Clinical Research Registries
Project lead by Benjamin Gathmann
Electronic registries that collect patient data are a central asset for PID research. These patient registries make it possible to analyze the epidemiology and natural course of primary immunodeficiencies, identify factors affecting the clinical course and evaluate the impact of therapeutic strategies. Furthermore, they are a central asset for studies investigating genotype-phenotype correlations. While single centers often have very limited cohort sizes, patient registries make it possible to define larger cohorts based on common clinical features and use these for genetic studies. Thereby, they provide researchers with sufficient numbers of cases for genetic research and clinical trials.
We have realized that there is a need for establishing patient registries both within the CCI as well as on the national and international level. In 2009, we set up an internal registry for our outpatient clinics which is used by physicians and study assistants to report clinical findings in a well-defined and structured way. The registry enables researchers in the CCI to quickly identify patients with specific features and analyze their data. It is also a data source for Health Services Research (e.g. the PID-CARE study).
On the next level, we coordinate the German national registry for primary immunodeficiencies as part of the PID-NET consortium (www.pid-net.org) which is funded by the German Federal Ministry of Education and Research (BMBF, 01GM0896). The national registry was set up to analyze the epidemiology and natural course of primary immunodeficiencies, assess the diagnostic delay for single PIDs, identify factors affecting the clinical course, evaluate the impact of therapeutic strategies and to compare and evaluate treatment regimes between medical centers in Germany. Furthermore, the registry is also intended for establishing links between medical centers within Germany and beyond. Data from the CCI outpatient clinic registry is electronically transferred to the national registry in regular intervals. As of November 2013, the national registry contains data on 1,867 patients. The distribution of patients on PID centers in Germany is visualized in Figure 1.
Figure 1: Geographic distribution of centers in Germany. Centers that are located in the same city are subsumed under the city’s name. Point markers are proportional to the number of reported patients. NB: The registration of current patients has not been completed in most of the centers.
The national PID registry is embedded within the European PID registry of the European Society for Immunodeficiencies (ESID, www.esid.org). The ESID Registry was established by Bodo Grimbacher in 2004 and is coordinated by the head of the ESID Registry Working Party. It is hosted and developed at the CCI and currently (Nov 2013) holds data on more than 18,800 patients from 30 countries (see Figure 2). The ESID Registry also maintains active ties with the United States' USIDnet Registry and the Latin American LASID Registry, as well as registries in Asia and Australia. Thereby, the contribution of the CCI to the German national registry provides links to immunodeficiency centers around the globe.
Figure 2: Registered (living) patients and PID prevalence in the ESID Database As of Sep 26, 2013; Total number of living patients: 16,264
Important Publications
Gathmann et al.: The German national registry for primary immunodeficiencies (PID). Clin Exp Immunol. 2013 Aug;173(2):372-80.
Gathmann et al.: ESID Registry Working Party. The European internet-based patient and research database for primary immunodeficiencies: results 2006-2008. Clin Exp Immunol. 2009 Sep;157 Suppl 1:3-11.
Contact
Benjamin Gathmann
ESID Online Database Coordinator
PID-NET Registry Coordinator
Tel.: 0761 270-78171
Fax: 0761 270-36960
benjamin.gathmann@uniklinik-freiburg.de