关键词: Bile duct proliferation Duct plate malformation Extra-hepatic biliary atresia Portal fibrosis Portal inflammation

来  源:   DOI:10.7860/JCDR/2016/19252.8035

Abstract:
BACKGROUND: Extrahepatic biliary atresia is a progressive disorder characterised by fibroinflammatory obliteration or stenosis of the extrahepatic biliary tree leading to obstruction of bile flow and cholestatic jaundice. It is the most common cause for cholestasis in newborn. Histopathological criteria for diagnosing biliary atresia from liver biopsy have not been clearly defined.
OBJECTIVE: This study was undertaken to analyse the significance of the various histopathological features in diagnosis and prognosis of extrahepatic biliary atresia from liver biopsy specimens.
METHODS: This was a retrospective study of 43 cases of extra-hepatic biliary atresia diagnosed and treated at a tertiary care hospital between January 2010 to December 2014. Formalin fixed paraffin embedded liver biopsy tissues were processed by standard technique and the slides were stained with haematoxylin and eosin. All the slides were reviewed and graded by a semi-quantitative scoring system. Features such as increased age at kasai\'s portoenterostomy, portal fibrosis, bile duct proliferation, cholestasis, portal inflammation and duct plate malformation were studied. Statistical analysis was worked out using SPSS 17.0 (statistical package for the social science software). Chi-square test was used to find association between various parameters with respect to mortality and Kaplan-Meier estimator was used for survival analysis of the population under study.
RESULTS: In this study comprising of 43 cases, only 6 cases (13.95%) were alive at the end of 6 months follow-up. Twenty patients who died and the 17 cases with poor survival had greater degrees of fibrosis, bile duct proliferation and cholestasis. Majority of the cases with duct plate malformation expired inspite of earlier surgical intervention. Thus proving the association of fibrosis, bile duct proliferation, cholestasis and duct plate malformation with the survival and prognosis of biliary atresia cases. Age at surgery did not show any correlation with prognosis as cases operated even at <60 days had poor survival.
CONCLUSIONS: From this study it can be concluded that in extrahepatic biliary atresia patients, presence of duct plate malformation, greater degrees of fibrosis, bile duct proliferation and cholestasis were strongly associated with poor prognosis.
摘要:
背景:肝外胆管闭锁是一种进行性疾病,其特征是肝外胆管树的纤维炎性闭塞或狭窄,导致胆汁流阻塞和胆汁淤积性黄疸。它是新生儿胆汁淤积的最常见原因。从肝活检中诊断胆道闭锁的组织病理学标准尚未明确定义。
目的:本研究从肝活检标本中分析各种组织病理学特征在肝外胆道闭锁诊断和预后中的意义。
方法:回顾性研究了2010年1月至2014年12月在某三级医院诊治的43例肝外胆道闭锁。通过标准技术处理福尔马林固定的石蜡包埋的肝活检组织,并用苏木精和伊红染色载玻片。通过半定量评分系统对所有载玻片进行审查和分级。特征,如在kasai的口肠造口术中年龄增加,门静脉纤维化,胆管增生,胆汁淤积,对门静脉炎症和管板畸形进行了研究。使用SPSS17.0(社会科学软件统计软件包)进行统计分析。卡方检验用于发现与死亡率有关的各种参数之间的关联,并且Kaplan-Meier估计器用于研究人群的生存分析。
结果:本研究共43例,在6个月的随访结束时,只有6例(13.95%)存活。死亡患者20例,生存率差的17例,纤维化程度较大,胆管增生和胆汁淤积。尽管进行了较早的手术干预,但大多数导管板畸形的病例都已过期。因此证明了纤维化的关联,胆管增生,胆汁淤积和导管板畸形与胆道闭锁病例的生存和预后。手术年龄与预后没有任何相关性,因为即使在<60天手术的病例生存率也很低。
结论:从这项研究可以得出结论,在肝外胆道闭锁患者中,管道板畸形的存在,纤维化程度更高,胆管增生和胆汁淤积与不良预后密切相关。
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