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Magda Carneiro-Sampaio, MD, PhD Hospital das Clínicas da Faculdade de Medicina

Magda Carneiro-Sampaio, MD, PhD Hospital das Clínicas da Faculdade de Medicina Universidade de São Paulo (FMUSP), Brazil. Primary Immunodeficiencies in 910 Brazilian patients of different age groups. > 30 years old: 227. 910 consecutive patients with well-defined PIDs*

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Magda Carneiro-Sampaio, MD, PhD Hospital das Clínicas da Faculdade de Medicina

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  1. Magda Carneiro-Sampaio, MD, PhD Hospital das Clínicas da Faculdade de Medicina Universidade de São Paulo (FMUSP), Brazil Primary Immunodeficiencies in 910 Brazilian patients of different age groups

  2. > 30 years old: 227 910 consecutive patients with well-defined PIDs* followed-up at Hospital das Clínicas da FMUSP and grouped according to age at diagnosis: • < 2 years old: 118 patients • 2 - 5 years old: 141 60 different PIDs were identified • 5 - 10 years old: 169 • 10 - 20 years old: 137 • 20 - 30 years old: 118 *Notarangelo et al. IUIS Experts Committee PID Classification JACI ; 124:1161, 2009

  3. Ethnicity of Brazilian people results from a mixture of native Indian, Portuguese and African descents, who have been merging since XVIth century

  4. In the last 120 years Brazil received significant Italian, Syrian-Lebanese and Japanese immigration Fotos de Priscila Oliveira, 2010

  5. Predominantly Antibody Deficiencies Combined T + B deficiencies Phagocyte number and/or function def Other Well defined PID syndromes Complement deficiencies Immune dysregulation diseases Innate immunity defects Autoinflammatory disorders Primary Immunodeficiencies (PID): Hospital das Clínicas da FMUSP’s Series (N=910) Magda Carneiro-Sampaio, Jorge E. Kalil, Alberto Duarte & cols, Primary Immunodeficiency Diseases in Brazilian Patients of Different Age Groups, 2010

  6. Distribution of HC-FMUSP PID patients (N = 910) according to age groups and the main IUIS PID categories

  7. 18% (XLA, THI) 5% 14% (HLH, IPEX) Distribution of the <2 years old patients (N=118) according to the main IUIS PID categories 14% (DiGeorge Syndrome) 2,5% 21% (CGD) 25% (SCID) 2,5%

  8. 1% 8% Distribution of the 2-5 years old patients (N=141) according to the main IUIS PID categories 16% 8% 14% 3,5% 48% (XLA, THI, IgAD) 1,5%

  9. Gender distribution in different age groups of HC-FMUSP PID series (N = 910) Age (years)

  10. Highly significant findings in the comparison between <5 years old X >5 years old PID groups Hospital das Clínicas FMUSP

  11. Faculdade de Medicina da Universidade de São Paulo Clinical staff in charge of PID patients follow-up at Hospital das Clínicas da FMUSP • Dept of Pediatrics (patients < 20 years-old): Magda Carneiro-Sampaio, Cristina Miuki A Jacob, Antonio Pastorino, Angela Bueno F Fomin, Mayra Dorna, Leticia Watanabe • Dept of Internal Medicine (patients >20 years-old): Jorge Kalil, Cristina Kokron, Myrthes Toledo Barros, Luiz Vicente Rizzo • Dept of Dermatology (patients with skin manifestations of all ages): Alberto Duarte, Dewton Moraes-Vasconcelos, Anete Sevciovic Grumach

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