São Paulo Advanced School on Primary Immunodeficiencies: Unraveling Human Immunophysiology

A 1st São Paulo Advanced School on Primary Immunodeficiencies: Unraveling Human Immunophysiology – I ESPCA-PID foi organizada pela Faculdade de Medicina da Universidade de São Paulo, com coordenação da Profa. Dra. Magda Maria Sales Carneiro-Sampaio, e fomentada pela FAPESP.

A proposta da I ESPCA-PID era uma estratégia inovadora para a compreensão da fisiologia e da fisiopatologia do sistema imunológico humano, através do estudo de crianças portadoras de Imunodeficiências Primárias.

Um comitê selecionou 80 alunos (27 estrangeiros e 53 brasileiros) para participar do evento, entre os 135 inscritos (70 deles brasileiros e 65 estrangeiros).

A I ESPCA-PID contou com a participação de 31 docentes brasileiros e 13 docentes estrangeiros.

PAÍSES REPRESENTADOS POR PALESTRANTES:
Argentina, Colômbia, Estados Unidos, França, Inglaterra, Israel e Portugal

  • Intituição

    Faculdade de Medicina / Universidade de São Paulo (USP)

  • Área de conhecimento

    Medicina

  • Pesquisador Responsável

    Magda Maria Sales Carneiro-Sampaio

  • Nº do Processo FAPESP

    09/54525-1

  • Período

    27/11/2010 a 04/12/2010

  • Inscrições

    Encerradas

  • Site

  • Cidade

    São Paulo

  • Palavras-chave

    Imunofisiologia humana, Imunodeficiências primárias, Medicina translacional, Imunidade anti-infecciosa, Tolerância imunológica, Genômica

  • Programa Ocultar

     

    November 28th (Sunday)

    19:00 h

    Welcome session

    Adress: Instituto da Criança - Hospital das Clínicas-FMUSP

    Av. Dr. Enéas de Carvalho Aguiar, 647 - Main Auditorium

    Introduction to the course - Magda Carneiro-Sampaio

    FAPESP: purposes and programs - Ricardo Brentani

     

    Opening lecture:

     

    Evolution and development of natural tolerance in the context of anti-infectious protection

     

    António Coutinho

    20:30 h

    Cocktail

       

    November 29th (Monday) - December 4 th (Saturday)

    Adress: Faculdade de Medicina de São Paulo - Av. Dr. Arnaldo, 455

    Auditório dos Paramédicos — 4° andar

    08:30h

    Lecture:

     

    Primary Immunodeficiency Diseases: "Experiments of Nature" - Hans Ochs (USA)

     

    Coordinator: Jorge E. Kalil (Brazil)

     

    Presentation and discussion of PID clinical cases:

     

    Ataxia telangiectasia with immunodeficiency: a series from Costa Rica – Dora Matus (Costa Rica)

    10:00h

    Coordinator – Hans Ochs (USA)

    10:00 - 10:30h

    Coffee break

    10:30h

    Lecture (joint session with Instituto de Infectologia Emilio Ribas):

     

    Congenital defects of innate immunity - Steven M Holland (USA)

     

    Coordinator: Alberto J. S. Duarte (Brazil)

     

    Presentation and discussion of PID clinical cases:

     

    Chronic Granulomatous Disease due to CYBB mutation - Antonio C. Pastorino (Brazil)

    12:00h

    Coordinator: Steven M. Holland (USA)

    12:00 - 13:30h

    Lunch and poster view

    13:30h

    Laboratory primers on basic tools for investigating human pathophysiology:

     

    1) Genomic Tools: DNA sequencing, Gene expression analysis using DNA microarrays, Comparative Genomic Hybridization using high density DNA microarrays – Carlos A. Moreira-Filho & Silvia Y. Bando (Brazil)

    15:30h

    2) Flow cytometry: João Bosco Oliveira (USA)

    15:30 - 16:00h

    Coffee break

    16:00h

    Lecture:

     

    An introduction to mouse models of PIDs – Jocelyne Demengeot (Portugal)

     

    Coordinator – Momtchilo Russo

     

    Lecture:

     

    Leukocyte Adhesion Deficiencies - Amos Etzioni (Israel)

    17:30h

    Coordinator: Luiz Vicente Rizzo (Brazil)

    November 30th (Tuesday)

    08:30h

    Lecture:

     

    PIDs and mycobacterial diseases: a model for understanding specific microorganism susceptibility - Sergio D. Rosenzweig (USA)

     

    Coordinators: Jorge Sampaio and Heloisa H. Souza Marques (Brazil)

     

    Presentation and discussion of PID clinical cases:

     

    Defects of the Interferon gamma / IL-12 axis with BCGitis - Dewton Moraes-Vasconcelos (Brazil)

    10:00h

    Coordinator: Sergio D. Rosenzweig (USA)

    10:00 - 10:30h

    Coffee break

    10:30h

    Lecture:

     

    Individual susceptibility to fulminant Hepatitis A: new genetic and pathophysiology factors revisited - Sergio D. Rosenzweig (USA)

     

    Coordinator – José Alexandre M. Barbuto (Brazil)

     

    Presentation and discussion of PID clinical cases:

     

    Severe Combined Immunodeficiency (SCID) - Jessica Rojas Restrepo (Colombia)

    12:00h

    Coordinator – Beatriz Costa Carvalho (Brazil)

    12:00 - 13:30h

    Lunch and poster view

    13:30h

    Lecture:

     

    Inherited defects of regulatory T cells - Hans Ochs (USA)

     

    Coordinator: Magda Carneiro-Sampaio (Brazil)

     

    Presentation and discussion of PID clinical cases:

     

    Immunedysregulation Polyendocrinopathy Enteropathy X-linked - IPEX – Letícia Watanabe and Juliana F. Fernandes (Brazil)

     

    Neonatal enteropathy due to IL-2 receptor alpha-chain (CD25) mutation – Federica Barzaghi (Italy)

     

    Comments: Mouse models of regulatory T cell deficiency – Jocelyne Demengeot (Portugal)

    16:00h

    Coordinator: Hans Ochs (USA)

    16:00 - 16:30h

    Coffee break

    16:30h

    Lecture:

     

    Immune defects leading to fungal infections - Amos Etzioni (Israel)

     

    Coordinator: Matias Oleastro (Argentina)

     

    Presentation and discussion of PID clinical cases:

     

    Decreased TH17 counts in patients with chronic Candida albicans infections – Ellen Renner (Germany)

    18:00h

    Coordinator: Amos Etzioni (Israel)

    December 1st (Wednesday)

    08:30h

    Lecture:

     

    Hemophagocytic lymphohistiocytosis (HLH): phenotype, molecular defects and diagnostic approach - Geneviève de Saint-Basile (France)

     

    Coordinator: Edécio Cunha-Neto (Brazil)

     

    Presentation and discussion of PID clinical cases:

     

    Hemophagocytic lymphohistiocytosis - HLH - Elma Ines Nievas (Argentina)

    10:00h

    Coordinator: Geneviève de Saint-Basile (France)

    10:00 - 10:30h

    Coffee break

    10:30h

    Lecture (joint session with the area of Pediatric Intensive Care):

     

    Hemophagocytic lymphohistiocytosis (HLH): pathophysiology and treatment Geneviève de Saint Basile (France)

     

    Coordinator: Werther Brunow de Carvalho and Edécio Cunha-Neto (Brazil)

     

    Lecture:

     

    Primary Immunodeficiencies in a Leishmaniosis endemic area - Edgar Marcelino de Carvalho (Brazil)

    12:30h

    Coordinator: Maria Irma Seixas Duarte (Brazil)

    12:30 - 14:00h

    Lunch and poster view

    14:00h

    Lecture:

     

    Human disorders of apoptosis: ALPS and beyond - João Bosco Oliveira (USA)

     

    Coordinator: Niels Olsen Saraiva Câmara (Brazil)

     

    Presentation and discussion of PID clinical cases:

    15:30h

    Autoimmune lymphoproliferative syndrome (ALPS) due to Fas mutation Maria Gabriela Simesen de Bielke (Argentina)

    Coordinator – João Bosco Oliveira (USA)

    15:30 - 16:00h

    Coffee break

    16:00h

    Presentation and discussion of PID clinical cases:

     

    Familial Mediterranean Fever – Adem Polat (Turkey)

     

    Deficiency of interleukin 1 receptor antagonist (DIRA): an autoinflammatory disease - Adriana Almeida de Jesus (Brazil)

     

    Comments about mouse models of PIDs discussed on the day – Jocelyne Demengeot (Portugal)

    18:00h

    Coordinators – Sonia Jancar and Clovis Artur Almeida Silva (Brazil)

    December 2st (Thursday)

    08:30h

    Lecture (joint session with “Comissão de Pesquisa da FMUSP”)

     

    AIRE - Diane Mathis (USA)

     

    Coordinator: Geraldo Passos and Durval Damiani (Brazil)

     

    Presentation and discussion of PID clinical cases:

     

    Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED) due to AIRE mutation: the prototype of central tolerance defects - Mayra B. Dorna (Brazil)

    10:00h

    Coordinator: Diane Mathis (USA)

    10:00 - 10:30h

    Coffee break

    10:30h

    Lecture:

     

    Molecular Bases for Autoimmunity in Down Syndrome - Magda Carneiro-Sampaio (Brazil)

     

    Coordinator: Antonio Coutinho (Portugal)

     

    Presentation and discussion of PID clinical cases:

     

    DiGeorge syndrome associated to 22q.11.2 deletion: a defect of thymus development– presentation of a series - Angela Bueno Fomin (Brazil)

    12:00h

    Coordinator: Esper Kallas (Brazil)

    12:00 - 13:30h

    Lunch and poster view

    13:30h

    Lecture:

     

    Complement Deficiencies - Luis Eduardo Coelho Andrade (Brazil)

     

    Coordinator: Magda Carneiro-Sampaio (Brazil)

     

    Presentation and discussion of PID clinical cases:

     

    Homozygous C1q deficiency: the strongest single factor for developing SLE - Natalia Umetsu (Brazil)

     

    Hereditary Angioedema – Maria Fernanda Ferraro (Brazil)

    15:30 h

    Coordinator – Luiza Karla Arruda and Virginia Ferriani (Brazil)

    15:30 - 16:00h

    Coffee break

     

    Lecture:

     

    Control of promiscuous gene expression in the thymus – Geraldo Passos (Brazil)

     

    Presentation and discussion of PID clinical cases:

     

    FOXN1 deficiency - Isabel Esteves (Portugal)

     

    Comments: Mouse models of thymic deficiencies – Jocelyne Demengeot (Portugal)

    18:00h

    Coordinator – Jocelyne Demengeot (Portugal)

    December 3rd (Friday)

    08:30h

    Lecture:

     

    Disorders of B cell development - José Luis Franco (Colombia)

     

    Coordinator: Virginia Paes Leme Ferriani (Brazil)

     

    Presentation and discussion of PID clinical cases:

     

    X-linked Agammaglobulinemia due to BTK mutation – Cristina Miuki Abe Jacob (Brazil)

    10:00h

    Coordinator: José Luis Franco (Colombia)

    10:00 - 10:30h

    Coffee break

    10:30h

    Presentation and discussion of PID clinical cases:

     

    Hyper IgM syndrome due to AID deficiency- Ana Paula Moschione Castro (Brazil)

     

    X-linked Hyper IgM syndrome due to CD40 ligand deficiency -Sonia Cristina Gaspar Lemos (Portugal)

     

    Common Variable Immunodeficiency (CVID): presentation of a large Brazilian cohort Ana Karolina B. Oliveira (Brazil)

    12:00h

    Coordinators: Michael Neuberger (UK) and Cristina M. Kokron (Brazil)

    12:00 - 13:30h

    Lunch and taking office ceremony of the new Dean of FMUSP (Prof. Giovanni G. Cerri)

    13:30h

    Lecture:

     

    Role of seric IgA and IgA Fc receptors in inflammation: inhibitory ITAMs as novel regulators of immunity – Renato C. Monteiro (France)

     

    Coordinators: Cristina Miuki Abe Jacob and Luiz Vicente Rizzo (Brazil)

     

    Presentation and discussion of PID clinical cases:

     

    Presentation of a large cohort of IgA-deficient patients with infectious, allergic and autoimmune manifestations - Myrthes Toledo-Barros (Brazil)

     

    Transfer of antibodies across placenta and in breast milk from mothers with CVID and IgA deficiency – Patricia Palmeira (Brazil)

    15:30h

    Coordinator: Renato C. Monteiro (France)

     

    Lecture: Rheumatoid Arthritis – Diane Mathis (USA)

    December 4th (Saturday)

    9:00h

    Closing lecture:

     

    Generation of antibody diversity: from patient to bench and back again Michael Neuberger (UK)

     

    Coordinator: António Coutinho (Portugal)

     

    Course evaluation and general discussion

     

    Coordinators - Jocelyne Demengeot (Portugal) and Crina Samarguitean (Finland)

    12:00h

    Concluding remarks (António Coutinho and Magda Carneiro-Sampaio)

    12:00h

    Closing Banquet - Brazilian barbecue