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Thymus transplantation in complete DiGeorge anomaly.

Publication ,  Journal Article
Markert, ML; Devlin, BH; Chinn, IK; McCarthy, EA
Published in: Immunol Res
2009

Complete DiGeorge anomaly is characterized by athymia, congenital heart disease, and hypoparathyroidism. This congenital disease is fatal by age 2 years unless immune reconstitution is successful. There are multiple underlying syndromes associated with complete DiGeorge anomaly including 22q11 hemizygosity in approximately 50%, CHARGE association in approximately 25%, and diabetic embryopathy in approximately 15%. Approximately one-third of patients present with rash and lymphadenopathy associated with oligoclonal "host" T cells. This condition resembles Omenn syndrome. Immunosuppression is necessary to control the oligoclonal T cells. The results of thymus transplantation are reported for a series of 50 patients, of whom 36 survive. The survivors develop naïve T cells and a diverse T cell repertoire.

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Published In

Immunol Res

DOI

ISSN

0257-277X

Publication Date

2009

Volume

44

Issue

1-3

Start / End Page

61 / 70

Location

United States

Related Subject Headings

  • Treatment Outcome
  • Thymus Gland
  • T-Lymphocyte Subsets
  • Liver
  • Kaplan-Meier Estimate
  • Infant
  • Immunology
  • Humans
  • DiGeorge Syndrome
  • Child, Preschool
 

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Markert, M. L., Devlin, B. H., Chinn, I. K., & McCarthy, E. A. (2009). Thymus transplantation in complete DiGeorge anomaly. Immunol Res, 44(1–3), 61–70. https://doi.org/10.1007/s12026-008-8082-5
Markert, M Louise, Blythe H. Devlin, Ivan K. Chinn, and Elizabeth A. McCarthy. “Thymus transplantation in complete DiGeorge anomaly.Immunol Res 44, no. 1–3 (2009): 61–70. https://doi.org/10.1007/s12026-008-8082-5.
Markert ML, Devlin BH, Chinn IK, McCarthy EA. Thymus transplantation in complete DiGeorge anomaly. Immunol Res. 2009;44(1–3):61–70.
Markert, M. Louise, et al. “Thymus transplantation in complete DiGeorge anomaly.Immunol Res, vol. 44, no. 1–3, 2009, pp. 61–70. Pubmed, doi:10.1007/s12026-008-8082-5.
Markert ML, Devlin BH, Chinn IK, McCarthy EA. Thymus transplantation in complete DiGeorge anomaly. Immunol Res. 2009;44(1–3):61–70.
Journal cover image

Published In

Immunol Res

DOI

ISSN

0257-277X

Publication Date

2009

Volume

44

Issue

1-3

Start / End Page

61 / 70

Location

United States

Related Subject Headings

  • Treatment Outcome
  • Thymus Gland
  • T-Lymphocyte Subsets
  • Liver
  • Kaplan-Meier Estimate
  • Infant
  • Immunology
  • Humans
  • DiGeorge Syndrome
  • Child, Preschool