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进行性家族性肝内胆汁淤积症的诊治进展 被引量:7

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作者 孙梅 郭亚琼
出处 《中国实用儿科杂志》 CSCD 北大核心 2008年第1期6-9,共4页 Chinese Journal of Practical Pediatrics
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参考文献18

  • 1Emmanuel Jacquemin.progressive familial intrahepatic cholestssis:genetic basis and treatment[J].Pediatric Liver,2000,4(4):753-763.
  • 2Karan M Emerick,MD.Progressive familial intrahepatic cholestasis.2006-06-19[2007-11-13].http://www.emedicine.com/ped/topic2771.htm.
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  • 4Nancy A.M van Doteghem,Leo W.J.Klomp,Gerard P.Benign recurrent intrahepatic cholestasis progressiving to progressive familial intrahepatic cholestasis:low GGT cholestasis is a clinical continuum[J].Hepatology,2002,36:439-443.
  • 5Chen F,Ananthanarayanan M,Emer S,et al.Progressive Familial Intrahepatic Cholestasis Type1,Is Associated With Decreased Farnesoid X Receptor Activity[J].Gastroenterology,2004,126:756-764.
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  • 7Ismail H,Kalicinski P,Markiewiez M,et al.Treatment of progressive familial intrahepatic cholestasis:liver transplantation or partial external biliary diversion[J].Pediatric Transplantation,1999,3:219-224.
  • 8陈朱波,姚定康.进行性家族性肝内胆汁淤积[J].国际消化病杂志,2006,26(4):279-281. 被引量:5
  • 9Demeilliers C,Jacquemin E,Barbu V,et al.Altered hepatobiliary gene expressions in PFIC1:ATP8B1 gene defect is associated with CFTR down regulation[J].Hepatology,2006,43 (5):1125-1134.
  • 10王中林,朱启镕,王建设,等.BSEP基因突变在低γ-GGT的进行性肝内胆汁淤积中的意义[J].第十四次全国儿科学术会议论文汇编,2007:64.

二级参考文献36

共引文献8

同被引文献63

  • 1王中林,朱启镕,王晓红,王建设.黄疸型婴儿肝炎预后与血清谷氨酰转肽酶水平变化的关系[J].肝脏,2005,10(4):271-274. 被引量:9
  • 2李晓瑜,杜敏联,庄思齐,莫恩明,马华梅,李燕虹,陈红珊,丘小汕.遗传性酪氨酸血症Ⅰ型10例的临床诊断分析[J].中华儿科杂志,2006,44(6):470-471. 被引量:13
  • 3朱世殊,张鸿飞,陈菊梅,杨晓晋,董漪,徐志强,陈大为,谢秋里.儿童肝衰竭病因及病理的研究[J].传染病信息,2006,19(3):132-134. 被引量:15
  • 4Liver Failure and Artficial Liver Group,Chinese Society of Infectious Diseases,Chinese Medical Association.肝衰竭诊疗指南[J].实用肝脏病杂志,2006,9(6):321-324. 被引量:190
  • 5Ozlem Yonem,Yusuf Bayraktar.Clinical characteristics of Caroli's syndrome[J].World Journal of Gastroenterology,2007,13(13):1934-1937. 被引量:26
  • 6VANMIL SWC,HOUWEN RHJ,KLOMP LWJ. Genetics of intrafamilial cholestasis syndromes [J]. J Med Genet,2005, 42: 449-463.
  • 7DEMEILLIERS C,JACQUEMIN E,BARBU V,et al. Altered hepatobiliary gene expressions in PFICI:ATPSB1 gene defect is associated with CFTR downregulation[J]. Hepatology,2006,43:1125-1134.
  • 8PAULUSMA CC,GROEN A,KUNNE C,et al. Atp8b1 deficiency in mice reduces resistance of the canalicular membrane to hydrophobic bile salts and impairs bile salt transport[J]. Hepatology, 2006,44: 195-204.
  • 9CAI SY,GAUTAM S,NGUYEN T,et al. ATP8B1 Deficiency disrupts the bile canalicular membrane bilayer structure in hepatocytes,but FXR expression and activity are maintained[J]. Gastroenterology, 2009,136:1060-1069.
  • 10OUDE ELFERINK RP,PAULUSMA CC,GROEN AK. Hepatocanalicular transport defects: pathophysiologic mechanisms of rare diseases[J]. Gastroenterology, 2006,130: 908-925.

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