Transcript Slide 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
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
 5 - 10 years old: 169
 10 - 20 years old: 137
60
different PIDs
were identified
 20 - 30 years old: 118
 > 30 years old: 227
*Notarangelo et al. IUIS Experts Committee PID Classification
JACI ; 124:1161, 2009
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
Other Well defined PID syndromes
Complement deficiencies
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
100%
80%
60%
40%
20%
0%
<2
2-5
5-10
10-20
20-30
> 30
All
groups
Age (years)
Antibody def
Immun Dysreg
Combined def
Complem Def
Phagocyte
Autoinflam syndr
Other Well-def syndr
Innate immun
Distribution of the <2 years old patients (N=118)
according to the main IUIS PID categories
14%
(HLH, IPEX)
2,5% 5%
Predominantly Antibody Deficiencies
18%
(XLA, THI)
Combined T + B deficiencies
Phagocyte number and/ or function def
Complement deficiencies
14%
(DiGeorge
Syndrome)
25%
(SCID)
Other Well defined PID syndromes
Immune dysregulation diseases
Innate immunity defects
2,5%
Autoinflammatory disorders
21%
(CGD)
Distribution of the 2-5 years old patients (N=141)
according to the main IUIS PID categories
Predominantly Antibody Deficiencies
8%
1%
Combined T + B deficiencies
1,5%
16%
Phagocyte number and/ or function def
48%
(XLA, THI, IgAD)
Complement deficiencies
Other Well defined PID syndromes
8%
Immune dysregulation diseases
Innate immunity defects
Autoinflammatory disorders
14%
3,5%
Gender distribution in different age groups
of HC-FMUSP PID series (N = 910)
100
%
80
F
60
M
40
20
0
<2
2-5
5-10
10-20
20-30
Age (years)
Age (years)
> 30
Highly significant findings in the comparison
between <5 years old X >5 years old PID groups
Frequency %
<5 years old
( N=259)
>5 years old
(N= 651)
p
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
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
Faculdade de Medicina da Universidade de São Paulo