Werner's Syndrome Associated with Cholangiocarcinoma

A 38-year-old Japanese man had cholangiocarcinoma in association with typical features of Werner's syndrome. Thereplicative capacity of fibroblasts in culture was characteristically reduced, and the in vitro natural killer cell activity was deficient. He died of massive G-I tract bleeding 8 mon...

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Published in:	Japanese Journal of Medicine Vol. 25; no. 2; pp. 179 - 183
Main Authors:	OKAMURA, Hideki, YANAGI, Yusuke, IMARI, Yasuhisa, KUDO, Jiro, ISHIBASHI, Hiromi, USHIJIMA, Masahiro, IMAMURA, Takashi
Format:	Journal Article
Language:	English
Published:	Japan The Japanese Society of Internal Medicine 1986
Subjects:	Adenoma, Bile Duct - etiology Adult Aging Bile Duct Neoplasms - etiology Cells, Cultured Cholangiocarcinoma Fibroblasts - metabolism Humans Killer Cells, Natural - immunology Male Mutation Natural killer cells Premature aging Werner Syndrome - complications Werner Syndrome - genetics Werner's syndrome
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Abstract	A 38-year-old Japanese man had cholangiocarcinoma in association with typical features of Werner's syndrome. Thereplicative capacity of fibroblasts in culture was characteristically reduced, and the in vitro natural killer cell activity was deficient. He died of massive G-I tract bleeding 8 months after admission.
AbstractList	A 38-year-old Japanese man had cholangiocarcinoma in association with typical features of Werner's syndrome. Thereplicative capacity of fibroblasts in culture was characteristically reduced, and the in vitro natural killer cell activity was deficient. He died of massive G-I tract bleeding 8 months after admission. A 38-year-old Japanese man had cholangiocarcinoma in association with typical features of Werner's syndrome. The replicative capacity of fibroblasts in culture was characteristically reduced, and the in vitro natural killer cell activity was deficient. He died of massive G-I tract bleeding 8 months after admission.
Author	ISHIBASHI, Hiromi KUDO, Jiro IMAMURA, Takashi OKAMURA, Hideki IMARI, Yasuhisa USHIJIMA, Masahiro YANAGI, Yusuke
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BackLink	https://www.ncbi.nlm.nih.gov/pubmed/3018336$$D View this record in MEDLINE/PubMed
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References	2) Nakao Y, Kishihara M, Yoshimi H, et al: Werner's syndrome: in vivo and in vitro characteristics as a model of aging. Am J Med 65: 919, 1978. 10) Goto M, Tanimoto K, Horiuchi Y, et al: Family analysis of Werner's syndrome: a survey of 42 Japanese families with a review of the literature. Clin Genet 19: 8, 1981. 9) Martin GM: Birth Defects: Original Article Series, Vol. XIV, No. 1, 1978, p 5. 5) Salk D: Werner's syndrome: A review of recent research with an analysis of connective tissue metabolism, growth control of cultured cells, and chromosomal aberrations. Human Genet 62: 1, 1982. 3) Tannhauser SJ: Werner's syndrome (progeria of the adult) and Rothmund's syndrome: two types of closely related heredo-familial atrophic dermatosis with juvenile cataracts and endocrine features. A critical study with five new cases. Ann Intern Med 23: 559, 1945. 4) Epstein GJ, Martin GM, Shultz AL, et al: Werner's syndrome: a review of its symptomatology, natural history, pathologic features, genetics and relationship to the natural aging process. Medicine 45: 177, 1966. 13) Hay flick L: The limited in vitro lifetime of human diploid cell strains. Exp Cell Res 37: 614, 1965. 14) Fujiwara Y, Higashikawa T, Tatsumi M: A retarded rate of DNA replication and normal level of DNA repair in Werner's syndrome fibroblasts in culture. J Cell Physiol 92: 365, 1977. 12) Herberman RB, Holden HT: Natural cell-mediated immunity. Adv Cancer Res 27: 305, 1978. 1) Beadle GF, Mackay IR, Whittingham S, et al: Werner's syndrome, a model of premature aging? J Med 9:377, 1978. 8) Hirschberg H, Skare H, Thorsby E: Cell mediated lympholysis: CML. A microplate technique requiring few target cells and employing a new method of supernatant collection. J Immunol Methods 16: 131, 1977. 7) Goldstein S, Singal DP: Alteration of fibroblast gene products in vitro from a subject with Werner's syndrome. Nature 251: 719, 1974. 11) Usui M, Ishii S, Yamawaki S, et al: The occurrence of soft tissue sarcomas in three siblings with Werner's syndrome. Cancer 54: 2580, 1984. 6) Holliday R, Porter field JS, Gibbs DD: Premature aging and occurrence of altered enzyme in Werner's syndrome fibroblasts. Nature 248: 762, 1974.
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Snippet	A 38-year-old Japanese man had cholangiocarcinoma in association with typical features of Werner's syndrome. Thereplicative capacity of fibroblasts in culture... A 38-year-old Japanese man had cholangiocarcinoma in association with typical features of Werner's syndrome. The replicative capacity of fibroblasts in culture...
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SubjectTerms	Adenoma, Bile Duct - etiology Adult Aging Bile Duct Neoplasms - etiology Cells, Cultured Cholangiocarcinoma Fibroblasts - metabolism Humans Killer Cells, Natural - immunology Male Mutation Natural killer cells Premature aging Werner Syndrome - complications Werner Syndrome - genetics Werner's syndrome
Title	Werner's Syndrome Associated with Cholangiocarcinoma
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