TY - JOUR
T1 - Postnatal development of T lymphocytes in a novel X-linked immunodeficiency disease
AU - Schmalstieg, Frank C.
AU - Wirt, Daniel P.
AU - Adkins, Linda T.
AU - Brooks, Edward G.
AU - Stansberry, Susan D.
AU - Swischuk, Leonard E.
AU - Goldman, Armond S.
N1 - Funding Information:
1 This work was presented to the Society for Pediatric Research and the American Pediatric Society on May 9, 1990, and was supported by the John Sealy Memorial Fund, the James W. McLaughlin Fellowship Fund, and Sandoz Pharmaceutics. 2 Abbreviations used: CID, combined immunodeficiency disease; SCID, severe combined immunodeficiency disease; CD, leukocyte cluster of differentiation; TCR, T cell antigen receptors.
PY - 1992/7
Y1 - 1992/7
N2 - We previously reported an X-linked combined immunode-ficiency disease (CID) characterized by immune deficiencies and complicating infections that were more moderate than those found in severe CID (SCID). Since other unstudied males in the family died in infancy, we questioned whether this T cell defect was more profound in early life. Subsequently, the development of blood T cells in an affected new-born male was examined. T cells were virtually undetectable at 48 hr. Over the next several months, CD4+ T cells (principally CD45RO+) rose to levels similar to those found in older affected males, but CD8+ T cells developed more slowly and never attained levels found in other affected males. Thus, this disease in early life mimics SCID and may pose a higher risk of fatal infections to affected individuals during that period. Finally, we speculate that the genetic defect may disrupt intrathymic development or selection of T cells.
AB - We previously reported an X-linked combined immunode-ficiency disease (CID) characterized by immune deficiencies and complicating infections that were more moderate than those found in severe CID (SCID). Since other unstudied males in the family died in infancy, we questioned whether this T cell defect was more profound in early life. Subsequently, the development of blood T cells in an affected new-born male was examined. T cells were virtually undetectable at 48 hr. Over the next several months, CD4+ T cells (principally CD45RO+) rose to levels similar to those found in older affected males, but CD8+ T cells developed more slowly and never attained levels found in other affected males. Thus, this disease in early life mimics SCID and may pose a higher risk of fatal infections to affected individuals during that period. Finally, we speculate that the genetic defect may disrupt intrathymic development or selection of T cells.
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U2 - 10.1016/0090-1229(92)90061-R
DO - 10.1016/0090-1229(92)90061-R
M3 - Article
C2 - 1606754
AN - SCOPUS:0026725359
SN - 0090-1229
VL - 64
SP - 71
EP - 77
JO - Clinical Immunology and Immunopathology
JF - Clinical Immunology and Immunopathology
IS - 1
ER -