Adler Amanda I, Shine Brian S F, Chamnan Parinya, Haworth Charles S, Bilton Diana
Addenbrooke's Hospital, Cambridge, UK.
Diabetes Care. 2008 Sep;31(9):1789-94. doi: 10.2337/dc08-0466. Epub 2008 Jun 5.
Longer survival of patients with cystic fibrosis has increased the occurrence of cystic fibrosis-related diabetes (CFRD). In this study we documented the incidence of CFRD and evaluated the association between mutations responsible for cystic fibrosis and incident CFRD, while identifying potential risk factors.
This was a population-based longitudinal study of 50 cystic fibrosis speciality clinics in the U.K. Subjects included 8,029 individuals aged 0-64 years enrolled in the U.K. Cystic Fibrosis Registry during 1996-2005. Of these, 5,196 with data and without diabetes were included in analyses of incidence, and 3,275 with complete data were included in analyses of risk factors. Diabetes was defined by physician diagnosis, oral glucose tolerance testing, or treatment with hypoglycemic drugs.
A total of 526 individuals developed CFRD over 15,010 person-years. The annual incidence was 3.5%. The incidence was higher in female patients and in patients with mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene in classes I and II. In a multivariate model of 377 cases of 3,275 patients, CFTR class (relative risk 1.70 [95% CI 1.16-2.49], class I or II versus others), increasing age, female sex, worse pulmonary function, liver dysfunction, pancreatic insufficiency, and corticosteroid use were independently associated with incident diabetes.
The incidence of CFRD is high in Britain. CFTR class I and II mutations increase the risk of diabetes independent of other risk factors including pancreatic exocrine dysfunction.
囊性纤维化患者生存期的延长增加了囊性纤维化相关糖尿病（CFRD）的发生率。在本研究中，我们记录了CFRD的发病率，评估了导致囊性纤维化的突变与CFRD发病之间的关联，并确定了潜在风险因素。
这是一项基于人群的纵向研究，对英国50家囊性纤维化专科诊所进行了调查。研究对象包括1996 - 2005年期间纳入英国囊性纤维化登记处的8029名0 - 64岁个体。其中，5196名有数据且无糖尿病的个体纳入发病率分析，3275名有完整数据的个体纳入风险因素分析。糖尿病通过医生诊断、口服葡萄糖耐量试验或使用降糖药物治疗来定义。
在15010人年中，共有526人发生了CFRD。年发病率为3.5%。女性患者以及囊性纤维化跨膜传导调节因子（CFTR）基因I类和II类突变的患者发病率更高。在3275例患者中的377例病例的多变量模型中，CFTR类别（相对风险1.70 [95%CI 1.16 - 2.49]，I类或II类与其他类别相比）、年龄增长、女性性别、肺功能较差、肝功能障碍、胰腺功能不全和使用皮质类固醇与糖尿病发病独立相关。
英国CFRD的发病率很高。CFTR I类和II类突变增加了糖尿病风险，独立于包括胰腺外分泌功能障碍在内的其他风险因素。
