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• W309596604 abstract "Autoimmune enteropathy is a relatively recently described entity consisting primarily of protracted diarrhea in young children, with circulating gut autoantibodies and, in most patients, extra-intestinal disease with a host of various autoimmune markers. Critical analysis of reported cases, which currently number approximately 100, reveals heterogeneity both at the clinical and laboratory levels. Affected patients appear to fall into two major categories: those with an underlying primary immunodeficiency, many of whom bear an X-linked syndrome of immune dysregulation, polyendocrinopathy and enteropathy, whereas the majority of reported cases have no demonstrable immune defects. Different types of circulating autoantibodies have been identified by indirect immunofluorescence using patients' sera, and several possible antigenic targets are being identified, though the causative role of these antibodies in mucosal injury remains unknown. A relatively high mortality rate as reported in the earlier cases has been replaced by cautious optimism in the face of more recent success using immunosuppressive agents such as cyclosporin and tacrolimus. This disorder appears to represent a syndrome of generalized uncontrolled immune activation, with the intestine as its primary target." @default.
• W309596604 created "2016-06-24" @default.
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• W309596604 date "2002-07-01" @default.
• W309596604 modified "2023-10-16" @default.
• W309596604 title "Autoimmune enteropathy" @default.
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• W309596604 doi "https://doi.org/10.1016/s1529-1049(02)00043-0" @default.
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