**2.1 Argentina**

360 Epidemiology Insights

conducted between 1996 to 2002 (Seymour *et al.,* 2005). To be assured diagnostic quality indicators should be generated which include patient registration data, which together form the field and field set and its variables called record. These cases are based registry designed to improve patient care, but are useful for studying diseases. An example is early records of 368 patients with chronic granulomatous disease (GCD) by American Foundation for Immunodeficiencies started in 1993, allowing a calculation of incidence of the disease for USA born in 1/200.000 (Winkelstein *et al.,* 2000). In 1997, this study has been expanded to Common Variable Immunodeficiency (CVID), Wiskott-Aldrich Syndrome (WAS), Severe Combined Immunodeficiency (SCID), Leukocyte Adhesion Deficiency (LAD), DiGeorge Syndrome, Hyper-IgM Syndrome (HIGM) (Winkelstein *et al.,* 2003) and X-Linked Agammaglobulinemia (XLA) (Winkelstein *et al.,* 2006), showing concern in development systems of records, population data for estimating incidence, prevalence and characteristics of disease. These data are important to maintain epidemiological studies and research and design of new clinical trials, reducing mortality, increasing survival and improved quality of life. In Europe, this service is performed by European Society for Immunodeficiency-ESID non-governmental organization that aims, to facilitate exchange of information between doctors, nurses, researchers, patients and their families, promote research into causes, mechanisms and treatment of PID (Sewel *et al.,* 2006; de Vries, 2006; Guzman *et al.,* 2007; Gathmann *et al.,* 2009). This group was established in 1993, Society in 1994, and receives data from 66 specialized centers in 26 European countries. In 2005 record showed amount of 10,000 patients from 26 countries with prevalence of 4-47/1.000.000, and the European internet-based patient and research database for primary immunodeficiencies results in 7,047 patients with PID in 30 countries (Knerr *et al.,*2008) and 7430 patients from 39 countries

have been documented in the ESID database (Gathmann *et al.,* 2009).

Gaspar, 2000) and Newcastle General Hospital (Slatter & Gennery, 2010).

In Latin America in 1993, was created the Latin American Group for Immunodeficiencies (LAGID) to study the prevalence of PID in different regions of Latin America and promote awareness of these diseases. There are also databases online with identification of mutations locus in specific cases of PID that are established by ESID and extended by others researchers. This was initiated in 1995 to collect data in Bruton's tyrosine kinase mutations (BTK) in XLA (Lindegren *et al.,* 2004). This occurred in Brazil, through the creation of Brazilian Group of Primary Immunodeficiencies (BRAGID), which through information campaigns, increased the number of 314 cases in 2000 to 536 cases in 2004 with Southeast region responsible for 70% total cases. Another relevant factor in understanding the epidemiology of PID is introduction of specialized care networks. In England there is the National Specialist Advisory Group Comissionary (NSCAG) of National Health System (NHS), which tracks PID complex cases, such the Great Ormond Street Hospital (Jones &

Several international initiatives are currently underway to promote awareness PID and increase number of diagnosis and registration of PID (http://www.primaryimmune.org/ resources/resources.htm) (Modell, 2007a; Modell, 2007b; Pickett *et al.,* 2008). Considering the increasingly frequent recognition of these diseases and their valuable contribution to understanding human immune system and mechanisms of defense against infections (more frequent medical care in worldwide) (Alcais *et al.,* 2009), it is essential establish the frequency and type in population (Casanova & Abel, 2007; Ballow *et al.,* 2009). The lack of proper diagnosis and treatment are the main problems in patients with PID in Latin America. These are related to lack of proper training of pediatrics, leading to misdiagnosis or late diagnosis, lack of proper screening, lack of government resources to implementation The main diagnostic centers in Argentina are located in Buenos Aires, and other centers are located in La Plata, Rosario, Cordoba and Mendoza. These centers are accessible and without cost to the community, provided that patients are referred by doctor. Private hospitals in Argentina offer only partial diagnosis, because no they have laboratories and professionals specialized in PID. Patients in Paraguay, Bolivia and Uruguay are also diagnosed and treated in Buenos Aires since these countries do not have adequate support. As their countries do not encourage their costs, or they do not have health insurance, this burden financially immunology centers in Argentina (Maceira *et al.,* 2010). Patients with PID who need IVIG are usually met in Buenos Aires, and treatment is not automatically continued in the pediatric patient who came into adults, it should be transferred from pediatric hospital to hospital for adults, interrupting treatment (Krasovec *et al.,* 2007). In Argentina, immunologists are not recognized experts by the Ministry of Health, and only two hospitals in Buenos Aires offer scholarships programs and post-graduate training in immunology funded by government agencies (Galicchio *et al.,* 2010; Condino-Neto *et al.,* 2011).
