E UROPEAN S OCIETY FOR I MMUNO D EFICIENCIES The ESID Online Patient- & Research Database

1. EUROPEAN SOCIETY FORIMMUNODEFICIENCIES The ESID Online Patient- & Research Database The ESID online database www.esid.org What is the ESID Registry and how can nurses use it to improve patient care? Bodo Grimbacher, MD Head of ESID Online Registry University Hospital Freiburg, Dep. Rheumatology & Clin. Immunology

2. AIMS • compile clinical and laboratory data of • patients with primary immunodeficiencies (PID) • improve diagnosis • improve classification • improve prognosis • improve therapy • provide continuous long-term compilation of data on PID • enable genetic and therapeutic trials within ESID • facilitate research across centres

3. Security and data protection Concept and architecture of an internet based ESID database network • SSL encrypted data transmission • Secure Server Net (audited Firewall of the Hospital of Freiburg) • User-Role Authentication • Passwords are stored in encrypted form • Multiple-User-Roles restrict views and presented data • Patient randomization (patient ID’s) • No demographical data or ‘free text’ that potentially directly identifies the patient is stored in the system • Coded and personalised version available • Implementation of a ‘multi-server solution’ in the personalised version

4. Security and data protection • Necessary documents • Signed patient consent form • Positive statement of ethics and data protection • Agreement ESID - documenting centre

5. Current status of Documenting Centres 64 centers signed an agreement with ESID 36 centers have already started documentation Russia (1) Total number of patients, Sept. 29 ,2006: 2755 NR NR NR NR Chilegid (Chile, NR) NR Turkey(4) Bragid (Brazil, NR) Iran(1) Egypt (1) NR = National Registry

6. Bonus system  Compensation for secretarial assistance (EUR 10 per patient per year if documented by June 30th) • Travel grants to ESID/INGID/IPOPI meetings and summer school (due day is June 30th every year) • 3 x EUR 500 for the center with the most registered patients • 2 x EUR 500 for the center with the second most registered patients • 1 x EUR 500 for the center with the third most registered patients  Publicationaward of max. EUR 15.000 for the best publications using data of the ESID Database

7. How to reach the database: www.esid.org For registered users only! For anyone interested Statistics! This is not the login to the database, but to the website!

8. New Features of the Database • pseudonymised and personalised version available • 28 extended subregistries • Search function for PID disease tree • Mutation Browser • Percentiles • SF 36 questionnaire • Predefined Queries • New website

9. Personalised vs. pseudonymised version Documenting centres choose between 2 versions: Pseudonymised version • personalised data on separate servers • easier and more comfortable working with the database • database becomes more attractive, can be used as electronic patient chart • data protection is guaranteed • no personal data in the database • centres maintain a separate list, matching IDs and names Personalised version

10. Subregistries already online • Common core dataset for more than 180 primary immunodeficiencies • Expert steering committees are developing disease-specific data models Disease-specific data models already available for... • Agamma with unknown genetic cause • ICOS deficiency • BLNK • TACI deficiency • Osteopetrosis • BAFF Receptor deficiency • MU-Chain • IG-Alpha (CD79A antigen deficiency) • Lambda 5 • Secondary selective IgA deficiency • CD19 deficiency • ICF Syndrome • Comel Netherton Syndrome • IPEX • CVID • XLA (BTK) • Selective IgA deficiency • Transient Hypogammaglobulinemia • Isolated IgG subclass deficiency • Ataxia Teleangiectasia • DiGeorge Syndrome • HIES • Nijmegan Breakage syndrome • Selective IgM deficiency • Deficiency of specific IgG • Secondary hypogammaglobulinemia

11. Search function for the „tree structure“ Enter a search string, e.g. CVID Or choose by clicking through the tree

12. Mutation Browser • in cooperation with the Institute of Medical Technology (IMT), • Tampere, Finland • gene mutations in 108 diseases can be entered • mutation data entered in the ESID database will be • validated and stored in the Finnish „IDbases“ • data is coded, contains no personal patient information • tool works with genomic DNA and cDNA

13. Mutation Browser Mutation displayed on ESID website

14. Percentiles • Weight, height and head circumference are part of the core dataset • Percentile curves are automatically drawn from this data • Helpful for paediatricians using the database as a patient chart

15. Patient’s percentile curve is drawn from values entered

16. SF36 • Medical Outcome Short Form (36) Health Survey • standardised measure of health care outcomes • designed for self-administration • Alternatively, face to face or telephone interview. • takes 5 –10 minutes to complete • Can be used for studies using the ESID Database! • Evaluation possible with the Database

17. SF36

18. Predefined Queries • How many patients in total are in the database? • What is their distribution regarding • documenting centres • diagnosis • categories • Age distribution • How many female patients? How many male patients? • How many patients does my centre have? • What is their distribution regarding diagnosis?

19. General numbers for all registered patients • Individual numbers for every centre

20. Contact us... Contact details • Email: registry@esid.org • Information, documents and demo version: www.esid.org  „ESID Registry“ • phone: +49 761 270 3445