Journal of Clinical Immunology

, Volume 8, Issue 6, pp 479–485

Primary immunodeficiencies in Switzerland: First report of the national registry in adults and children

  • O. Ryser
  • A. Morell
  • W. H. Hitzig
Original Articles

Abstract

This first report of a Swiss registry includes 313 patients with primary immunodeficiency syndromes (PIDS) who were observed between January 1975 and January 1985. Diagnosis of specific PIDS was made according to WHO criteria. The most frequent disorders were IgA deficiency (33%) and common variable immunodeficiency (22%), followed by selective deficiency of other immunoglobulin isotypes (9%), severe combined immunodeficiency (9%), infantile sex-linked agammaglobulinemia (7%), and Wiskott-Aldrich syndrome (6%). Frequencies of other types of PIDS varied between 0.3 and 4%. Half of the patients were in the pediatric age group. Male patients predominated (63%). In addition to respiratory and urogenital tract infections, autoimmune disorders were observed in 14 patients with IgA deficiency or common variable immunodeficiency. IgA deficiency was, furthermore, associated with atopic and neurological disorders. A comparison with other national registries revealed some differences: the frequency of severe combined immunodeficiency was high (incidence, 24.3 cases per 106 live births), and that of ataxia teleangiectasia was particularly low (1.4 per 106 live births) in Switzerland. Frequencies of the three major PIDS groups of (i) predominantly antibody defects, (ii) predominantly cell-mediated defects, and (iii) PIDS associated with other major defects agreed with those reported in the other European studies.

Key words

Primary immunodeficiency in Switzerland 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Rosen FS, Cooper MD, Wedgwood RJP: The primary immunodeficiencies. N Engl J Med 311:235–242, 300–310, 1984Google Scholar
  2. 2.
    Conley ME, Brown P, Pickard AR, Buckley RH, Miller DS, Raskind WH, Singer JW, Fialkow PJ: Expression of the gene defect in X-linked agammaglobulinemia. N Engl J Med 315:564–567, 1986Google Scholar
  3. 3.
    Hayakawa H, Jwata T, Kobayashi N: Primary Immunodeficiency syndrome in Japan. I. Overview of a national-wide survey on primary immunodeficiency. J Clin Immunol 1:31–39, 1981Google Scholar
  4. 4.
    Hayakawa H, Matsumoto S, Kabayashi N, Yata J: Genetically determined immunodeficiencies.In The Nature, Cellular, and Biochemical Basis and Management of Immunodeficiencies, Symposia Medica Hoechst 21, RA Good, E Lindenlaub (eds). Stuttgart, New York, Schattauer Verlag, 1987, pp 39–60Google Scholar
  5. 5.
    Fasth A: Primary immunodeficiency disorders in Sweden: Cases among children 1974–1979. J Clin Immunol 2:86–92, 1982Google Scholar
  6. 6.
    Fasth A: Immunodeficiency in children in Sweden 1974–1983. From the Swedish register of primary immunodeficiency disorders.In Progress in Immunodeficiency Research and Therapy, Vol I, C Griscelli, J Vossen (eds), Amsterdam, New York, Elsevier, 1984, pp 461–467Google Scholar
  7. 7.
    Luzi G, Businco L, Aiuti F: Primary immunodeficiency syndromes in Italy: A report of the National Register in children and adults. J Clin Immunol 3:316–321, 1983Google Scholar
  8. 8.
    World Health Organization: Report of a scientific group on immunodeficiency. Clin Immunol Immunopathol 28:450–475, 1983Google Scholar
  9. 9.
    Bundesamt für Statistik: Statistisches Jahrbuch der Schweiz. Basel, Birkhäuser Verlag, 1984Google Scholar
  10. 10.
    Morell A, Muehlheim E, Schaad U, Skvaril F, Rossi E: Susceptibility to infections in children with selective IgA-and IgA-IgG subclass deficiency. Eur J Pediatr 145:199–203, 1986Google Scholar
  11. 11.
    Fontana A, Grob PJ: Immunoglobulin deficiency, HLA antigens and seizures. Ann Intern Med 88:429, 1978Google Scholar
  12. 12.
    Clark, JA, Callicoat PA, Brenner NA, Bradley CA, Smith DM: Selective IgA deficiency in blood donors. Am J Clin Pathol 80:210–213, 1983Google Scholar
  13. 13.
    Kanoh T, Mizumoto T, Yasuda N, Koya M, Ohno Y, Uchino H, Yoshimura K, Ohkubo Y, Yamaguchi H: Selective IgA deficiency in Japanese blood donors: Frequency and statistical analysis. Vox Sang 50:81–86, 1986Google Scholar
  14. 14.
    Kinlen LJ, Webster ADB, Bird AG, Haile R, Soothill JF, Thompson RA: Prospective study of cancer in patients with hypogammaglobulinemia. Lancet 1:263–266, 1985Google Scholar
  15. 15.
    Filipovich AH, Spector BD, Kersey J: Immunodeficiency in humans as a risk factor in the development of malignancy. Prev Med 9:252–259, 1980Google Scholar
  16. 16.
    Spector BD, Perry GS III, Kersey JH: Genetically determined immunodeficiency disease (GDID) and malignancy: Report from the immunodeficiency-cancer registry. Clin Immunol Immunopathol 11:12–29, 1978Google Scholar
  17. 17.
    Cunningham-Rundles C, Siegal FP, Smithwick EM, Lion-Boulé A, Cunningham-Rundles S, O'Malley J, Barandun S, Good RA: Efficacy of intravenous immunoglobulin in primary humoral immunodeficiency disease. Ann Intern Med 101:435–439, 1984Google Scholar
  18. 18.
    O'Reilly RJ, Brochstein J, Dinsmore R, Kirkpatrick D: Morrow transplantation for congenital disorders. Semin Hematol 21:188–221, 1984Google Scholar
  19. 19.
    Fischer A, Griscelli C, Friedrich W, Kubanek B, Levinsky R, Morgan G, Vossen J, Wagemaker G, Landais P: Bone marrow transplantation for immunodeficiencies and osteopetrosis: European survey, 1968–1985. Lancet 2:1080–1084, 1986Google Scholar
  20. 20.
    Beatty PG, Clift RA, Michelson EM, Nisperos BB, Flournoy N, Martin PJ, Sanders JE, Stewart P, Buckner CD, Storb R, Thomas ED, Hansen JA: Marrow transplantation from related donors other than HLA-identical siblings. N Engl J Med 313:765–771, 1985Google Scholar
  21. 21.
    Friedrich W, Goldmann SF, Kleihauer E: Treatment of primary immunodeficiencies by bone marrow transplantation.In The Nature, Cellular, and Biochemical Basis and Management of Immunodeficiencies, Symposia Medica Hoechst 21, RA Good, E Lindenlaub (eds). Stuttgart, New York, Schattauer Verlag, 1987, pp 95–105Google Scholar

Copyright information

© Plenum Publishing Corporation 1988

Authors and Affiliations

  • O. Ryser
    • 1
  • A. Morell
    • 1
  • W. H. Hitzig
    • 2
  1. 1.Institute for Clinical and Experimental Cancer ResearchUniversity of BerneBerneSwitzerland
  2. 2.Department of PediatricsUniversity of ZurichZurichSwitzerland

Personalised recommendations