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首页> 外文期刊>Medical Sciences >Remodeling of Intrahepatic Ducts in a Model of Caroli Syndrome: Is Scar Carcinoma a Consequence of Laplace’s Law?
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Remodeling of Intrahepatic Ducts in a Model of Caroli Syndrome: Is Scar Carcinoma a Consequence of Laplace’s Law?

机译:在Caroli综合征模型中肝内导管的重塑:疤痕癌是拉普拉斯定律的结果吗?

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Caroli syndrome, characterized by saccular dilatation of intrahepatic ducts and congenital hepatic fibrosis, is without therapy in part due to its ultra-rare prevalence and the apparent lack of availability of a suitable experimental model. While the PCK rat has long been used as a model of fibropolycystic kidney disease, hepatobiliary biophysics in this animal model is incompletely characterized. Compared to age-matched, wild-type controls, the PCK rat demonstrated severe hepatomegaly and large saccular dilated intrahepatic ducts. Nevertheless, hepatic density was greater in the PCK rat, likely due to severe duct wall sclerosis accompanied by scarring across the hepatic parenchyma. Extracellular matrix accumulation appeared proportional to duct cross-sectional area and liver volume and appeared compensatory in nature. The PCK rat livers exhibited both cholangiocarcinoma and hepatocellular carcinoma coincident with areas of increased extracellular matrix deposition. Together, these data suggest that the PCK rat model mimics at least in part the spectrum of hepatobiliary pathology observed in Caroli syndrome and highlights the attendant risk associated with this disease.
机译:Caroli综合征的特征是肝内导管囊状扩张和先天性肝纤维化,因此没有进行治疗,部分原因是其超罕见的患病率以及明显缺乏合适的实验模型。虽然PCK大鼠长期以来一直被用作纤维多囊性肾脏疾病的模型,但该动物模型中的肝胆生物物理学尚未完全表征。与年龄匹配的野生型对照相比,PCK大鼠表现出严重的肝肿大和大的囊状扩张肝内导管。尽管如此,PCK大鼠的肝脏密度更高,这可能是由于严重的管壁硬化并伴有整个肝实质的瘢痕形成所致。细胞外基质的积累与导管的横截面积和肝脏体积成正比,并且本质上是补偿性的。 PCK大鼠肝脏同时显示胆管癌和肝细胞癌,同时细胞外基质沉积增加。总之,这些数据表明,PCK大鼠模型至少部分模拟了在Caroli综合征中观察到的肝胆病理学谱,并突出了与该疾病相关的伴随风险。

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