PiNSALink September 2004

IPOPI Board Visit to Aventis Behring in Marburg , Germany

At the end of April the IPOPI Board members met for a Subcutaneous IgG Workshop hosted by Aventis Behring in Marburg , Germany . Marburg is an old German town which traces its roots back to the 9 th century, is famous for its university and boasts of scholars such as Boris Pasternak, Jacob and Wilhelm Grimm, Robert Bunsen and Emil Von Behring. It is also well known for a magnificent Gothic building, the Church of St Elizabeth situated on the way to Old Town . It is a very picturesque city, set against a hillside with Landgrave ' s Castle at the top. The loving restoration of half-timbered houses gives Marburg an air of gracious history. In the centre of the Old Town is a Market Square , a popular venue for political meetings and cultural events. Some of the Board members experienced the Mayor singing in the spring at midnight of the first of May, an annual tradition.

The Board was privileged to be extremely well looked after by Aventis Behring and they certainly went out of their way to make us feel welcome. On the first day we were given a presentation on the company and shown the office and Nobel Prize for Medicine of Emil Von Behring. We then had a tour of the IgG production building and were impressed by the stringent measures in place to ensure hygiene and safety.

The following day we enjoyed a series of scientific presentations. Amongst these were the Safety of CE1200; the Aventis Behring Safety System by F. Niemann and the Production process/Virus safety/pasteurisation by T Nowak. Clinical Studies relating to CE1200 both in Scandinavia and in the EU and US by P. Kiessling and health related quality of life by U. Nicolay. There was then a discussion of patient ' s needs and the day finished with a Board meeting.

The Board would like to thank Aventis Behring and in particular Stefan Haag for the organization of this trip and also Christiane Gorski. It was a wonderful experience for all concerned and we came away with a greater appreciation for the amount of expertise and meticulous measures in place in the provision of blood products.

The ESID Online Patient and Research Registry
Barbara Frisch

The European Society for Immunodeficiencies (ESID) is about to launch a high-capacity and comprehensive modern online database system for immune deficiency diseases (PID). The project is named the ESID Online Patient and Research Registry.

The primary aim of the ESID Online Registry project is to increase the accessibility of data from patients with PID to facilitate and optimize research in these rare diseases and monitor the efficacy of awareness programs for PID. Moreover, the implementation of standards for diagnosis and therapy with help of the registry will be to the best advantage of patients. However, by engaging in the spirit of ESID it is also the intended outcome of the ESID Online Registry project to direct the attention to patient ' s situation and needs. Therefore, a group of specialists is about to elaborate a specific Quality of Life sub-registry in collaboration with PID patients and nurses. A specifically designed part of the database system may also be accessible for PID patients.

Data will be collected in disease specific sub-registries for PID, containing information such as the diagnosis, the gender, the age at onset of symptoms, the age at diagnosis, laboratory data, the past and current medication, adverse side effects of treatment, results of immunizations, genetic mutation detection etc. These sub-registries shall be developed on the basis of disease specific data models. In a second step, existing databases and national registries shall be integrated into the ESID Online Registry. The registry is intended as a clinical data management system that holds data-subsets of numerous participating centres and presents these data only to authenticated users (controlled by User-Password authentication). The implementation and integration of new modules and data fields is possible and existing modules and data structures are modifiable.

The ESID Online Registry is capable of assigning any follow-up data to a specific coded case. Therefore, in some countries (e.g. Germany ) a patient ' s consent form must be signed before patients data may be entered into the internet system. The data, centrally stored, shall be used for clinical data management, for research and some limited data will also be used for funding. In addition, specific features of the ESID Online Registry will be useful for physicians to supervise the treatment of documented patients.

Since April 1 st 2004, the first of approximately 125 different disease specific sub-registries is online, and entered into a month-long test stage. The lead off sub-registry is on CVID (common variable immunodeficiency), which meets with interests and needs of a multitude of patients as well as the requirements of physicians and researchers who are engaged in this complex disorder. We are looking forward to an exiting and very promising project.