Alain Fischer

Alain Fischer

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Alain Fischer

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Ttc7a regulates hematopoietic stem cell functions while controlling the stress-induced response.

Haematologica 2020 Jan 19;105(1):59-70. Epub 2019 Apr 19.

Laboratory of Normal and Pathological Homeostasis of the Immune System, INSERM UMR 1163, Imagine Institute, Paris

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http://www.haematologica.org/lookup/doi/10.3324/haematol.201
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http://dx.doi.org/10.3324/haematol.2018.207100DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6939534PMC
January 2020

Gene therapy for severe combined immunodeficiencies and beyond.

J Exp Med 2020 Jan;217(2)

Unité de Technologies Chimiques et Biologiques pour la Santé, UMR8258 Centre National de la Recherche Scientifique - U1267 Institut National de la Santé et de la Recherche Médicale, Faculté de Pharmacie de Paris, Université Paris Descartes, Paris, France.

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http://dx.doi.org/10.1084/jem.20190607DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7041706PMC
January 2020

Antibody-coated microbiota in nasopharynx of healthy individuals and IVIg-treated patients with hypogammaglobulinemia.

J Allergy Clin Immunol 2020 Jan 16. Epub 2020 Jan 16.

Innate Immunity Unit, Institut Pasteur, Paris, France; Institut National de la Santé et de la Recherche Médicale (INSERM) U1223, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.jaci.2020.01.002DOI Listing
January 2020

Concomitant and deficiencies cause chronic active Epstein-Barr virus infection of T cells.

J Exp Med 2019 Dec 19;216(12):2800-2818. Epub 2019 Sep 19.

Laboratory of Lymphocyte Activation and Susceptibility to EBV Infection, Institut National de la Santé et la Recherche Médicale, Unité Mixte de Recherche 1163, Paris, France

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http://dx.doi.org/10.1084/jem.20190678DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6888974PMC
December 2019

Benefit Corporation: a path to affordable gene therapies?

Nat Med 2019 12;25(12):1813-1814

I3h Institute, Université libre de Bruxelles, Brussels, Belgium.

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http://dx.doi.org/10.1038/s41591-019-0676-zDOI Listing
December 2019

Somatic genetic rescue in Mendelian haematopoietic diseases.

Nat Rev Genet 2019 10 11;20(10):582-598. Epub 2019 Jun 11.

Paris Descartes-Sorbonne Paris Cité University, Imagine Institute, Paris, France.

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http://dx.doi.org/10.1038/s41576-019-0139-xDOI Listing
October 2019

A 1-Year Prospective French Nationwide Study of Emergency Hospital Admissions in Children and Adults with Primary Immunodeficiency.

Authors:
Hélène Coignard-Biehler Nizar Mahlaoui Benoit Pilmis Vincent Barlogis Pauline Brosselin Nathalie De Vergnes Marianne Debré Marion Malphettes Pierre Frange Emilie Catherinot Isabelle Pellier Isabelle Durieu Antoinette Perlat Bruno Royer Alain Le Quellec Eric Jeziorski Alain Fischer Olivier Lortholary Laurent Aaron Daniel Adoue Claire Aguilar Nathalie Aladjidi Alexandre Alcais Zahir Amoura Philippe Arlet Corinne Armari-Alla Brigitte Bader-Meunier Sophie Bayart Yves Bertrand Boris Bienvenu Stéphane Blanche Damien Bodet Bernard Bonnotte Raphaël Borie Patrick Boutard Claire Briandet Jean-Paul Brion Jacques Brouard Sarah Cohen-Beaussant Laurence Costes Louis-Jean Couderc Pierre Cougoul Virginie Courteille Geneviève de Saint Basile Catherine Devoldere Anne Deville Jean Donadieu Eric Dore Fabienne Dulieu Christine Edan Natacha Entz-Werle Claire Fieschi Amandine Forestier Fanny Fouyssac Vincent Gajdos Lionel Galicier Virginie Gandemer Martine Gardembas Catherine Gaud Gaelle Guillerm Eric Hachulla Mohamed Hamidou Olivier Hermine Cyrille Hoarau Sébastien Humbert Arnaud Jaccard Serge Jacquot Jean-Philippe Jais Roland Jaussaud Pierre-Yves Jeandel Kamila Kebaili Anne-Sophie Korganow Olivier Lambotte Fanny Lanternier Claire Larroche Anne-Sophie Lascaux Emmanuelle Le Moigne Vincent Le Moing Yvon Lebranchu Marc Lecuit Guillaume Lefevre Richard Lemal Valérie Li Thiao Te Aude Marie-Cardine Nicolas Martin Silva Agathe Masseau Christian Massot Françoise Mazingue Etienne Merlin Gérard Michel Frédéric Millot Béatrice Monlibert Fabrice Monpoux Despina Moshous Luc Mouthon Martine Munzer Bénédicte Neven Raphaëlle Nove-Josserand Eric Oksenhendler Marie Ouachée-Chardin Caroline Oudot Anne Pagnier Jean-Louis Pasquali Marlène Pasquet Yves Perel Capucine Picard Christophe Piguet Dominique Plantaz Johan Provot Pierre Quartier Frédéric Rieux-Laucat Pascal Roblot Pierre-Marie Roger Pierre-Simon Rohrlich Hervé Rubie Valéry Salle Françoise Sarrot-Reynauld Amélie Servettaz Jean-Louis Stephan Nicolas Schleinitz Felipe Suarez Laure Swiader Sophie Taque Caroline Thomas Olivier Tournilhac Caroline Thumerelle François Tron Jean-Pierre Vannier Jean-François Viallard

J Clin Immunol 2019 Oct 10;39(7):702-712. Epub 2019 Aug 10.

Service de Maladies Infectieuses et Tropicales, Centre d'Infectiologie Necker Pasteur, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Université de Paris, Paris, France.

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http://dx.doi.org/10.1007/s10875-019-00658-9DOI Listing
October 2019

Platelets are the Achilles' heel of Wiskott-Aldrich syndrome.

Authors:
Alain Fischer

J Allergy Clin Immunol 2019 Sep 13;144(3):668-670. Epub 2019 Jul 13.

Collège de France, Paris, France; Immunology and Pediatric Hematology Department, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM UMR 1163, Institut Imagine, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.jaci.2019.06.039DOI Listing
September 2019

Signaling pathways involved in the T-cell-mediated immunity against Epstein-Barr virus: Lessons from genetic diseases.

Immunol Rev 2019 09;291(1):174-189

University Paris Descartes Sorbonne Paris Cité, Imagine Institut, Paris, France.

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http://dx.doi.org/10.1111/imr.12791DOI Listing
September 2019

Hematopoietic stem cell transplantation for CD40 ligand deficiency: Results from an EBMT/ESID-IEWP-SCETIDE-PIDTC study.

J Allergy Clin Immunol 2019 Jun 17;143(6):2238-2253. Epub 2019 Jan 17.

Department of Pediatric Immunology and HSCT, Great North Children's Hospital, Newcastle upon Tyne, United Kingdom; Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom.

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https://linkinghub.elsevier.com/retrieve/pii/S00916749193003
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http://dx.doi.org/10.1016/j.jaci.2018.12.1010DOI Listing
June 2019

Lymphoproliferative disease in patients with Wiskott-Aldrich syndrome: Analysis of the French Registry of Primary Immunodeficiencies.

J Allergy Clin Immunol 2019 Jun 20;143(6):2311-2315.e7. Epub 2019 Feb 20.

Clinical Hematology, Necker University Hospital, AP-HP, Paris, France; Paris Descartes University, Sorbonne Paris Cité University, Imagine Institute, Paris, France; INSERM UMR1163 & CNRS URL 8254, Laboratory of cellular and molecular mechanisms of hematological disorders and therapeutical implications, Paris, France; French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker University Hospital, AP-HP, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.jaci.2019.01.046DOI Listing
June 2019

Disease Evolution and Response to Rapamycin in Activated Phosphoinositide 3-Kinase δ Syndrome: The European Society for Immunodeficiencies-Activated Phosphoinositide 3-Kinase δ Syndrome Registry.

Front Immunol 2018 16;9:543. Epub 2018 Mar 16.

Center for Chronic Immunodeficiency, Medical Center - University of Freiburg, Freiburg, Germany.

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http://journal.frontiersin.org/article/10.3389/fimmu.2018.00
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http://dx.doi.org/10.3389/fimmu.2018.00543DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5863269PMC
May 2019

Genetic diagnosis of primary immunodeficiencies: A survey of the French national registry.

J Allergy Clin Immunol 2019 Apr 9;143(4):1646-1649.e10. Epub 2019 Jan 9.

French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Pediatric Immuno-Haematology and Rheumatology Unit, Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Paris Descartes University, Sorbonne Paris Cité, Imagine Institute, INSERM UMR 1163, Paris, France; Collège de France, Paris, France. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S00916749193000
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http://dx.doi.org/10.1016/j.jaci.2018.12.994DOI Listing
April 2019

Intestinal dysbiosis in inflammatory bowel disease associated with primary immunodeficiency.

J Allergy Clin Immunol 2019 02 10;143(2):775-778.e6. Epub 2018 Oct 10.

French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker-Enfants Malades University Hospital, APHP, Paris, France; Paediatric Haematology-Immunology and Rheumatology Department, Hôpital Necker-Enfants Malades, APHP, Paris, France; INSERM UMR 1163, Imagine Institute, Paris, France; Paris Descartes University, Sorbonne Paris Cité, Imagine Institute, Paris, France; Collège de France, Paris, France. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S00916749183142
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http://dx.doi.org/10.1016/j.jaci.2018.09.021DOI Listing
February 2019

RASGRP1 mutation in autoimmune lymphoproliferative syndrome-like disease.

J Allergy Clin Immunol 2018 08 15;142(2):595-604.e16. Epub 2017 Nov 15.

Department of Paediatrics, The University of Hong Kong-Shenzhen Hospital, Shenzhen, China; Department of Paediatrics and Adolescent Medicine, The University of Hong Kong, Hong Kong, China; Shenzhen Engineering Laboratory of Primary Immunodeficiency Diagnosis and Therapy, Shenzhen, China. Electronic address:

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http://dx.doi.org/10.1016/j.jaci.2017.10.026DOI Listing
August 2018

Diagnostic Yield of Next-generation Sequencing in Very Early-onset Inflammatory Bowel Diseases: A Multicentre Study.

J Crohns Colitis 2018 Aug;12(9):1104-1112

INSERM, UMR1163, Laboratory of Intestinal Immunity, and Imagine Institute, Paris, France.

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http://dx.doi.org/10.1093/ecco-jcc/jjy068DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6113703PMC
August 2018

Reply to 'Comment on: Evidence of innate lymphoid cell redundancy in humans'.

Nat Immunol 2018 08;19(8):789-790

APHP, Hôpital Universitaire Necker-Enfants Malades, Centre de Référence Déficits Immunitaires Héréditaires, Paris, France.

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http://dx.doi.org/10.1038/s41590-018-0165-4DOI Listing
August 2018

Kinesin-1 Is a New Actor Involved in Platelet Secretion and Thrombus Stability.

Arterioscler Thromb Vasc Biol 2018 05 8;38(5):1037-1051. Epub 2018 Mar 8.

INSERM, UMR_S 1163, Laboratory of Normal and Pathological Homeostasis of the Immune System, Paris, France (M.K., I.M., A.F., G.d.S.B., G.M.).

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http://dx.doi.org/10.1161/ATVBAHA.117.310373DOI Listing
May 2018

What do primary immunodeficiencies tell us about the essentiality/redundancy of immune responses?

Semin Immunol 2018 04 8;36:13-16. Epub 2017 Dec 8.

INSERM UMR 1163, Institut Imagine, Paris, France.

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http://dx.doi.org/10.1016/j.smim.2017.12.001DOI Listing
April 2018

Burden of Poor Health Conditions and Quality of Life in 656 Children with Primary Immunodeficiency.

J Pediatr 2018 03 6;194:211-217.e5. Epub 2017 Dec 6.

French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Pediatric Immuno-Haematology and Rheumatology Unit, Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM UMR1163, Imagine Institute, Sorbonne Paris Cité, University Paris Descartes, Paris, France; Division of Médecine Expérimentale, Collège de France, Paris, France.

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http://dx.doi.org/10.1016/j.jpeds.2017.10.029DOI Listing
March 2018

Pediatric-onset Evans syndrome: Heterogeneous presentation and high frequency of monogenic disorders including LRBA and CTLA4 mutations.

Clin Immunol 2018 03 10;188:52-57. Epub 2018 Jan 10.

INSERM UMR 1163, Laboratory of Immunogenetics of pediatric autoimmune diseases, Paris, France; Paris Descartes-Sorbonne Paris Cité University, Imagine Institute, Paris, France; Pediatric immuno-hematology and rhumatology unit, RAISE reference centre for pediatric inflammatory rheumatic diseases and systemic autoimmune diseases, Necker-Enfants Malades University Hospital, Assistance publique - Hôpitaux de Paris, France.

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http://dx.doi.org/10.1016/j.clim.2017.12.009DOI Listing
March 2018

Prevalence of primary immunodeficiencies in France is underestimated.

J Allergy Clin Immunol 2017 12 18;140(6):1731-1733. Epub 2017 Jul 18.

French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker-Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Pediatric Immuno-Haematology and Rheumatology Unit, Necker-Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Paris Descartes University, Sorbonne Paris Cité, Imagine Institute, Paris, France; INSERM UMR 1163, Paris, France; Collège de France, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.jaci.2017.06.020DOI Listing
December 2017

Birth of gene therapy

Authors:
Alain Fischer

Rev Prat 2017 11;67(9):1036-1042

Collège de France, Paris, France.

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November 2017

Autoimmune and inflammatory manifestations occur frequently in patients with primary immunodeficiencies.

J Allergy Clin Immunol 2017 Nov 10;140(5):1388-1393.e8. Epub 2017 Feb 10.

Centre de Référence Déficits Immunitaires Héréditaires (CEREDIH), Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France; Unité d'Immuno-Hématologie et Rhumatologie pédiatrique, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France; Paris Descartes-Sorbonne Paris Cité University, Imagine Institute, Paris, France; Laboratory of Human Genetics of Infectious Diseases, INSERM UMR 1163, Paris, France.

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http://dx.doi.org/10.1016/j.jaci.2016.12.978DOI Listing
November 2017

Gene Therapy: From Birth to Maturity Requires Commitment to Science and Ethics.

Authors:
Alain Fischer

Hum Gene Ther 2017 11;28(11):958

1 Collège de France , Paris, France.

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http://dx.doi.org/10.1089/hum.2017.29053.afrDOI Listing
November 2017

Finding patients using similarity measures in a rare diseases-oriented clinical data warehouse: Dr. Warehouse and the needle in the needle stack.

J Biomed Inform 2017 09 25;73:51-61. Epub 2017 Jul 25.

INSERM, Centre de Recherche des Cordeliers, UMR 1138 Equipe 22, Université Paris Descartes, Sorbonne Paris Cité, Paris, France; Département d'informatique médicale, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Sorbonne Paris Cité, France; Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Sorbonne Paris Cité, France.

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http://dx.doi.org/10.1016/j.jbi.2017.07.016DOI Listing
September 2017

Intrinsic antiproliferative activity of the innate sensor STING in T lymphocytes.

J Exp Med 2017 06 8;214(6):1769-1785. Epub 2017 May 8.

Immunity and Cancer Department, Institut Curie, PSL Research University, Institut National de la Santé et de la Recherche Médicale U932, 75005 Paris, France

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http://dx.doi.org/10.1084/jem.20161674DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5461003PMC
June 2017

Life-Threatening Pneumopathy and in a STAT3-Deficient Hyper-IgE Syndrome Patient.

Pediatrics 2017 Jun 17;139(6). Epub 2017 May 17.

Departments of Pediatric Respiratory and Allergy Diseases and.

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http://dx.doi.org/10.1542/peds.2016-0845DOI Listing
June 2017

Mammalian target of rapamycin inhibition counterbalances the inflammatory status of immune cells in patients with chronic granulomatous disease.

J Allergy Clin Immunol 2017 May 1;139(5):1641-1649.e6. Epub 2016 Oct 1.

Biotherapy Department, Necker-Enfants Malades Hospital, Assistance Publique-Hôpitaux de Paris, AP-HP, Paris, France; University Paris Descartes Sorbonne Paris Cité, Institut Imagine, Paris, France; Inserm UMR 1163, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.jaci.2016.08.033DOI Listing
May 2017

Physical health conditions and quality of life in adults with primary immunodeficiency diagnosed during childhood: A French Reference Center for PIDs (CEREDIH) study.

J Allergy Clin Immunol 2017 Apr 30;139(4):1275-1281.e7. Epub 2016 Sep 30.

French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Pediatric Immuno-Haematology and Rheumatology Unit, Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM UMR1163, Imagine Institute, Necker Medical School, Sorbonne Paris Cité, University Paris Descartes, Paris, France; Collège de France, Paris, France.

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http://dx.doi.org/10.1016/j.jaci.2016.08.027DOI Listing
April 2017

Different Immunological Pathways Underlie the Immune Response to Pneumococcal Polysaccharides.

J Clin Immunol 2017 04 16;37(3):277-278. Epub 2017 Mar 16.

Experimental Laboratory Immunology, Department of Microbiology and Immunology, KU Leuven, Leuven, Belgium.

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http://dx.doi.org/10.1007/s10875-017-0380-zDOI Listing
April 2017

Hematopoietic stem cell transplant in patients with activated PI3K delta syndrome.

J Allergy Clin Immunol 2017 03 12;139(3):1046-1049. Epub 2016 Nov 12.

Great North Children's Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom; Primary Immunodeficiency Group, Institute of Cellular Medicine, Newcastle upon Tyne University, Newcastle upon Tyne, United Kingdom.

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http://dx.doi.org/10.1016/j.jaci.2016.09.040DOI Listing
March 2017

Gene therapy: Myth or reality?

Authors:
Alain Fischer

C R Biol 2016 Jul-Aug;339(7-8):314-8. Epub 2016 May 31.

Paris Descartes-Sorbonne Paris Cité University, Imagine Institute, 75015 Paris, France; Immunology and Pediatric Hematology Department, Assistance publique-Hôpitaux de Paris, 75015 Paris, France; Inserm UMR 1163, 75015 Paris, France; Collège de France, 75005 Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.crvi.2016.04.011DOI Listing
February 2017

Clinical spectrum and features of activated phosphoinositide 3-kinase δ syndrome: A large patient cohort study.

J Allergy Clin Immunol 2017 02 16;139(2):597-606.e4. Epub 2016 Jul 16.

Department of Paediatric Immunology, Newcastle upon Tyne hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom; Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne Hospitals NHS Trust, Newcastle upon Tyne, United Kingdom.

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http://dx.doi.org/10.1016/j.jaci.2016.06.021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5292996PMC
February 2017

[The vaccination in danger?]

Authors:
Alain Fischer

Med Sci (Paris) 2017 Feb 27;33(2):119-120. Epub 2017 Feb 27.

Collège de France Institut Imagine-Inserm Hôpital Necker-Enfants Malades 149, rue de Sèvres 75015 Paris, France.

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http://dx.doi.org/10.1051/medsci/20173302001DOI Listing
February 2017

X-linked primary immunodeficiency associated with hemizygous mutations in the moesin (MSN) gene.

J Allergy Clin Immunol 2016 12 4;138(6):1681-1689.e8. Epub 2016 Jun 4.

Laboratory of Human Lymphohematopoiesis, INSERM UMR 1163, Paris, France; Paris Descartes-Sorbonne Paris Cité University, Imagine Institute, Paris, France; Biotherapy Clinical Investigation Center, Necker Children's Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.jaci.2016.04.032DOI Listing
December 2016

Efficacy of the Janus kinase 1/2 inhibitor ruxolitinib in the treatment of vasculopathy associated with TMEM173-activating mutations in 3 children.

J Allergy Clin Immunol 2016 12 20;138(6):1752-1755. Epub 2016 Aug 20.

Pediatric Hematology-Immunology and Rheumatology Department, Hôpital Necker-Enfants Malades, Paris, France; Paris Descartes University, Sorbonne-Paris-Cité, Institut Imagine, Paris, France; INSERM UMR 1163, Laboratory of Immunogenetics of Pediatric Autoimmunity, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.jaci.2016.07.015DOI Listing
December 2016

Primary immunodeficiencies suggest redundancy within the human immune system.

Sci Immunol 2016 Dec;1(6)

Paris Descartes-Sorbonne Paris Cité University, Imagine Institute, Paris, France.

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http://dx.doi.org/10.1126/sciimmunol.aah5861DOI Listing
December 2016

LRBA deficiency with autoimmunity and early onset chronic erosive polyarthritis.

Clin Immunol 2016 07 5;168:88-93. Epub 2016 Apr 5.

INSERM UMR 1163, Laboratory of Immunogenetics of Pediatric autoimmune Diseases, 75015 Paris, France; Paris Descartes-Sorbonne Paris Cité University, Imagine Institute, 75015 Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.clim.2016.03.006DOI Listing
July 2016

Therapeutic effect of JAK1/2 blockade on the manifestations of hemophagocytic lymphohistiocytosis in mice.

Blood 2016 07 24;128(1):60-71. Epub 2016 May 24.

INSERM UMR1163, Laboratory of Normal and Pathological Homeostasis of the Immune System, Paris, France; Paris Descartes University-Sorbonne Paris Cité, Imagine Institute, Paris, France; Centre d'Etudes des Déficits Immunitaires, Assistance Publique-Hôpitaux de Paris, Hôpital Necker, Paris, France.

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http://dx.doi.org/10.1182/blood-2016-02-700013DOI Listing
July 2016

Polygenic mutations in the cytotoxicity pathway increase susceptibility to develop HLH immunopathology in mice.

Blood 2016 04 10;127(17):2113-21. Epub 2016 Feb 10.

INSERM Unité Mixte de Recherche 1163, Laboratory of Normal and Pathological Homeostasis of the Immune System, Paris, France; Paris Descartes University-Sorbonne Paris Cité, Imagine Institute, Paris, France; Centre d'Etudes des Déficits Immunitaires, Assistance Publique-Hôpitaux de Paris, Hôpital Necker, Paris, France.

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http://dx.doi.org/10.1182/blood-2015-12-688960DOI Listing
April 2016

Genetic, Cellular and Clinical Features of ICF Syndrome: a French National Survey.

J Clin Immunol 2016 Feb 6;36(2):149-59. Epub 2016 Feb 6.

Study Center for Primary Immunodeficiencies, Necker-Enfants Malades Hospital, Assistance Publique Hôpitaux de Paris (APHP), University Paris Descartes, 149 rue de Sevres, 75015, Paris, France.

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http://dx.doi.org/10.1007/s10875-016-0240-2DOI Listing
February 2016

SYK expression endows human ZAP70-deficient CD8 T cells with residual TCR signaling.

Clin Immunol 2015 Dec 14;161(2):103-9. Epub 2015 Jul 14.

Laboratory of Lymphocyte Activation and Susceptibility to EBV Infection, INSERM UMR1163, Institut IMAGINE, Paris, France; Paris Descartes University, Sorbonne Paris Cité, Imagine Institut, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.clim.2015.07.002DOI Listing
December 2015

An in vivo genetic reversion highlights the crucial role of Myb-Like, SWIRM, and MPN domains 1 (MYSM1) in human hematopoiesis and lymphocyte differentiation.

J Allergy Clin Immunol 2015 Dec 26;136(6):1619-1626.e5. Epub 2015 Jul 26.

INSERM UMR 1163, Laboratory of Genome Dynamics in the Immune System, Paris, France; Paris Descartes-Sorbonne Paris Cité University, Imagine Institute, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.jaci.2015.06.008DOI Listing
December 2015

A neuropathological study of cerebrovascular abnormalities in a signal transducer and activator of transcription 3-deficient patient.

J Allergy Clin Immunol 2015 Nov 30;136(5):1418-21.e1-5. Epub 2015 Jun 30.

Sorbonne Paris Cité, Paris Descartes University, Imagine Institute, Paris, France; Centre de référence des déficits immunitaires héréditaires (CEREDIH), Necker Children's Hospital, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France; Study Centre for Primary Immunodeficiencies, Necker Children's Hospital, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France; Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR 1163, IMAGINE Institute, Paris, France.

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http://dx.doi.org/10.1016/j.jaci.2015.05.021DOI Listing
November 2015

Cytotoxic granule secretion by lymphocytes and its link to immune homeostasis.

F1000Res 2015 30;4(F1000 Faculty Rev):930. Epub 2015 Sep 30.

INSERM UMR1163, Laboratory of Normal and Pathological Homeostasis of the Immune System, Paris, F-75015, France ; Paris Descartes University-Sorbonne Paris Cité, Imagine Institute, Paris, F-75015, France ; Immunology and Pediatric Hematology Department, Necker Children's Hospital, AP-HP, Paris, France ; Collège de France, Paris, F-75005, France.

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http://dx.doi.org/10.12688/f1000research.6754.1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4648190PMC
November 2015

Severe combined immunodeficiencies and related disorders.

Nat Rev Dis Primers 2015 10 29;1:15061. Epub 2015 Oct 29.

Division of Allergy, Immunology and Blood and Marrow Transplantation, Department of Pediatrics, University of California at San Francisco, San Francisco, California, USA.

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http://dx.doi.org/10.1038/nrdp.2015.61DOI Listing
October 2015

Early-onset hypogammaglobulinemia: A survey of 44 patients.

J Allergy Clin Immunol 2015 Oct 8;136(4):1097-9.e2. Epub 2015 May 8.

INSERM UMR 1163, Paris, France; Paris Descartes University - Sorbonne Paris Cité, Imagine Institute, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.jaci.2015.03.038DOI Listing
October 2015

A novel hypomorphic mutation in STIM1 results in a late-onset immunodeficiency.

J Allergy Clin Immunol 2015 Sep 30;136(3):816-819.e4. Epub 2015 Apr 30.

Paris Descartes University - Sorbonne Paris Cité, Paris, France; Study Center for Immunodeficiency, Necker-Enfants Malades Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR 1163, Institut IMAGINE, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.jaci.2015.03.009DOI Listing
September 2015

Hepatitis E virus in hematopoietic stem cell donors: Towards a systematic HEV screening of donors?

J Infect 2015 Jul 26;71(1):141-4. Epub 2015 Feb 26.

Unité d'Immunologie, Hématologie et Rhumatologie pédiatriques, AP-HP, Hôpital Necker - Enfants malades, Paris, France; Institut Imagine, Université Paris Descartes, Sorbonne Paris Cité, Paris, France; EA08, Université Paris Descartes, Sorbonne Paris Cité, Paris, France.

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http://dx.doi.org/10.1016/j.jinf.2015.02.008DOI Listing
July 2015

Recent advances in understanding the pathophysiology of primary T cell immunodeficiencies.

Authors:
Alain Fischer

Trends Mol Med 2015 Jul 4;21(7):408-16. Epub 2015 May 4.

Paris Descartes-Sorbonne Paris Cité University, Imagine Institute, Paris, France; Immunology and Pediatric Hematology Department, Assistance Publique-Hôpitaux de Paris, Paris, France; Institut National de la Santé et de la Recherche Médicale (INSERM) Unité Mixte de Recherche 1163, Paris, France; Collège de France, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.molmed.2015.04.002DOI Listing
July 2015

Pulmonary manifestations in adult patients with chronic granulomatous disease.

Eur Respir J 2015 Jun 22;45(6):1613-23. Epub 2015 Jan 22.

Service de Pneumologie, Hôpital Foch, Suresnes, France UPRES EA 220, Suresnes, France Faculté des Sciences de la Santé Simone Veil, Université Versailles-Saint-Quentin-en-Yvelines, Versailles, France.

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http://dx.doi.org/10.1183/09031936.00118414DOI Listing
June 2015

Systematic neonatal screening for severe combined immunodeficiency and severe T-cell lymphopenia: Analysis of cost-effectiveness based on French real field data.

J Allergy Clin Immunol 2015 Jun 1;135(6):1589-93. Epub 2015 Apr 1.

URC Eco (Clinical Research Unit in Health Economics), Assistance Publique-Hôpitaux de Paris, Hôtel Dieu Hospital, Paris, France; Public Health Unit, Henri Mondor Hospital, Assistance Publique-Hôpitaux de Paris, INSERM UMR 1123, Créteil, France. Electronic address:

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http://dx.doi.org/10.1016/j.jaci.2015.02.004DOI Listing
June 2015

Faster T-cell development following gene therapy compared with haploidentical HSCT in the treatment of SCID-X1.

Blood 2015 Jun 13;125(23):3563-9. Epub 2015 Apr 13.

Département de Biothérapie, Centre d'Investigation Clinique intégré en Biothérapies, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Institut Imagine, Paris, France; INSERM UMR1163, Paris, France;

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http://dx.doi.org/10.1182/blood-2014-12-616003DOI Listing
June 2015

A novel immunoregulatory role for NK-cell cytotoxicity in protection from HLH-like immunopathology in mice.

Blood 2015 Feb 18;125(9):1427-34. Epub 2014 Dec 18.

Laboratory of Normal and Pathological Homeostasis of the Immune System, INSERM Unité Mixte de Recherche 1163, Paris France; Imagine Institute, Paris Descartes University-Sorbonne Paris Cité, Paris France; Centre d'Etudes des Déficits Immunitaires, Assistance Publique-Hôpitaux de Paris, Hôpital Necker, Paris, France.

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http://www.bloodjournal.org/content/early/2014/12/17/blood-2
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http://www.bloodjournal.org/content/bloodjournal/125/9/1427.
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http://www.bloodjournal.org/cgi/doi/10.1182/blood-2014-09-60
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http://dx.doi.org/10.1182/blood-2014-09-602946DOI Listing
February 2015