title: Primary immunodeficiency diseases in Latin America: the second report of the LAGID registry.
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.
authors.affiliation: Department of Pediatrics, LSU Health Sciences Center, New Orleans, Louisiana, USA. lleiva@lsuhsc.edu
authors.forename: Lily E
authors.initials: LE
authors.lastname: Leiva
authors.forename: Marta
authors.initials: M
authors.lastname: Zelazco
authors.forename: Matías
authors.initials: M
authors.lastname: Oleastro
authors.forename: Magda
authors.initials: M
authors.lastname: Carneiro-Sampaio
authors.forename: Antonio
authors.initials: A
authors.lastname: Condino-Neto
authors.forename: Beatriz Tavares
authors.initials: BT
authors.lastname: Costa-Carvalho
authors.forename: Anete Sevciovic
authors.initials: AS
authors.lastname: Grumach
authors.forename: Arnoldo
authors.initials: A
authors.lastname: Quezada
authors.forename: Pablo
authors.initials: P
authors.lastname: Patiño
authors.forename: José Luis
authors.initials: JL
authors.lastname: Franco
authors.forename: Oscar
authors.initials: O
authors.lastname: Porras
authors.forename: Francisco Javier
authors.initials: FJ
authors.lastname: Rodríguez
authors.forename: Francisco Javier
authors.initials: FJ
authors.lastname: Espinosa-Rosales
authors.forename: Sara Elva
authors.initials: SE
authors.lastname: Espinosa-Padilla
authors.forename: Diva
authors.initials: D
authors.lastname: Almillategui
authors.forename: Celia
authors.initials: C
authors.lastname: Martínez
authors.forename: Juan Rodríguez
authors.initials: JR
authors.lastname: Tafur
authors.forename: Marilyn
authors.initials: M
authors.lastname: Valentín
authors.forename: Lorena
authors.initials: L
authors.lastname: Benarroch
authors.forename: Rosy
authors.initials: R
authors.lastname: Barroso
authors.forename: Ricardo U
authors.initials: RU
authors.lastname: Sorensen
mesh_terms: D001723:Birth Rate; D003625:Data Collection; D003710:Demography; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000145:classification / Q000453:epidemiology* / Q000276:immunology; D007843:Latin America / Q000453:epidemiology; D008297:Male; D010641:Phenotype; D015995:Prevalence; D012042:Registries; D011795:Surveys and Questionnaires