A Mystery Diagnosis: Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Recessive

Document Type

Article

Publication Date

5-1-2009

Department

Management and International Business

Abstract

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX), a rare disease, has recently been shown to occur more frequently than previously suspected. The clinical phenotype of IPEX is a direct result of an overactive immune system caused by the proliferation of autoaggressive T cells and autoantibody-producing B cells. Without prompt diagnosis and treatment, the disease can be fatal within 1 year.

Publication Title

Laboratory Medicine

Volume

40

Issue

5

First Page

303

Last Page

306

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