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先天性肝外胆道闭锁

Congenital extrahepatic biliary atresia.

作者信息

Scotto J M, Stralin H G

出版信息

Arch Pathol Lab Med. 1977 Aug;101(8):416-9.

PMID:577675
Abstract

Ten cases of congenital extrahepatic biliary atresia were studied ultrastructurally. Samples of liver were obtained from each and in six of the cases, fibrous tracts, which we hoped would contain extrahepatic bile ducts, were also secured. The observed extrahepatic biliary structures were real, but hypoplastic, bile ducts. In places, necrosed epithelial cells, without obvious inflammatory processes, could be observed. The ductular cell cytoplasmic changes and the inflammatory reaction are different according to whether extrahepatic or intrahepatic sites are considered. These differences, as well as the cytoplasmic modifications of liver parenchymal cells, seem to be the result of impaired bile flow. On the contrary, extrahepatic bile duct hypoplasia and necrosis seem to be directly related to the unknown origin of this disease. Whether the nuclear changes of hepatocytes are the expression of direct injury of the liver is another important question. If there is direct injury, it is possible that the disease might evolve as an independent liver disease despite a correctly performed and uncomplicated surgical intervention.

摘要

对10例先天性肝外胆道闭锁病例进行了超微结构研究。从每例获取肝脏样本,其中6例还获取了我们希望含有肝外胆管的纤维束。观察到的肝外胆道结构是真实的,但为发育不全的胆管。在某些部位,可以观察到坏死的上皮细胞,且无明显炎症过程。根据是考虑肝外还是肝内部位,小胆管细胞的细胞质变化和炎症反应有所不同。这些差异以及肝实质细胞的细胞质改变似乎是胆汁流动受损的结果。相反,肝外胆管发育不全和坏死似乎与该疾病的未知起源直接相关。肝细胞的核变化是否是肝脏直接损伤的表现是另一个重要问题。如果存在直接损伤,那么尽管手术操作正确且未出现并发症,该疾病仍有可能发展为一种独立的肝脏疾病。

相似文献

1
Congenital extrahepatic biliary atresia.先天性肝外胆道闭锁
Arch Pathol Lab Med. 1977 Aug;101(8):416-9.
2
Bile ductule formation in fetal, neonatal, and infant livers compared with extrahepatic biliary atresia.胎儿、新生儿和婴儿肝脏中的胆小管形成与肝外胆道闭锁的比较。
Hepatology. 1996 Sep;24(3):568-74. doi: 10.1002/hep.510240318.
3
[Histomorphological liver changes due to extrahepatic biliary stresia].[肝外胆管闭锁所致的肝脏组织形态学改变]
Dtsch Z Verdau Stoffwechselkr. 1979;39(5):226-33.
4
[Atresia of the extrahepatic bile ducts. Etiological hypothesis founded on a histological study of 130 fibrous remnants].
Arch Fr Pediatr. 1979 Nov;36(9 Suppl):III-XII.
5
[Liver ultrastructure in congenital atresia of the extrahepatic bile ducts].
Acta Gastroenterol Latinoam. 1984;14(4):265-71.
6
[Histopathologic study of the extrahepatic biliary system in uncorrectable congenital biliary atresia: its pathogenesis and type of atresia].[不可纠正性先天性胆道闭锁肝外胆道系统的组织病理学研究:其发病机制及闭锁类型]
Nihon Geka Gakkai Zasshi. 1985 May;86(5):587-601.
7
Significance of the anomalous arrangement of the pancreaticobiliary duct in the etiology of biliary atresia.胰胆管异常排列在胆道闭锁病因学中的意义。
Eur J Pediatr Surg. 2007 Apr;17(2):96-9. doi: 10.1055/s-2007-965028.
8
Immunohistochemistry of the liver and biliary tree in extrahepatic biliary atresia.肝外胆道闭锁时肝脏和胆道树的免疫组织化学
J Pediatr Surg. 2001 Jul;36(7):1017-25. doi: 10.1053/jpsu.2001.24730.
9
Polysplenia syndrome and splenic hypoplasia associated with extrahepatic biliary atresia.多脾综合征及脾发育不全与肝外胆道闭锁相关。
Arch Pathol Lab Med. 1980 Apr;104(4):212-4.
10
[Congenital atresia of the extrahepatic bile-ducts in two siblings (author's transl)].两例同胞先天性肝外胆管闭锁(作者译)
Klin Padiatr. 1978 Sep;190(5):512-8.

引用本文的文献

1
Intranuclear vermicellar bodies in human osteosarcoma and ossifying fibroma cells.人骨肉瘤和骨化性纤维瘤细胞中的核内蠕虫状小体。
J Cancer Res Clin Oncol. 1983;106(1):74-6. doi: 10.1007/BF00399902.
2
Bile duct and liver pathology in biliary atresia.胆道闭锁中的胆管和肝脏病理学
World J Surg. 1978 Sep;2(5):561-9. doi: 10.1007/BF01556047.