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Title: Primary immunodeficiency diseases in Latin America: the second report of the LAGID registry
Authors: Leiva, Lily E.
Zelazco, Marta
Oleastro, Matias
Carneiro-Sampaio, Magda
Condino-Neto, Antonio
Costa-Carvalho, Beatriz Tavares
Grumach, Anete Sevciovic
Quezada, Arnoldo
Patino, Pablo
Franco, Jose Luis
Porras, Oscar
Rodriguez, Francisco Javier
Espinosa-Rosales, Francisco Javier
Espinosa-Padilla, Sara Elva
Almillategui, Diva
Martinez, Celia
Tafur, Juan Rodriguez
Valentin, Marilyn
Benarroch, Lorena
Barroso, Rosy
Sorensen, Ricardo U.
Louisiana State Univ
Hosp Nacl Ped Juan P Garrahan
Universidade de São Paulo (USP)
Universidade Federal de São Paulo (UNIFESP)
Univ Chile
Univ Antioquia
Hosp Nacl Ninos Dr Carlos Saenz Herrera
Inst Nacl Seguridad Social
Inst Nacl Pediat
Hosp Ninos Panama
Inst Invest Ciencias Salud
Univ Nacl Mayor San Marcos
Hosp Pediatr Pereira Rossell
Hosp Clin
Clin Leopoldo Aguerrevere
Keywords: primary immunodeficiency
Latin America
immunodeficiency epidemiology
Issue Date: 1-Jan-2007
Publisher: Springer
Citation: Journal of Clinical Immunology. New York: Springer/plenum Publishers, v. 27, n. 1, p. 101-108, 2007.
Abstract: This is the second report on the continuing efforts of LAGID to increase the recognition and registration of patients with primary immunodeficiency diseases in 12 Latin American countries: Argentina, Brazil, Chile, Colombia, Costa Rica, Honduras, Mexico, Panama, Paraguay, Peru, Uruguay, and Venezuela. This report reveals that from a total of 3321 patients registered, the most common form of primary immunodeficiency disease was predominantly antibody deficiency (53.2%) with IgA deficiency reported as the most frequent phenotype. This category was followed by 22.6% other well-defined ID syndromes, 9.5% combined T- and B-cell inmunodeficiency, 8.6% phagocytic disorders, 3.3% diseases of immune dysregulation, and 2.8% complement deficiencies. All countries that participated in the first publication in 1998 reported an increase in registered primary immunodeficiency cases, ranging between 10 and 80%. A comparison of the estimated minimal incidence of X-linked agammaglobulinemia, chronic granulomatous disease, and severe combined immunodeficiency between the first report and the present one shows an increase in the reporting of these diseases in all countries. in this report, the estimated minimal incidence of chronic granulomatous disease was between 0.72 and 1.26 cases per 100,000 births in Argentina, Chile, Costa Rica, and Uruguay and the incidence of severe combined immunodeficiency was 1.28 and 3.79 per 100,000 births in Chile and Costa Rica, respectively. However, these diseases are underreported in other participating countries. in addition to a better diagnosis of primary immunodeficiency diseases, more work on improving the registration of patients by each participating country and by countries that have not yet joined LAGID is still needed.
ISSN: 0271-9142
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