Benedicte Neven

Benedicte Neven

UNVERIFIED PROFILE

Are you Benedicte Neven?   Register this Author

Register author
Benedicte Neven

Benedicte Neven

Publications by authors named "Benedicte Neven"

Are you Benedicte Neven?   Register this Author

100Publications

3691Reads

12Profile Views

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.

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S00916749193003
Publisher Site
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:

View Article

Download full-text PDF

Source
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.

View Article

Download full-text PDF

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

Hematopoietic Stem Cell Transplant for the Treatment of X-MAID.

Front Pediatr 2019 14;7:170. Epub 2019 May 14.

Division of Allergy and Immunology, The Children's Hospital of Philadelphia, Philadelphia, PA, United States.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3389/fped.2019.00170DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6527778PMC
May 2019

Efficacy of JAK1/2 inhibition in the treatment of chilblain lupus due to TREX1 deficiency.

Ann Rheum Dis 2019 Mar 3;78(3):431-433. Epub 2018 Oct 3.

Department of Paediatric Haematology-Immunology and Rheumatology, Hôpital Necker-Enfants Malades, AP-HP, Paris, France

View Article

Download full-text PDF

Source
http://ard.bmj.com/lookup/doi/10.1136/annrheumdis-2018-21403
Publisher Site
http://dx.doi.org/10.1136/annrheumdis-2018-214037DOI Listing
March 2019

Outcomes and Treatment Strategies for Autoimmunity and Hyperinflammation in Patients with RAG Deficiency.

J Allergy Clin Immunol Pract 2019 Mar 12. Epub 2019 Mar 12.

University of South Florida and Johns Hopkins All Children's Hospital, Saint Petersburg, Fla; Division of Allergy and Immunology, Massachusetts General Hospital for Children, Boston, Mass. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaip.2019.02.038DOI Listing
March 2019

Intestinal dysbiosis in inflammatory bowel disease associated with primary immunodeficiency.

J Allergy Clin Immunol 2019 Feb 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:

View Article

Download full-text PDF

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

Outcome of domino hematopoietic stem cell transplantation in human subjects: An international case series.

J Allergy Clin Immunol 2018 Nov 5;142(5):1628-1631.e4. Epub 2018 Jul 5.

Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands; Department of Pediatric Hematology and Stem Cell Transplantation, Wilhelmina Children's Hospital, Utrecht, The Netherlands. Electronic address:

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S00916749183099
Publisher Site
http://dx.doi.org/10.1016/j.jaci.2018.06.030DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6226332PMC
November 2018

JAK 1/2 Blockade in MDA5 Gain-of-Function.

J Clin Immunol 2018 Nov 15;38(8):844-846. Epub 2018 Nov 15.

Centre for Genomic and Experimental Medicine, MRC Institute of Genetics and Molecular Medicine, The University of Edinburgh, Edinburgh, UK.

View Article

Download full-text PDF

Source
http://link.springer.com/10.1007/s10875-018-0563-2
Publisher Site
http://dx.doi.org/10.1007/s10875-018-0563-2DOI Listing
November 2018

Model of population pharmacokinetics of cidofovir in immunocompromised children with cytomegalovirus and adenovirus infection.

J Antimicrob Chemother 2018 Sep;73(9):2422-2429

Unité d'Immunologie, Hématologie et Rhumatologie pédiatriques, AP-HP, Hôpital universitaire Necker - Enfants malades, Paris, France.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/jac/dky192DOI Listing
September 2018

Towards a new set of classification criteria for PFAPA syndrome.

Pediatr Rheumatol Online J 2018 Sep 21;16(1):60. Epub 2018 Sep 21.

Unité Romande d'Immuno-rhumatologie Pédiatrique, CHUV, University of Lausanne, Lausanne and HUG, Geneva, Switzerland.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12969-018-0277-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6151014PMC
September 2018

Epithelial barrier dysfunction in desmoglein-1 deficiency.

J Allergy Clin Immunol 2018 Aug 27;142(2):702-706.e7. Epub 2018 Apr 27.

Laboratory of Genetics of Monogenic Auto-inflammatory Diseases, Necker Branch, U1163, Necker-Enfants Malades Hospital (AP-HP), Paris Descartes-Sorbonne Paris Cité University, Imagine Institute, Paris, France. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaci.2018.04.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6078820PMC
August 2018

The Autoimmune Lymphoproliferative Syndrome with Defective FAS or FAS-Ligand Functions.

J Clin Immunol 2018 Jul 17;38(5):558-568. Epub 2018 Jun 17.

Laboratory of Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163-Institut Imagine, 24 boulevard du Montparnasse, 75015, Paris, France.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s10875-018-0523-xDOI Listing
July 2018

A case of reversible pulmonary arterial hypertension associated with incontinentia pigmenti.

Pulm Circ 2018 Oct-Dec;8(4):2045894018793983. Epub 2018 Jul 23.

1 Department of Pediatric Cardiology, Necker Enfants Malades Hospital, Paris, France.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/2045894018793983DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6131310PMC
July 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.

View Article

Download full-text PDF

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

Long-term follow-up of IPEX syndrome patients after different therapeutic strategies: An international multicenter retrospective study.

J Allergy Clin Immunol 2018 Mar 11;141(3):1036-1049.e5. Epub 2017 Dec 11.

Division of Stem Cell Transplantation and Regenerative Medicine, Department of Pediatrics, Stanford University School of Medicine, Stanford, Calif. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaci.2017.10.041DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6050203PMC
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.

View Article

Download full-text PDF

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

Abnormalities of T-cell receptor repertoire in CD4 regulatory and conventional T cells in patients with RAG mutations: Implications for autoimmunity.

J Allergy Clin Immunol 2017 12 31;140(6):1739-1743.e7. Epub 2017 Aug 31.

Laboratory of Clinical Immunology and Microbiology, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaci.2017.08.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5911433PMC
December 2017

Corrigendum: Natural Killer Cells from Patients with Recombinase-Activating Gene and Non-Homologous End Joining Gene Defects Comprise a Higher Frequency of CD56 NKG2A Cells, and Yet Display Increased Degranulation and Higher Perforin Content.

Front Immunol 2017 10;8:1244. Epub 2017 Oct 10.

Laboratory of Host Defenses, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United States.

View Article

Download full-text PDF

Source
http://journal.frontiersin.org/article/10.3389/fimmu.2017.01
Publisher Site
http://dx.doi.org/10.3389/fimmu.2017.01244DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5641412PMC
October 2017

Brief Report: Blockade of TANK-Binding Kinase 1/IKKɛ Inhibits Mutant Stimulator of Interferon Genes (STING)-Mediated Inflammatory Responses in Human Peripheral Blood Mononuclear Cells.

Arthritis Rheumatol 2017 07 5;69(7):1495-1501. Epub 2017 Jun 5.

Paris Descartes University, Sorbonne-Paris-Cité, Institut Imagine and INSERM UMR 1163, Laboratory of Neurogenetics and Neuroinflammation, Paris, France.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/art.40122DOI Listing
July 2017

Natural Killer Cells from Patients with Recombinase-Activating Gene and Non-Homologous End Joining Gene Defects Comprise a Higher Frequency of CD56 NKG2A Cells, and Yet Display Increased Degranulation and Higher Perforin Content.

Front Immunol 2017 17;8:798. Epub 2017 Jul 17.

Laboratory of Host Defenses, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United States.

View Article

Download full-text PDF

Source
http://journal.frontiersin.org/article/10.3389/fimmu.2017.00
Publisher Site
http://dx.doi.org/10.3389/fimmu.2017.00798DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5511964PMC
July 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

View Article

Download full-text PDF

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

View Article

Download full-text PDF

Source
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.

View Article

Download full-text PDF

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

Array-CGH predicts prognosis in plasma cell post-transplantation lymphoproliferative disorders.

Genes Chromosomes Cancer 2017 03 1;56(3):221-230. Epub 2016 Nov 1.

Service de Pathologie, Hôpital Necker - Enfants Malades, Assistance Publique - Hôpitaux de Paris (AP-HP), Université Paris Descartes Sorbonne Cité, Paris, France.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/gcc.22428DOI Listing
March 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.

View Article

Download full-text PDF

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

Gene Therapy in a Patient with Sickle Cell Disease.

N Engl J Med 2017 03;376(9):848-855

From the Departments of Biotherapy (J.-A.R., A.M., E.M., L.C., M.C.), Clinical Pharmacy (P. Bourget), Pediatric Neuroradiology (D.G.), General Pediatrics (M.M.), and Pediatric Immunology-Hematology Unit (B.N., S.B.), Necker Children's Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Biotherapy Clinical Investigation Center, Groupe Hospitalier Universitaire Ouest, AP-HP, INSERM (J.-A.R., A.M., E.M., L.C., L.W., M.C.), Unité de Technologies Chimiques et Biologiques pour la Santé, Centre National de la Recherche Scientifique Unité Mixte de Recherche 8258, INSERM Unité 1022, Faculté de Pharmacie de Paris, Université Paris Descartes, Chimie ParisTech (S.H.-B.-A.), Immunology Laboratory, Groupe Hospitalier Universitaire Paris-Sud, Hôpital Kremlin-Bicêtre, AP-HP, Le Kremlin-Bicêtre (S.H.-B.-A.), the Institute of Emerging Diseases and Innovative Therapies, Imagine Institute, Université Paris Descartes, Sorbonne Paris Cité University (M.S., B.N., L.W., M.C.), Mère-Enfant Clinical Investigation Center, Groupe Hospitalier Necker Cochin (M.S.), Université Paris Diderot, Sorbonne Paris Cité University, INSERM Institut National de Transfusion Sanguine, Unité Biologie Intégrée du Globule Rouge, Laboratoire d'Excellence GR-Ex (W.E.N.), and Laboratoires de Virologie, Hôpital Cochin (J.-F.M.), Paris, Atomic and Alternative Energy Commission, Université Paris-Sud, Fontenay-aux-Roses (E.P., Y.B., S.C., P.L.), Institut Mondor de Recherche Biomédicale, Equipe 2, Centre de Référence des Syndromes Drépanocytaires Majeurs, Centre Hospitalier Universitaire Henri Mondor, AP-HP, Laboratoire d'Excellence GR-Ex, Créteil (P. Bartolucci), and Université Paris Diderot, Sorbonne Paris Cité University, INSERM Unité 1149, Hôpital Louis-Mourier, AP-HP, Laboratoire d'Excellence GR-Ex, Colombes (H.P., T.L.) - all in France; Bluebird Bio, Cambridge (R.W.R., O.N., G.V., L.S., S.S.), and Brigham and Women's Hospital and Harvard Medical School, Boston (P.L.) - both in Massachusetts; and Ramathibodi Hospital, Mahidol University, Bangkok, Thailand (P.L.).

View Article

Download full-text PDF

Source
http://www.nejm.org/doi/10.1056/NEJMoa1609677
Publisher Site
http://dx.doi.org/10.1056/NEJMoa1609677DOI Listing
March 2017

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:

View Article

Download full-text PDF

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

JAK inhibition in STING-associated interferonopathy.

Ann Rheum Dis 2016 12 12;75(12):e75. Epub 2016 Oct 12.

Laboratory of Neurogenetics and Neuroinflammation, INSERM UMR1163, Institut Imagine, Paris, France.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1136/annrheumdis-2016-210504DOI Listing
December 2016

Specific T cells for the treatment of cytomegalovirus and/or adenovirus in the context of hematopoietic stem cell transplantation.

J Allergy Clin Immunol 2016 09 30;138(3):920-924.e3. Epub 2016 Apr 30.

Biotherapy Department, Necker Children's Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Immunology and Pediatric Hematology Department, Necker Children's Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Human Lymphohematopoiesis-INSERM UMR1163, Paris Descartes-Sorbonne Paris Cité University, Imagine Institute, Paris, France. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaci.2016.03.032DOI Listing
September 2016

Prospective Evaluation of Serum β-Glucan Testing in Patients With Probable or Proven Fungal Diseases.

Open Forum Infect Dis 2016 Sep 16;3(3):ofw128. Epub 2016 Jun 16.

Unitéde Parasitologie-Mycologie, Service de Microbiologie Clinique; Université Paris Descartes, Sorbonne Paris-Cité,; Unité Biologie et Pathogénicité Fongiques, Département de Mycologie, Institut Pasteur, Paris; Institut National de la Recherche Agronomique, Unité Sous Contrat 2019, Paris, France.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/ofid/ofw128DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4942764PMC
September 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:

View Article

Download full-text PDF

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

Is colchicine an effective treatment in periodic fever, aphtous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome?

Joint Bone Spine 2016 Jul 7;83(4):406-11. Epub 2016 Apr 7.

Service de rhumatologie pédiatrie, CHU Kremlin Bicêtre, AP-HP, 78, rue du Général-Leclerc, 94270 Le Kremlin-Bicêtre, France; Centre de référence des maladies auto-inflammatoires (MAI), 94270 Le Kremlin-Bicêtre, France.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jbspin.2015.08.017DOI Listing
July 2016

Evaluation of antithymocyte globulin pharmacokinetics and pharmacodynamics in children.

J Allergy Clin Immunol 2016 Jan 17;137(1):306-309.e4. Epub 2015 Jul 17.

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

View Article

Download full-text PDF

Source
http://search.proquest.com/openview/ca42ac96763a00e67aac143f
Web Search
http://linkinghub.elsevier.com/retrieve/pii/S009167491500790
Publisher Site
http://dx.doi.org/10.1016/j.jaci.2015.06.003DOI Listing
January 2016

Performance of Different Diagnostic Criteria for Familial Mediterranean Fever in Children with Periodic Fevers: Results from a Multicenter International Registry.

J Rheumatol 2016 Jan 15;43(1):154-60. Epub 2015 Nov 15.

From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3899/jrheum.141249DOI Listing
January 2016

Obstructive sleep apnea syndrome after hematopoietic stem cell transplantation in children with mucopolysaccharidosis type I.

Mol Genet Metab 2015 Dec 22;116(4):275-80. Epub 2015 Oct 22.

Pediatric Noninvasive Ventilation and Sleep Unit, AP-HP, Hôpital Necker Enfants-Malades, Paris, France; Paris Descartes University, Paris, France; Inserm U 955, Team 13, Créteil University, Paris XII, Créteil, France. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ymgme.2015.10.004DOI Listing
December 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.

View Article

Download full-text PDF

Source
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:

View Article

Download full-text PDF

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

Use of Eculizumab in Patients With Allogeneic Stem Cell Transplant-Associated Thrombotic Microangiopathy: A Study From the SFGM-TC.

Transplantation 2015 Sep;99(9):1953-9

1 Hématologie Greffe de Moelle, Hôpital Saint Louis, APHP, Université Paris Diderot, Paris, France. 2 Service d'Hématologie Pédiatrique, Hôpital de la Timone, Marseille, France. 3 Service d'Onco-hematologie Pédiatrique, Hôpital Arnaud de Villeneuve, Centre Hospitalier Universitaire de Montpellier, Montpellier, France. 4 Service d'Hématologie, CHU de Purpan, Toulouse, France. 5 Département de Néphrologie et Transplantation d'organe, CHU de Rangueil, Toulouse, France. 6 Service d'Hématologie, Hôpital de la Pitié Salpetrière, APHP, Université Pierre et Marie Curie, Paris. 7 Service de Thérapie Cellulaire et d'Hématologie Clinique, Hôpital d'Estaing, CHU Clermont Ferrand, Université d'Auvergne, Clermont-Ferrand, France. 8 Service d'Immuno-hématologie et Rhumatologie pédiatrique, Hôpital Necker-Enfants Malades, Laboratoire INSERM U768, Institut Imagine, Université Sorbonne Paris-cité, Paris, France. 9 Centre de Néphrologie et Transplantation Rénale, Hôpital de la Conception, APHM, Université d'Aix Marseille, Marseille, France. 10 Service d'Hématologie, Hôpital Hautepierre, Hôpitaux Universitaire de Strasbourg, Strasbourg, France. 11 Département de Biostatistiques, Hôpital Saint Louis, APHP, Université Paris Diderot, Paris, France. 12 Service d'Immunologie Biologique, Hôpital Européen George Pompidou, APHP, CHU Paris Ouest, Paris, France. 13 Service d'Hématologie Biologique, Hôpital Antoine Beclère, APHP, Université Paris Sud, Clamart, France. 14 Service d'Hématologie, Centre de Référence des Microangiopathies Thrombotiques, Hôpitaux Universitaires de l'Est Parisien, APHP, Université Pierre et Marie Curie, Paris, France.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/TP.0000000000000601DOI 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.

View Article

Download full-text PDF

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

View Article

Download full-text PDF

Source
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;

View Article

Download full-text PDF

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

CD45RA depletion in HLA-mismatched allogeneic hematopoietic stem cell transplantation for primary combined immunodeficiency: A preliminary study.

J Allergy Clin Immunol 2015 May 3;135(5):1303-9.e1-3. Epub 2014 Oct 3.

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

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaci.2014.08.019DOI Listing
May 2015

Stem cell transplantation for primary immunodeficiencies: the European experience.

Curr Opin Allergy Clin Immunol 2014 Dec;14(6):516-20

aBiotherapy Department, Necker Children's Hospital, Assistance Publique-Hôpitaux de Paris bBiotherapy Clinical Investigation Center, Groupe Hospitalier Universitaire Ouest, Assistance Publique-Hôpitaux de Paris, INSERM cParis Descartes - Sorbonne Paris Cité University, Imagine Institute dINSERM UMR 1163, Laboratory of Human Lymphohematopoiesis eImmunology and Pediatric Hematology Department, Assistance Publique-Hôpitaux de Paris fCollège de France, Paris, France.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/ACI.0000000000000119DOI Listing
December 2014

Circulating endothelial cells as markers of endothelial dysfunction during hematopoietic stem cell transplantation for pediatric primary immunodeficiency.

J Allergy Clin Immunol 2014 Nov 16;134(5):1203-6. Epub 2014 Jul 16.

Service d'Hématologie Biologique, Hôpital George Pompidou, Assistance Publique-Hôpitaux de Paris, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France.

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S00916749140080
Publisher Site
http://dx.doi.org/10.1016/j.jaci.2014.05.039DOI Listing
November 2014

A modified γ-retrovirus vector for X-linked severe combined immunodeficiency.

N Engl J Med 2014 Oct;371(15):1407-17

From the Departments of Biotherapy (S.H.-B.-A., J. Blondeau, L.C., F.T., M.C.) and Immunology and Pediatric Hematology (S.B., G.C., D.M., B.N., C.P., F.T., A.F.) and the Centre d'Étude des Déficits Immunitaires (C.P.), Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris (AP-HP), the Biotherapy Clinical Investigation Center, Groupe Hospitalier Universitaire Ouest, AP-HP, INSERM (S.H.-B.-A., J. Blondeau, L.C., F.T., M.C.), Unité de Technologies Chimiques et Biologiques pour la Santé, Centre National de la Recherche Scientifique, 8258-INSERM Unité 1022, Faculté des Sciences Pharmaceutiques et Biologiques, Université Paris Descartes (S.H.-B.-A.), Immunology Laboratory, Groupe Hospitalier Universitaire Paris-Sud, AP-HP, Le Kremlin-Bicêtre (S.H.-B.-A.), Imagine Institute, Paris Descartes-Sorbonne Paris Cité University (S.B., J. Blondeau, L.C., D.M., B.N., C.P., E.S., A.F., M.C.), INSERM Unités Mixtes de Recherche 1163, Laboratory of Human Lymphohematopoiesis (J. Blondeau, L.C., E.S., F.T., A.F., M.C.), Groupe Immunoscope, Immunology Department, Institut Pasteur (A.L.), and Collège de France (A.F.) - all in Paris; Division of Hematology-Oncology (S.-Y.P., H.B., D.G., C.E.H., G.H., L.E.L., W.B.L., D.A.W.) and Division of Immunology (L.D.N.), Boston Children's Hospital, Department of Pediatric Oncology, Dana-Farber Cancer Institute (S.-Y.P., D.G., L.E.L., W.B.L., D.A.W.), Harvard Medical School (S.-Y.P., M.A., L.E.L., W.B.L., J.R., L.E.S., A.T., L.D.N., D.A.W.), Center for Human Cell Therapy, Program in Cellular and Molecular Medicine, Boston Children's Hospital (M.A., J.R., L.E.S., A.T.), Division of Hematologic Malignancies, Dana-Farber Cancer Institute (J.R.), and the Manton Center for Orphan Disease Research (L.D.N.) - all in Boston; Great Ormond Street Hospital for Children NHS Foundation Trust (H.B.G., J.X.-B., A.J.T.) and Section of Molecular and Cellular Immunology, University College London Institute of Child Health (H.B.G., K.F.B., A.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1056/NEJMoa1404588DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4274995PMC
October 2014

Granulomatous inflammation: The overlap of immune deficiency and inflammation.

Best Pract Res Clin Rheumatol 2014 Apr;28(2):191-212

Nemours/Alfred I. duPont Hospital for Children, 1600 Rockland Road, Wilmington, DE 19803-3607, USA.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.berh.2014.03.006DOI Listing
April 2014

RAS-associated lymphoproliferative disease evolves into severe juvenile myelo-monocytic leukemia.

Blood 2014 Mar;123(12):1960-3

INSERM UMR 1163, Laboratory of Immunogenetics of Pediatric Autoimmune Diseases, Paris, France Paris Descartes-Sorbonne Paris Cité University, Imagine Institute, Paris, France.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1182/blood-2014-01-548958DOI Listing
March 2014

Temporal and spatial compartmentalization of drug-resistant cytomegalovirus (CMV) in a child with CMV meningoencephalitis: implications for sampling in molecular diagnosis.

J Clin Microbiol 2013 Dec 9;51(12):4266-9. Epub 2013 Oct 9.

Unité d'immunologie, hématologie et rhumatologie pédiatrique, Assistance publique-Hôpitaux de Paris (AP-HP), Hôpital Necker-Enfants malades, Paris, France.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1128/JCM.02411-13DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3838023PMC
December 2013

Phenotypic characterization of very early-onset IBD due to mutations in the IL10, IL10 receptor alpha or beta gene: a survey of the Genius Working Group.

Inflamm Bowel Dis 2013 Dec;19(13):2820-8

*Université Paris Descartes, Sorbonne Paris Cité, Institut Imagine, Paris, France; †Assistance Publique-Hôpitaux de Paris, Hôpital Necker-Enfants Malades, Service de Gastroentérologie pédiatrique, Paris, France; ‡Department of Pediatric Gastroenterology, ErasmusMC-Sophia Children's Hospital, Rotterdam, the Netherlands; §Department of Gastroenterology, Great Ormond Street Hospital for Sick Children and Institute of Child Health, London, United Kingdom; ‖Pediatric Gastroenterology Unit, Centro Hospitalar do Porto, Porto, Portugal; ¶Marienhospital, Bonn, Germany; **Department of Gastroenterology, Hepatology and Feeding Disorders, The Children's Memorial Health Institute, Warsaw, Poland; ††Gastroenterologia, Endoscopia Digestiva, Epatologia e Cura del Bambino con Trapianto di Fegato, Dipartimento Salute della Donna e del Bambino, Padova, Italy; ‡‡Assistance Publique-Hôpitaux de Paris, Hôpital Necker-Enfants Malades, Service d'anatomopathologie, Paris, France; §§Assistance Publique-Hôpitaux de Paris, Hôpital Necker-Enfants Malades, Service de radiologie, Paris, France; ‖‖INSERM U989, Paris, France; and ¶¶INSERM U768, Paris, France.

View Article

Download full-text PDF

Source
http://pdfs.journals.lww.com/ibdjournal/2013/12000/Phenotypi
Web Search
http://content.wkhealth.com/linkback/openurl?sid=WKPTLP:land
Publisher Site
http://dx.doi.org/10.1097/01.MIB.0000435439.22484.d3DOI Listing
December 2013