both pulmonary and extrapulmonary) occur in these adult patients including pulmonary disease, cystic fibrosis-related diabetes, renal disease, metabolic bone disease, cancers, drug allergies and toxic effects, and complications associated with lung transplantation.
在过去的二十年中，囊性纤维化患者的治疗和管理取得了几项重大进展。关键药物的引入、协调的护理套餐、改善的营养支持以及抗生素的密集使用，使得生存状况得到了实质性的改善。在此期间，囊性纤维化患者的年龄结构发生了巨大变化——一些国家现在成年患者比儿科患者多。随着年龄的增长和更严重的肺部疾病，这些成年患者会出现一些重要的后遗症（肺部和肺外），包括肺部疾病、囊性纤维化相关糖尿病、肾脏疾病、代谢性骨病、癌症、药物过敏和毒性作用，以及与肺移植相关的并发症。
