Results from the European CGD registry funded by the CGD Research Trust were presented recently at the 13th International Congress of Immunology in Rio de Janeiro, Brazil.
Dr Merlijn van den Berg of the Emma Children’s Hospital in Amsterdam gave a summary of the analysis of clinical data obtained between 2000 and 2003 on 430 patients affected by CGD. Data were collected from 12 centres in ten European countries (but excluded the data from the UK).
The results, which will be interesting for patients and families, have attracted lots of interest from clinicians working with CGD patients as well as basic scientists.
Dr Van den Berg explained that the data on people affected by CGD is being collected because:“We need to know if current therapies are working and improving survival rates for people with CGD.”
New insights about the two forms of CGD have been gained: “Our data suggest that the non-X-linked form of CGD − termed autosomal recessive and affecting both males and females - is diagnosed in children at a later age than the X-linked form of CGD. We found evidence supporting a less severe clinical course in autosomal recessive patients as compared to the X-linked group.”
The database is also sharpening our understanding of which parts of the body are most vulnerable, and to which organisms: Dr Van den Berg said they found that “the most prevalent manifestations of CGD occurred in the lungs, followed by skin, lymph nodes and gastro-intestinal tract and that the vast majority of infections were caused by Staphylococcus aureus, Aspergillus and Salmonella species”.
Dr Van den Berg outlined some of the next steps to make the most of the database:
“Our aim is to improve the treatment and clinical outcome for people with CGD. Funding by the CGD Research Trust has enabled us to collect valuable data on people with CGD in mainland Europe. This information will be entered into an on-line database under the umbrella of the European Society for Immunodeficiencies. The CGD subregistry database, currently under construction, will allow clinicians to update the data on their patients and register details of recently diagnosed cases of CGD. Among the many questions we have regarding the variation of the disease, analysis of these prospective data will help us for instance to link the clinical course and survival of CGD patients to prophylactic [preventative] treatment and thus improve clinical care in the future.”
Download the abstract of Dr van den Berg’s findings presented at the meeting.IMPORTANT NOTE :
The information contained on this website is intended only as a guideline, not as a substitute for medical advice. Always consult your doctor if you or your child has any CGD symptoms or concerns.
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