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进行性家族性肝内胆汁淤积症外科治疗 被引量：3

Surgical treatment for progressive familial intrahepatic cholestasis
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摘要 进行性家族性肝内胆汁淤积症（progressive familial intrahepatic cholestasis，PFIC）以严重的肝内胆汁淤积为主要特征，是一种罕见的常染色体隐性遗传病，常发生在新生儿期或1岁内，多表现为渐进性黄疸、瘙痒、皮肤结节、营养不良和生长发育障碍，严重者可因肝功能衰竭而死亡。
作者 李龙 刘垚
机构地区 首都儿科研究所附属儿童医院小儿外科
出处 《中国实用儿科杂志》 CSCD 北大核心 2013年第4期260-262,共3页 Chinese Journal of Practical Pediatrics
关键词 胆汁淤积 婴儿 外科 治疗 cholestasis infant surgery therapy
分类号 R72 [医药卫生—儿科]
  • 相关文献

参考文献12

  • 1Emerick KM, Elias MS, Melin-Aldana H, et al.Bile composi- tion in alagille syndrome and PFIC patients having partial exter- nal biliary diversion [J ].BMC Gastroenterol, 2008, 8:47-52.
  • 2Melter M, Rodeck B, Kardorff R, et al. Progressive familial in- trahepatic cholestasis: partial biliary diversion normalizes serum lipids and improves growth in noncirrhotic patients [J]. Am J Gastroenterol, 2000,95( 12):3522-3528.
  • 3Arnell H, Papadogiannakis N, Zemack H, et al. Follow-up in children with progressive familial intrahepatic cholestasis after partial external biliary diversio[J]. J Pediatr Gastroenterol Nu- tr, 2010,51 (4):494-499.
  • 4Kalicinski PJ, Ismail H, Jankowska I, et al. Surgical treatment of progressive familial intrahepatic cholestasis: comparison of partial external biliary diversion and ileal bypass [ J ]. Eur J Pedi- atr Surg, 2003,13:307-311.
  • 5Bustorff-Silva J, Sbraggia Neto L, Olimpio H, et al. Partial in- ternal biliary diversion through a cholecystojejunocolonic anasto- mosis-a novel surgical approach for patients with progressive familial intrahepatic cholestasis: a preliminary report [J]. Pedi- atr Surg, 2007,42(8):1337-1340.
  • 6Grin F, Erginel B, Durmaz O, et al. An outstanding non-trans- plant surgical intervention in progressive familial intrahepatic cholestasis: partial internal biliary diversion [J]. Pediatr Surg Int, 2010,26(8):831-834.
  • 7Ganesh R, Suresh N, Sathiyasekeran M, et al. Partial internal biliary diversion: a solution for intractable pruritus in progres- sive familial intrahepatic cholestasis type 1 [J]. Saudi J Gastro- enterol,2011,17:212-214.
  • 8Schukfeh N, Metzelder ML, Petersen C, et al. Normalization of serum bile acids after partial external biliary diversion indicates an excellent long-term outcome in children with progressive fa- milial intrahepatie cholestasis [J]. J Pediatr Surg, 2012, 47: 501-505.
  • 9Yang H, Porte R J, Verkade HJ, et al. Partial external biliary diversion in children with progressive familial intrahepatie cho- lestasis and Alagille disease [J].J Pediatr Gastroenterol Nutr, 2009,49:216-221.
  • 10李龙,张金山,张军,刘树立,刁美.胆囊结肠吻合治疗进行性家族性肝内胆汁淤积症的探讨[J].中华小儿外科杂志,2011,32(10):741-745. 被引量：6

二级参考文献8

  • 1李龙,余奇志,刘刚,黄柳明,雷宇,贾军,王平.经腹腔镜行先天性胆总管囊肿切除肝管空肠Roux-Y吻合术的探讨[J].临床小儿外科杂志,2002,1(1):54-56. 被引量：56
  • 2Jacquemin E. Progressive familial intrahepatic eholestasis: genetic basis and treatment. Clin Liver Dis, 2000,4 (4) : 753-763.
  • 3Hollands CM, Rivera-Pedrogo FJ, Oonzalez-Vallina R, et al. Ileal exclusion for Byler's disease: an alternative surgical approach with promision early results for prutitus. J Pediatr Surg, 1998, 33(2):220-224.
  • 4Soubrane O, Gauthier F, DeVictor D, et al. Otthotopie liver transplantation for byler disease. Transplantation, 1990, 50 ( 5 ) : 804-806.
  • 5Whitington PF, Whitington GL. Partial external diversion of bile for the treatment of intractable prutitus associated with intrahepatic cholestasis. Gastroenterology, 1988, 95 (1) : 130- 136.
  • 6Kurbegov AC, Setchell KD, Haas JE, et al. Billiary diversion for progressive familial intrahepatic cholestasis: improved liver morphology and bile acid profiie. Gastroenterology, 2003, 125 (4):1227-1234.
  • 7Bustorff-Silva J, Sbraggia Neto L, Olimpio H, et al. Partial internal biliary diversion through a cholecystojejunocolonic anastomosis : a novel surgical approach for patients with progressive familial intrahepatic cholestasis:a preliminary report. J Pediatr Surg,2007,42 (8) : 1337-1340.
  • 8Ismail H, Kalicinski P, Markiewiez M, et al. Treatment of progressive familial intrahepatic cholestasis: liver transplantation or partial external biliary diversion. Pediatr Transplant, 1999,3 (3) :219-224.

共引文献5

同被引文献26

  • 1Shneider BL. Progressive intrahepatic cholestasis: mechanisms, diagnosis and therapy[J]. Pediatr Transplant,2004,8 (6): 609-612.
  • 2Berumen J, Feinberg E, Todo T, et al. Complications following liver transplantation for progressive familial intrahepatic cholestasis[J]. Dig Dis Sci, 2014,59 (11): 2649-2452.
  • 3Morotti RA, Suchy FJ, Magid MS. Progressive familial intrahepatic cholestasis (PFIC) type 1,2, and 3 : a review of the liver pathology findings[J]. Semin Liver Dis,2011,31(1) :3-10.
  • 4Englert C,Grabhorn E, Richter A, et al. Liver transplantation in children with progressive familial intrahepatic cholestasis [J]. Transplantation, 2007,84(10): 1361-1363.
  • 5Ismail H, Kalicinski P, Markiewicz M, et al. Treatment of progressive familial intrahepatie eholestasis: liver transplantation or partial external biliary diversion[J]. Pediatr Transplant, 1999,3(3):219-224.
  • 6Kasahara M, Kiuchi T, Inomata Y, et al. Living-related liver transplantation for Alagille syndrome [J]. Transplantation, 2003,75(12) :2147-2150.
  • 7Hori T,Egawa H,Miyagawa-Hayashino A, et al. Living-donor liver transplantation for progressive familial intrahepatic cholestasis[J]. World J Surg, 2011,35 (2) : 393-402.
  • 8Jacquemin E. Progressive familial intrahepatic cholestasis[J]. Clin Res Hepatol Gastroentero, 2012,36 (Suppl 1 ): S26-35.
  • 9Naoi S, Hayashi H, Inoue T, et al. Improved liver function and relieved pruritus after 4-phenylbutyrate therapy in a patient with progressive familial intrahepatic cholestasis type 2[J]. J Pediatr,2014,164(5): 121J9-1227.
  • 10Hori T, Egawa H, Takada Y, et al. Progressive familial intrahepatic cholestasis: a single-center experience of living- donor liver transplantation during two decades in Japan[J]. Clin Transplant,2011,25(5) :776-785.

引证文献3

二级引证文献10

中国实用儿科杂志
2013年 第4期

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