Signals for Hepatic Figrogenesis in Pediatric Cholestatic Liver Disease: Review and Hypothesis
Signals for Hepatic Figrogenesis in Pediatric Cholestatic Liver Disease: Review and Hypothesis
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小儿胆汁淤积性肝病中肝纤维生成的信号：回顾和假设
DOI：
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发表时间：
2004
期刊：
影响因子：
--
通讯作者：
P. Lewindon
中科院分区：
文献类型：
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作者：
G. Ramm;Anita C. Hoskins;S. Greco;T. Pereira;P. Lewindon
新生儿胆汁淤积性肝病 儿童胆汁淤积性肝病的发生是由于肝细胞胆汁形成的改变或胆汁通过肝内胆管或肝外胆管流出肝细胞的中断所致[1]。肝脏疾病通常出现在出生后的最初几周内。许多疾病在新生儿中表现出胆汁淤积性黄疸，包括（a）多种胆管病，例如肝外胆道闭锁、囊性纤维化（CF）、胆总管囊肿、α1-抗胰蛋白酶缺乏症和阿拉吉尔综合征，（b）胆囊的几种异常，例如胆石症和胆汁淤积性黄疸。 胆囊炎，以及 (c) 胆汁酸转运障碍。新生儿胆汁淤积最常见的形式是胆道闭锁，相对频率约为 30% [1]。为了实施有效的治疗干预，早期诊断至关重要。这可能很困难，因为许多不同形式的新生儿胆汁淤积的许多表型表现相似，甚至可能重叠。
Neonatal Cholestatic Liver Diseases Cholestatic liver disease in children occurs as a result of either an alteration in hepatocyte bile formation or disruption of bile flow out of the hepatocyte through intrahepatic bile ductules or extrahepatic bile ducts [1]. Liver disease usually appears within the first few weeks following birth. A large number of disorders exhibit cholestatic jaundice in neonatal life including (a) numerous cholangiopathies, such as extrahepatic biliary atresia, cystic fibrosis (CF), choledochal cyst, alpha1-Antitrypsin deficiency and Alagille's syndrome, (b) several abnormalities of the gall bladder, such as cholelithiasis and cholecystitis, and (c) bile acid transport disorders. The most commonly occurring form of neonatal cholestasis is biliary atresia, representing a relative frequency of approximately 30% [1]. In order to administer effective therapeutic intervention early diagnosis is critical. This can prove difficult as a number of phenotypic manifestations of the many different forms of neonatal cholestasis are similar and may even overlap.
膳食脂质和维生素 E 对胆管结扎大鼠线粒体脂质过氧化和肝损伤的影响。
影响因子：
6.5
作者：
Sokol,RJ;Devereaux,M;Khandwala,RA
通讯作者：
Khandwala,RA