Benedicte Neven

Summary

Country: France

Publications

  1. doi request reprint A survey of 90 patients with autoimmune lymphoproliferative syndrome related to TNFRSF6 mutation
    Benedicte Neven
    Inserm U768, Hopital Necker Enfants Malades, Paris, France
    Blood 118:4798-807. 2011
  2. doi request reprint Long-term efficacy of the interleukin-1 receptor antagonist anakinra in ten patients with neonatal-onset multisystem inflammatory disease/chronic infantile neurologic, cutaneous, articular syndrome
    Benedicte Neven
    Assistance Publique Hopitaux de Paris, Hopital Necker Enfants Malades, Inserm U768, Universite Rene Descartes Paris 5, Paris, France
    Arthritis Rheum 62:258-67. 2010
  3. ncbi request reprint [International classification of primary immunologic deficiencies]
    Benedicte Neven
    Service d Immuno Hématologie Pédiatrique, Hopital Necker Enfants Malades, 75743 Paris 15
    Rev Prat 57:1646-51. 2007
  4. doi request reprint Cryopyrinopathies: update on pathogenesis and treatment
    Benedicte Neven
    Hopital Necker Enfants Malades, Paris, France
    Nat Clin Pract Rheumatol 4:481-9. 2008
  5. pmc HIV-1 activates Cdc42 and induces membrane extensions in immature dendritic cells to facilitate cell-to-cell virus propagation
    Damjan S Nikolic
    Department of Dermatology and Venereology, Microbiology and Molecular Medicine, University Hospitals and Medical School of Geneva, Geneva, Switzerland
    Blood 118:4841-52. 2011
  6. ncbi request reprint Human leucocyte antigen-identical haematopoietic stem cell transplantation in major histocompatiblity complex class II immunodeficiency: reduced survival correlates with an increased incidence of acute graft-versus-host disease and pre-existing viral infe
    Raffaele Renella
    Unité d Immunologie et Hématologie Pédiatrique, Faculté de Médecine Université Réné Descartes, Paris, France
    Br J Haematol 134:510-6. 2006
  7. ncbi request reprint Hematopoietic stem cell transplantation in hemophagocytic lymphohistiocytosis: a single-center report of 48 patients
    Marie Ouachée-Chardin
    Department of Pediatric Immuno Hematology, Necker Enfants Malades Hospital, Paris, France
    Pediatrics 117:e743-50. 2006
  8. ncbi request reprint Hematopoietic stem cell transplantation in Griscelli syndrome type 2: a single-center report on 10 patients
    Jana Pachlopnik Schmid
    INSERM, Unité U768, Laboratoire du Développement Normal et Pathologique du Système Immunitaire, Paris, France
    Blood 114:211-8. 2009
  9. doi request reprint Long-term outcome after hematopoietic stem cell transplantation of a single-center cohort of 90 patients with severe combined immunodeficiency
    Benedicte Neven
    Unité d Immuno Hématologie et Rhumatologie Pédiatrique, Hopital Necker Enfants Malades, Assistance Publique Hopitaux de Paris, Paris, France
    Blood 113:4114-24. 2009
  10. doi request reprint FAS-L, IL-10, and double-negative CD4- CD8- TCR alpha/beta+ T cells are reliable markers of autoimmune lymphoproliferative syndrome (ALPS) associated with FAS loss of function
    Aude Magerus-Chatinet
    Inserm U768, Paris, France
    Blood 113:3027-30. 2009

Detail Information

Publications28

  1. doi request reprint A survey of 90 patients with autoimmune lymphoproliferative syndrome related to TNFRSF6 mutation
    Benedicte Neven
    Inserm U768, Hopital Necker Enfants Malades, Paris, France
    Blood 118:4798-807. 2011
    ..We also noted a significantly greater occurrence of disease-related symptoms in male than in female patients...
  2. doi request reprint Long-term efficacy of the interleukin-1 receptor antagonist anakinra in ten patients with neonatal-onset multisystem inflammatory disease/chronic infantile neurologic, cutaneous, articular syndrome
    Benedicte Neven
    Assistance Publique Hopitaux de Paris, Hopital Necker Enfants Malades, Inserm U768, Universite Rene Descartes Paris 5, Paris, France
    Arthritis Rheum 62:258-67. 2010
    ..This study was undertaken to assess the long-term efficacy and safety of anakinra treatment in patients with NOMID/CINCA syndrome...
  3. ncbi request reprint [International classification of primary immunologic deficiencies]
    Benedicte Neven
    Service d Immuno Hématologie Pédiatrique, Hopital Necker Enfants Malades, 75743 Paris 15
    Rev Prat 57:1646-51. 2007
  4. doi request reprint Cryopyrinopathies: update on pathogenesis and treatment
    Benedicte Neven
    Hopital Necker Enfants Malades, Paris, France
    Nat Clin Pract Rheumatol 4:481-9. 2008
    ..The development of therapeutic options for these cryopyrinopathies illustrates effective translation of basic science to clinical practice and the convergence of human genetics and targeted therapies...
  5. pmc HIV-1 activates Cdc42 and induces membrane extensions in immature dendritic cells to facilitate cell-to-cell virus propagation
    Damjan S Nikolic
    Department of Dermatology and Venereology, Microbiology and Molecular Medicine, University Hospitals and Medical School of Geneva, Geneva, Switzerland
    Blood 118:4841-52. 2011
    ..Our results demonstrate that HIV-1 binding on immature dendritic cells enhances the formation of membrane extensions that facilitate HIV-1 transfer to CD4(+) T lymphocytes...
  6. ncbi request reprint Human leucocyte antigen-identical haematopoietic stem cell transplantation in major histocompatiblity complex class II immunodeficiency: reduced survival correlates with an increased incidence of acute graft-versus-host disease and pre-existing viral infe
    Raffaele Renella
    Unité d Immunologie et Hématologie Pédiatrique, Faculté de Médecine Université Réné Descartes, Paris, France
    Br J Haematol 134:510-6. 2006
    ..We suggest that the reduced survival after HLA-identical HSCT may be caused by the high incidence of pre-existing viral infections and associated with the onset of severe acute GVHD...
  7. ncbi request reprint Hematopoietic stem cell transplantation in hemophagocytic lymphohistiocytosis: a single-center report of 48 patients
    Marie Ouachée-Chardin
    Department of Pediatric Immuno Hematology, Necker Enfants Malades Hospital, Paris, France
    Pediatrics 117:e743-50. 2006
    ..Chemotherapy- or immunotherapy-based treatments can achieve remission. Hematopoietic stem cell transplantation (HSCT), however, is the only curative option, but optimal modalities and long-term outcome are not yet well known...
  8. ncbi request reprint Hematopoietic stem cell transplantation in Griscelli syndrome type 2: a single-center report on 10 patients
    Jana Pachlopnik Schmid
    INSERM, Unité U768, Laboratoire du Développement Normal et Pathologique du Système Immunitaire, Paris, France
    Blood 114:211-8. 2009
    ..Additional studies are required to improve treatment...
  9. doi request reprint Long-term outcome after hematopoietic stem cell transplantation of a single-center cohort of 90 patients with severe combined immunodeficiency
    Benedicte Neven
    Unité d Immuno Hématologie et Rhumatologie Pédiatrique, Hopital Necker Enfants Malades, Assistance Publique Hopitaux de Paris, Paris, France
    Blood 113:4114-24. 2009
    ..In most cases, HSCT enables long-term survival with infrequent sequelae. However, the occurrence of relatively late-onset complications is a concern that requires specific means of prevention and justifies careful patient follow-up...
  10. doi request reprint FAS-L, IL-10, and double-negative CD4- CD8- TCR alpha/beta+ T cells are reliable markers of autoimmune lymphoproliferative syndrome (ALPS) associated with FAS loss of function
    Aude Magerus-Chatinet
    Inserm U768, Paris, France
    Blood 113:3027-30. 2009
    ..Overall, we showed that determination of the FAS-L represents, together with the IL-10 concentration and the DNT cell percentage, a reliable tool for the diagnosis of ALPS...
  11. doi request reprint Severe cutaneous bacillus Calmette-Guérin infection in immunocompromised children: the relevance of skin biopsy
    Amélie Gantzer
    Department of Dermatology, Hopital Necker Enfants Malades, Universite Rene Descartes, APHP, Paris, France
    J Cutan Pathol 40:30-7. 2013
    ..Skin biopsy is a useful part of the diagnostic workup for disseminated BCGitis, directing the clinician toward severe immunodeficiency. Moreover, skin biopsy may be a useful means of monitoring immune restoration for prognostic purposes...
  12. doi request reprint Characteristics and outcome of early-onset, severe forms of Wiskott-Aldrich syndrome
    Nizar Mahlaoui
    Unité d immunologie et d hématologie pédiatrique, Hôpital Universitaire Necker Enfants Malades, Assistance Publique des Hopitaux de Paris AP HP, Paris, France
    Blood 121:1510-6. 2013
    ..We suggest that HSCT is a curative strategy in this subgroup of patients and should be performed as early in life as possible, even when a fully matched donor is lacking...
  13. ncbi request reprint Immune reconstitution after haematopoietic stem cell transplantation: obstacles and anticipated progress
    Marina Cavazzana-Calvo
    Assistance Publique Hopitaux de Paris AP HP, Department of Biotherapy, Hopital Necker Enfants Malades, Paris, France
    Curr Opin Immunol 21:544-8. 2009
    ..If these objectives could be achieved, the haploidentical procedure would become more readily available to patients affected by acquired or inherited disorders of the haematopoietic system...
  14. pmc Temporal and spatial compartmentalization of drug-resistant cytomegalovirus (CMV) in a child with CMV meningoencephalitis: implications for sampling in molecular diagnosis
    Pierre Frange
    Unite d immunologie, Hématologie et Rhumatologie Pédiatrique, Assistance Publique Hopitaux de Paris AP HP, Hopital Necker Enfants Malades, Paris, France
    J Clin Microbiol 51:4266-9. 2013
    ..However, wild-type cytomegalovirus was evidenced in blood when the meningoencephalitis was diagnosed. Treatment of meningoencephalitis should be adapted to all previously identified resistance mutations in any compartment. ..
  15. pmc Autoimmune lymphoproliferative syndrome caused by a homozygous null FAS ligand (FASLG) mutation
    Aude Magerus-Chatinet
    Unité Inserm 768, Hopital Necker Enfants Malades, Paris, France
    J Allergy Clin Immunol 131:486-90. 2013
    ..Autoimmune lymphoproliferative syndrome (ALPS) is characterized by chronic nonmalignant lymphoproliferation, accumulation of double-negative T cells, hypergammaglobulinemia G and A, and autoimmune cytopenia...
  16. pmc Onset of autoimmune lymphoproliferative syndrome (ALPS) in humans as a consequence of genetic defect accumulation
    Aude Magerus-Chatinet
    Inserm U768, Universite Paris Descartes, Hopital Necker Enfants Malades, Paris, France
    J Clin Invest 121:106-12. 2011
    ..This observation provides the molecular bases of a nonmalignant autoimmune disease development in humans and may shed light on the mechanism underlying the occurrence of other autoimmune diseases...
  17. pmc Ocular modifications in a young girl with cryopyrin-associated periodic syndromes responding to interleukin-1 receptor antagonist anakinra
    Celine Terrada
    Department of Ophthalmology, APHP, Pitie Salpetriere Hospital, 75013, Paris, France
    J Ophthalmic Inflamm Infect 1:133-6. 2011
    ..Ophthalmologic signs normalized. This ophthalmologic description (optic nerve and cornea) has never been illustrated, even if ocular affections are classic in the cryopyrin-associated periodic syndromes...
  18. doi request reprint Reduced expression of FOXP3 and regulatory T-cell function in severe forms of early-onset autoimmune enteropathy
    Nicolette Moes
    Universite Paris Descartes, Paris, France
    Gastroenterology 139:770-8. 2010
    ..We analyzed the molecular basis of neonatal or early postnatal AIE using clinical, genetic, and functional immunological studies...
  19. doi request reprint A Mendelian predisposition to B-cell lymphoma caused by IL-10R deficiency
    Benedicte Neven
    Unité d Immuno Hématologie Pédiatrique, Hopital Necker Enfant Malades, Assistance publique des Hôpitaux de Paris APHP, Paris, France
    Blood 122:3713-22. 2013
    ..Hence, IL-10R deficiency is associated with a high risk of developing B-cell lymphoma. Our results revealed an unexpected role of the IL-10R pathway in lymphomagenesis. ..
  20. pmc Diagnosis of autoimmune lymphoproliferative syndrome caused by FAS deficiency in adults
    Olivier Lambotte
    Service de Medecine Interne, Assistance Publique Hopitaux de Paris, Hôpital du Kremlin Bicêtre, Le Kremlin Bicetre, France
    Haematologica 98:389-92. 2013
    ..Autoimmune lymphoproliferative syndrome may well be diagnosed in adulthood. The occurrence of additional genetic events may account for the delayed disease onset...
  21. doi request reprint Transplantation in patients with SCID: mismatched related stem cells or unrelated cord blood?
    Juliana F Fernandes
    EUROCORD, Hopital Saint Louis, Paris, France
    Blood 119:2949-55. 2012
    ..For children with SCID and no HLA-identical sibling donor, both UCBT and MMRDT represent available HSC sources for transplantation with quite similar outcomes...
  22. ncbi request reprint Characteristics of HIV-infected children recently diagnosed in Paris, France
    Eugenia Macassa
    Unité d Immunologie Hématologie Pédiatriques, Hopital Necker Enfants Malades, 149 rue de Sevres, 75015, Paris, France
    Eur J Pediatr 165:684-7. 2006
    ..HIV-1 infection should be taken into consideration in children born in countries with a high prevalence of HIV, even if they have been living in France for several years and present no symptoms...
  23. ncbi request reprint Molecular basis of the spectral expression of CIAS1 mutations associated with phagocytic cell-mediated autoinflammatory disorders CINCA/NOMID, MWS, and FCU
    Benedicte Neven
    INSERM U429, Hopital Necker Enfants Malades, Paris, France
    Blood 103:2809-15. 2004
    ....
  24. doi request reprint Mutation in the SLC29A3 gene: a new cause of a monogenic, autoinflammatory condition
    Isabelle Melki
    Department of Pediatric Immunology and Rheumatology UIH, Hopital Necker Enfants Malades, 149 rue de Sevres, F 75743 Paris, France
    Pediatrics 131:e1308-13. 2013
    ....
  25. ncbi request reprint Allogeneic bone marrow transplantation in mevalonic aciduria
    Benedicte Neven
    Unité d Immuno Hématologie et Rhumatologie Pédiatrique, Assistance Publique Hopitaux de Paris, Paris, France
    N Engl J Med 356:2700-3. 2007
    ..We observed sustained remission of febrile attacks and inflammation during a 15-month follow-up period...
  26. doi request reprint Morbidity and mortality from ataxia-telangiectasia are associated with ATM genotype
    Romain Micol
    CEREDIH, Hopital Necker Enfants Malades, Assistance Publique Hopitaux de Paris, 75743 Paris Cedex 15, France
    J Allergy Clin Immunol 128:382-9.e1. 2011
    ..The course of the disease is characterized by neurologic manifestations, infections, and cancers...
  27. ncbi request reprint Long-term T-cell reconstitution after hematopoietic stem-cell transplantation in primary T-cell-immunodeficient patients is associated with myeloid chimerism and possibly the primary disease phenotype
    Marina Cavazzana-Calvo
    Institut National de la Sante et de la Recherche Medicale, Unité 768, Paris, France
    Blood 109:4575-81. 2007
    ....
  28. ncbi request reprint Immunotherapy of familial hemophagocytic lymphohistiocytosis with antithymocyte globulins: a single-center retrospective report of 38 patients
    Nizar Mahlaoui
    Unité d Immunologie et Hématologie Pédiatrique, Hopital Necker Enfants Malades, 149 rue de Sevres, 75015 Paris, France
    Pediatrics 120:e622-8. 2007
    ....