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Magda Carneiro-Sampaio, MD, PhD Hospital das Clínicas da Faculdade de Medicina Universidade de São Paulo (FMUSP), Brazil Primary Immunodeficiencies in.

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Presentation on theme: "Magda Carneiro-Sampaio, MD, PhD Hospital das Clínicas da Faculdade de Medicina Universidade de São Paulo (FMUSP), Brazil Primary Immunodeficiencies in."— Presentation transcript:

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

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

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

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

5 Primary Immunodeficiencies (PID): Hospital das Clínicas da FMUSP’s Series (N=910) Predominantly Antibody Deficiencies Combined T + B deficiencies Phagocyte number and/or function def Complement deficiencies Other Well defined PID syndromes Immune dysregulation diseases Innate immunity defects Autoinflammatory disorders 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 2,5% Distribution of the <2 years old patients (N=118) according to the main IUIS PID categories 5% 18% (XLA, THI) 14% (DiGeorge Syndrome) 21% (CGD) 2,5% 14% (HLH, IPEX) 25% (SCID)

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

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

10 Highly significant findings in the comparison between 5 years old PID groups Frequency % p <5 years old ( N=259) >5 years old (N= 651) Predominantly Antibody deficiencies 34%75%<0.001 Combined T+ B deficiencies13%3%<0.001 Phagocyte number and/ or function defects 17%5%<0.001 Immune dysregulation diseases10%2%<0.001 Male gender71%52%< Hospital das Clínicas FMUSP

11  Dept of Internal Medicine (patients >20 years-old): Jorge Kalil, Cristina Kokron, Myrthes Toledo Barros, Luiz Vicente Rizzo  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 Dermatology (patients with skin manifestations of all ages): Alberto Duarte, Dewton Moraes-Vasconcelos, Anete Sevciovic Grumach Clinical staff in charge of PID patients follow-up at Hospital das Clínicas da FMUSP Faculdade de Medicina da Universidade de São Paulo


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