In the last framework programme, the coordinator of this FP7 project, Prof. Grimbacher headed the development and the establishment of a clinical online patient registry. After 4 years already more than 9.000 patients with primary immunodeficiency diseases were registered into the ESID online database, (59%) of which belonging to the group of PAD (www.esid.org/esid_registry.php). This project will be taken further to create a platform for the exchange of clinical information and research data complemented by quality of life data from the actual patients. To this end, the database shall be complemented by a sample bank.
The ESID Online Registry (www.esid.org/esid_registry.php) is capable of registering clinical and laboratory data on all 206 primary immunodeficiency diseases currently listed in the IUIS publication (1). The particular strength of this database is that follow-up data is being collected on a yearly basis. The data currently being collected however only comprises a common dataset of 22 fields which are considered to give the basic information needed for assessment of an individual patient. In this WP we will extend the datasets of each PAD to facilitate future observational trials.
 Notarangelo LD, Fischer A, Geha RS, Casanova JL, Chapel H, Conley ME, Cunningham-Rundles C, Etzioni A, Hammarström L, Nonoyama S, Ochs HD, Puck J, Roifman C, Seger R, Wedgwood J. Primary immunodeficiency diseases: 2009 update. International Union of Immunological Societies Expert Committee on Primary Immunodeficiencies. J Allergy Clin Immunol. 2009; 124(6):1161-1178.
 Guzman D, Veit D, Knerr V, Kindle G, Gathmann B, Eades-Perner A, Grimbacher B. The ESID Online Database network. Bioinformatics. 2007;23:654-5.
 Eades-Perner AM, Gathmann B, Knerr V, Guzman D, Veit D, Kindle G, Grimbacher B; ESID Registry Working Party. The European internet-based patient and research database for primary immunodeficiencies: results 2004-06. Clin Exp Immunol. 2007;147:306-12.
Lung disease is one of the most frequent complications and causes of death of the patients with a primary antibody deficiency syndrome (PAD). Longitudinal data suggest that lung disease starts during childhood and leads to progressive structural lung changes as demonstrated by computed tomography (CT) of the chest. The goals of project 4 are i) to assess lung pathology of PID patients with spirometry and computed chest tomography and ii) to determine the sensitivity and specificity of Multiple Breath Washout (MBW) technique as an alternative for detection of early lung disease in paediatric PAD patients.