Department of Respiratory Medicine, University Children's Hospital Zurich, Switzerland; Departments of Pulmonology and Paediatric Pulmonology, Cantonal Hospital Winterthur, Switzerland.
Department of Respiratory Medicine, University Children's Hospital Zurich, Switzerland; Department of Pulmonology, University Children's Hospital Basel, Switzerland.
J Cyst Fibros. 2024 May;23(3):506-511. doi: 10.1016/j.jcf.2023.08.015. Epub 2023 Nov 22.
Good data quality is essential when rare disease registries are used as a data source for pharmacovigilance studies. This study investigated data quality of the Swiss cystic fibrosis (CF) registry in the frame of a European Cystic Fibrosis Society Patient Registry (ECFSPR) project aiming to implement measures to increase data reliability for registry-based research.
All 20 pediatric and adult Swiss CF centers participated in a data quality audit between 2018 and 2020, and in a re-audit in 2022. Accuracy, consistency and completeness of variables and definitions were evaluated, and missing source data and informed consents (ICs) were assessed.
The first audit included 601 out of 997 Swiss people with CF (60.3 %). Data quality, as defined by data correctness ≥95 %, was high for most of the variables. Inconsistencies of specific variables were observed because of an incorrect application of the variable definition. The proportion of missing data was low with <5 % for almost all variables. A considerable number of missing source data occurred for CFTR variants. Availability of ICs varied largely between centers (10 centers had >5 % of missing documents). After providing feedback to the centers, availability of genetic source data and ICs improved.
Data audits demonstrated an overall good data quality in the Swiss CF registry. Specific measures such as support of the participating sites, training of data managers and centralized data collection should be implemented in rare disease registries to optimize data quality and provide robust data for registry-based scientific research.
当罕见病登记处被用作药物警戒研究的数据来源时，良好的数据质量至关重要。本研究在欧洲囊性纤维化协会患者登记处（ECFSPR）项目的框架内调查了瑞士囊性纤维化（CF）登记处的数据质量，旨在实施措施以提高基于登记的研究的数据可靠性。
2018 年至 2020 年间，所有 20 家瑞士儿科和成人 CF 中心参与了数据质量审核，并于 2022 年进行了重新审核。评估了变量和定义的准确性、一致性和完整性，并评估了缺失的源数据和知情同意书（ICs）。
第一次审核包括 997 名瑞士 CF 患者中的 601 名（60.3%）。数据质量，定义为数据正确性≥95%，对于大多数变量都很高。由于变量定义的不正确应用，观察到特定变量的不一致。几乎所有变量的缺失数据比例都很低，<5%。CFTR 变体的大量源数据缺失。ICs 的可用性在中心之间差异很大（10 个中心有>5%的缺失文件）。在向中心提供反馈后，遗传源数据和 ICs 的可用性得到了改善。
数据审核表明瑞士 CF 登记处的数据质量总体良好。在罕见病登记处中，应实施特定措施，如支持参与站点、培训数据管理员和集中数据收集，以优化数据质量并为基于登记的科学研究提供可靠的数据。
