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Journal of Clinical Immunology

, Volume 27, Issue 1, pp 101–108 | Cite as

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

  • LILY E. LEIVAEmail author
  • MARTA ZELAZCO
  • MATÍAS OLEASTRO
  • MAGDA CARNEIRO-SAMPAIO
  • ANTONIO CONDINO-NETO
  • BEATRIZ TAVARES COSTA-CARVALHO
  • ANETE SEVCIOVIC GRUMACH
  • ARNOLDO QUEZADA
  • PABLO PATIÑO
  • JOSÉ LUIS FRANCO
  • OSCAR PORRAS
  • FRANCISCO JAVIER RODRÍGUEZ
  • FRANCISCO JAVIER ESPINOSA-ROSALES
  • SARA ELVA ESPINOSA-PADILLA
  • DIVA ALMILLATEGUI
  • CELIA MARTÍNEZ
  • JUAN RODRÍGUEZ TAFUR
  • MARILYN VALENTÍN
  • LORENA BENARROCH
  • ROSY BARROSO
  • RICARDO U. SORENSEN
OriginalArticle

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.

KEY WORDS

Primary immunodeficiency Latin America LAGID Immunodeficiency epidemiology 

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Copyright information

© Springer Science+Business Media, LLC 2006

Authors and Affiliations

  • LILY E. LEIVA
    • 1
    • 17
    Email author
  • MARTA ZELAZCO
    • 2
  • MATÍAS OLEASTRO
    • 2
  • MAGDA CARNEIRO-SAMPAIO
    • 3
  • ANTONIO CONDINO-NETO
    • 3
  • BEATRIZ TAVARES COSTA-CARVALHO
    • 4
  • ANETE SEVCIOVIC GRUMACH
    • 5
  • ARNOLDO QUEZADA
    • 6
  • PABLO PATIÑO
    • 7
  • JOSÉ LUIS FRANCO
    • 7
  • OSCAR PORRAS
    • 8
  • FRANCISCO JAVIER RODRÍGUEZ
    • 9
  • FRANCISCO JAVIER ESPINOSA-ROSALES
    • 10
  • SARA ELVA ESPINOSA-PADILLA
    • 10
  • DIVA ALMILLATEGUI
    • 11
  • CELIA MARTÍNEZ
    • 12
  • JUAN RODRÍGUEZ TAFUR
    • 13
  • MARILYN VALENTÍN
    • 14
  • LORENA BENARROCH
    • 15
  • ROSY BARROSO
    • 16
  • RICARDO U. SORENSEN
    • 1
  1. 1.Department of PediatricsLSU Health Sciences CenterNew OrleansUSA
  2. 2.Hospital Nacional de Pediatría “Juan P. Garrahan”Buenos AiresUSA
  3. 3.Instituto de Ciências Biomédicas da Universidade de São PauloSão PauloBrazil
  4. 4.Departamento de Pediatria da UNIFESP-EPMSão PauloBrazil
  5. 5.Department of DermatologyUniversity of Sao Paulo, Primary immunodeficiency Outpatient GroupSao PauloBrazil
  6. 6.Departamento de PediatriaFacultad de Medicina, Universidad de ChileSantiagoChile
  7. 7.Facultad de MedicinaUniversidad de AntioquiaMedellínColombia
  8. 8.Hospital Nacional de Niños “Dr. Carlos Saenz Herrera”San JoseCosta Rica
  9. 9.Departamento de PediatríaInstituto Nacional de Seguridad SocialTegucigalpaHonduras
  10. 10.Instituto Nacional de PediatríaMexico CityMexico
  11. 11.Servicio de Inmunología y AlergiaHospital de Niños de PanamáEl DoradoPanamá
  12. 12.Instituto de Investigación de Ciencias de la SaludAsunciónParaguay
  13. 13.Facultad de MedicinaUniversidad Nacional Mayor de San MarcosLimaPeru
  14. 14.Hospital Pediátrico “Pereira Rossell”MontevideoUruguay
  15. 15.Hospital de ClínicasCaracasVenezuela
  16. 16.Clínica “Leopoldo Aguerrevere”CaracasVenezuela
  17. 17.The Research Institute for ChildrenChildren’s HospitalNew OrleansUSA

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