1887
Volume 1998, Issue 2
  • ISSN: 0253-8253
  • EISSN: 2227-0426

Abstract

Abstract

Omenn syndrome is a rare form of severe combined immune deficiency (SCID) with distinctive clinical and immunologic features(1). It was first described by Omenn in 1965 under the title of: Familial Reticuloen-Dotheliosis

with Eosinophilia(2),. Since then, over 45 cases has been described(1). In this paper; we are reporting the first case of Omenn's syndrome in Qatar community.

Loading

Article metrics loading...

/content/journals/10.5339/qmj.1998.2.21
2014-04-01
2024-03-28
Loading full text...

Full text loading...

References

  1. Gomez L., Le Deist F., Blanche S.;. et al.: Treatment of Omenn Syndrome by bone marrow transplantation. J Pediatrics 127::1995:7681.
    [Google Scholar]
  2. Omenn G.S: Familial reticuloendotheliosis with eosinophilia. New Engl J Med 273::1965:427432.
    [Google Scholar]
  3. Cederbaum S.D., Niwayama G., Stiehm E.R., et al.: Combined immune deficiency presenting as Letterer-Siwe Syndrome. J Pediatrics 85::1974:466471.
    [Google Scholar]
  4. Wyss M., Von Flienner V., Jacot-des-Combes E., et al.: A Lympho-proliferative Syndrome Cutaneous dystrophy and combined immune deficiency with lack of helper T-cell factor. Clin Immunol Immunopathol 23::1982:3449.
    [Google Scholar]
  5. Barth R.F., Vergara G.G., Khurana S.K., Lowman I.T.: Rapidly fatal histiocytosis associated with eosinophilia and primary immunological deficiency. Lancet 2::1972:503506.
    [Google Scholar]
  6. Ammann A.J.: Combined antibody (B-cells) and cellular (Tcells) immune deficiency disorders in basic and clinical immunology. Stites D.P., Terr A.J., Appleton & Lange, Norwalk, Connecticut/San Mateo1991:347355. California.
    [Google Scholar]
  7. Vossbeck S., Knobloch C., Heymer B., et al.: Severe combined immune defect. Presentation of exfoliative dermatitis with eosinophilia and lymphadenopathy. Monatsschr Kinderheilkd 140::1992:188193.
    [Google Scholar]
  8. Tachinami T., Koizumi S., et al.: Yachie.Immune status in two brothers with Omenn's Syndrome: No discernible chimerism on FACS analysis using a monoclonal antibody specific for a maternally restricted HLA antigen. Am J Pediatr Hematol Oncol 12::1990:343350.
    [Google Scholar]
  9. Appleton A.L., Curtis A., Wilken J., et al.: Differentiation of materno-fetal GVHD from Omenn's Syndrome in pre-BMT patients with severe combined immune deficiency. Bone Marrow Transplantation 14::1994:157159.
    [Google Scholar]
  10. Romagrani S.:.: Thl and Th2 in human diseases. Clin Immunol Immunopathol 80::1996:225235.
    [Google Scholar]
  11. De Saint-Basile G., Le Deist F., de Villartay J.P., et al.: Restricted heterogenity of T-lymphocytes in combined immune deficiency with hyper eosinophilia (Omenn's syndrome). J Clin Invest 87::1991:13521359.
    [Google Scholar]
  12. Cavazzana-Calvo M., Le Deist F., De Saint-Basile G.;., et al.: Increased radio sensitivity of granulocyte macrophage colony-forming units and skin fibroblasts in human autosomal recessive severe combined immune deficiency. J Clin Ivest 91::1993:12141218.
    [Google Scholar]
  13. Le Deist F., de Saint-Basile G., Mazerolles F.;., et al.: Primary membrane T-cell immune deficiencies. Clin Immunol Immunopathol 61::1991:550560.
    [Google Scholar]
http://instance.metastore.ingenta.com/content/journals/10.5339/qmj.1998.2.21
Loading
  • Article Type: Case Report
This is a required field
Please enter a valid email address
Approval was a Success
Invalid data
An Error Occurred
Approval was partially successful, following selected items could not be processed due to error