TY - JOUR
T1 - Proinflammatory cytokinemia associated with transient myeloproliferative disorder in down syndrome
AU - Shimada, Akira
AU - Maruyama, Kenichi
AU - Shitara, Toshiji
AU - Kato, Masahiko
AU - Cho, Kazutoshi
AU - Kobayashi, Tohru
AU - Fujiu, Toru
AU - Tsuchida, Yoshiaki
AU - Nishida, Akira
AU - Hayashi, Yasuhide
AU - Minakami, Hisanori
AU - Kozawa, Kunihisa
AU - Kimura, Hirokazu
PY - 2004
Y1 - 2004
N2 - 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.
AB - 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.
KW - Down syndrome
KW - Proinflammatory cytokines
KW - Transient myeloproliferative disorder
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U2 - 10.1159/000075376
DO - 10.1159/000075376
M3 - Article
C2 - 14671434
AN - SCOPUS:12144290017
SN - 0006-3126
VL - 85
SP - 167
EP - 172
JO - Biology of the Neonate
JF - Biology of the Neonate
IS - 3
ER -