Mosaicism in X-linked severe combined immunodeficiency.

Ting, S S; Ziegler, J; Leigh, D; Kemp, A; Lindeman, R · J Pediatr · 1998

case_report · Level V

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Abstract

We performed gamma c gene analysis of a boy with severe combined immunodeficiency whose brother died of the same condition. A base pair deletion in exon 6 was found, which was absent in his mother, indicating maternal mosaicism with important implications for genetic counseling and demonstrating the importance of mutation analysis in boys with severe combined immunodeficiency.

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