JOURNAL OF CLINICAL IMMUNOLOGY, vol.34, no.6, pp.601-606, 2014 (Journal Indexed in SCI)
Article / Article
Title of Journal :
JOURNAL OF CLINICAL IMMUNOLOGY
IPEX, FOXP3, clinical heterogeneity, lung involvement, REGULATORY T-CELLS, IMMUNE DYSREGULATION, IPEX SYNDROME, FOXP3, MUTATIONS, GENES
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.