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| Thursday October 16, 2008 |
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| 7.45 - 8.45 |
ESIDjuniors Breakfast Session
"Meet the expert" |
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Location for this session only: Hotel Central (click
here for route
and map) |
| MTE 1 |
BMT |
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Andrew J. Cant (Newcastle, United
Kingdom) |
| MTE 2 |
Innate immunity |
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Steve Holland (Bethesda, USA) |
| MTE 3 |
B-cells |
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Helen Chapel (Oxford, United Kingdom) |
| MTE 4 |
T-cells |
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Hans D. Ochs (Seattle, USA) |
| MTE 5 |
Diagnostic protocols in PID |
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Esther de Vries (`s-Hertogenbosch, The
Netherlands) |
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|
| 9.00 – 10.30 |
Educational Day Session I: CVID |
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Opening and welcome |
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A case
of agammaglobulinemia with normal count of B cells, associated with
severe lung and liver fibrosis. A new disease? |
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A.
Trizzino (Palermo, Italy) |
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Serum antibody response
to tetanus toxoid in children with Down syndrome |
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M.A.A. Kusters (‘s-Hertogenbosch, Netherlands) |
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B-cell development and immunophenotyping |
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Jacques J.M. van Dongen (Rotterdam, The
Netherlands) |
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Clinical aspects of CVID |
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Helen Chapel (Oxford, United Kingdom) |
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| 11.00 – 12.30 |
Educational Day Session II: Autoimmunity
/ Immune Dysregulation |
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Atypical onset of APECED (Autoimmune Polyendocrinopathy, Candidiasis,
Ectodermal Dystrophy) in two siblings |
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D.
Montin (Torino, Italy) |
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Chronic
inflammatory bowel disease is a new phenotype of Artemis deficiency |
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J.C.
Rohr (Freiburg, Germany) |
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Hyper-IgE Syndromes: Progress in defining the genetic basis for these
clinical entities |
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E.D.
Renner (Munich, Germany) |
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Reconstitution of thymic function after gene therapy for ADA-SCID:
preliminary observations by imaging and immunological studies |
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F.
Ferrua (Milan, Italy) |
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Diseases caused by lack of immune
regulation |
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Hans D. Ochs (Seattle, USA) |
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| 13.45 – 14.45 |
Educational Day Session III:
Deficit of innate immunity |
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Disease related to NEMO and IRAK4
deficiency |
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Jordan Orange (Philadelphia, USA) |
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Neutrophil disorders |
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A.R. Gennery (Newcastle, United Kingdom) |
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|
| 14.45 – 16.00 |
Educational Day Session IV: Infectious
diseases / PID |
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A
patient affected by SCN with long term CSF3R acquired mutation and no
progression in MDS/AML |
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L.
Giunti (Firenze, Italy) |
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Complement factor B deficiency associated with recurrent asceptic
meningitis |
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F.
Haerynck (Ghent, Belgium) |
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Infections defining PID |
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Andrew J. Cant (Newcastle, United
Kingdom) |
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| 16.00 – 16.30 |
Poster viewing |
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| 16.30 – 18.00 |
Symposium "Women in PID" |
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Esther de Vries (`s-Hertogenbosch, The
Netherlands), Eleonora Gambineri (Florence, Italy), Helen Chapel
(Oxford, United Kingdom) |
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Guest speaker: t.b.a. |
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| 18.15 – 19.45 |
ESID-INGID-IPOPI Opening Session |
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Chair:
Esther De Vries (The Netherlands) |
| 18.15 |
Meeting President, ESID President, INGID
President, IPOPI President |
| 18.45 |
Keynote Lecture:
Trends in primary immunodeficiencies |
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Alain Fischer (Paris, France) |
| 19.30 |
Music |
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| 20.00 – 22.00 |
Welcome reception at Jheronimus Bosch
Art Center |
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| Friday October 17, 2008 |
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| 8.30 – 10.30 |
ESID Parallel Session Ia: The thymus,
T-cell development and auto-immunity |
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Chairs:
Naomi Taylor (France) and Maarten J.D. Van Tol (The Netherlands) |
| 8.30 |
T-cell selection in relation to
auto-immunity |
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Georg Holländer (Basel, Switzerland) |
| 9.00 |
Fingerprints of Human Cytomegalovirus on
the immune system |
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René A.W. van Lier (Amsterdam, The
Netherlands) |
| 9.30 |
Clinical experience in T-cell deficient
patients |
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Andrew J. Cant (Newcastle, United
Kingdom) |
| 10.00 |
Long-term thymic reconstitution following intrathymic transfer of
progenitor cells in a model of ZAP-70-deficient scid |
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R.
Vicente (Montepellier, France) |
| 10.10 |
Impact
of thymic epithelium on immunopathology in lymphopenic mice
reconstituted with hypomorphic Rag2 haemopoietic progenitors |
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V.
Marrella (Segrate, Italy) |
| 10.20 |
Genetic
defects of T-cell development affect organization and maturation of
thymic epithelial and dendritic cells in humans and mice: Implications
for leaky SCID and Omenn syndrome |
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L.D.
Notarangelo (Boston, USA) |
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|
| 8.30 – 10.30 |
ESID Parallel Session Ib: Innate
immunity |
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Chairs:
Bodo Grimbacher (United Kingdom) and
Jos Van der Meer (The Netherlands) |
| 8.30 |
Signaling defect due to the
mutations in Tyk2 and STAT3 resulting in hyper-IgE syndrome |
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Yoshiyuki Minegishi (Tokyo, Japan) |
| 9.00 |
The patients of Job: STAT3,
IL-17 and the new face of an old disease |
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Steve Holland (Bethesda, USA) |
| 9.30 |
Identification of novel genes underlying the development of Herpes
Simplex Encephalitis |
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V.
Sancho-Shimizu (Paris, France) |
| 9.40 |
Defects
of IFN? receptors 1 and 2 impair the phagocytic NADPH oxidase function
and CYBB, CYBA, and NCF1 gene expression |
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C.C.M.
Prando (Campinas, Brazil) |
| 9.50 |
EDA-ID
due to NEMO mutation complicated by disseminated mycobacterium
infection: Successful haematopoietic stem cell transplantation |
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M.
Abinun (Newcastle Upon Tyne, United Kingdom) |
| 10.00 |
Severe
score 5 WAS in infants: clinical and biological presentation and
outcome. Analysis of the french national registry/CEREDIH network |
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N.
Mahlaoui (Paris, France) |
| 10.10 |
A
germline but macrophage-tropic CYBB mutation in patients with X-linked
predisposition to mycobacterial diseases |
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J.C.B.
Bustamante (Paris, France) |
| 10.20 |
A new
form of Osteoclast poor Osteopetrosis due to mutations in RANK gene
|
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A.
Villa (Segrate, Italy) |
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|
| 10.30 – 11.00 |
Poster viewing |
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| 11.00 – 12.30 |
ESID Plenary Session II: Extended
frontiers in immunodeficiency |
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Chairs:
Jean-Laurent Casanova (France) and Bert Gerritsen (The Netherlands) |
| 11.00 |
Crohn’s is an
immunodeficiency disease |
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Anthony W. Segal (London,United Kingdom) |
| 11.30 |
Hemolytic uremic syndrome -
a primary immunodeficiency? |
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Marina Botto (London, United Kingdom) |
| 12.00 |
Impaired ex vivo interferon production in a patient with severe
varicella |
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S.Y.
Zhang (Paris, France) |
| 12.10 |
Hereditary multiple intestinal atresia with immunodeficiency:
Immunologic findings and differential gene expression. |
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K. Chen
(Los Angeles, USA) |
| 12.20 |
A
unique haplotype of the MUNC13-4 gene associated with familial
hemophagocytic lymphohistiocytosis and macrophage activation syndrome in
systemic juvenile idiopathic arthritis |
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K.Z.
Zhang (Cincinnati, USA) |
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| 13.00 – 14.30 |
Satellite Symposium: Baxter |
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Click
here for the symposium invitation and program |
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| 13.00 – 14.30 |
Satellite Symposium: Octapharma |
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Click
here for the program |
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| 12.30 – 14.30 |
Poster viewing |
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| 14.30 – 16.00 |
ESID General Assembly (members only) |
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| 16.00 – 16.30 |
Poster viewing |
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| 16.30 – 18.00 |
Workshop 1: Management of PID |
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Chair: Anna Sediva (Prague, Czech
Republic) |
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| 16.30 – 18.00 |
Workshop 2: Gene therapy |
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Chair: Bobby Gaspar (London, United
Kingdom) |
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| 18.30 – 19.30 |
Cultural Event at St. John's Cathedral |
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| Saturday October 18, 2008 |
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| 7.30 – 8.30 |
Breakfast Session II: Sanquin |
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More autonomy for immunodeficient patients, new developments
in home treatment |
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Chair: Esther de Vries
(The Netherlands) |
|
07.30 |
Treating immunodeficient patients, home therapy is the future? |
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Helen Chapel (Oxford,
United Kingdom) |
|
07.35 |
From hospital to home treatment, the Dutch approach |
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Esther de Vries
('s-Hertogenbosch,The
Netherlands) |
|
08.05 |
In my own hands, a patient’s view on home therapy |
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Merlijn van Rij
(Amsterdam,The
Netherlands) |
|
08.15 |
Sanquin Home Service, a tailor made service for intravenous and
subcutaneous therapy
Video about the transition to home treatment of a Dutch family |
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|
| 8.30 – 9.30 |
ESID Parallel Session IIIa: Migration
and regulation |
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Chairs:
Laszlo Marodi (Hungary) and Arjan Lankester (The Netherlands) |
| 8.30 |
Migration and regulation of
the immune response: Lessons learned from LCH |
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Nicola E. Annels (London, United Kingdom) |
| 9.00 |
MAS |
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Gritta Janka-Schaub (Hamburg, Germany) |
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| 9.30 – 10.30 |
ESID Parallel Session IIIb:
Visualization of the immune response |
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Chairs:
Naomi Taylor (France) and C. Weemaes (The Netherlands) |
| 9.30 |
Monocytes and dendritic cells |
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Frédéric Geissmann (Paris, France) |
| 10.00 |
Imaging T cell activation |
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Andrey Shaw (St. Louis,
USA) |
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|
| 8.30 – 10.30 |
ESID Parallel Session IIIc: Stem cell
regeneration |
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Chairs:
Mario Abinun (United Kingdom) and Ilhan Tezcan (Turkey) |
| 8.30 |
Delineating the cellular and molecular
pathways of hematopoietic lineage commitment |
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Sten E.W. Jacobsen (Oxford,
UK and Lund, Sweden) |
| 9.00 |
Divide, differentiate or
die? Manipulating hematopoietic stem cell fate decisions through
molecular reprogramming. |
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Paul Coffer (Utrecht, The Netherlands) |
| 9.30 |
Immunomodulation by mesenchymal stem
cells |
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Katarina Le Blanc, (Stockholm, Sweden) |
| 10.00 |
Hematopoietic stem cell gene therapy for Wiskott Aldrich syndrome |
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K. Boztug (Hannover, Germany) |
| 10.10 |
Stem cell transplantation for Nijmegen breakage syndrome – experience in
5 patients |
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M.H. Albert (München, Germany) |
| 10.20 |
Immune reconstitution in ADA-SCID patients treated with hematopoietic
stem cell gene therapy |
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I. Brigida (Milan, Italy) |
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| 11.00 – 12.30 |
ESID Parallel Session IVa: New insights
in B-cell development |
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Chairs:
Bobby Gaspar (United Kingdom) and Lennart Hammarström (Sweden) |
| 11.00 |
Dynamics of B-cell development |
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Jacques J.M. van Dongen (Rotterdam, The
Netherlands) |
| 11.30 |
B-cells, genes and disease |
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Mary-Ellen Conley (Memphis, USA) |
| 12.00 |
Human CD20 deficiency impairs the ability to mount T cell independent
antibody responses. |
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T.W.
Kuijpers (Amsterdam, The Netherlands) |
| 12.10 |
Accumulation of specific memory B-cell subsets in peripheral blood of
healthy adults in response to pneumococcal vaccination. |
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R.S. Abraham (Rochester, USA) |
| 12.20 |
Human PMS2 deficiency is associatedwith
impaired immunoglobulin
class switch recombination |
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A. Durandy
(Paris, France) |
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| 11.00 – 12.30 |
ESID Parallel Session IVb: NK-cells |
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Chairs:
Laszlo Marodi (Hungary) and Nico Hartwig (The Netherlands) |
| 11.00 |
The role of NK-cells in shaping the immune
response |
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Eric Vivier (Marseille, France) |
| 11.30 |
Insights into NK-cell
function from primary immunodeficiency disorders |
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Jordan Orange (Philadelphia, USA) |
| 12.00 |
Human NK cells can control CMV-infection in the absence of T cells |
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T.W.
Kuijpers (Amsterdam, The Netherlands) |
| 12.10 |
Dendritic cells require CD18 for normal podosome formation |
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S. Burns (London, United Kingdom) |
| 12.20 |
Wasp confers selective advantage for specific hematopoietic cell
populations and serves a unique role in marginal zone development and
function |
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L. Westerberg
(Boston, USA) |
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|
| 13.00 – 14.30 |
Satellite Symposium: CSL – Behring |
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Click
here for the program |
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| 13.00 – 14.30 |
Satellite Symposium: Grifols |
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Click
here for the program |
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| 14.30 – 16.00 |
Workshop 3: Diagnosis of PID |
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Chairs: Esther de Vries
(`s-Hertogenbosch,The Netherlands), Mirjam van der Burg (Rotterdam,The
Netherlands) |
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|
| 14.30 – 16.00 |
Workshop 4: The ESID online registry:
How can we use it to the full? |
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Chairs: Gerhard Kindle (Freiburg,
Germany), Taco W. Kuijpers (Amsterdam, The Netherlands) |
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|
| 14.30 – 16.00 |
Workshop 5: Stem cell transplantation |
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Chairs: Mario Abinun (Newcastle, United
Kingdom), Andrew J.Cant (Newcastle, United Kingdom) |
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| 16.30 – 18.00 |
ESID Parallel Session Va: Long-term
management in PID |
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Chairs:
Eleonora Gambineri (Italy) and Jaap.Van Dissel (The Netherlands) |
| 16.30 |
The adult PID patient |
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Isabella Quinti (Rome, Italy) |
| 17.00 |
Granulomatous disease in CVID |
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P.
Martin van Hagen (Rotterdam, The
Netherlands) |
| 17.30 |
Long-term analysis of immune reconstitution and clinical status after
hematopoietic cell transplantation for severe T-cell immunodeficiencies |
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D. De
Martiis (Brescia, Italy |
| 17.40 |
Long term cognitive outcome following HSCT for severe congenital
immunodeficiency: what factors are associated with long term
difficulties?. |
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P. Titman (London, United Kingdom) |
| 17.50 |
Clinical, biological and genetic characteristics of 242 patients
affected with ataxia-telangiectasia: A retrospective analysis from the
French primary immunodeficiency registry (CEREDIH) |
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R. Micol (Paris, France) |
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|
| 16.30 – 18.00 |
ESID Parallel Session Vb: Th17 and
Tregs in immune regulation |
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Chairs:
Bodo Grimbacher (United Kingdom)
and Nico Wulffraat (The Netherlands) |
| 16.30 |
Th17 cells |
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Gitta Stockinger (Mill Hill,United
Kingdom) |
| 17.00 |
What do T regulatory cells
see and do |
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Ethan M. Shevach (Bethesda, USA) |
| 17.30 |
Hyper-IgE associated mutant STAT3 proteins exert a dominant negative
effect on STAT3 transcriptional activity via different mechanisms
depending on the domain of the protein affected |
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T.R. Torgerson (Seatlle, USA) |
| 17.40 |
Mutations in STAT3 and IL12RB1mpair the development of human
IL-17-producing T cells |
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L. De Beaucoudrey (Paris, France) |
| 17.50 |
Impaired Th-17 responses in patients with chronic mucocutaneous
candidiasis |
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D. Lilic (Durham / Newcastle Upon Tyne, United Kingdom) |
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| 16.30 – 18.00 |
Workshop 6: PID diagnosis and management
beyond Europe |
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Chair: Yu Lung Lau, Hong Kong (China) |
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| 20.00 – 24.00 |
Gala dinner (ESID-INGID-IPOPI members
and their accompanying persons only) at the Orangerie |
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| Sunday October 19, 2008 |
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|
| 08.30 – 10.30 |
ESID Plenary Session VI: Novel Primary
Immunodeficiencies & Late Breakers |
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Chairs:
Jean-Laurent Casanova (France) and Andrew J. Cant (United
Kingdom) |
| 8.30 |
A novel
clinical syndrome associating severe congenital neutropenia and complex
developmental aberrations caused by deficiency of G6PC3 |
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K. Boztug
(Hannover, Germany) |
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LAD-1/variant
syndrome is caused by mutations in FERMT3 |
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D.
Roos(Amsterdam, The Netherlands) |
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Kindlin 3 - A
new gene involved in the pathogenesis of LAD III |
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A. Etzioni
(Haifa, Israel) |
| 9.10 |
Human adenylate
kinase 2 deficiency causes a very profound haematopoietic defect
associated with sensorineural deafness (Reticular Dysgenesis) |
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M.C.C.
Cavazzana-Calvo (Paris, France) |
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New
non-homologous end joining defect in radiosensitive T-B- SCID:
Identification of the first human DNA-PKcs mutation |
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M. Van der
Burg (Rotterdam, The Netherlands) |
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Human CD27
deficiency: a novel immune-deficiency causing EBV induced
lymphoproliferative disease |
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J.M. Van
Montfrans (Utrecht, The Netherlands) |
| 9.50 |
Antibody-deficiency and Acute Nephritic Syndrome in a Patient with
Homozygous Disruption of the CD81 Gene |
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M.C. Van
Zelm (Rotterdam, The Netherlands) |
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Pyogenic
bacterial infections in humans with MyD88 deficiency |
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H. Von
Bernuth (Paris, France) |
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Chronic
mucocutaneous candidiasis is associated with a homozygous mutation in
CARD9 |
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B.
Grimbacher (London, United Kingdom) |
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|
| 11.00
- 12.00 |
ESID Working Party meetings:
SCT & GT, Clinical, Genetics, Education, PID-care in development,
ESIDjuniors and ESID website demonstration |
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|
| 12.00 – 13.00 |
ESID-INGID-IPOPI Closing Session |
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Chair:
Esther de Vries (The Netherlands) |
| 12.00 |
Keynote Closing Lecture: Future
developments in innate immune deficiency |
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Dirk Roos (Amsterdam, The Netherlands) |
| 12.45 |
IPOPI President, INGID President, ESID
President, Meeting President |