Escola São Paulo de Ciência Avançada

November 28^th (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 30^th (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 1^st (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 2^st (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 3^rd (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