1. Progress report of the ESID online registry Bodo Grimbacher, MD Head of ESID patient registries www.esid-registry.org

2. Input requires a positive statement from the local data protection officer and a signed consent from the patient(s) or legal tutor (approved form provided in 9 languages) 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) Only coded data allowed (ONLY the patient’s physician can match a specific data-set with his patients) No demographical data or ‘free text’ that potentially directly identifies the patient is stored in the system Implementation of the ‘2-server solution’ Thereof follows: - Signed consent form from patients - Positive statement of ethics and data protection - Agreement between ESID and documenting centre e.g. on core dataset (red fields) etc. Security and Dataprotection Concept and architecture of an internet based ESID database network

3. Consent form for patients available in 17 languages

4. Signed by 46 documenting centers from 24 countries Agreement ESID Documenting Center

5. http://www.esid-registry.org The ESID Main Registry

6. http://www.esid-registry.org The ESID Main Registry

7. Show all Patients The ESID Main Registry

8. The ESID Main Registry

9. Building up lists

10. The ESID Main Registry

11. Please document EITHER the core dataset (red fields are subject to financial compensation) OR the complete patient chart below. ESID-main vs. ESID-subregistries

12. ESID Main Registry & Subregistries ESID Main Patient Registry CVID Subregistry DGS Subregistry NBS Subregistry XLA Subregistry HIGM Subregistry SCID Subregistry Neutropenia Subregistry...additional Subregistries

13. The DGS Subregistry

14. Time schedule June 15th, 2004 ESID main registry online October 2004CVID subregistry online October 2004DGS subregistry online October 2004IPEX subregistry online January 2005NBS subregistry online January 2005ICF subregistry online February 2005HIES subregistry online February 2005ICOS subregistry online February 2005drug-induced hypogamma online Summer 2005SCID subregistry TEST online Summer 2005XLA subregistry TEST online Summer 2005HIGM subregistry TEST online Summer 2005IgAD subregistry TEST online Summer 2005Osteopetrosis subregistry TEST online http://www.esid-registry.org/TEST.jsp

15. Functionalitis of the database ICD-10 code implementationactive Extensible drop-downs [+]active Tool-Tips: exact definition of fields [?]active Mutation detection Toolactive Color codingactive Report-generation Toolactive SQL query Toolactive Recall-function06/2005 Query by Example Tool06/2005 Easy doable Free-Query tool2006

16. The CVID Subregistry

17. Functionalitis of the database ICD-10 code implementationactive Extensible drop-downs [+]active Tool-Tips: exact definition of fields [?]active Mutation detection Toolactive Color codingactive Report-generation Toolactive SQL query Toolactive Recall-function06/2005 Query by Example Tool06/2005 Easy doable Free-Query tool2006

18. [+] and [?]

19. Functionalitis of the database ICD-10 code implementationactive Extensible drop-downs [+]active Tool-Tips: exact definition of fields [?]active Mutation detection Toolactive Color codingactive Report-generation Toolactive SQL query Toolactive Recall-function06/2005 Query by Example Tool06/2005 Easy doable Free-Query tool2006

20. The IPEX Subregistry

22. ESID Mutation Web Service

23. Functionalitis of the database ICD-10 code implementationactive Extensible drop-downs [+]active Tool-Tips: exact definition of fields [?]active Mutation detection Toolactive Color codingactive SQL query Toolactive Report-generation Tool06/2005 Recall-function06/2005 Query by Example Tool06/2005 Easy doable Free-Query tool2006

25. Functionalitis of the database ICD-10 code implementationactive Extensible drop-downs [+]active Tool-Tips: exact definition of fields [?]active Mutation detection Toolactive Color codingactive SQL query Toolactive Report-generation Tool06/2005 Recall-function06/2005 Query by Example Tool06/2005 Easy doable Free-Query tool2006 please send an Email to: frisch@medizin.ukl.uni-freiburg.de

26. Functionalitis of the database ICD-10 code implementationactive Extensible drop-downs [+]active Tool-Tips: exact definition of fields [?]active Mutation detection Toolactive Color codingactive SQL query Toolactive Report-generation Tool09/2005 Recall-function10/2005 Query by Example Tool12/2005 Easy doable Free-Query tool2006

27. Documentalist/Physician/User Web Application Patient List (PL) coded Data (cD) Accesses Web App Sends data collection to user WebApp retrieves patient data from the PL Database WebApp retrieves data from the cD Database DB Server 1 DB Server 2 Application Server Firewall 1 Firewall 2

28. Functionalitis of the database ICD-10 code implementationactive Extensible drop-downs [+]active Tool-Tips: exact definition of fields [?]active Mutation detection Toolactive Color codingactive SQL query Toolactive Report-generation Tool09/2005 Recall-function10/2005 Query by Example Tool12/2005 Easy doable Free-Query tool2006

29. Number of Patients per Documenting Center

30. Number of entries per diagnosis

31. Subregistries

32. Info for ESID members Bonus system:  Compensation for secretarial assistance (EUR 10 per patient per year if documented by Jun.30th, 2005)  Publication award 2005 of max. EUR 15.000 for the best publications using data of the ESID Registry  2005 Travel grants to ESID meetings and summer school: 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  Extra EUR 2000 for the first 15 centers to reach 50 fully documented patients

33. The database network taking clinical patient registries to the next level..... Bodo Grimbacher, MD University Hospital Freiburg Head of ESID patient registries grimbacher@medizin.ukl.uni-freiburg.de