Genetic determinants and epidemiology of cystic fibrosis-related diabetes: results from a British cohort of children and adults.
Genetic determinants and epidemiology of cystic fibrosis-related diabetes: results from a British cohort of children and adults.
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DOI：
10.2337/dc08-0466
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发表时间：
2008-09
期刊：
影响因子：
16.2
通讯作者：
Bilton D
中科院分区：
文献类型：
--
作者：
Adler AI;Shine BS;Chamnan P;Haworth CS;Bilton D
目的：囊性纤维化患者的生存期延长增加了囊性纤维化相关糖尿病(CFRD)的发生率。在这项研究中，我们记录了CFRD的发生率，并评估了导致囊性纤维化的突变与CFRD事件之间的关联，同时确定了潜在的危险因素。研究设计和方法--这是一项以人群为基础的纵向研究，涉及英国50家囊性纤维化专科诊所。研究对象包括1996年至2005年在英国囊性纤维化登记中心登记的8,029名0-岁的患者。其中，5196名有数据和没有糖尿病的患者被纳入发病率分析，3275名数据完整的患者被纳入危险因素分析。糖尿病的定义是医生诊断、口服葡萄糖耐量测试或降血糖药物治疗。结果--在15010人年中，共有526人患上了CFRD。年发病率为3.5%。在3275名患者的377例多因素模型中，CFTR分级(相对风险1.70[95%可信区间1.16-2.49]，I级或II级与其他类型相比)、年龄增加、女性、肺功能差、肝功能障碍、胰腺功能不全和皮质类固醇的使用与糖尿病的发生独立相关。结论--CFRD在英国的发病率很高。CFTRI和II类突变增加了糖尿病的风险，独立于其他风险因素，包括胰腺外分泌功能障碍。
OBJECTIVE—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. RESEARCH DESIGN AND METHODS—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. RESULTS—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. CONCLUSIONS—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.
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影响因子：
24.5
作者：
Ahmed, N;Corey, M;Durie, P
通讯作者：
Durie, P
影响因子：
16.2
作者：
Milla, CE;Billings, J;Moran, A
通讯作者：
Moran, A
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3.8
作者：
Cucinotta, D;De Luca, F;Arrigo, T
通讯作者：
Arrigo, T
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5.1
作者：
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通讯作者：
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影响因子：
105.7
作者：
LANNG, S;HANSEN, A;KOCH, C
通讯作者：
KOCH, C