胆汁淤积性肝病的发生机制及治疗进展PPT.ppt

Pruritus was scored by a semiquantitative scale of 1–4, with grade 1 ?occasional pruritus, grade 2 ?daily intermittent pruritus with preponderance of asymptomatic periods, grade 3 ?daily intermittent pruritus with preponderance of symptomatic periods and grade 4 ?persistent pruritus, day and night. * * Early cholestasis after liver transplantation diagnosis flowchart. Fibrosing cholestatic hepatitis, FCH; liver transplantation, LT; primary biliary cirrhosis, PBC; primary sclerosing cholangitis, PSC; ultrasound, US. * * Late cholestasis after liver transplantation diagnosis flowchart. Fibrosing cholestatic hepatitis, FCH; liver transplantation, LT; primary biliary cirrhosis, PBC; primary sclerosing cholangitis, PSC; ultrasound, US. * * * * * 正常人血清胆红素浓度为：5.0-20.8μmol/l，一般认为超过25.7 μmol/l有临床意义，但此时巩膜和皮肤尚不易察觉，称为“隐性黄疸”。当血清胆红素超过25.7～34.0 μmol/l临床上就出现黄疸。34-170 μmol/l为轻度黄疸，170-340 μmol/l为中度黄疸；大于340 μmol/l为高度黄疸。 结合胆红素/总胆红素大于35%为阻塞性或肝细胞性黄疸，比值小于20%为溶血性黄疸。 * INR大于1.2为异常（患者与正常人PT的比值） * 肝总管直径约0.5cm，胆总管直径约0.6～0.8cm。如ERCP失败可考虑经皮肝内胆道造影（PTC);如诊断基本明确，不必做肝活检。 * * * * * 急性期应限制脂肪摄人量，每天食物中脂肪含量不宜超过40g，长期重度黄疸时，应给予易吸收的中链脂肪酸 食物中胆固醇含量增加，胆汁中胆固醇也增加，使胆汁黏度增加，胆汁流量减少，加重胆汁淤积，故对胆汁淤积的患者应限制胆固醇的摄取。 应给予高热量，每天8400～12600kJ。 对有严重消化道症状者可由静脉输入10％葡萄糖溶液，症状好转后停用，但不必以此作为常规治疗。 慢性肝炎、肝硬化合并胆汁淤积时，适当减少蛋白量，以防止氨中毒，诱发肝性脑病的发生。 * * * * UDCA a is hydrophilic, non-cytotoxic bile acid, which is normally present in human bile, but in very low concentrations only. It is the major bile acid of the bear. In primary cholangiopathic liver diseases, such as PBC, UDCA protects the injured cholangiocytes by making the bile acid composition of bile hydrophilic. It stimulates biliary secretion and it inhibits bile acid induced apoptosis. Recently, it has been suggested that UDCA may stimulate - via binding to the nuclear receptor SXR/PXR the metabolism of hydrophobic bile acids by cytochrome-P3A4 to hydroxylated, hydrophilic and less cytotoxic bile acids. These bile acids can more easily be excreted via the kidney. * * * * * * 常用剂量：1~