The PCD Registry

Welcome


Welcome to the international registry for Primary Ciliary Dyskinesia (PCD).
PCD is a rare disorder of mucociliary clearance caused by defective hair like organelles (cilia). The purpose of the PCD Registry is to measure, survey and compare different aspects of PCD manifestation, course and treatment, to provide data for epidemiological research and to identify special patient groups suitable for multi-centre trials.
The PCD Registry is part of the European Reference Network for Rare Respiratory Diseases ERN-LUNG. We follow the recommendations of the EU Expert Committee on Rare Diseases including an international interoperatility of registries and databases to pool and exchange knowledge and data on rare disesases.

Heymut Omran, Petra Pennekamp, Johanna Raidt and Simone Helms (For the ERN-LUNG Network and the BESTCILIA consortium)

Bestcilia ERN LUNG
ERN on pages of European Commission

Login to the PCD Registry


If you are already part of the PCD Registry and had access to the previous data entry system, you should have received an email with new login information. If you did not receive such an email or want to become part of the PCD Registry, please contact the PCD Registry administrator.

FAQ


As internet browser we recommend the use of Mozilla Firefox or Google Chrome. Internet Explorer is not recommended.

How can the data be entered into the registry?

The data can either be entered into the PCD Registry directly after login, or imported via a specific *.CSV file that we provide in advance (download option after Log In or e-mail request to the PCD Registry administrator).
Under special circumstances (e.g. import of existing datasets from other registries/cohorts) we provide support to enter your data.
The PCD Registry is accessible from the web using the electronic data capture system REDCap. Each authorised operator is able to enter the data for their country/centre and export own center specific data to different formats (CSV/Microsoft Excel, PDF, SAS, Stata, R, ODM or SPSS).
A highly protected system will ensure data protection through the encryption of data. Identifying patient data are not collected in the PCD Registry.

Where are the data stored?

At the University of Münster, Institute of Medical Informatics - Informatics for personalised medicine (IMI). A server is located in secure premises, where access is allowed to authorized personnel only. Data storage is in agreement with EU data protection legislation.

How to become a registry centre?

In order to get assigned as a referring centre please contact the PCD Registry administrator.

Information for PCD Patients

Why do we need a PCD Registry?

Registries are the only way to pool data in the field of Rare Diseases (RD) such as PCD in order to achieve a sufficient sample size for epidemiological and/or clinical research. RD Registries such as the PCD Registry constitute key instruments to develop clinical research in the field of RD, to improve patient care and healthcare planning. RD Registries such as the PCD Registry are vital to assess the feasibility of clinical trials, to facilitate the planning of appropriate clinical trials and to support the enrolment of patients.

Why should I as a patient participate?

Patient involvement is the key factor in the establishment of any patient registry, especially RD Registries. For the purpose of the above aims, we need as many patients in the PCD Registry as possible in order to make sure that data are representative of the whole spectrum of the disease and to have enough patients to render research possible – research that may benefit you or other patients in the future.
If you – as a patient – have further questions please do not hesitate to contact us.

Which data are collected?

We collect demographic data (e.g. age, diagnostic findings and symptoms at diagnosis, current age, sex) but also clinical data which are recorded (e.g. lung function, weight, height, infections, treatment and complications). The data are collected using a common set of definitions, following the recommendations of the EU Expert Committee on Rare Diseases. This ensured international interoperability of registries and databases to pool and exchange knowledge and data on rare diseases.

Ways to register

Registration will be performed by the treating physician via password-secured access. The access authorization will be passed on by the PCD Registry administrator.

Information about data security

To ensure that patients are not accidentally registered twice and to take into account a changing of caring physician, personal data such as first name, last name, date of birth, and optionally middle name and place of birth have to be included in the first step of patient registration. These will be directly transferred into a Patient identification-Code (PID) and a pseudonym generated. Tracking a patient's identification with the PID is impossible. The registry administration uses the Mainzelliste, a web-based pseudonymisation service of the first level, which enables the Patient identification-Code (PID) from personal identifying data.
The medical health data is linked to the patient identification code (PID) and then stored in a central medical database in a data center physically separated from the Mainzelliste server used for PID generation. Personal identifying data and personal medical health data are always stored on different servers; a combination of data stored in these two databases is only possible for authorized treating physicians of the PCD treatment center.
Data saving underlies the regulations for data security laws and the medical confidentiality. International regulations for good clinical practice (GCP) always apply. Unauthorized third parties will not have access to registry data.
Mainzelliste publication: Lablans M, Borg A, √úckert F: A RESTful interface to pseudonymization services in modern web applications. BMC Med Inform Decis Mak. 2015 Feb 7;15:2. doi: 10.1186/s12911-014-0123-5.

Inclusion criteria

The PCD Registry is a longitudinal study. It recruits all prevalent subjects with PCD and all subsequent incident cases. Patients who fulfil the diagnostic criteria below should be included in the registry:

  • Clinical presentation consistent with PCD and
  • Confirmation of the diagnosing by at least two of the following methods:
    • abnormally low nasal NO concentration/production
    • abnormal high-speed video microscopic (HVMA) finding
    • abnormal immunofluorescence finding
    • demonstration of biallelic disease-causing mutations by genotyping (In those cases, where genetic diagnosis is not possible because of financial or structural issues, genetic diagnosis can be offered in the framework of a research project)
    • abnormal transmission electron microscopy (TEM) finding

(Note: if only high-speed video microscopy and nasal NO concentration/production are abnormal, high-speed video microscopy should be repeated at least three times and show the same abnormal results each time).
Given the complexity of diagnosing PCD, not all patients will meet the above mentioned definite diagnostic criteria. Therefore, individuals with typical clinical symptoms and only one abnormal diagnostic test are also eligible to enter the registry.

Ethics and Patient Confidentiality

Obtaining the necessary patient consents is a prerequisite for entering data in the PCD Registry. As legislation differs in each participating country, it is important to check the requirements with your local Data Protection Officers and Ethics Committees. If you have any questions, please contact the PCD Registry administrator. You will get support for the Ethics Committee approval procedure, e.g. sample consent forms for Patient Information and Consent. Patients can only be entered into the PCD Registry once you have received their signed, informed consent to do so. Please store all the consent forms that your patients have signed safely at your local institution. It is the responsibility of the reporting centres/countries/registries to have the acquired permissions to export/report data to the PCD Registry.

Data Security

Due to data protection regulations, the data stored in the server of the PCD Registry must be anonymous (i.e. the patient must not be identifiable). Therefore, the authorised operator has to keep a logbook with the identifying patient data and the code generated by the registry software. It is your responsibility to make sure that access to this list of names and matching patient codes is restricted to authorized personnel only. Furthermore, please note that if your list of matching codes and full names is lost, it will be impossible to retrieve the data identification by means of the PCD Registry software. Moreover, data are encrypted (i.e. not de-codable) when transmitted. The PCD Registry is protected according to EU Data Protection legislation, both physically and technically, and backup is secured.

Funding

The establishment of the PCD Registry was part of the EU funded BESTCILIA project (FP7/2007-2013) under grant agreement n° 305404 (BESTCILIA). Funding continues within several projects including REGISTRY WAREHOUSE (HORIZON2020, GA n° 777295).

About us

The PCD Registry has been designed and established by the BESTCILIA consortium headed by Heymut Omran, University Children's Hospital Münster, Department of General Pediatrics, Münster, Germany and is part of the European Reference Network on Rare Respiratory Diseases ERN-LUNG. For more information, please contact the PCD Registry administrator.

Impressum

Publisher and technical Implementation

Institute of Medical Informatics
Acting Head: Univ.-Prof. Dr. med. Julian Varghese
Albert-Schweitzer-Campus 1, Building A11
48149 Münster, Germany
Tel.: +49 251 83 - 5 52 61
Fax: 0251 / 83 - 5 22 59
imi(at)uni-muenster(dot)de

Responsible for the content:

University Children's Hospital Münster
Department of General Pediatrics
Director: Univ.-Prof. Dr. med. Heymut Omran
Albert-Schweitzer-Campus 1, Building A1
48149 Münster, Germany

Westfälische Wilhelms-Universität Münster
Faculty of Medicine
Working Group: Univ.-Prof. Dr. med. Heymut Omran
Albert-Schweitzer-Campus 1, Building A1
48149 Münster, Germany

Telephone (secretariat): +49 251 83 - 4 77 32
Fax (secretariat): +49 251 83 - 4 77 35
E-mail (secretariat): Sekretariat-Kinderklinik(at)ukmuenster(dot)de
E-mail (project): pcdregistry.eu(at)ukmuenster(dot)de

Dr. rer. nat. Petra Pennekamp
Telephone: +49 251 83 - 4 10 97

Simone Helms
Telephone: +49 251 83 - 4 83 58

Mandatory Information according to §5 Telemediengesetz (TMG, Telemedia Act):

The following mandatory information concerns the leading physicians and directors of the hospital and institutes.

For all leading physicians and directors the following applies:

The respective legal job title was awarded in Germany.
The physicians are members of the Medical Association Westfalen-Lippe (Ärztekammer Westfalen Lippe).
The supervisory authority within the scope of the activities of the statutory health insurance physicians is the Association of statutory health insurance physicians Westfalen-Lippe (Kassenärztliche Vereinigung Westfalen Lippe).
The professional regulation is the professional code of conduct (Berufsordnung) of the Ärztekammer Westfalen Lippe.

VAT ID
University of Münster: DE 126 118 759

Disclaimer
As a service provider we are responsible according to §7 para.1 TMG for our own contents on these pages (incl. pcdregistry.uni-muenster.de) according to the general laws. According to §§ 8 to 10 TMG, we are not obliged to monitor transmitted or stored third-party information or to investigate circumstances that indicate illegal activity.
Obligations to remove or block the use of information in accordance with general laws remain unaffected by this. However, liability in this respect is only possible from the time of knowledge of a concrete violation of the law. As soon as we become aware of such infringements, we will remove the content immediately.

On this website links to third party websites are used on whose contents we have no influence. We explicitly distance ourselves from contents of those websites (according to the verdict of the Landgericht Hamburg from May 12th 1998) and point out that we neither have bearing on the content of those websites nor attempt to adopt it. The owners ot these respective websites are solely liable for illegal, faulty or incomplete contents.