immune dysregulation, polyendocrinopathy, enteropathy

immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome

[MIM304790] a syndrome of variable autoimmune features including eczema, enteropathies, hemolytic anemia, and endocrinopathies (diabetes mellitus and thyroid pathology), abnormal responses to viral infections; associated death in infancy or early childhood is common.
References in periodicals archive ?
Canioni et al., "Treatment of the immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX) by allogeneic bone marrow transplantation," The New England Journal of Medicine, vol.
Piccirillo, "The immunological and genetic basis of immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome," Current Opinion in Allergy and Clinical Immunology, vol.
A group of Japanese researchers noted that [CD4.sup.+][CD25.sup.+][CD127.sup.low] T cells isolated from IPEX (Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked) patients also exhibited appreciable suppressive activity, although less than that exhibited by Treg cells from healthy controls.
Clinical and molecular profile of a new series of patients with immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome: inconsistent correlation between forkhead box protein 3 expression and disease severity.
Furthermore, an inadequate number of T reg lymphocytes can lead to autoimmunity in humans, as it is clearly shown in patients with immune dysregulation, polyendocrinopathy, enteropathy, and X-linked (IPEX) syndrome, who completely lack T reg cells as results of a mutation in FoxP3 [33].
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