Reincke M, Petersenn S, Buchfelder M, Gerbert B, Skrobek-Engel G, Franz H, Lohmann R, Quabbe H-J
Medical Center, Division of Endocrinology, University of Munich.
Exp Clin Endocrinol Diabetes. 2006 Oct;114(9):498-505. doi: 10.1055/s-2006-948313.
Patient registries are valuable tools to study long-term morbidity and mortality of rare diseases. Acromegaly is rare (incidence 3-4/mill/year, prevalence 40-70/mill; approx. 300 new patients/yr and up to 5700 patients in Germany). Diagnostic and therapeutic possibilities have considerably improved, but treatment results remain often unsatisfactory. The main cause is residual disease activity after surgery, most importantly due to invasive macroadenomas. The German Acromegaly Registry is an initiative of the Pituitary Study Group of the German Endocrine Society (DGE). Formally established in January 2003 by the Board of the DGE, long-term financial support is guaranteed by an unrestricted grant from Novartis Pharma GmbH to the DGE. The registry cooperates closely with the United Kingdom and the Austrian registries. The aim of the German Acromegaly Registry is to establish a database of sufficient epidemiological strength in order to (1) document co-morbidity and mortality, (2) provide data on diagnostic and therapeutic procedures/effectiveness, (3) enable comparison of procedures in different national centres, (4) provide information for patient support groups/interaction with health care providers, (5) enable comparison with other national registries within Europe. The registry has at present 82 participating centres, and 42 have included patients (20 university clinics, 8 non-university hospitals, 14 centres in private practice). The database aims to include all acromegalic patients in Germany who are cared for and treated at present. Up to December 2005 1543 patients have been entered in a retrospective manner. Data collection is by external monitoring by highly trained study nurses who visit the individual centres. Inclusion is planned to continue at a rate of 500 per year. Starting in 2005 centres are revisited every 3 years at a rate of 500 per year (prospective phase of the registry). Quality of the data has been validated by an independent monitoring team which demonstrated high data concordance.
Initial results of the German Acromegaly Registry show that it was possible to include a large number of patients within 3 years into the registry. Data quality has been validated and shown to be satisfactory. Therefore, the registry will be a useful tool to study long-term morbidity and mortality in a large series of patients.
患者登记库是研究罕见病长期发病率和死亡率的重要工具。肢端肥大症较为罕见（发病率为每年3 - 4/百万，患病率为40 - 70/百万；德国每年约有300名新患者，总计达5700名患者）。诊断和治疗方法虽有显著改善，但治疗效果往往仍不尽人意。主要原因是手术后疾病仍有残留活动，最重要的是侵袭性大腺瘤所致。德国肢端肥大症登记库是德国内分泌学会（DGE）垂体研究小组发起的项目。2003年1月由DGE董事会正式设立，诺华制药有限公司向DGE提供无限制赠款，确保了长期资金支持。该登记库与英国和奥地利的登记库密切合作。德国肢端肥大症登记库的目标是建立一个具有足够流行病学规模的数据库，以便（1）记录合并症和死亡率，（2）提供诊断和治疗程序/有效性的数据，（3）便于比较不同国家中心的程序，（4）为患者支持团体提供信息/促进与医疗服务提供者的互动，（5）便于与欧洲其他国家登记库进行比较。该登记库目前有82个参与中心，其中42个已纳入患者（20个大学诊所，8个非大学医院，14个私人执业中心）。该数据库旨在纳入目前在德国接受护理和治疗的所有肢端肥大症患者。截至2005年12月，已有1543名患者以回顾性方式录入。数据收集由训练有素的研究护士进行外部监测，他们走访各个中心。计划每年以500例的速度持续纳入患者。从2005年开始，每3年以每年500例的速度对各中心进行回访（登记库的前瞻性阶段）。数据质量已由一个独立监测团队验证，显示出高度的数据一致性。
德国肢端肥大症登记库的初步结果表明，在3年内有可能将大量患者纳入登记库。数据质量已得到验证且令人满意。因此，该登记库将成为研究大量患者长期发病率和死亡率的有用工具。
