Chercheur

    Françoise Le Deist , M.D. , Ph.D.

    francoise.le.deist@umontreal.ca
    Françoise Le Deist
    Axe de recherche
    Maladies virales, immunitaires et cancers
    Thème de recherche
    Maladies inflammatoires et de l'immunité: étude des mécanismes physiopathologiques et de l'approche
    Adresse
    CHUSJ

    Téléphone
    514 345-4931 #3534

    Fax
    514 345-7860

    Titres

    • Professeur titulaire, Département de microbiologie et immunologie, Université de Montréal.
    • Chercheur, Centre de recherche CHU Sainte-Justine

    Formation

    • PhD en sciences, Université Paris VII, 1983.
    • MD, Université Paris VI, 1982.

    Intérêts de recherche

    De 1988 à 2005 j'ai été responsable du Centre d'étude des déficits immunitaires à l'Hôpital Necker - enfants malades à Paris, période pendant laquelle j'ai étudié la physiopathologie de certaines maladies immunitaires héréditaires incluant des déficits immunitaires et des maladies auto-immunes.

    Dans la prolongation de ma carrière en tant que chercheur au Centre de recherche CHU Sainte-Justine depuis 2005, mes intérêts de recherche sont :

    • Immunité innée et infections : certaines infections observées dans la population sont elles dues à une susceptibilité particulière génétiquement déterminée?
    • Déficits immunitaires héréditaires : élucider de nouveaux mécanismes à l'origine de déficit immunitaire non encore élucidé.
    • Reconstitution immunitaire après greffe de cellules souches hématopoïétiques : la greffe de cellules souches hématopoïétiques constitue un modèle de différenciation des lymphocytes in vivo.
    • Pharmacodynamie des immunosuppresseurs : comprendre les mécanismes à l'origine de la variabilité de susceptibilité à ces traitements prescrits chez les patients vivant avec un organe greffé.

    Présentations

    • Lapeyraque AL, Durrieu V, Phan V, Alvarez F, Raboisson MJ, Haddad E, Brito RM, Lajeunesse J, Théorêt Y, Le Deist F. New tools for the pharmacodynamic monitoring of immunosuppressive drugs in pediatric kidney, heart and liver transplant patients. 11th International Congress of Therapeutic Drug Monitoring & Clinical Toxicology, Montréal, 3-8 octobre, 2009.
    • Rottembourg D, Le Deist F, Carel JC Lacroix A, Mallone R, Deal C. T cell epitope identification in auto-immune adrenal deficiency. Annual Meeting of The Endocrine Society, Washington D. C., juin 2009.

Publications

Invasive group A Streptococcus disease in French-Canadian children is not associated with a defect in MyD88/IRAK4-pathway - Fernandes I, Brito RM, Bidet P, Rallu F, Laferrière C, Ovetchkine P, Le Deist F. Invasive group A Streptococcus disease in French-Canadian children is not associated with a defect in MyD88/IRAK4-pathway. Allergy Asthma Clin Immunol 2014; 5: 10(1):9. doi: 10.1186/1710-1492-10-9.

Implication of different effector mechanisms by cord blood-derived and peripheral blood-derived cytokine-induced killer cells to kill precursor B acute lymphoblastic leukemia cell lines - Durrieu L, Lemieux W, Dieng Mame M, Fontaine F, Duval M, Le Deist F, Haddad E. Implication of different effector mechanisms by cord blood-derived and peripheral blood-derived cytokine-induced killer cells to kill precursor B acute lymphoblastic leukemia cell lines. Cytotherapy 2014; 16(6): 845-856.

ICON: The Early Diagnosis of Congenital Immunodeficiencies - Routes J, Abinun M, Al-Herz W, Bustamante J, Condino-Neto A, De La Morena MT, Etzioni A, Gambineri E, Haddad E, Kobrynski L, Le Deist F, Nonoyama S, Oliveira JB, Perez E, Picard C, Rezaei N, Sleasman J, Sullivan KE, Torgerson T. ICON: The Early Diagnosis of Congenital Immunodeficiencies. J Clin Oncol 2014; (sous presse).

Ceftazidime-induced drug reaction with eosinophilia and systemic symptoms (DRESS) complicated by hemophagocytic lymphohistiocytosis - Picard M, Fernandez M, Des Roches A, Bégin P, Paradis J, Paradis L, Le Deist F. Ceftazidime-induced drug reaction with eosinophilia and systemic symptoms (DRESS) complicated by hemophagocytic lymphohistiocytosis. J Allergy Clin Immunol Pract 2013; 1(4): 409-412.

Inflammatory bowel disease and T cell lymphopenia in G6PC3 deficiency - Bégin P, Patey N, Mueller P, Rasquin A, Sirard A, Klein C, Haddad E, Drouin E, Le Deist F. Inflammatory bowel disease and T cell lymphopenia in G6PC3 deficiency. J Clin Immunol 2013; 33(3): 520-525.

Cord blood-derived mesenchymal stromal cells down-modulate CD4+ T-cell activation by inducing IL-10 producing Th1 cells - Selleri S, Dieng Mame M, Nicoletti S, Louis I, Beauséjour C, Le Deist F, Haddad E. Cord blood-derived mesenchymal stromal cells down-modulate CD4+ T-cell activation by inducing IL-10 producing Th1 cells. Stem Cells Dev 2013; 22(7): 1063-1075.

Exome sequencing identifies mutations in the gene TTC7A in French-Canadian cases with hereditary multiple intestinal atresia - Samuels ME, Majewski J, Aljazaeri A, Fernandez I, Casals F, Patey N, Decaluwé H, Gosselin I, Haddad E, Hodgkinson A, Idaghdour Y, Marchand V, Michaud JL, Rodrigue MA, Desjardins S, Dubois S, Le Deist F, Awadalla P, Raymond V, Maranda B. Exome sequencing identifies mutations in the gene TTC7A in French-Canadian cases with hereditary multiple intestinal atresia. J Med Genet 2013; 50(5): 324-329.

Whole-exome sequencing reveals a rapid change in the frequency of rare functional variants in a founding population of humans - Casals F, Hodgkinson A, Hussin J, Idaghdour Y, Bruat V, de Malliard T, Grenier JC, Gbeha E, Hamdan F, Girard S, Spinella JF, Larivière M, Saillour V, Healy J, Fernandes I, Sinnett D, Michaud JL, Rouleau GA, Haddad E, Le Deist F, Awadalla P. Whole-exome sequencing reveals a rapid change in the frequency of rare functional variants in a founding population of humans. PLoS Genet 2013; 9(9): e1003815.

Reconstitution of maturating and regulatory lymphocyte subsets after cord blood and BMT in children - Charrier E, Cordeiro P, Brito RM, Mezziani S, Herblot S, Le Deist F, Duval M. Reconstitution of maturating and regulatory lymphocyte subsets after cord blood and BMT in children. Bone Marrow Transplant 2013; 48(3): 376-382.

Fatal Mycobacterium colombiense/cytomegalovirus coinfection associated with acquired immunodeficiency due to autoantibodies against interferon gamma: a case report - Poulin S, Corbeil C, Nguyen M, St-Denis A, Côté L, Le Deist F, Carignan A. Fatal Mycobacterium colombiense/cytomegalovirus coinfection associated with acquired immunodeficiency due to autoantibodies against interferon gamma: a case report. BMC Infect Dis 2013; 13: 24. doi: 10.1186/1471-2334-13-24.

A homozygous mucosa-associated lymphoid tissue 1 (MALT1) mutation in a family with combined immunodeficiency - Jabara HH, Ohsumi T, Chou J, Massaad MJ, Benson H, Megarbane A, Chouery E, Mikhael R, Gorka O, Gewies A, Portales P, Nakayama T, Hosokawa H, Revy P, Herrod H, Le Deist F, Lefranc G, Ruland J, Geha RS. A homozygous mucosa-associated lymphoid tissue 1 (MALT1) mutation in a family with combined immunodeficiency. J Allergy Clin Immunol 2013; 132(1): 151-158.

Cord blood-derived and peripheral blood-derived cytokine-induced killer cells are sensitive to Fas-mediated apoptosis - Durrieu L, Dieng Mame M, Le Deist F, Haddad E. Cord blood-derived and peripheral blood-derived cytokine-induced killer cells are sensitive to Fas-mediated apoptosis. Biol Blood Marrow Tr 2013; 19(9): 1407-1411.

Secondary pulmonary alveolar proteinosis after unrelated cord blood hematopoietic cell transplantation - Ansari M, Rougemont A-L, Le Deist F, Ozsahin H, Duval M, Champagne M, Fournet JC. Secondary pulmonary alveolar proteinosis after unrelated cord blood hematopoietic cell transplantation. Pediatr Transplant 2012; 16(5): E146–E149.

Reconstitution of protective immune responses against cytomegalovirus and varicella zoster virus does not require disease development in pediatric recipients of umbilical cord blood transplantation - Merindol N, Salem Fourati I, Brito RM, Grenier A, Charrier E, Cordeiro P, Caty M, Mezziani S, Malette B, Duval M, Alfieri C, Ovetchkine P, Le Deist F, Soudeyns H. Reconstitution of protective immune responses against cytomegalovirus and varicella zoster virus does not require disease development in pediatric recipients of umbilical cord blood transplantation. J Immunol 2012; 189(10): 5016-5028.

Human interferon-alpha increases the cytotoxic effect of CD56(+)cord blood-derived cytokine-induced killer cells on human B-acute lymphoblastic leukemia cell lines - Durrieu L, Grégoire-Gauthier J, Dieng Mame M, Fontaine F, Le Deist F, Haddad E. Human interferon-alpha increases the cytotoxic effect of CD56(+)cord blood-derived cytokine-induced killer cells on human B-acute lymphoblastic leukemia cell lines. Cytotherapy 2012; 14(10): 1245-1257.

Ataxia-telangiectasia presenting with a novel immunodeficience - Perreault S, Bernard G, Lortie A, Le Deist F, Decaluwé H. Ataxia-telangiectasia presenting with a novel immunodeficience. Pediatr Neurol 2012; 46(5): 322-324.

[Immunodeficiencies and pathologies associated with mutations in STIM/ORAI, a membrane complex in the heart of calcium signalling] - Le Deist F, Capiod T. [Immunodeficiencies and pathologies associated with mutations in STIM/ORAI, a membrane complex in the heart of calcium signalling]. Méd Sci (Paris) 2011; 27(8-9): 737-745.

Eculizumab in severe Shiga-toxin-associated HUS - Lapeyraque AL, Malina M, Frémeaux-Bacchi V, Boppel T, Kirschfink M, Oualha M, Proulx F, Clermont MJ, Le Deist F, Niaudet P, Schaefer F. Eculizumab in severe Shiga-toxin-associated HUS. N Engl J Med 2011; 364(26): 2561-2563.

High prevalence of primary immune deficiencies in children with autoimmune disorders - Barsalou J, Saint-Cyr C, Drouin E, Le Deist F, Haddad E. High prevalence of primary immune deficiencies in children with autoimmune disorders. Clin Exp Rheumatol 2011; 29(1): 125-130.

Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency) - Pachlopnik Schmid J, Canioni D, Moshous D, Touzot F, Mahlaoui N, Hauck F, Kanegane H, Lopez-Granados E, Mejstrikova E, Pellier I, Galicier L, Galambrun C, Barlogis V, Bordigoni P, Fourmaintraux A, Hamidou M, Dabadie A, Le Deist F, Haerynck F, Ouachee-Chardin M, Rohrlich P, Stephan JL, Lenoir C, Rigaud S, Lambert N, Milili M, Schiff C, Chapel H, Picard C, de Saint Basile G, Blanche S, Fischer A, Latour S. Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency). Blood 2011; 117(5): 1522-1529.

21-Hydroxylase epitopes are targeted by CD8 T cells in autoimmune Addison's disease - Rottembourg D, Deal C, Lambert M, Mallone R, Carel JC, Lacroix A, Caillat-Zucman S, Le Deist F. 21-Hydroxylase epitopes are targeted by CD8 T cells in autoimmune Addison's disease. J Autoimmun 2010; 35(4): 309-315.

ZAP70: a master regulator of adaptive immunity - Fischer A, Picard C, Chemin K, Dogniaux S, Le Deist F, Hivroz C. ZAP70: a master regulator of adaptive immunity. Semin Immunopathol 2010; 32(2): 107-116.

Length of storage and in vitro immunomodulation induced by prestorage leukoreduced red blood cells - Karam O, Tucci M, Toledano B, Robitaille N, Cousineau J, Thibault L, Lacroix J, Le Deist F. Length of storage and in vitro immunomodulation induced by prestorage leukoreduced red blood cells. Transfusion 2009; 49(11): 2326-2334.

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 - Magerus-Chatinet A, Stolzenberg MC, Loffredo MS, Neven B, Schaffner C, Ducrot N, Arkwright PD, Bader-Meunier B, Barbot J, Blanche S, Casanova JL, Debre M, Ferster A, Fieschi C, Florkin B, Galambrun C, Hermine O, Lambotte O, Solary E, Thomas C, Le Deist F, Picard C, Fischer A, Rieux-Laucat F. 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. Blood 2009; 113(13): 3027-3030.

STIM1 mutation associated with a syndrome of immunodeficiency and autoimmunity - Picard C, McCarl CA, Papolos A, Khalil S, Luthy K, Hivroz C, Le Deist F, Rieux-Laucat F, Rechavi G, Rao A, Fischer A, Feske S. STIM1 mutation associated with a syndrome of immunodeficiency and autoimmunity. N Engl J Med 2009; 360(19): 1971-1980.

Hematopoietic engraftment of XLA bone marrow CD34(+) cells in NOG/SCID mice - Moreau T, Bardin F, Barlogis V, Le Deist F, Chabannon C, Tonnelle C. Hematopoietic engraftment of XLA bone marrow CD34(+) cells in NOG/SCID mice. Cytotherapy 2009; 11(2): 198-205.

Relative CD4 lymphopenia and a skewed memory phenotype are the main immunologic abnormalities in a child with Omenn syndrome due to homozygous RAG1-C2633T hypomorphic mutation - McCusker C, Hotte S, Le Deist F, Hirschfeld AF, Mitchell D, Nguyen VH, Gagnon R, Mazer B, Turvey SE, Jabado N. Relative CD4 lymphopenia and a skewed memory phenotype are the main immunologic abnormalities in a child with Omenn syndrome due to homozygous RAG1-C2633T hypomorphic mutation. Clin Immunol 2009; 131(3): 447-455.

Hypomorphic mutation of ZAP70 in human results in a late onset immunodeficiency and no autoimmunity - Picard C, Dogniaux S, Chemin K, Maciorowski Z, Lim A, Mazerolles F, Rieux-Laucat F, Stolzenberg MC, Debre M, Magny JP, Le Deist F, Fischer A, Hivroz C. Hypomorphic mutation of ZAP70 in human results in a late onset immunodeficiency and no autoimmunity. Eur J Immunol 2009; 39(7): 1966-1976.

Long-term outcome after hematopoietic stem cell transplantation of a single-center cohort of 90 patients with severe combined immunodeficiency - Neven B, Leroy S, Decaluwé H, Le Deist F, Picard C, Moshous D, Mahlaoui N, Debre M, Casanova JL, Dal Cortivo L, Madec Y, Hacein-Bey-Abina S, de Saint Basile G, de Villartay JP, Blanche S, Cavazzana-Calvo M, Fischer A. Long-term outcome after hematopoietic stem cell transplantation of a single-center cohort of 90 patients with severe combined immunodeficiency. Blood 2009; 113(17): 4114-4124.

Munc18-2 deficiency causes familial hemophagocytic lymphohistiocytosis type 5 and impairs cytotoxic granule exocytosis in patient NK cells - Côté M, Menager M, Burgess E, Mahlaoui N, Picard C, Schaffner C, Al-Manjomi F, Al-Harbi M, Alangari A, Le Deist F, Gennery AR, Prince N, Cariou A, Nitschke P, Blank U, El-Ghazali G, Menasche G, Latour S, Fischer A, de Saint Basile G. Munc18-2 deficiency causes familial hemophagocytic lymphohistiocytosis type 5 and impairs cytotoxic granule exocytosis in patient NK cells. J Clin Invest 2009; 119(12): 3765-3773.

Long-term outcome after hematopoietic stem cell transplantation of a single-center cohort of 90 patients with severe combined immunodeficiency - Neven B, Leroy S, Decaluwé H, Le Deist F, Picard C, Moshous D, Mahlaoui N, Debre M, Casanova JL, Dal Cortivo L, Madec Y, Hacein-Bey-Abina S, de Saint Basile G, de Villartay JP, Blanche S, Cavazzana-Calvo M, Fischer A. Long-term outcome after hematopoietic stem cell transplantation of a single-center cohort of 90 patients with severe combined immunodeficiency. Blood 2009; 113(17): 4114-4124.

Renal granuloma and immunoglobulin M-complex glomerulonephritis: a case of common variable immunodeficiency? - Benoit G, Lapeyraque AL, Sartelet H, St-Cyr C, Le Deist F, Haddad E. Renal granuloma and immunoglobulin M-complex glomerulonephritis: a case of common variable immunodeficiency?. Pediatr Nephrol 2009; 24(3): 601-604.

Chronic active gastritis in X-linked lymphoroliferative disease - Rougemont A-L, Fournet JC, Martin S, de Saint Basile G, Latour S, Primeau M, Rubbia-Brandt L, Haddad E, Le Deist F. Chronic active gastritis in X-linked lymphoroliferative disease. Am J Surg Pathol 2008; 32(2): 323-328.

Combined T- and B-cell immunodeficiencies - Le Deist F, Moshous D, Howe S, Nahum A, Kavadas F, Lavine E, Roifman CM, Fischer A. Combined T- and B-cell immunodeficiencies. Dans: Rezaei N, Aghamohammadi A, Notarangelo LD (eds). Primary Immunodeficiency Diseases, Definition, Diagnosis and Management. Springer, 2008; 39-78.

ICF syndrome, a genetic disese related to DNMT3B a de novo DNA methyltransferase - Bourc'his C, Le Deist F, Viegas-Péquignot E. ICF syndrome, a genetic disese related to DNMT3B a de novo DNA methyltransferase. Dans: Epstein CJ, Erickson PP, Wynshaw-Boris A (eds). Inborn Errors of Human Development. Oxford University Press, 2008; 974-977.

Long-term outcome following hematopoietic stem cell transplantation in Wiskott-Aldrich syndrome: collaborative study of the European Society for Immunodeficiencies and the European Group for Blood and Marrow Transplantation - Ozsahin H, Cavazzana-Calvo M, Notarangelo LD, Schulz A, Trasher A, Mazzarello T, Slatter M, Le Deist F, Blanche S, Veys P, Fasth A, Bredius R, Sedlacek P, Wulffraat N, Ortega J, Heilmann C, O'Meara A, Wachowiak J, Kalwak K, Matthes-Martin S, Gungor T, Ikinciogullari A, Landais P, Cant A, Friedrich WAF. Long-term outcome following hematopoietic stem cell transplantation in Wiskott-Aldrich syndrome: collaborative study of the European Society for Immunodeficiencies and the European Group for Blood and Marrow Transplantation. Blood 2008; 111(1): 439-445.

[Expression anomalies of the CD3-TCR complex expression and immunodeficiencies] - Le Deist F, de Saint Basile G, Rieux-Laucat F, Hivroz C, Fischer A. [Expression anomalies of the CD3-TCR complex expression and immunodeficiencies]. Méd Sci (Paris) 2007; 23(2): 161-166.

T-cell immunodeficiencies - Le Deist F, Fischer A. T-cell immunodeficiencies. Dans: Rich R (ed.). Clinical Immunology, 3e édition. St-Louis. Mosby, 2007; 531-551.

Déficits immunitaires primaires - Des Roches A, Haddad E, Le Deist F. Déficits immunitaires primaires. Dans: Turgeon J, Bernard-Bonnin AC, Gervais P, Ovetchkine P, Gauthier M (eds). Dictionnaire de thérapeutique pédiatrique Weber, 2e édition. Boucherville. Gaëtan Morin, 2007; 346-353.

Rituximab therapy for childhood Evans syndrome - Bader-Meunier B, Aladjidi N, Bellmann F, Monpoux F, Nelken B, Robert A, Armari-Alla C, Picard C, Le Deist F, Munzer M, Yacouben K, Bertrand Y, Pariente A, Chaussé A, Perel Y, Leverger G. Rituximab therapy for childhood Evans syndrome. Haematologica 2007; 92(12): 1691-1694.

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 - Cavazzana-Calvo M, Carlier F, Le Deist F, Morillon E, Taupin P, Gautier D, Radford-Weiss I, Caillat-Zucman S, Neven B, Blanche S, Cheynier R, Fischer A, Hacein-Bey-Abina S. 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. Blood 2007; 109(10): 4575-4581.

Is xenotransplantation of embryonic stem cells a realistic option? - Bonnevie L, Bel A, Sabbah L, Al Attar N, Pradeau P, Weill B, Le Deist F, Bellamy V, Peyrard S, Menard C, Desnos M, Bruneval P, Binder P, Hagege AA, Puceat M, Menasche P. Is xenotransplantation of embryonic stem cells a realistic option?. Transplantation 2007; 83(3): 333-335.

Perforin-dependent apoptosis functionally compensates Fas-deficiency in Activation-induced cell-death of human T-lymphocytes - Mateo V, Menager M, de Saint Basile G, Stolzenberg MC, Roquelaure B, Andre N, Florkin B, Le Deist F, Picard C, Fischer A, Rieux-Laucat F. Perforin-dependent apoptosis functionally compensates Fas-deficiency in Activation-induced cell-death of human T-lymphocytes. Blood 2007; 110(13): 4285-4292.

Inherited and somatic CD3zeta mutations in a patient with T-cell deficiency - Rieux-Laucat F, Hivroz C, Lim A, Mateo V, Pellier I, Selz F, Fischer A, Le Deist F. Inherited and somatic CD3zeta mutations in a patient with T-cell deficiency. N Engl J Med 2006; 354(18): 1913-1921.

[Hypomorphic RAG1 mutations and CMV infection: a new phenotype of severe combined immunodeficiency] - Le Deist F, de Villartay JP, Lim A, Dechanet-Merville J, Fischer A. [Hypomorphic RAG1 mutations and CMV infection: a new phenotype of severe combined immunodeficiency]. Méd Sci (Paris) 2006; 223: 239-240.

[Auto-immune hemolytic anemia and dyserythropoiesis as the presenting signs of Fas-deficient condition in 3 children] - Guitton C, Le Deist F, Tchernia G, Bader-Meunier B. [Auto-immune hemolytic anemia and dyserythropoiesis as the presenting signs of Fas-deficient condition in 3 children]. Arch Pediatr 2006; 134: 367-370.

T cell-dependent activation of dendritic cells requires IL-12 and IFN-{gamma} signaling in T cells - Miro F, Nobile C, Blanchard N, Lind M, Filipe-Santos O, Fieschi C, Chapgier A, Vogt G, de Beaucoudrey L, Kumararatne DS, Le Deist F, Casanova JL, Amigorena S, Hivroz C. T cell-dependent activation of dendritic cells requires IL-12 and IFN-{gamma} signaling in T cells. J Immunol 2006; 177(6): 3625-3634.

[Defect in lytic granule exocytosis: several causes, a same effect] - Menasche G, Menager M, Le Deist F, Fischer A, de Saint Basile G. [Defect in lytic granule exocytosis: several causes, a same effect]. Méd Sci (Paris) 2006; 228(9): 733-738.

Familial NK cell deficiency associated with impaired IL-2- and IL-15-dependent survival of lymphocytes - Eidenschenk C, Jouanguy E, Alcais A, Mention JJ, Pasquier B, Fleckenstein IM, Puel A, Gineau L, Carel JC, Vivier E, Le Deist F, Casavant S. Familial NK cell deficiency associated with impaired IL-2- and IL-15-dependent survival of lymphocytes. J Immunol 2006; 177(12): 8835-8843.

Mycophenolate mofetil as an alternate immunosuppressor for autoimmune lymphoproliferative syndrome - Kossiva L, Theodoridou M, Mostrou G, Vrachnou E, Le Deist F, Rieux-Laucat F, Kanariou MG. Mycophenolate mofetil as an alternate immunosuppressor for autoimmune lymphoproliferative syndrome. J Pediatr Hematol Oncol 2006; 28(12): 824-826.

XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome - Rigaud S, Fondaneche MC, Lambert N, Pasquier B, Mateo V, Soulas P, Galicier L, Le Deist F, Rieux-Laucat F, Revy P, Fischer A, de Saint Basile G, Latour S. XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome. Nature 2006; 444(7115): 110-114.

Hematopoietic stem cell transplantation in hemophagocytic lymphohistiocytosis: a single-center report of 48 patients - Ouachee-Chardin M, Elie C, de Saint Basile G, Le Deist F, Mahlaoui N, Picard C, Neven B, Casanova JL, Tardieu M, Cavazzana-Calvo M, Blanche S, Fischer A. Hematopoietic stem cell transplantation in hemophagocytic lymphohistiocytosis: a single-center report of 48 patients. Pediatrics 2006; 117(4): E743-750.

[Epidemiology of autoimmune haemolytic anemia in children: French data] - Aladjidi N, Leverger G, Pariente A, Bader-Meunier B, Le Deist F, Colin Y, Michel G, Quartier P, Pondare C, Monpoux F, Leblanc T, Nelken B, Lutz P, Blouin P, Yacouben K, Robert A, Stephan JL, Perel Y. [Epidemiology of autoimmune haemolytic anemia in children: French data]. Arch Pediatr 2006; 13(6): 511-514.

Kaposi's sarcoma in a child with Wiskott-Aldrich syndrome - Picard C, Mellouli F, Duprez R, Chedeville G, Neven B, Fraitag S, Delaunay J, Le Deist F, Fischer A, Blanche S, Bodemer C, Gessain A, Casanova JL, Bejaoui M. Kaposi's sarcoma in a child with Wiskott-Aldrich syndrome. Eur J Pediatr 2006; 165(7): 453-457.

Successful allogeneic hemopoietic stem cell transplantation in a child who had anhidrotic ectodermal dysplasia with immunodeficiency - Dupuis-Girod S, Cancrini C, Le Deist F, Palma P, Bodemer C, Puel A, Livadiotti S, Picard C, Bossuyt X, Rossi P, Fischer A, Casanova JL. Successful allogeneic hemopoietic stem cell transplantation in a child who had anhidrotic ectodermal dysplasia with immunodeficiency. Pediatrics 2006; 118(1): e205-211.

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 infections - Renella R, Picard C, Nevanlinni H, Ouachee-Chardin M, Casanova JL, Le Deist F, Cavazzana-Calvo M, Blanche S, Fischer A. 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 infections. Brit J Haematol 2006; 134(5): 510-516.

Severe combined immunodeficiency and microcephaly in siblings with hypomorphic mutations in DNA ligase IV - Buck D, Moshous D, de Chasseval R, Ma Y, Le Deist F, Cavazzana-Calvo M, Fischer A, Casanova C, Leiber MR, de Villartay JP. Severe combined immunodeficiency and microcephaly in siblings with hypomorphic mutations in DNA ligase IV. Eur J Immunol 2006; 36(1): 224-235.

Cernunnos, a novel nonhomologous end-joining factor, is mutated in human immunodeficiency with microcephaly - Buck D, Malivert L, de Chasseval R, Barraud A, Fondaneche MC, Sanal O, Plebani A, Stephan JL, Hufnagel M, Le Deist F, Fischer A, Durandy A, de Villartay JP, Revy P. Cernunnos, a novel nonhomologous end-joining factor, is mutated in human immunodeficiency with microcephaly. Cell 2006; 124(2): 287-299.

Omenn syndrome in an infant with IL7RA gene mutation - Giliani S, Bonfirm C, de Saint Basile G, Lanzi G, Brousse N, Koliski A, Malvezzi A, Fischer A, Notarangelo LD, Le Deist F. Omenn syndrome in an infant with IL7RA gene mutation. J Pediatr 2006; 148(2): 272-274.

[Severe combined immunodeficiency: susceptibility to HPV?] - Le Deist F, Laffort C, Orth G, Bodemer C, Fischer A. [Severe combined immunodeficiency: susceptibility to HPV?]. Méd Sci (Paris) 2005; 21(2): 125-127.

Autoimmune lymphoproliferative syndrome and perforin - Rieux-Laucat F, Le Deist F, de Saint Basile G. Autoimmune lymphoproliferative syndrome and perforin. N Engl J Med 2005; 352(3): 306-307; author reply 306-307.

Coombs' negative haemolytic anaemia as a first manifestation of autoimmune lymphoproliferative disease - Guitton C, Le Deist F, Bader-Meunier B. Coombs' negative haemolytic anaemia as a first manifestation of autoimmune lymphoproliferative disease. Brit J Haematol 2005; 129(3): 442-443.

Severe combined immunodeficiency. A model disease for molecular immunology and therapy - Fischer A, Le Deist F, Hacein-Bey-Abina S, Andre-Schmutz I, de Saint Basile G, de Villartay JP, Cavazzana-Calvo M. Severe combined immunodeficiency. A model disease for molecular immunology and therapy. Immunol Rev 2005; 203: 98-109.

Interleukin-7 receptor alpha (IL-7Ralpha) deficiency: cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients - Giliani S, Mori L, de Saint Basile G, Le Deist F, Rodriguez-Perez C, Forino C, Mazzolari E, Dupuis S, Elhasid R, Kessel A, Galambrun C, Gil J, Fischer A, Etzioni A, Notarangelo LD. Interleukin-7 receptor alpha (IL-7Ralpha) deficiency: cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients. Immunol Rev 2005; 203: 110-126.

Ca2+ ionophores trigger membrane remodeling without a need for store-operated Ca2+ entry - Galitzine M, Capiod T, Le Deist F, Meyer D, Freyssinet JM, Kerbiriou-Nabias D. Ca2+ ionophores trigger membrane remodeling without a need for store-operated Ca2+ entry. Biochem Biophys Res Commun 2005; 327(1): 335-341.

Severe and progressive encephalitis as a presenting manifestation of a novel missense perforin mutation and impaired cytolytic activity - Feldmann J, Menasche G, Callebaut I, Minard-Colin V, Bader-Meunier B, Le Clainche L, Fischer A, Le Deist F, Tardieu M, de Saint Basile G. Severe and progressive encephalitis as a presenting manifestation of a novel missense perforin mutation and impaired cytolytic activity. Blood 2005; 105(7): 2658-2663.

WHIM syndromes with different genetic anomalies are accounted for by impaired CXCR4 desensitization to CXCL12 - Balabanian K, Lagane B, Pablos JL, Laurent L, Planchenault T, Verola O, Lebbe C, Kerob D, Dupuy A, Hermine O, Nicolas JF, Latger-Cannard V, Bensoussan D, Bordigoni P, Baleux F, Le Deist F, Virelizier JL, Arenzana-Seisdedos F, Bachelerie F. WHIM syndromes with different genetic anomalies are accounted for by impaired CXCR4 desensitization to CXCL12. Blood 2005; 105(6): 2449-2457.

Transient familial haemophagocytic lymphohistiocytosis reactivation post-CD34 haematopoietic stem cell transplantation - Almousa H, Ouachee-Chardin M, Picard C, Radford-Weiss I, Caillat-Zucman S, Cavazzana-Calvo M, Blanche S, de Saint Basile G, Le Deist F, Fischer A. Transient familial haemophagocytic lymphohistiocytosis reactivation post-CD34 haematopoietic stem cell transplantation. Brit J Haematol 2005; 130(3): 404-408.

The repair of DNA damages/modifications during the maturation of the immune system: lessons from human primary immunodeficiency disorders and animal models - Revy P, Buck D, Le Deist F, de Villartay JP. The repair of DNA damages/modifications during the maturation of the immune system: lessons from human primary immunodeficiency disorders and animal models. Adv Immunol 2005; 87: 237-295.

CD3 deficiencies - Fischer A, de Saint Basile G, Le Deist F. CD3 deficiencies. Curr Opin Allergy Clin Immunol 2005; 5(6): 491-495.

[Evans' syndrome: a retrospective study from the ship (French Society of Pediatric Hematology and Immunology) (36 cases)] - Blouin P, Auvrignon A, Pagnier A, Thuret I, Antoni G, Bader-Meunier B, Le Deist F, Chastagner P, Aladjidi N, Pellier I, Bertrand Y, Behar C, Landmann-Parker J, Leverger G, Perel Y. [Evans' syndrome: a retrospective study from the ship (French Society of Pediatric Hematology and Immunology) (36 cases)]. Arch Pediatr 2005; 12(11): 1600-1607.

A novel immunodeficiency associated with hypomorphic RAG1 mutations and CMV infection - de Villartay JP, Lim A, Al-Mousa H, Dupont S, Dechanet-Merville J, Cournau-Gatbois E, Gougeon ML, Lemainque A, Eidenschenk C, Jouanguy E, Abel L, Casanova JL, Fischer A, Le Deist F. A novel immunodeficiency associated with hypomorphic RAG1 mutations and CMV infection. J Clin Invest 2005; 115(11): 3291-3299.

Failure of SCID-X1 gene therapy in older patients - Thrasher AJ, Hacein-Bey-Abina S, Gaspar HB, Blanche S, Davies EG, Parsley K, Gilmour K, King D, Howe S, Sinclair J, Hue C, Carlier F, von Kalle C, de Saint Basile G, Le Deist F, Fischer A, Cavazzana-Calvo M. Failure of SCID-X1 gene therapy in older patients. Blood 2005; 105(11): 4255-4257.

Long-term survival in severe combined immune deficiency: the role of persistent maternal engraftment - Tezcan I, Ersoy F, Sanal O, Turul T, Uckan D, Balci S, Hicsonmez G, Prieur M, Caillat-Zucman S, Le Deist F, de Saint Basile G. Long-term survival in severe combined immune deficiency: the role of persistent maternal engraftment. J Pediatr 2005; 146(1): 137-140.

Clonal evidence for the transduction of CD34+ cells with lymphomyeloid differentiation potential and self-renewal capacity in the SCID-X1 gene therapy trial - Schmidt M, Hacein-Bey-Abina S, Wissler M, Carlier F, Lim A, Prinz C, Glimm H, Andre-Schmutz I, Hue C, Garrigue A, Le Deist F, Lagresle C, Fischer A, Cavazzana-Calvo M, von Kalle C. Clonal evidence for the transduction of CD34+ cells with lymphomyeloid differentiation potential and self-renewal capacity in the SCID-X1 gene therapy trial. Blood 2005; 105(7): 2699-2706.

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