Histological findings in the livers of patients with neonatal intrahepatic cholestasis caused by citrin deficiency
Histological findings in the livers of patients with neonatal intrahepatic cholestasis caused by citrin deficiency
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DOI：
10.1111/j.1872-034x.2009.00594.x
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发表时间：
2010-03-01
影响因子：
4.2
通讯作者：
Saheki, Takeyori
中科院分区：
文献类型：
--
作者：
Kimura, Akihiko;Kage, Masayoshi;Saheki, Takeyori
目的：对30例胆汁性胆汁淤积症(NICCD)患儿的肝组织进行SLC25A13基因分析，以了解胆汁淤积症(NICCD)患儿肝脏的组织学特点。方法：对肝活检标本进行苏木精-伊红、天青和柏林蓝染色。不同标本的炎症程度不同，一半出现中度或重度炎症变化。几乎所有的标本都观察到肝细胞中的脂肪沉积，其中20例(67%)有严重的脂肪肝。两种类型的肝细胞混合有大泡或微泡脂肪滴，胆汁淤积率为77%。含铁血黄素主要分布于汇管区的门静脉周围肝细胞和巨噬细胞，占57%。结论：大泡和微泡混合脂肪肝细胞和上述表现，如脂肪肝、胆汁淤积、坏死性炎症反应和铁沉积，在婴幼儿和成人的其他肝病中几乎从未见过。我们认为NICCD是一种具有独特肝脏病理特征的疾病。
Aim:To characterize the histological features of the livers of patients with neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD), we studied specimens from 30 patients diagnosed with NICCD by genetically analyzing the SLC25A13 gene.Methods:Liver biopsy specimens were subjected to hematoxylin-eosin, Azan, and Berlin-blue staining.Results:Most specimens showed varying degrees of fibrosis. The degree of inflammation varied among the specimens, with half showing moderate or severe inflammatory changes. Fat deposition in hepatocytes was observed in almost all of the specimens, and severe fatty liver was noted in 20 (67%) of them. There was a mixture of two types of hepatocytes with macrovesicular or microvesicular fat droplets, and cholestasis was observed at a rate of 77%. Hemosiderin deposition, mostly mild and localized in periportal hepatocytes and macrophages in portal areas, was observed in 57% of the specimens.Conclusion:A combination of mixed macrovesicular and microvesicular fatty hepatocytes and the above-described findings, such as fatty liver, cholestasis, necroinflammatory reaction and iron deposition, are almost never observed in other liver diseases in infants and adults. We believe that NICCD is a disease with characteristic hepatopathological features.