Garattini E, Bilton D, Cremona G, Hodson M
Servizio di Pneumologia, Istituto Scientifico San Raffaele, Milano, Italy.
Monaldi Arch Chest Dis. 2011 Sep;75(3):178-84. doi: 10.4081/monaldi.2011.219.
Cystic fibrosis (CF) is the most common autosomal recessive inherited disease of Caucasian populations. As a result of a variety of diagnostic and therapeutic strategies there has been a dramatic increase in the life expectancy of patients with CF in the last decades and 50% of patients are now adults. This review will focus on the disease in adults and the provision of appropriate care. The complex care required to improve the survival and quality of life in the adult patients can best be provided in a dedicated adult cystic fibrosis unit. These units currently exist in many European countries, but more are needed in Italy.
囊性纤维化（CF）是白种人群中最常见的常染色体隐性遗传病。由于多种诊断和治疗策略，在过去几十年中，CF患者的预期寿命显著增加，现在50%的患者已成年。本综述将聚焦于成年患者的疾病情况及适当护理的提供。改善成年患者生存和生活质量所需的复杂护理，最好在专门的成人囊性纤维化病房提供。目前许多欧洲国家都有这样的病房，但意大利还需要更多。
