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Erschienen in: Immunologic Research 1-3/2009

01.07.2009

Thymus transplantation in complete DiGeorge anomaly

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

Erschienen in: Immunologic Research | Ausgabe 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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Metadaten
Titel
Thymus transplantation in complete DiGeorge anomaly
verfasst von
M. Louise Markert
Blythe H. Devlin
Ivan K. Chinn
Elizabeth A. McCarthy
Publikationsdatum
01.07.2009
Verlag
Humana Press Inc
Erschienen in
Immunologic Research / Ausgabe 1-3/2009
Print ISSN: 0257-277X
Elektronische ISSN: 1559-0755
DOI
https://doi.org/10.1007/s12026-008-8082-5

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