title: Osteopontin expression in the liver with severe perisinusoidal fibrosis: autopsy case of Down syndrome with transient myeloproliferative disorder.
abstract: Down syndrome with transient myeloproliferative disorder (TMD) is often associated with perinatal liver fibrosis. The authors recently encountered an autopsy case of this disease with a characteristic severe perisinusoidal liver fibrosis. Osteopontin (OPN) is a molecule that plays an important role in diverse fibro-inflammatory diseases. The purpose of the present report was to examine the involvement of OPN in development of the Down syndrome-associated liver fibrosis. Histology indicated severe perisinusoidal fibrosis and ductular arrangements of hepatocytes in the liver. Appearance of atypical megakaryocytes in the liver, a feature of TMD associated with Down syndrome, was not evident. On immunohistochemistry expression of OPN was observed in hepatocytes often having ductular arrangements and infiltrating macrophages. In contrast, a small number of transforming growth factor-beta1 (TGF-beta1)-positive mononuclear cells were present in the liver. Numerous activated hepatic stellate cells (HSC) expressing alpha-smooth muscle actin (alpha-SMA) were seen in the perisinusoidal area. A recent report indicated that OPN could directly activate the HSC. Thus, it is suggested that OPN produced by hepatocytes and macrophages induces activation of the HSC, and leads to the development of perisinusoidal liver fibrosis.
authors: Department of Pathology and Immunology, Division of Molecular Pathology and Tumor Pathology, Akita University School of Medicine, Akita, Japan.
authors: Takuo
authors: T
authors: Tokairin
authors: Yuji
authors: Y
authors: Nishikawa
authors: Hitoshi
authors: H
authors: Watanabe
authors: Yuko
authors: Y
authors: Doi
authors: Yasufumi
authors: Y
authors: Omori
authors: Toshiaki
authors: T
authors: Yoshioka
authors: Youhei
authors: Y
authors: Yamamoto
authors: Masayuki
authors: M
authors: Yoshida
authors: Takuya
authors: T
authors: Nishimura
authors: Qinchang
authors: Q
authors: Li
authors: Hirokazu
authors: H
authors: Arai
authors: Akira
authors: A
authors: Ishida
authors: Goro
authors: G
authors: Takada
authors: Katsuhiko
authors: K
authors: Enomoto
mesh_terms: D001344:Autopsy; D004314:Down Syndrome / Q000150:complications / Q000378:metabolism* / Q000473:pathology; D022781:Hepatocytes / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry; D007231:Infant, Newborn; D008103:Liver Cirrhosis / Q000378:metabolism* / Q000473:pathology; D008297:Male; D009196:Myeloproliferative Disorders / Q000209:etiology / Q000378:metabolism*; D053495:Osteopontin / Q000096:biosynthesis*; D053773:Transforming Growth Factor beta1 / Q000096:biosynthesis
chemical_list: D053773:Transforming Growth Factor beta1; D053495:Osteopontin