title: Proinflammatory cytokinemia associated with transient myeloproliferative disorder in down syndrome.
abstract: A transient myeloproliferative disorder (TMD) occurs in 10% of the infants with Down syndrome. While most cases resolve within a few months, in 20% of them TMDs are life-threatening or fatal. We encountered 4 patients with TMD, including 1 patient who died of liver failure and disseminated intravascular coagulation. Suspecting involvement of proinflammatory cytokines, we serially assayed them in patients' sera. Cytokines were significantly more abundant in patients than in controls. Interleukins 1 and 2, tumor necrosis factor alpha, interferon gamma, and granulocyte-macrophage colony-stimulating factor were greatly increased, especially in the infant who died. Sustained cytokinemia is likely to participate in TMD pathophysiology, and very high serum concentrations might predict a poor outcome.
authors.affiliation: Gunma Children's Medical Center, Gunma, Japan. ashimada@gcmc.pref.gunma.jp
authors.forename: Akira
authors.initials: A
authors.lastname: Shimada
authors.forename: Kenichi
authors.initials: K
authors.lastname: Maruyama
authors.forename: Toshiji
authors.initials: T
authors.lastname: Shitara
authors.forename: Masahiko
authors.initials: M
authors.lastname: Kato
authors.forename: Kazutoshi
authors.initials: K
authors.lastname: Cho
authors.forename: Tohru
authors.initials: T
authors.lastname: Kobayashi
authors.forename: Toru
authors.initials: T
authors.lastname: Fujiu
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Tsuchida
authors.forename: Akira
authors.initials: A
authors.lastname: Nishida
authors.forename: Yasuhide
authors.initials: Y
authors.lastname: Hayashi
authors.forename: Hisanori
authors.initials: H
authors.lastname: Minakami
authors.forename: Kunihisa
authors.initials: K
authors.lastname: Kozawa
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Kimura
mesh_terms: D016207:Cytokines / Q000097:blood*; D004314:Down Syndrome / Q000150:complications* / Q000276:immunology; D005260:Female; D006801:Humans; D008297:Male; D009196:Myeloproliferative Disorders / Q000150:complications* / Q000276:immunology
chemical_list: D016207:Cytokines