**Section 8**

**Epidemiology of Primary Immunodeficiency Diseases** 

354 Epidemiology Insights

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**17** 

*Brazil* 

**Primary Immunodeficiency Diseases in Latin** 

Primary immunodeficiencies (PID) are genetic disorders of immunity whose incidence varies from 1:250 to 1:1,000,000 depending of disease and study population. Because of incomplete records of immunodeficiency in the world, is estimated that the prevalence of 1:5,000 to 1:100,000 (Geha *et al.,* 2007; Boyle & Buckley, 2007; Notarangelo,*et al.,* 2010). Thus, the PID is a important group to public health as other genetic diseases that rely government support in a neonatal screening program such phenylketonuria (PKU) with incidence of 1/15.000 (de Carvalho *et al.*, 2007) and congenital hypothyroidism, with incidence of 1/4.000 (American Academy of Pediatrics, 1993; Olivieri, 2009). The PID are classified in defective immune deficiency prevalent in predominantly antibody; combined immunodeficiencies, cellular immunodeficiencies, phagocyte defects, immune deficiencies associated with lymphoproliferative diseases, and deficiencies of complement system or secondary immunodeficiencies associated with other diseases. This classification is updated periodically by the International Union of Immunological Societies (IUIS), associated with

the World Health Organization (WHO) (Geha *et al.,* 2007; Notarangelo,*et al.,* 2010).

dysplasia with immunodeficiency) have emerged as reflecting new PID.

This classification is progressively adjusting to the rapid evolution of the field. Many new phenotypes (e.g. hemophagocytosis, thrombotic purpura, herpes encephalitis, Mendelian susceptibility to mycobacterial infection, epidermodysplasia verruciformis, chronic mucocutaneous candidiasis, autoinflammatory disorders, and anhidrotic ectodermal

Knowledge of PID is still deficient in many countries and within many countries, since doctors and health authorities are often poorly informed about their clinical presentations, diagnosis, importance and health impact of these diseases, and geographic factors that influence the release of same around the world (Sewell, 2006). Recent estimates of PID made by the European Parliament showed that approximately 1 in 800 to 10,000 people have PID that significantly affects your health, PID affect at least 10 million people worldwide, the true prevalence of PID in some forms of general population is estimated between 1 in 250 and 1 in 500, data comparable with type I diabetes (1 in 700) and multiple sclerosis (1 in

**1. Introduction** 

 \*

Corresponding Author

**America: Epidemiology and Perspectives** 

Paolo Ruggero Errante and Antonio Condino-Neto\* *Department of Immunology, Institute of Biomedical Sciences* 

*University of São Paulo, São Paulo* 
