Severe Combined Immunodeficiency—Classification, Microbiology Association and Treatment

Author:

Justiz-Vaillant Angel A.1,Gopaul Darren2ORCID,Akpaka Patrick Eberechi13,Soodeen Sachin1,Arozarena Fundora Rodolfo34ORCID

Affiliation:

1. Department of Paraclinical Sciences, Faculty of Medical Sciences, The University of the West Indies, St. Augustine, Trinidad and Tobago

2. Department of Internal Medicine, Port of Spain General Hospital, The University of the West Indies, St. Augustine, Trinidad and Tobago

3. Eric Williams Medical Sciences Complex, North Central Regional Health Authority, Champs Fleurs, Trinidad and Tobago

4. Department of Clinical and Surgical Sciences, Faculty of Medical Sciences, The University of the West Indies, St. Augustine, Trinidad and Tobago

Abstract

Severe combined immunodeficiency (SCID) is a primary inherited immunodeficiency disease that presents before the age of three months and can be fatal. It is usually due to opportunistic infections caused by bacteria, viruses, fungi, and protozoa resulting in a decrease in number and impairment in the function of T and B cells. Autosomal, X-linked, and sporadic forms exist. Evidence of recurrent opportunistic infections and lymphopenia very early in life should prompt immunological investigation and suspicion of this rare disorder. Adequate stem cell transplantation is the treatment of choice. This review aimed to provide a comprehensive approach to the microorganisms associated with severe combined immunodeficiency (SCID) and its management. We describe SCID as a syndrome and summarize the different microorganisms that affect children and how they can be investigated and treated.

Publisher

MDPI AG

Subject

Virology,Microbiology (medical),Microbiology

Reference117 articles.

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5. Severe combined immunodeficiency: From its discovery to the perspective;Kanegane;Nihon Rinsho Meneki. Gakkai Kaishi,2017

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