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Reliable, easy-to-read patient information on common medical conditions β written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
IPEX
Introduction
IPEX stands for immunodysregulation polyendocrinopathy enteropathy X-linked syndrome and it is a rare disease linked to the dysfunction of the transcriptional activator FoxP3. [1]
It leads to the dysfunction of regulatory T-cells and the subsequent autoimmunity.
This autoimmunity is called IPEX and it is the attack from the body’s own immune system against the body’s own tissues and organs. Early age onset of this disease in males causes severe enlargement of the secondary lymphoid organs, insulin dependent diabetes, eczema, food allergies, and infections. There has been limited success in treating the syndrome by bone marrow transplantation.[2]
References
References
- β Yong PL, Russo P, Sullivan KE (2008). “Use of Sirolimus in IPEX and IPEX-Like Children”. J. Clin. Immunol. doi:10.1007/s10875-008-9196-1. PMIDΒ 18481161. Unknown parameter
|month=ignored (help) - β Clinical and molecular features of the immunodysregulation, polyendocrinopathy, enteropathy, X linked (IPEX) syndrome
