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Immunologic Research
Published in: Immunologic Research 1-3/2009

01-07-2009

Thymus transplantation in complete DiGeorge anomaly

Authors: M. Louise Markert, Blythe H. Devlin, Ivan K. Chinn, Elizabeth A. McCarthy

Published in: Immunologic Research | Issue 1-3/2009

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Abstract

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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Metadata
Title
Thymus transplantation in complete DiGeorge anomaly
Authors
M. Louise Markert
Blythe H. Devlin
Ivan K. Chinn
Elizabeth A. McCarthy
Publication date
01-07-2009
Publisher
Humana Press Inc
Published in
Immunologic Research / Issue 1-3/2009
Print ISSN: 0257-277X
Electronic ISSN: 1559-0755
DOI
https://doi.org/10.1007/s12026-008-8082-5

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