Registry Working Party
Registry WP - Newsletter August 2015
by Nizar Mahlaoui
The ESID registry has been a great achievement of the ESID society since its creation in 2004 and has gathered information on a growing number of patients collected through 100+ centers across Europe. It is thus the largest database on PID worldwide and one of the major component of our Society.
You can have more information here: http://esid.org/Working-Parties/Registry
Together with the Registry WP-Steering Committee Elect (Bodo Grimbacher, Germany - Isabella Quinti, Italy - Matthew Buckland, United Kingdom - Markus Seidel, Austria - Joris van Montfrans, The Netherlands) and Mrs. Jose Drabwell, IPOPI President, along with Gerhard Kindle and Benjamin Gathmann, both from Freiburg University, we continue to work on the new Registry platform.
Our SC meeting was held in Freiburg on May 22nd 2015.
Some recent achievements
- 1. Data transfer to the new registry (Level 1):
Since the launch of the new system, more than 6,200 patients have been validated and registered, among whom more than 1,200 are newly registered patients.
This includes a quality control process that is implemented during the transfer of data from the old to the new registry database.
The UK Registry based on the ESID Registry recently went live and the patients should be migrated to the new Registry very soon (more than 3,500 patients).
We hope to get the Spanish Registry transfer their patients and the Italian centers document directly to the ESID Registry in the forthcoming months.
We, and the NMOs (with the contribution of IPOPI), had contacts with some Scandinavian centers that expressed interest in contributing their patient to the Level 1 in the near future.
- 2. ESID Registry Clinical Diagnosis Criteria:
The ESID Registry WP has developed new working clinical diagnosis criteria for PID patients lacking a genetic documentation ( http://esid.org/Working-Parties/Registry/Diagnosis-criteria) in order to enhance the quality of registration of patient.
- 3. ESID Database data access and publication rules:
These have been recently updated. Please visit our website for further information.
- 4. Level 3 studies:
- The APDS project (PIs: Prof Stephan Ehl, Prof. Alison Condliffe, Dr. Sven Kracker). This collaborative study is financially supported by 3 pharmaceutical companies: Novartis, HSK and UCB. More information here: http://esid.org/Working-Parties/Registry/Studies/APDS-Registry
- The unPAD (unclassified Predominantly Antibody Disorder, PI: Prof. Esther de Vries). The initiation of this study is financially supported by PPTA.
- 5. Six papers using the ESID Registry have been published this year so far:
- Marschall K et al. Clin Exp Immunol. The Swiss National Registry for Primary Immunodeficiencies: Report on the first 6 years' activity 2008-2014. (PMID: 26031847)
- Jonkman-Berk BM et al. Clin Immunol. Primary immunodeficiencies in the Netherlands: National patient data demonstrate the increased risk of malignancy. (PMID: 25451158)
- Turley AJ et al. J Clin Immunol. Spectrum and Management of Complement Immunodeficiencies (Excluding Hereditary Angioedema) Across Europe. (PMID: 25663093)
- Aydin SE et al. J Clin Immunol. DOCK8 Deficiency: Clinical and Immunological Phenotype and Treatment Options - a Review of 136 Patients. (PMID: 25627830)
- Salvator H et al. Eur Respir J. Pulmonary manifestations in adult patients with CGD. (PMID: 25614174)
- Suarez F et al. J Clin Oncol. Incidence, presentation, and prognosis of malignancies in ataxia-telangiectasia: a report from the French national registry of primary immune deficiencies. (PMID: 25488969)
In the near future, we plan to:
- Complete the working diagnostic criteria
- Implement the first level 2 registration forms
- Implement the link to a high quality genetics module, along with the ESID Genetics WP.
- Foster clinical and translational research on PIDs
- Implement the first Level 3 study
- Perform the first analysis of the new registry (epidemiological data, therapy data…)
- Secure the Registry funding through Level3 projects along other potential sources, including with the ESID support
Thank you to all contributors to the ESID Registry.
Have a great summer!
REGISTRY WP Chairman, on behalf of the ESID Registry WP Steering Committee.
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