M
Marianne Debré
Researcher at Necker-Enfants Malades Hospital
Publications - 61
Citations - 6318
Marianne Debré is an academic researcher from Necker-Enfants Malades Hospital. The author has contributed to research in topics: Immunodeficiency & Population. The author has an hindex of 37, co-authored 61 publications receiving 5785 citations. Previous affiliations of Marianne Debré include Louisiana State University in Shreveport & Paris Descartes University.
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Journal ArticleDOI
Efficacy of Gene Therapy for X-Linked Severe Combined Immunodeficiency
Salima Hacein-Bey-Abina,Julia Hauer,Annick Lim,Capucine Picard,Gary P. Wang,Charles C. Berry,Chantal Martinache,Frédéric Rieux-Laucat,Sylvain Latour,Bernd H. Belohradsky,Lily E. Leiva,Ricardo U. Sorensen,Marianne Debré,Jean-Laurent Casanova,Stéphane Blanche,Anne Durandy,Frederic D. Bushman,Alain Fischer,Marina Cavazzana-Calvo +18 more
TL;DR: After nearly 10 years of follow-up, gene therapy was shown to have corrected the immunodeficiency associated with SCID-X1 and may be an option for patients who do not have an HLA-identical donor for hematopoietic stem-cell transplantation and for whom the risks are deemed acceptable.
Journal ArticleDOI
Phosphoinositide 3-Kinase δ Gene Mutation Predisposes to Respiratory Infection and Airway Damage
Ivan Angulo,Oscar Vadas,Fabien Garçon,Edward Banham-Hall,Vincent Plagnol,Timothy Ronan Leahy,Timothy Ronan Leahy,Helen Baxendale,Tanya I. Coulter,Tanya I. Coulter,James Curtis,Changxin Wu,Katherine G. Blake-Palmer,Olga Perisic,Deborah J. Smyth,Mailis Maes,Christine A Fiddler,Jatinder K. Juss,Deirdre Cilliers,Gašper Markelj,Anita Chandra,George Farmer,Anna Kielkowska,Jonathan Clark,Sven Kracker,Sven Kracker,Marianne Debré,Capucine Picard,Capucine Picard,Isabelle Pellier,Nada Jabado,James Morris,Gabriela Barcenas-Morales,Alain Fischer,Alain Fischer,Len R. Stephens,Phillip T. Hawkins,Jeffrey C. Barrett,Mario Abinun,Menna R. Clatworthy,Anne Durandy,Rainer Doffinger,Edwin R. Chilvers,Andrew J. Cant,Dinakantha S. Kumararatne,Klaus Okkenhaug,Roger L. Williams,Alison M. Condliffe,Sergey Nejentsev +48 more
TL;DR: Activated PI3K-δ syndrome (APDS), a PID associated with a dominant gain-of-function mutation in which lysine replaced glutamic acid at residue 1021 (E1021K) in the p110δ protein, the catalytic subunit of phosphoinositide 3-kinase δ (PI3Kδ), encoded by the PIK3CD gene is described.
Journal ArticleDOI
Immunodeficiency, autoinflammation and amylopectinosis in humans with inherited HOIL-1 and LUBAC deficiency
Bertrand Boisson,Emmanuel Laplantine,Carolina Prando,Silvia Giliani,Elisabeth Israelsson,Zhaohui Xu,Avinash Abhyankar,Laura Israel,Laura Israel,Giraldina Trevejo-Nunez,Dusan Bogunovic,Alma-Martina Cepika,Donna A. MacDuff,Maya Chrabieh,Maya Chrabieh,Marjorie Hubeau,Marjorie Hubeau,Fanny Bajolle,Marianne Debré,Evelina Mazzolari,Donatella Vairo,Fabrice Agou,Herbert W. Virgin,Xavier Bossuyt,Caroline Rambaud,Fabio Facchetti,Damien Bonnet,Damien Bonnet,Pierre Quartier,Pierre Quartier,Jean-Christophe Fournet,Jean-Christophe Fournet,Virginia Pascual,Damien Chaussabel,Damien Chaussabel,Luigi D. Notarangelo,Anne Puel,Anne Puel,Alain Israël,Jean-Laurent Casanova,Capucine Picard +40 more
TL;DR: It is suggested that LUBAC regulates NF-κB–dependent IL-1β responses differently in different cell types, consistent with the unique association of autoinflammation and immunodeficiency in patients from two kindreds.
Journal ArticleDOI
Efficacy of etanercept for the treatment of juvenile idiopathic arthritis according to the onset type
Pierre Quartier,Pierre Taupin,Franck Bourdeaut,Irène Lemelle,Pascal Pillet,Michel Bost,Jean Sibilia,Isabelle Koné-Paut,Sylvie Gandon-Laloum,Marc LeBideau,Brigitte Bader-Meunier,Richard Mouy,Marianne Debré,Paul Landais,Anne-Marie Prieur +14 more
TL;DR: Although most patients initially respond to etanercept, this initial response is not always followed by sustained improvement over longer periods of time, and the higher rate of treatment failure in the group with systemic-onset JIA indicates that these patients in particular may require alternative treatments.
Journal ArticleDOI
Infusion of Fcγ fragments for treatment of children with acute immune thrombocytopenic purpura
Marianne Debré,M C Bonnet,Wolf H. Fridman,E Carosella,N. Philippe,P Reinert,Etienne Vilmer,C Kaplan,Jean-Luc Teillaud,Claude Griscelli +9 more
TL;DR: The infusion of Fcγ fragments is an efficient treatment of acute ITP in children and strengthens the hypothesis that F cγR blockade is the main mechanism of action of IVIG in ITP, although other immunoregulatory mechanisms triggered by the presence of increased sCD16 concentrations in serum could be involved in the clinical benefit observed.