Contents Obituary 2 Introduction 3 ESID information 4 President’s letter 5 Secretary’s report 5 Treasurer’s report 7 News & Views 7 Journal of Experimental Medicine 7 Genetics and Mechanisms of Susceptibility to Infectious Diseases” Pasteur Institute, aris 7 21-24 November 2007 The 6th Prague Spring ESID Meeting , Prague, May 14 and 15, 2007 8 Suggestions to the ESID community for the Journal of Primary Immunodeficiency? 9 ESID Prague Spring Meeting, May 14 and 15, 2007 FORM 10 Scheduled J Project Meetings (No. 19 to 21) 2007 11 Report on the 1st J Project Meeting in Bucharest 11 J Project meeting in Zaporozhye, East-Ukraine, 19-20 April 2007 12 Laboratory of Human Genetics of Infectious Diseases, post-doc positions 12 Working Party reports 13 Educational 13 Genetics 13 Registries 14 Juniors 19 PID care in development 19 Interesting Papers 19 Interesting Cases 21 Young Researchers’ Corner 23 Roland Levinsky, 1943-2007 Roland was a pioneer and inspirational leader in the field of primary immunodeficiency. Although originally from South Africa, Roland spent his adult life in the UK, 26 years of it at the Insti- tute of Child Health (ICH) and Great Ormond Street Hospital (GOS) in London. Roland attended medical school in London (University College London) and then trained in paediatrics in Birmingham and London before moving to Great Ormond Street Hospital in London in 1973. He subsequently became Senior Lecturer and Honorary Consultant Immunologist at GOS and Hugh Greenwood Pro- fessor of Immunology in 1985, a Chair he held at the Institute of Child Health, until he left London in 2002 to be Vice-Chancellor of Plymouth University. Initially planning to become a Nephrologist, Roland became interested in the immunology of renal disease at GOS. This led on to immunology and in particular, the primary immunodeficiency disor- ders (PIDs), for which he pioneered the application of bone marrow transplantation (BMT) in the UK. This was also at a time when many similarly minded clinician scientists were interested in utilising the new techniques that were being developed in molecular biology and genetics to understand the molecular basis of disease. Roland was among a select group who understood the power of these new techniques and how they could eventually be used to “cure” these life-threatening diseases. Together with other like-minded individuals an informal grouping was established in 1983, the Euro- pean Group for Immunodeficiency Disease (EGID), which was the forerunner of what is known as ESID today. Using the large cohorts of patients, it was possible to use the new genetic techniques to identify the molecular basis of primary diseases and Roland was among the first to see this. This resulted in suc- cessful collaborations to identify the genes for X-linked agammaglobulinemia and X-linked hyper IgM syndrome. Roland also saw the potential for using this genetic knowledge to develop the techniques to treat PID using gene therapy. Roland was an enthusiastic advocate of gene therapy, being in- volved in the early European trials of gene therapy for adenosine deaminase deficiency. He was in- volved in the first research projects to obtain funding in the UK to develop gene therapy techniques for PID. His enthusiasm and vision lead directly to the successful gene therapy programme at GOS today. Roland was a highly efficient administrator. He became Dean of the ICH in 1990 when it became apparent to him that he was the only person who had the vision and strength of will to lead it forward. Roland set about a complete reorganisation of the Institute’s academic structure and abolished nearly all committees. He recruited many young clinical and non-clinical scientists, whose research interests ranged across diverse fields, from genetics to public health, but who all shared one thing: a desire to apply top quality science to diagnosis and treatment of children’s disease. This resulted in one of Roland’s greatest achievements; turning the ICH into a word-class children’s research organi- sation, and a legacy of highly trained and motivated medical research scientists all over the world, many of whom hold University Chairs in their own right. This success led to his appointment in 1999 as the Vice Provost for Biomedicine and Head of the Graduate School at University College London, and then Vice Chancellor of Plymouth University. Under his vision and leadership the university flourished and leapt up the rankings of the UK’s uni- versities. Roland will be widely missed by everyone who knew him. He was a giant in every sense, a tremen- dous family man, whose wisdom and kindness touched the lives of many. He was a mentor to many of us, and will be sorely missed for his friendship, wisdom, and humour. Christine Kinnon, Adrian Thrasher and Bobby Gaspar 2 The ESID Newsletter is made for the members of ESID – the European Society for Immuno Deficiencies. Dear ESID members, It is published under the responsibility of the ESID Board, and at this moment it is edited by Esther de We are very happy to present you a new issue of Vries (editor in chief), the ESID Newsletter. We hope you will find many Lucia Bianchi, Ales Janda, things that interest you. Gustavo Lazo, Nima Rezaei, and Crina We are very sorry to have lost such an Samarghitean. inspirational colleague as Roland Levinsky due to an unfortunate accident, and will all remember him. Any ESID member who is interested in publishing The ESIDjuniors have done their part - and his or her views, research, more! - to give you material to read and think about. new ideas or other Please give them your reactions, whether you are junior material in the ESID or senior yourself. Newsletter is cordially invited to submit copy to There are lots of interesting meetings to read the Editor. Suitability for about, and all kinds of information from the Working publication is assessed by Parties. the Editor in consultation with the other members Feel free to send us your ideas and comments, of the ESID Board. and contributions to the ESID Newsletter! Editorial address: Don’t miss the ESID Summer School Dr. Esther de Vries, advertisement on the back of this issue !! Please alert pediatric immunologist, all young people in the field you know of to this event. Jeroen Bosch Hospital loc Applications should be made online, all information about GZG, P.O. Box 90153, that can be found on the ESID website www.esid.org. 5200 ME ’s-Hertogen bosch, the Netherlands, tel. +31-73-6992965, fax I wish you all a beautiful springtime, +31-73-6992948. Please only use my new email address: esid@ Esther DE VRIES estherdevries.nl Front page: Dutch tulips. 3 registry, ESID decided to develop a new state-of-the-art database for primary immunodeficiencies. This online registry was launched in 2004 and contains subregistries for more than 150 primary immunodeficiencies. It combines both clinical and laboratory data of PID patients and offers the possibility to document genetic data as well. Up to date, more than 2,000 patients have been registered in that ESID is the European Society for database. Information, database statistics Immunodeficiencies. It was formed in 1994. and a demo version of the registry can be The forerunner of ESID, the informal found at www.esid.org/registry.php, or send an European Group for Immunodeficiencies email to [email protected]. (EGID) was established in 1983. The aims of this society are, among others, to facilitate The new ESID Online Registry is the exchange of ideas and information among connected to the mutation databases physicians, scientists and other investigators (IDbases) in Tampere, Finland. These were who are concerned with immunodeficiencies created since 1995, when the first locus- and to promote the research on these specific immunodeficiency mutation database diseases. Anyone who is interested in primary accessible through the internet was immunodeficiency diseases can become a established (BTKbase for X-linked member of ESID. Registration is possible agammaglobulinemia). Since then, more than online at www.esid.org/members.php. 100 additional locus-specific databases have been established. Information is available at Within ESID, seven Working http://bioinf.uta.fi. Parties are actively engaged in coordinating the member’s joined efforts in patient care ESID organizes a biennial and research in primary immunodeficiency congress to facilitate international contact diseases: Stem cell transplantation and gene between primary immunodeficiency specialists. therapy (chair: Mario Abinun), Registries The last congress was organised in 2006 in (chair: Gerhard Kindle), Clinical (chair: Budapest, Hungary , and the next one will be Bobby Gaspar), Genetics (chair: Naomi October 16-19 in `s-Hertogenbosch, The Taylor), Education (chair: Andrew Cant), PID- Netherlands, in 2008. Information is available care in development (chair: Laszlo Marodi), at www.esid2008.org. and ESIDjuniors (chair: Eleonora Gambineri). Anyone who is interested in participating in one or more of these Working Parties is invited to do so. Please contact the chairman of the relevant Working Party (contact = ESID Information = information is available at www.esid.org /board.php). In 1994, a main registry of patients with various forms of immunodeficiency in Europe was established. Altogether, data from some 10,000 patients from 26 countries was compiled until 2002. However, given various shortcomings of this 4 ESID Poll on changes to the constitution. Dear ESID members, In Budapest October 2006, during the GA, it was decided that ESID needs to amend As you may recall, we decided during its Constitution in order to reflect new the last General Assembly in Budapest, that challenges for the rapidly growing Society. the ESID should amend its Constitution. We Thus, after many discussions, the ESID Board thought the new Constitution should meet asked all ESID members to vote on the the new challenges facing a rapidly growing questions listed below and endorse the Board to Society.