Article
Clinical heterogeneity of immunodysregulation, polyendocrinopathy, enteropathy, X-linked: pulmonary involvement as a non-classical disease manifestation.
Journal of clinical immunology - 1 Aug 2014
Baris Safa, Schulze Ilka, Ozen Ahmet, Karakoç Aydıner Elif, Altuncu Emel, Karasu Gulsun Tezcan, Ozturk Nilufer, Lorenz Myriam, Schwarz Klaus, Vraetz Thomas, Ehl Stephan, Barlan Isil B
Abstract excerpt
PURPOSE: IPEX (Immunodysregulation, Polyendocrinopathy, Enteropathy, X-linked) is a rare X-linked recessive life-threatening disorder characterized by autoimmunity and early death. Pulmonary complication related with IPEX has not been elucidated exactly. Here, we report 4 IPEX patients, 3 of which died from severe pulmonary disease. METHODS: Clinical data and laboratory findings including autoantibodies,...
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