magda carneiro-sampaio, md, phd hospital das clínicas da faculdade de medicina universidade de são...
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Magda Carneiro-Sampaio, MD, PhDHospital das Clínicas da Faculdade de Medicina
Universidade de São Paulo (FMUSP), Brazil
Primary ImmunodeficienciesPrimary Immunodeficiencies
in 910 Brazilian patients in 910 Brazilian patients
of different age groups of different age groups
910 consecutive patients with well-defined PIDs* followed-up at Hospital das Clínicas da FMUSP and grouped according to age at diagnosis:
5 - 10 years old: 169
2 - 5 years old: 141
< 2 years old: 118 patients
10 - 20 years old: 137
20 - 30 years old: 118
> 30 years old: 227
*Notarangelo et al. IUIS Experts Committee PID Classification JACI ; 124:1161, 2009
60different PIDs were identified
Ethnicity of Brazilian people results from a mixture of native Indian, Portuguese and African descents, who have been merging since XVIth century
In the last 120 years Brazil received significant Italian, Syrian-Lebanese and Japanese immigration
Fotos de Priscila Oliveira, 2010
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
Distribution of HC-FMUSP PID patients (N = 910) according to age groups and the main IUIS PID categories
0%
20%
40%
60%
80%
100%
< 2 2-5 5-10 10-20 20-30 > 30 Allgroups
Age (years)
Antibody def Combined def Phagocyte Other Well-def syndrImmun Dysreg Complem Def Autoinflam syndr Innate immun
2,5%
Distribution of the <2 years old patients (N=118)according to the main IUIS PID categories
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
5%18%
(XLA, THI)
14%(DiGeorgeSyndrome)
21%(CGD)
2,5%
14%(HLH, IPEX)
25% (SCID)
1,5%
Distribution of the 2-5 years old patients (N=141)according to the main IUIS PID categories
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
8%1%
8%
3,5%14%
16%
48%(XLA, THI, IgAD)
Age (years)
Gender distribution in different age groups of HC-FMUSP PID series (N = 910)
0
20
40
60
80
100
%
< 2 2-5 5-10 10-20 20-30 > 30
Age (years)
F
M
Highly significant findings in the comparison between <5 years old X >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 deficiencies 13% 3% <0.001
Phagocyte number and/ or function defects
17% 5% <0.001
Immune dysregulation diseases 10% 2% <0.001
Male gender 71% 52% <0.0001
Hospital das Clínicas FMUSP
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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