Long-term disease course in a patient with severe neonatal IPEX syndrome - 07/09/15
Summary |
We report here on the clinical, histological and immunological findings regarding a patient with immunodysregulation polyendocrinopathy enteropathy X-linked syndrome who was treated for the first 21years with a combination of immunosuppressant agents (IS). The potential modalities of care and treatment options in this rare and severe immune-mediated disorder are discussed. So, long-term outcome for IPEX patients can be obtained with immunosuppressive treatment, which is important since the outcome of haematopoietic stem cell transplantation for this population is variable.
Le texte complet de cet article est disponible en PDF.Abbreviations : Foxp3, GI, HSCT, IPEX, MFM
Plan
Vol 39 - N° 4
P. e43-e47 - septembre 2015 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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