What are the racial predilections of severe combined immunodeficiency (SCID)?

Updated: Aug 11, 2020
  • Author: Francisco J Hernandez-Ilizaliturri, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Although there is no racial predilection for combined B-cell and T-cell disorders, some forms of combined immunodeficiency have been reported more in some ethnic groups, such as the following [7] :

  • JAK3 mutations in Italy
  • MHC class II deficiency of North African origin
  • ADA-SCID in the Somali population has an incidence of 1 in 5,000 [10]
  • ZAP70 mutations in the Mennonite population
  • Artemis gene product–deficiency in Navajo Indians of Athabasca descent has a reported incidence of 1 in 2,000 [10]
  • RAG1/ RAG2–deficient SCID in Europe
  • CHH in the Finnish population and the old Amish order in the United States

The disorders associated with the X chromosome typically manifest only in males, whereas females are carriers. Approximately 50% of SCID cases are X-linked.

Most patients with these disorders become symptomatic with recurrent infections, failure to thrive, or both in the first months of life.


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