Thymus transplantation in complete DiGeorge anomaly.

Published

Journal Article

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.

Full Text

Duke Authors

Cited Authors

  • Markert, ML; Devlin, BH; Chinn, IK; McCarthy, EA

Published Date

  • 2009

Published In

Volume / Issue

  • 44 / 1-3

Start / End Page

  • 61 - 70

PubMed ID

  • 19066739

Pubmed Central ID

  • 19066739

International Standard Serial Number (ISSN)

  • 0257-277X

Digital Object Identifier (DOI)

  • 10.1007/s12026-008-8082-5

Language

  • eng

Conference Location

  • United States