CUREHLH
"European initiative to improve knowledge, treatment and survival of haemophagocytic syndromes in children"
| Coordinatore | UNIVERSITAETSKLINIKUM HAMBURG-EPPENDORF
Organization address
address: Martinistrasse 52 contact info |
| Nazionalità Coordinatore | Germany [DE] |
| Sito del progetto | http://www.uke.de/curehlh |
| Totale costo | 3˙917˙550 € |
| EC contributo | 2˙994˙942 € |
| Programma | FP7-HEALTH
Specific Programme "Cooperation": Health |
| Code Call | FP7-HEALTH-2007-A |
| Funding Scheme | CP-FP |
| Anno di inizio | 2008 |
| Periodo (anno-mese-giorno) | 2008-06-01 - 2011-05-31 |
Partecipanti
| # | ||||
|---|---|---|---|---|
| 1 |
UNIVERSITAETSKLINIKUM HAMBURG-EPPENDORF
Organization address
address: Martinistrasse 52 contact info |
DE (HAMBURG) | coordinator | 0.00 |
| 2 |
AZIENDA OSPEDALIERO-UNIVERSITARIA ANNA MEYER
Organization address
address: VIALE PIERACCINI 24 contact info |
IT (FIRENZE) | participant | 0.00 |
| 3 |
FORSCHUNGSZENTRUM BORSTEL
Organization address
address: Parkallee 1-40 contact info |
DE (BORSTEL) | participant | 0.00 |
| 4 |
INSTITUT NATIONAL DE LA SANTE ET DE LA RECHERCHE MEDICALE (INSERM)
Organization address
address: 101 Rue de Tolbiac contact info |
FR (PARIS) | participant | 0.00 |
| 5 |
ISTITUTO GIANNINA GASLINI
Organization address
address: VIA GEROLAMO GASLINI 5 contact info |
IT (GENOVA) | participant | 0.00 |
| 6 |
KAROLINSKA INSTITUTET
Organization address
address: Nobels Vag 5 contact info |
SE (STOCKHOLM) | participant | 0.00 |
| 7 |
THE CHANCELLOR, MASTERS AND SCHOLARS OF THE UNIVERSITY OF CAMBRIDGE
Organization address
address: The Old Schools, Trinity Lane contact info |
UK (CAMBRIDGE) | participant | 0.00 |
| 8 |
UNIVERSITA DEGLI STUDI DI BRESCIA
Organization address
address: Piazza Del Mercato 15 contact info |
IT (BRESCIA) | participant | 0.00 |
| 9 |
UNIVERSITAETSKLINIKUM FREIBURG
Organization address
address: HUGSTETTER STRASSE 49 contact info |
DE (FREIBURG) | participant | 0.00 |
Mappa
Word cloud
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Obiettivo del progetto (Objective)
'Haemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening inherited immune disorder of early childhood characterised by an uncontrolled immune response. Three disease causing genes have been identified in about half of the patients. Diagnosis is difficult, pathophysiology poorly understood, and treatment unsatisfactory with about 40% of the children dying from treatment failure or toxicity. All European scientists active in research and treatment of HLH have joined in a consortium to enforce collaboration, combine the various expertises, and provide access to material and data. In vitro studies and several murine models for HLH and diseases predisposing to HLH, will be used to get more insight into the pathophysiology of the diverse genetic defects. Studies will address the role of triggers and antigen-presenting cells, the influence of cytokines in the initiation and propagation of the uncontrolled immune response, the interaction of immune effector cells and soluble factors, and the mechanism of cytotoxicity in lymphocytes and natural killer cells. Data about geno-phenotype will be combined to evaluate how known and unknown genetic defects reflect on the clinical course and immunophenotype of the patients. A diagnostic algorithm, incorporating knowledge from the known defects, will be developed and optimised in all countries to facilitate earlier and reliable diagnosis, and to separate patients with unknown defects or acquired HLH. The identification of risk factors for treatment failure and toxicity will be used to design improved treatment strategies. An experimental treatment will be tested in a murine model and will be the basis for a less toxic treatment in the future. Earlier diagnosis, better insight into the pathophysiology, and development of less toxic treatments are the steps towards a higher cure rate in HLH. In a rare disease as HLH, only combined efforts on an European-wide level will be able to achieve these goals.'
Introduzione (Teaser)
Leading European immunologists teamed up to solve the enigma of haemophagocytic syndromes in children. By identifying triggering factors, exploring the role of cytokines and unveiling the mechanisms implicated in immune cell cytotoxicity, scientists wished to improve existing diagnostics and treatment options for patients.