Article
Use of sirolimus in IPEX and IPEX-like children.
Journal of clinical immunology - 1 Sept 2008
Yong Pierre L, Russo Pierre, Sullivan Kathleen E
Abstract excerpt
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. METHODS: We reviewed the effect of sirolimus use in our cohort of IPEX and IPEX-like patients (n = 7). RESULTS AND DISCUSSION: Our patients exhibited...
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