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先天性肝纤维化10例临床分析 被引量:4

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摘要 先天性肝纤维化(congenital hepatic fibrosis,CHF)是一种常染色体隐性遗传病,主要累及肝胆系统和肾,以肝纤维化、门静脉高压、肾囊性病变为特征。病理学上定义为不同程度的肝门静脉周围纤维化和不规则形态增生的胆管[1],临床主要表现为肝脾大、食管胃底静脉曲张破裂出血等,而肝功能正常或轻度损伤[2]。
出处 《北京医学》 CAS 2018年第4期364-365,368,共3页 Beijing Medical Journal
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  • 1赵新颜,王宝恩,贾继东.36例先天性肝纤维化的临床病理特点[J].中华消化杂志,2005,25(12):748-749. 被引量:19
  • 2Kerr DNS, Harrison CV, Sherlock S, et al. Congenital hepatic fibrosis[J]. QJ MED, 1961,30(1):91 -117.
  • 3Poddar U, Thapa BR, Vashishta RK, et al. Congenital hepatic fibrosis in Indian children [J]. J Gastroenterol Hepatol, 1999, 14 (12): 1192 -1196.
  • 4Desmet VJ. Ludwig symposium on biliary disorders- part Ⅰ. Pathogenesis of ductal plate abnormalities[ J ]. Mayo Clin Proc, 1998,73 (1):80-89.
  • 5Gunay - Aygun M, Avner ED, Bacallao RL, et al. Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis:summary statement of a first national institutes of health/office of rare diseases conference [ J ]. J Pediatr, 2006,149 ( 2 ) : 159 - 164.
  • 6Turkbey B, Ocak i, Daryanani K,et al. Autosomal recessive polycystic kidney clisease and congenital hepatic fibrosis (ARPKD/CHF) [J]. PediatrRadio, 2009 ,39(2):100-111.
  • 7Nakanuma Y,Harada K, Sato Y, et al. Recent progress in the etiopathogenesis of pediatric biliaty disease, particularly Caroli's disease with congenital hepatic fibrosis and biliary atresia [ J]. Histol Histopathol, 2010,25 ( 2 ) : 223 - 235.
  • 8Gunay- Aygun M. Liver and kidney disease in ciliopathies[ J]. Am J Med Genet C Semin Med Genet, 2009,151C (4) : 296 - 306.
  • 9张水军,赵永福,苟建军,马秀现,许培钦.布加综合征460例误诊误治原因探讨[J].中华普通外科杂志,1999,14(3):165-167. 被引量:14

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