Omenn’s Syndrome : A rare primary immunodeficiency disorder

Ibtisam B Elnour, Shakeel Ahmed, Kamal Halim, V Nirmala

Abstract


Over the last 17 years different forms of severe combined immunodeficiency have been diagnosed at Sultan Qaboos University Hospital, Muscat. Omenn’s syndrome is a rare autosomal recessive form of severe combined immunodeficiency. We report a 6 weeks old Omani infant who presented with the characteristic clinical and immunological phenotype of Omenn’s syndrome. We take the opportunity to discuss and review the immunological aspect of this rare syndrome. 



Keywords


Omenn’s Syndrome; Immunodeficiency, severe, combined; Immunodeficiency, primary; Case report; Oman.

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Sultan Qaboos University Medical Journal, College of Medicine and Health Sciences, Sultan Qaboos University, PO Box 35, Postal Code 123, Al-Khod, Muscat, Oman

ISSN (Print edition): 2075-051X ISSN (Internet edition): 2075-0528

Copyright SQUMJ 2019. This journal and its content is licensed under a Creative Commons Attribution No Derivatives 4.0 International license.

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