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Article Dans Une Revue Clinics and Research in Hepatology and Gastroenterology Année : 2015

Long-term disease course in a patient with severe neonatal IPEX syndrome

Résumé

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 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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hal-01911483 , version 1 (02-11-2018)

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Rémi Duclaux-Loras, Sophie Collardeau-Frachon, Stéphane Nancey, Nicole Fabien, Dominique Kaiserlian, et al.. Long-term disease course in a patient with severe neonatal IPEX syndrome. Clinics and Research in Hepatology and Gastroenterology, 2015, 39 (4), pp.e43-47. ⟨10.1016/j.clinre.2015.03.006⟩. ⟨hal-01911483⟩
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