Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
3286079 | Clinics and Research in Hepatology and Gastroenterology | 2015 | 5 Pages |
Abstract
SummaryWe 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 21 years 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.
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Authors
Rémi Duclaux-Loras, Sophie Collardeau-Frachon, Stephane Nancey, Nicole Fabien, Dominique Kaiserlian, Alain Lachaux,