囊性纤维化相关性糖尿病的特征：来自欧洲囊性纤维化学会患者登记处和德国/奥地利糖尿病前瞻性随访登记处的两个不同来源的数据。
Characteristics of cystic fibrosis-related diabetes: Data from two different sources the European cystic fibrosis society patient registry and German/Austrian diabetes prospective follow-up registry.
机构信息
Institute of Epidemiology and Medical Biometry, Central Institute for Biomedical Technology, University of Ulm, Ulm, Germany.
German Center for Diabetes Research (DZD), Neuherberg, Germany.
出版信息
Pediatr Diabetes. 2019 May;20(3):255-262. doi: 10.1111/pedi.12831. Epub 2019 Mar 7.
BACKGROUND
Standardized patient registries provide a unique basis to get insight into cystic fibrosis (CF)-related diabetes (CFRD), the most common comorbidity in CF.
METHODS
A total of 3853 CFRD patients from the European CF Society Patient Registry (ECFSPR) and 752 from the German/Austrian diabetes prospective follow-up (diabetes patienten verlaufsdokumentation [DPV]) were studied. To adjust for age and sex, multivariable regression was used (SAS 9.4).
RESULTS
DPV subjects were younger (26.5 [20.2-32.6] vs 28.3 [21.7-36.0] years, P < 0.001) and more often female (59.6 vs 50.9%, P < 0.001). In both registries, F508del homozygotes were most frequent, with higher proportion in DPV (80.9 vs 57.8%, P = 0.003). After adjustment, lung-transplantation (LTX) was more common in ECFSPR (18.9 vs 4.9%, P < 0.001), although duration since LTX (4.8 ± 0.2 vs 5.5 ± 0.7 years, P = 0.33) did not differ. In DPV patients without LTX, a lower BMI (19.6 ± 0.1 vs 21.0 ± 0.1 kg/m , P < 0.001), higher proportion of underweight (41.2 vs 20.2%, P < 0.001) and a tendency towards worse lung function (%FEV : 42.3 ± 4.2 vs 48.3 ± 0.5%, P = 0.16) were observed.
CONCLUSIONS
Between both registries, demographic and clinical differences of CFRD were present. Besides different kind of data sources, diverse treatment structures between countries may play a role. The results may further indicate a more serious illness in patients treated in specialized diabetes clinics, documenting their data in DPV.
背景
标准化患者注册为深入了解囊性纤维化（CF）相关糖尿病（CFRD）提供了独特的基础，这是 CF 最常见的合并症。
方法
共研究了来自欧洲 CF 学会患者注册处（ECFSPR）的 3853 例 CFRD 患者和来自德国/奥地利糖尿病前瞻性随访（糖尿病 patienten verlaufsdokumentation [DPV]）的 752 例患者。为了调整年龄和性别，使用多变量回归（SAS 9.4）。
结果
DPV 组患者年龄较小（26.5 [20.2-32.6] 岁与 28.3 [21.7-36.0] 岁，P<0.001），女性比例更高（59.6%与 50.9%，P<0.001）。在两个登记处，F508del 纯合子最常见，DPV 中的比例更高（80.9%与 57.8%，P=0.003）。调整后，肺移植（LTX）在 ECFSPR 中更为常见（18.9%与 4.9%，P<0.001），尽管 LTX 后的时间（4.8±0.2 年与 5.5±0.7 年，P=0.33）没有差异。在没有 LTX 的 DPV 患者中，体重指数（BMI）较低（19.6±0.1 千克/米与 21.0±0.1 千克/米，P<0.001），体重不足比例较高（41.2%与 20.2%，P<0.001），肺功能更差（%FEV：42.3±4.2%与 48.3±0.5%，P=0.16）。
结论
在这两个登记处之间，CFRD 的人口统计学和临床差异存在。除了不同类型的数据源外，国家之间不同的治疗结构也可能起作用。结果可能进一步表明，在专门的糖尿病诊所接受治疗的患者病情更严重，他们的数据记录在 DPV 中。
Zotero 插件