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首页> 外文期刊>Metabolism: Clinical and Experimental >Effects of bezafibrate on dyslipidemia with cholestasis in children with familial intrahepatic cholestasis-1 deficiency manifesting progressive familial intrahepatic cholestasis.
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Effects of bezafibrate on dyslipidemia with cholestasis in children with familial intrahepatic cholestasis-1 deficiency manifesting progressive familial intrahepatic cholestasis.

机译：苯扎贝特对表现为家族性肝内胆汁淤积的家族性肝内胆汁淤积-1缺乏症患儿的血脂异常合并胆汁淤积的影响。

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摘要

No appropriate pharmaceutical therapy has been established for dyslipidemia with cholestasis in progressive familial intrahepatic cholestasis (PFIC)-1. We evaluated the efficacy of bezafibrate in PFIC-1. We monitored the clinical presentation and lipoprotein metabolism of 3 patients, aged 3, 4, and 8 years, with FIC1 deficiency, manifesting PFIC-1, over 12 months of bezafibrate therapy. Pruritus was substantially alleviated in the 3 patients after initiation of bezafibrate. Cholestasis was alleviated in 2 of them. Serum high-density lipoprotein cholesterol and low-density lipoprotein cholesterol increased 1.6- to 2.0-fold and 1.1- to 1.2-fold, respectively; but the values remained low and normal, respectively. Serum lipoprotein X, which was at normal levels before treatment, was elevated to levels above the upper limit of the reference range. High serum triglyceride levels decreased by 15% to 30%, to normal levels, after treatment initiation. The activities of lipoprotein lipase and hepatic triglyceride lipase were increased, but those of high-density lipoprotein regulators remained unchanged. Liver expression of multidrug resistance protein-3, which regulates lipoprotein X synthesis, was enhanced by bezafibrate therapy. Bezafibrate treatment favorably affected pruritus, dyslipidemia, and cholestasis in PFIC-1.
机译：对于进行性家族性肝内胆汁淤积（PFIC）-1的胆汁淤积性血脂异常，尚未建立适当的药物治疗方法。我们评估了苯扎贝特在PFIC-1中的疗效。在苯扎贝特治疗12个月期间，我们监测了3名3、4、8岁，FIC1缺乏，表现为PFIC-1的患者的临床表现和脂蛋白代谢。苯扎贝特开始后3例患者的瘙痒得到了明显缓解。其中2例胆汁淤积得到缓解。血清高密度脂蛋白胆固醇和低密度脂蛋白胆固醇分别增加1.6到2.0倍和1.1到1.2倍。但该值分别保持较低和正常水平。在治疗前处于正常水平的血清脂蛋白X升高至高于参考范围上限的水平。治疗开始后，高血清甘油三酸酯水平降低了15％至30％，降至正常水平。脂蛋白脂肪酶和肝甘油三酸酯脂酶的活性增加，但高密度脂蛋白调节剂的活性保持不变。苯扎贝特治疗可增强调节脂蛋白X合成的多药耐药蛋白3的肝表达。苯扎贝特治疗有利于影响PFIC-1中的瘙痒，血脂异常和胆汁淤积。

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