title: Long-term survival in severe combined immune deficiency: the role of persistent maternal engraftment.
abstract: Two siblings with severe combined immune deficiency, one with maternal engraftment and detectable immunologic functions who was alive at the age of 8 years are presented. Both patients had the same JAK3 gene mutation, suggesting that maternal engraftment may result in immune competence leading to long-term survival in patients with severe combined immune deficiency.
authors.affiliation: Ihsan Dogramaci Children's Hospital, Sihhiye, Ankara, Turkey.
authors.forename: Ilhan
authors.initials: I
authors.lastname: Tezcan
authors.forename: Fugen
authors.initials: F
authors.lastname: Ersoy
authors.forename: Ozden
authors.initials: O
authors.lastname: Sanal
authors.forename: Tuba
authors.initials: T
authors.lastname: Turul
authors.forename: Duygu
authors.initials: D
authors.lastname: Uckan
authors.forename: Sevim
authors.initials: S
authors.lastname: Balci
authors.forename: Gonul
authors.initials: G
authors.lastname: Hicsonmez
authors.forename: M
authors.initials: M
authors.lastname: Prieur
authors.forename: S
authors.initials: S
authors.lastname: Caillat-Zucmann
authors.forename: Francois
authors.initials: F
authors.lastname: Le Deist
authors.forename: Genevieve
authors.initials: G
authors.lastname: De Saint Basile
mesh_terms: D002648:Child; D006801:Humans; D007112:Immunity, Maternally-Acquired; D053616:Janus Kinase 3; D008297:Male; D010375:Pedigree; D011505:Protein-Tyrosine Kinases / Q000235:genetics; D016511:Severe Combined Immunodeficiency / Q000235:genetics / Q000276:immunology* / Q000401:mortality; D015996:Survival Rate
chemical_list: D011505:Protein-Tyrosine Kinases; C507933:JAK3 protein, human; D053616:Janus Kinase 3