CUREHLH

"European initiative to improve knowledge, treatment and survival of haemophagocytic syndromes in children"

Coordinatore	UNIVERSITAETSKLINIKUM HAMBURG-EPPENDORF    more  Organization address address: Martinistrasse 52 city: HAMBURG postcode: 20246 contact info Titolo: Dr. Nome: Kai Cognome: Lehmberg Email: send email Telefono: +49 40 7410 54209 Fax: +49 40 7410 54601
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

#	participant	country	role	EC contrib. [€]
1	UNIVERSITAETSKLINIKUM HAMBURG-EPPENDORF  Organization address address: Martinistrasse 52 city: HAMBURG postcode: 20246 contact info Titolo: Dr. Nome: Kai Cognome: Lehmberg Email: send email Telefono: +49 40 7410 54209 Fax: +49 40 7410 54601	DE (HAMBURG)	coordinator	0.00
2	AZIENDA OSPEDALIERO-UNIVERSITARIA ANNA MEYER  Organization address address: VIALE PIERACCINI 24 city: FIRENZE postcode: 50139 contact info Titolo: Dr. Nome: Massimo Cognome: Braganti Email: send email Telefono: 0039-055-5662301 Fax: 0039-055-5662379	IT (FIRENZE)	participant	0.00
3	FORSCHUNGSZENTRUM BORSTEL  Organization address address: Parkallee 1-40 city: BORSTEL postcode: 23845 contact info Titolo: Ms. Nome: Susann Cognome: Schrader Email: send email Telefono: 0049(0)-40-4537188220 Fax: 0049(0)-40-4537188721	DE (BORSTEL)	participant	0.00
4	INSTITUT NATIONAL DE LA SANTE ET DE LA RECHERCHE MEDICALE (INSERM)  Organization address address: 101 Rue de Tolbiac city: PARIS postcode: 75654 contact info Titolo: Ms. Nome: Annick Cognome: Bertault Email: send email Telefono: 0033-1-40784901 Fax: 0033-1-40784998	FR (PARIS)	participant	0.00
5	ISTITUTO GIANNINA GASLINI  Organization address address: VIA GEROLAMO GASLINI 5 city: GENOVA postcode: 16147 contact info Titolo: Dr. Nome: Patrizia Cognome: Fabrizi Email: send email Telefono: 0039 010 563 6461 Fax: 0039 010 377 6590	IT (GENOVA)	participant	0.00
6	KAROLINSKA INSTITUTET  Organization address address: Nobels Vag 5 city: STOCKHOLM postcode: 17177 contact info Titolo: Mr. Nome: Thomas Cognome: Walstam Email: send email Telefono: 0046-8-51773487 Fax: 0046-8-51771774	SE (STOCKHOLM)	participant	0.00
7	THE CHANCELLOR, MASTERS AND SCHOLARS OF THE UNIVERSITY OF CAMBRIDGE  Organization address address: The Old Schools, Trinity Lane city: CAMBRIDGE postcode: CB2 1TN contact info Titolo: Ms. Nome: Dawn Cognome: Barker Email: send email Telefono: +44-1223 333543 Fax: 0044-1223 332988	UK (CAMBRIDGE)	participant	0.00
8	UNIVERSITA DEGLI STUDI DI BRESCIA  Organization address address: Piazza Del Mercato 15 city: BRESCIA postcode: 25121 contact info Titolo: Prof. Nome: Raffaele Cognome: Badolato Email: send email Telefono: 0039 0303995717 Fax: 0039 0303388099	IT (BRESCIA)	participant	0.00
9	UNIVERSITAETSKLINIKUM FREIBURG  Organization address address: HUGSTETTER STRASSE 49 city: FREIBURG postcode: 79106 contact info Nome: Gerhard Cognome: Henninger Email: send email Telefono: 0049-761-270-1920 Fax: -2969	DE (FREIBURG)	participant	0.00

Mappa

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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.