Thymus transplantation in complete DiGeorge anomaly.
Journal Article (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
- PMC4951183
International Standard Serial Number (ISSN)
- 0257-277X
Digital Object Identifier (DOI)
- 10.1007/s12026-008-8082-5
Language
- eng
Conference Location
- United States