共查询到20条相似文献,搜索用时 33 毫秒
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Aleixo M Muise Dan Turner Eytan Wine Peter Kim Margaret Marcon Simon C Ling 《Clinical gastroenterology and hepatology》2006,4(11):1411-1414
BACKGROUND & AIMS: To illustrate the limitations of the embryonic and perinatal classification system of biliary atresia (BA), we present a child with an antenatal diagnosis of choledochal cyst (CC) associated with BA and review the published literature. METHODS: Medline and Pubmed were searched for "BA and CC," "cystic biliary atresia," "BA and cysts," and "biliary cystic malformations." RESULTS: A 7-week-old with an antenatal diagnosis of CC was found to have BA associated with CC. The literature search identified 88 cases of BA with CC. Sixty-seven cases had type 1 BA (atresia of the common bile duct), 2 had type 2 BA (atresia of the common hepatic duct), and 19 had type 3 BA (atresia of the porta hepatis). Of the 27 cases of antenatal diagnosis of BA with CC only 1 had associated congenital anomalies. Outcome analysis showed poor outcomes were significantly more common (P = .009) and occurred earlier (P = .0249) in patients with type 3 BA. Children with type 3 BA were 5.4 times more likely to develop poor outcomes compared with type 1 (hazard ratio, 5.4; 95% confidence interval, 1.03-27.8). CONCLUSIONS: BA associated with CC forms a distinct subtype of BA, characterized by a preponderance of type 1 BA, a relatively good clinical outcome after surgery, and an absence of associated congenital anomalies. Antenatal diagnosis of many affected infants supports their inclusion within the embryonic BA group and suggests that a broader interpretation of the embryonic phenotype and further classification of BA based on genetic susceptibility may be required. 相似文献
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Induction of interferon-stimulated gene expression and antiviral responses require protein deacetylase activity 总被引:3,自引:0,他引:3 下载免费PDF全文
Chang HM Paulson M Holko M Rice CM Williams BR Marié I Levy DE 《Proceedings of the National Academy of Sciences of the United States of America》2004,101(26):9578-9583
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Selective loss of a DNase I hypersensitive site upstream of the tyrosine aminotransferase gene in mice homozygous for lethal albino deletions. 下载免费PDF全文
K S Zaret P Milos M Lia D Bali S Gluecksohn-Waelsch 《Proceedings of the National Academy of Sciences of the United States of America》1992,89(14):6540-6544
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Broad KD Keverne EB 《Proceedings of the National Academy of Sciences of the United States of America》2011,108(37):15237-15241
The fetal genome regulates maternal physiology and behavior via its placenta, which produces hormones that act on the maternal hypothalamus. At the same time, the fetus itself develops a hypothalamus. In this study we show that many of the genes that regulate placental development also regulate the developing hypothalamus, and in mouse the coexpression of these genes is particularly high on embryonic days 12 and 13 (days E12-13). Such synchronized expression is regulated, in part, by the maternally imprinted gene, paternally expressed gene 3 (Peg3), which also is developmentally coexpressed in the hypothalamus and placenta at days E12-13. We further show that challenging this genomic linkage of hypothalamus and placenta with 24-h food deprivation results in disruption to coexpressed genes, primarily by affecting placental gene expression. Food deprivation also produces a significant decrease in Peg3 gene expression in the placenta, with consequences similar to many of the placental gene changes induced by Peg3 mutation. Such genomic dysregulation does not occur in the hypothalamus, where Peg3 expression increases with food deprivation. Thus, changes in gene expression brought about by food deprivation are consistent with the fetal genome's maintaining hypothalamic development at a cost to its placenta. This biased change to gene dysregulation in the placenta is linked to autophagy and ribosomal turnover, which sustain, in the short term, nutrient supply for the developing hypothalamus. Thus, the fetus controls its own destiny in times of acute starvation by short-term sacrifice of the placenta to preserve brain development. 相似文献
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