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

Cystic fibrosis Data quality Data quality audit Patient registries

Journal

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
ISSN: 1873-5010
Titre abrégé: J Cyst Fibros
Pays: Netherlands
ID NLM: 101128966

Informations de publication

Date de publication:
22 Nov 2023
Historique:
received: 02 05 2023
revised: 03 08 2023
accepted: 31 08 2023
medline: 24 11 2023
pubmed: 24 11 2023
entrez: 23 11 2023
Statut: aheadofprint

Résumé

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.

Sections du résumé

BACKGROUND 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 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 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 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.

Identifiants

pubmed: 37996316
pii: S1569-1993(23)00904-9
doi: 10.1016/j.jcf.2023.08.015
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

Copyright © 2023. Published by Elsevier B.V.

Déclaration de conflit d'intérêts

Declaration of Competing Interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Kathleen Jahn, Andreas Jung, Philipp Latzin, Alexander Moeller, Christian Murer, Lutz Naehrlich, Alain Sauty and Jakob Usemann have received honoraria from Vertex Pharmaceuticals. Andreas Jung has received consulting fees and honoraria from EffRx Pharmaceuticals and Viatris. Philipp Latzin has received honoraria from CSL Vifor.

Auteurs

Lara Wolf (L)

Department of Respiratory Medicine, University Children's Hospital Zurich, Switzerland; Departments of Pulmonology and Paediatric Pulmonology, Cantonal Hospital Winterthur, Switzerland.

Jakob Usemann (J)

Department of Respiratory Medicine, University Children's Hospital Zurich, Switzerland; Department of Pulmonology, University Children's Hospital Basel, Switzerland.

Eugénie Collaud (E)

Department of Respiratory Medicine, University Children's Hospital Zurich, Switzerland.

Marie-France Derkenne (MF)

Department of Pulmonology, Lausanne University Hospital, Switzerland.

Reta Fischer (R)

Quartier Bleu, Pulmonology Outpatient Clinic, Berne, Switzerland.

Maxime Hensen (M)

Department of Paediatrics, Cantonal Hospital Fribourg, Switzerland.

Michael Hitzler (M)

Department of Paediatric Pulmonology, Cantonal Hospital Lucerne, Switzerland.

Markus Hofer (M)

Departments of Pulmonology and Paediatric Pulmonology, Cantonal Hospital Winterthur, Switzerland.

Demet Inci (D)

Department of Respiratory Medicine, University Children's Hospital Zurich, Switzerland.

Sarosh Irani (S)

Department of Pulmonology, Cantonal Hospital Aarau, Switzerland.

Kathleen Jahn (K)

Clinic of Respiratory Medicine and Pulmonary Cell Research, University Hospital Basel, Switzerland.

Angela Koutsokera (A)

Department of Pulmonology, Lausanne University Hospital, Switzerland.

Rachel Kusche (R)

Department of Pulmonology, Cantonal Hospital Aarau, Switzerland; Department of Paediatric Pulmonology, Cantonal Hospital Aarau, Switzerland.

Thomas Kurowski (T)

Department of Pulmonology, University Hospital Zurich, Switzerland.

Philipp Latzin (P)

Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics, Inselspital Berne University Hospital, Switzerland.

Dagmar Lin (D)

Department of Pulmonology, Inselspital Berne University Hospital, Switzerland.

Laurence Mioranza (L)

Paediatric Pulmonology and Cystic Fibrosis Unit, Division of Paediatrics, Department Woman-Mother-Child, Lausanne University Hospital, Switzerland.

Alexander Moeller (A)

Department of Respiratory Medicine, University Children's Hospital Zurich, Switzerland.

Anne Mornand (A)

Department of Paediatric Pulmonology, Geneva University Hospital, Switzerland.

Dominik Mueller-Suter (D)

Department of Paediatric Pulmonology, Cantonal Hospital Aarau, Switzerland.

Christian Murer (C)

Department of Pulmonology, Cantonal Hospital Lucerne, Switzerland.

Lutz Naehrlich (L)

Department of Paediatrics, Justus-Liebig-University Giessen, Germany; European Cystic Fibrosis Society Patient Registry, Karup, Denmark.

Jérôme Plojoux (J)

Deparment of Pulmonology, Geneva University Hospital, Switzerland.

Nicolas Regamey (N)

Department of Paediatric Pulmonology, Cantonal Hospital Lucerne, Switzerland.

Romy Rodriguez (R)

Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics, Inselspital Berne University Hospital, Switzerland.

Isabelle Rochat (I)

Paediatric Pulmonology and Cystic Fibrosis Unit, Division of Paediatrics, Department Woman-Mother-Child, Lausanne University Hospital, Switzerland.

Alain Sauty (A)

Department of Pulmonology, Réseau Hospitalier Neuchâtelois, Switzerland.

Macé Schuurmans (M)

Department of Pulmonology, University Hospital Zurich, Switzerland.

Michaela Semmler (M)

Department of Pulmonology, Inselspital Berne University Hospital, Switzerland.

Daniel Trachsel (D)

Department of Pulmonology, University Children's Hospital Basel, Switzerland.

Anna-Lena Walter (AL)

Department of Pulmonology, Cantonal Hospital St. Gallen, Switzerland.

Andreas Jung (A)

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. Electronic address: andreas.jung@kispi.uzh.ch.

Classifications MeSH