A Mystery Diagnosis: Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Recessive
Management and International Business
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.
Gischel, M. E.,
(2009). A Mystery Diagnosis: Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Recessive. Laboratory Medicine, 40(5), 303-306.
Available at: http://aquila.usm.edu/fac_pubs/1239