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

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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REFERENCES

  1. 1

    Buckley R: Primary immunodeficiency diseases: Dissectors of the immune system. Immunol Rev 185:206–219, 2002

    PubMed  Article  CAS  Google Scholar 

  2. 2

    Lindegren ML, Kobrynski L, Rasmussen SA, Moore CA, Grosse SD, Vanderford ML, et al.: Applying public health strategies to primary immunodeficiency diseases: A potential approach to genetic disorders. MMWR Recomm Rep 53(RR-1):1–29, 2004

    PubMed  Google Scholar 

  3. 3

    Hayakawa H, Iwata T, Yata J, Kobayashi N: Primary immunodeficiency syndrome in Japan. I. Overview of a nationwide survey on primary immunodeficiency syndrome. J Clin Immunol 1(1):31–39, 1981

    PubMed  Article  CAS  Google Scholar 

  4. 4

    Luzi G, Businco L, Aiuti F: Primary immunodeficiency syndromes in Italy: A report of the National Register in Children and Adults. J Clin Immunol 3(4):316–320, 1983

    PubMed  Article  CAS  Google Scholar 

  5. 5

    Ryser O, Morell A, Hitzig W: Primary immunodeficiencies in Switzerland: First report of the National Registry in Adults and Children. J Clin Immunol 8(6):479–485, 1988

    PubMed  Article  CAS  Google Scholar 

  6. 6

    Matamoros F, Mila Llambi J, Espanol Boren T, Raga Borja S, Fontan Casariego G: Primary immunodeficiency syndrome in Spain: First report of the National Registry in children and adults. J Clin Immunol 17(4):333–339, 1997

    Article  Google Scholar 

  7. 7

    Lappalainen I, Ollila J, Smith C, Vihinen M: Registries of immuno deficiency patients and mutations. Hum Mutat 10(4):261–267, 1997

    PubMed  Article  CAS  Google Scholar 

  8. 8

    Baumgart K, Britton W, Kemp A, French M, Roberton D: The spectrum of primary immunodeficiency disorders in Australia. J Allergy Clin Immunol 100(3):415–423, 1997

    PubMed  Article  CAS  Google Scholar 

  9. 9

    Stray-Pedersen A, Abrahamsen T, Froland S: Primary immunodeficiency diseases in Norway. J Clin Immunol 20(6):477–485, 2000

    PubMed  Article  CAS  Google Scholar 

  10. 10

    Llambi M, Etxagobe G, Matamoros F: The Spanish Registry of Primary Immunodeficiencies (REDIP). Allergol Immunopathol 29(3):122–125, 2001

    Google Scholar 

  11. 11

    Samarghitean C, Valiaho J, Vihinen M: Online registry of genetic and clinical immunodeficiency diagnostic laboratories, IDiagnostics. J Clin Immunol 24(1):53–61, 2004

    PubMed  Article  Google Scholar 

  12. 12

    Zelazco M, Carneiro-Sampaio M, Cornejo de Luigi M, Garcia de Olarte D, Porras Madrigal O, Berrón Perez R, et al.: Primary immunodeficiency diseases in Latin America: First report from eight countries participating in the LAGID. J Clin Immunol 18(2):161–166, 1998

    Article  Google Scholar 

  13. 13

    Bureau UC: International Data Base. Washingon, DC, 2006

  14. 14

    Organization WH: Primary immunodeficiency diseases. Report of a WHO Scientific Group. Clin Exp Immunol 99(Suppl 1):1–24, 1995

    Google Scholar 

  15. 15

    ESID. European Society for Immunodeficiencies [Website], 2006

  16. 16

    Notarangelo L, Casanova J-L, Conley M, Chapel H, Fischer A, Puck J, et al.: Primary immunodeficiency diseases: An update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting in Budapest. J Allergy Clin Immunol 117:883–896, 2006

    PubMed  Article  Google Scholar 

  17. 17

    Carneiro-Sampaio M, Carbonare S, Sezentraum R, Araujo M, Ribeiro M, Porto M: Frequency of selective IgA deficiency among Brazilian blood donors and healthy pregnant women. Allergol Immunopathol 17:213–216, 1989

    CAS  Google Scholar 

  18. 18

    Nunez R: Primary immunodeficiency in Colombian children. Allergol Immunopathol 16(4):273–275, 1988

    CAS  Google Scholar 

  19. 19

    Craviotto R, Feldman G, Giraudi V, Oleastro M, Pérez N, Rivas M, et al.: Immunodeficiencias primarias: Informe del Registro Argentino. Arch Argent Pediatr 99(3):263–268, 2001

    Google Scholar 

  20. 20

    Lim D, Thong B, Ho S, Shek L, Lou J, Leong K, et al.: Primary immunodeficiency diseases in Singapore—The last 11 years. Singapore Med J 44(11):579–586, 2003

    PubMed  CAS  Google Scholar 

  21. 21

    Aghamohammadi A, Moein M, Farhoudi A, Pourpak Z, Rezaei N, Abolmaali K, et al.: Primary immunodeficiency in Iran: First report of the National Registry of PID in Children and Adults. J Clin Immunol 22(6):375–380, 2002

    PubMed  Article  Google Scholar 

  22. 22

    Urrejola P, Cattani A, Heusser F, Talesnik E: Sindrome deDiGeorge. Rev Child Pediatr 62:381–385, 1991

    CAS  Google Scholar 

  23. 23

    Passero L, Contreras P, Gonzalez B, Carrion F, Navarrete C, Mendez C, et al.: Características clínicas e inmunológicas de veinte pacientes con síndrome de DiGeorge. Rev Esp Pediatr 61:201–206, 2005

    Google Scholar 

  24. 24

    Patiño PJ, Perez JE, Lopez JA, Condino-Neto A, Grumach A, Botero JH, et al.: Molecular analysis of chronic granulomatous disease caused by defects in gp91-phox. Hum Mutat 13:29–37, 1999

    PubMed  Article  Google Scholar 

  25. 25

    Agudelo-Florez P, Prando-Andrade CC, Lopez JA, Costa-Carvalho BT, Quezada A, Espinosa FJ, et al.: Chronic granulomatous disease in Latin American patients: Clinical spectrum and molecular genetics. Pediatr Blood Cancer 46(2):243–252, 2006

    PubMed  Article  Google Scholar 

  26. 26

    Danielian S, El-Hakeh J, Basilico G, Oleastro M, Rosenzweig S, Feldman G, et al.: Bruton tyrosine kinase gene mutations in Argentina. Hum Mutat 21(4):451–456, 2003

    PubMed  Article  Google Scholar 

  27. 27

    El-Hakeh J, Rosenzweig S, Oleastro M, Basack N, Berozdnik L, Molina F, et al.: Wiskott–Aldrich syndrome in Argentina: 17 unique, including nine novel, mutations. Hum Mutat 19(2):186–187, 2002

    PubMed  Article  Google Scholar 

  28. 28

    Winkelstein JA, Marino MC, Johnston RB Jr, Boyle J, Curnutte J, Gallin JI, et al.: Chronic granulomatous disease. Report on a National Registry of 368 patients. Medicine (Baltimore) 79(3):155–169, 2000

    Article  CAS  Google Scholar 

  29. 29

    Smith C, Witte O: X-linked agammaglobulinemia: A disease of Btk tyrosine kinase. In Primary Immunodeficiency Diseases, H Ochs, C Smith, J Puck (eds). New York, Oxford University Press, 1999, pp 263–284

    Google Scholar 

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Correspondence to LILY E. LEIVA.

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Latin American Group for Primary Immunodeficiency Diseases

APPENDIX

APPENDIX

In addition to the authors, the following collaborators participated in this study. Argentina: Bozzola C, Garip E, Krasovec S, Miño O, Orellana J, Sierra de Criscuolo A, Gambarte L. Brazil: de Moraes Vasconcelos D, da Silva Duarte AJ. Colombia: Ortega MC. Honduras: Almendarez C. Peru: Vega H.

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LEIVA, L.E., ZELAZCO, M., OLEASTRO, M. et al. Primary Immunodeficiency Diseases in Latin America: The Second Report of the LAGID Registry. J Clin Immunol 27, 101–108 (2007). https://doi.org/10.1007/s10875-006-9052-0

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KEY WORDS

  • Primary immunodeficiency
  • Latin America
  • LAGID
  • Immunodeficiency epidemiology