Primary Immunodeficiency Diseases in Latin America: The Second Report of the LAGID Registry

Springer Science and Business Media LLC - Tập 27 - Trang 101-108 - 2006
LILY E. LEIVA1,2, MARTA ZELAZCO3, MATÍAS OLEASTRO3, MAGDA CARNEIRO-SAMPAIO4, ANTONIO CONDINO-NETO4, BEATRIZ TAVARES COSTA-CARVALHO5, ANETE SEVCIOVIC GRUMACH6, ARNOLDO QUEZADA7, PABLO PATIÑO8, JOSÉ LUIS FRANCO8, OSCAR PORRAS9, FRANCISCO JAVIER RODRÍGUEZ10, FRANCISCO JAVIER ESPINOSA-ROSALES11, SARA ELVA ESPINOSA-PADILLA11, DIVA ALMILLATEGUI12, CELIA MARTÍNEZ13, JUAN RODRÍGUEZ TAFUR14, MARILYN VALENTÍN15, LORENA BENARROCH16, ROSY BARROSO17, RICARDO U. SORENSEN1
1Department of Pediatrics, LSU Health Sciences Center, New Orleans, USA
2The Research Institute for Children, Children’s Hospital, New Orleans, USA
3Hospital Nacional de Pediatría “Juan P. Garrahan”, Buenos Aires, USA
4Instituto de Ciências Biomédicas da Universidade de São Paulo, São Paulo, Brazil
5Departamento de Pediatria da UNIFESP-EPM, São Paulo, Brazil
6Department of Dermatology, University of Sao Paulo, Primary immunodeficiency Outpatient Group, Sao Paulo, Brazil
7Departamento de Pediatria, Facultad de Medicina, Universidad de Chile, Santiago, Chile
8Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia
9Hospital Nacional de Niños “Dr. Carlos Saenz Herrera”, San Jose, Costa Rica
10Departamento de Pediatría, Instituto Nacional de Seguridad Social, Tegucigalpa, Honduras
11Instituto Nacional de Pediatría, Mexico City, Mexico
12Servicio de Inmunología y Alergia, Hospital de Niños de Panamá, El Dorado, Panamá
13Instituto de Investigación de Ciencias de la Salud, Asunción, Paraguay
14Facultad de Medicina, Universidad Nacional Mayor de San Marcos, Lima, Peru
15Hospital Pediátrico “Pereira Rossell”, Montevideo, Uruguay
16Hospital de Clínicas, Caracas, Venezuela
17Clínica “Leopoldo Aguerrevere”, Caracas, Venezuela

Tóm tắt

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.

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