Data accuracy, consistency and completeness of the national Swiss cystic fibrosis patient registry: Lessons from an ECFSPR data quality project,Journal of Cystic Fibrosis

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Data accuracy, consistency and completeness of the national Swiss cystic fibrosis patient registry: Lessons from an ECFSPR data quality project
Journal of Cystic Fibrosis ( IF 5.2 ) Pub Date : 2023-11-22 , DOI: 10.1016/j.jcf.2023.08.015
Lara Wolf ₁ , Jakob Usemann ₂ , Eugénie Collaud ₃ , Marie-France Derkenne ₄ , Reta Fischer ₅ , Maxime Hensen ₆ , Michael Hitzler ₇ , Markus Hofer ₈ , Demet Inci ₃ , Sarosh Irani ₉ , Kathleen Jahn ₁₀ , Angela Koutsokera ₄ , Rachel Kusche ₁₁ , Thomas Kurowski ₁₂ , Philipp Latzin ₁₃ , Dagmar Lin ₁₄ , Laurence Mioranza ₁₅ , Alexander Moeller ₃ , Anne Mornand ₁₆ , Dominik Mueller-Suter ₁₇ , Christian Murer ₁₈ , Lutz Naehrlich ₁₉ , Jérôme Plojoux ₂₀ , Nicolas Regamey ₇ , Romy Rodriguez ₁₃ , Isabelle Rochat ₁₅ , Alain Sauty ₂₁ , Macé Schuurmans ₁₂ , Michaela Semmler ₁₄ , Daniel Trachsel ₂₂ , Anna-Lena Walter ₂₃ , Andreas Jung ₂₄

Affiliation

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.
Department of Respiratory Medicine, University Children's Hospital Zurich, Switzerland.
Department of Pulmonology, Lausanne University Hospital, Switzerland.
Quartier Bleu, Pulmonology Outpatient Clinic, Berne, Switzerland.
Department of Paediatrics, Cantonal Hospital Fribourg, Switzerland.
Department of Paediatric Pulmonology, Cantonal Hospital Lucerne, Switzerland.
Departments of Pulmonology and Paediatric Pulmonology, Cantonal Hospital Winterthur, Switzerland.
Department of Pulmonology, Cantonal Hospital Aarau, Switzerland.
Clinic of Respiratory Medicine and Pulmonary Cell Research, University Hospital Basel, Switzerland.
Department of Pulmonology, Cantonal Hospital Aarau, Switzerland; Department of Paediatric Pulmonology, Cantonal Hospital Aarau, Switzerland.
Department of Pulmonology, University Hospital Zurich, Switzerland.
Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics, Inselspital Berne University Hospital, Switzerland.
Department of Pulmonology, Inselspital Berne University Hospital, Switzerland.
Paediatric Pulmonology and Cystic Fibrosis Unit, Division of Paediatrics, Department Woman-Mother-Child, Lausanne University Hospital, Switzerland.
Department of Paediatric Pulmonology, Geneva University Hospital, Switzerland.
Department of Paediatric Pulmonology, Cantonal Hospital Aarau, Switzerland.
Department of Pulmonology, Cantonal Hospital Lucerne, Switzerland.
Department of Paediatrics, Justus-Liebig-University Giessen, Germany; European Cystic Fibrosis Society Patient Registry, Karup, Denmark.
Deparment of Pulmonology, Geneva University Hospital, Switzerland.
Department of Pulmonology, Réseau Hospitalier Neuchâtelois, Switzerland.
Department of Pulmonology, University Children's Hospital Basel, Switzerland.
Department of Pulmonology, Cantonal Hospital St. Gallen, Switzerland.
Department of Respiratory Medicine, University Children's Hospital Zurich, Switzerland; Departments of Pulmonology and Paediatric Pulmonology, Cantonal Hospital Winterthur, Switzerland; European Cystic Fibrosis Society Patient Registry, Karup, Denmark.

Background

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.

Methods

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.

Results

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.

Conclusions

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.

中文翻译：

瑞士国家囊性纤维化患者登记处的数据准确性、一致性和完整性：ECFSPR 数据质量项目的经验教训

背景

当罕见疾病登记处被用作药物警戒研究的数据源时，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，旨在采取措施提高基于登记处的研究的数据可靠性。

方法

瑞士所有 20 个儿科和成人 CF 中心均参加了 2018 年至 2020 年的数据质量审计，并于 2022 年进行了重新审计。评估了变量和定义的准确性、一致性和完整性，以及缺失的源数据和知情同意书 (IC)进行了评估。

结果

第一次审计纳入了 997 名瑞士 CF 患者中的 601 名（60.3%）。大多数变量的数据质量（数据正确性≥95%）都很高。由于变量定义的应用不正确，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 变体存在大量源数据缺失。IC 的可用性在各个中心之间差异很大（10 个中心的文件丢失率超过 5%）。向中心提供反馈后，遗传源数据和 IC 的可用性得到改善。