title: [Recent advances in the pathogenesis of hyper IgE syndrome].
abstract: Hyperimmunoglobulin-E syndrome (HIES) is one of the primary immunodeficiency with the manifestations of recurrent infections especially with Staphylococcus aureus, characteristic facies, hyperextensibility of joints, multiple bone fractures, scoliosis, and delayed shedding of the primary teeth. It is a multisystem disease of autosomal dominant or recessive inheritance. Recently, the genetic causes of HIES (STAT3, TYK2, and DOCK8) were clarified.
authors.affiliation: Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Japan.
authors.forename: Hidetoshi
authors.initials: H
authors.lastname: Takada
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Minegishi
mesh_terms: D006801:Humans; D007589:Job Syndrome / Q000235:genetics*; D013203:Staphylococcal Infections