Use of Sirolimus in IPEX and IPEX-Like Children

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• 作者：Pierre L. Yong ; Pierre Russo and Kathleen E. Sullivan
• 关键词：IPEX ; enteropathy ; regulatory T cells ; sirolimus ; rapamycin
• 刊名：Journal of Clinical Immunology
• 出版年：2008
• 出版时间：September, 2008
• 年：2008
• 卷：28
• 期：5
• 页码：581-587
• 全文大小：177.9 KB

文摘

Introduction IPEX (immune dysregulation, polyendocrinopathy, enteropathy, and X-linked syndrome), a rare inflammatory disease caused by mutations of Foxp3, destroys the immunoregulatory environment of affected male infants. Data on optimal therapy are limited.