多烯磷脂酰胆碱联合谷胱甘肽治疗妊娠期肝内胆汁淤积症患者的效果及对围产结局的影响

目的探讨多烯磷脂酰胆碱联合谷胱甘肽治疗妊娠期肝内胆汁淤积症(ICP)患者的效果及对围产结局的影响。方法选取2015年1月～2018年12月ICP患者124例,随机分为两组。两组均予以卧床休息、间断吸氧、高渗葡萄糖、维生素C及能量合剂等治疗。对照组在此基础上予以多烯磷脂酰胆碱针15 mL静脉滴注,1次/d;观察组在对照组基础上再加谷胱甘肽针2.4 g静脉滴注,1次/d。两组均连用2周。观察两组治疗后临床症状及血清生化指标改善情况,并比较其围产结局。结果治疗2周后,两组瘙痒评分均较治疗前显著下降(P0.01或P0.05),且观察组下降幅度较对照组更明显(P0.05);观察组黄疸和瘙痒消失时间明显少于或短于对照组(P0.05)。治疗2周后,两组血清ALT、TBA和AST水平均较治疗前明显下降(P0.01或P0.05),且观察组下降幅度较对照组更明显(P0.05)。同时观察组宫内窘迫、早产和新生儿窒息发生率明显低于对照组,分娩出血量明显少于对照组,新生儿体重明显大于对照组(P0.05)。结论多烯磷脂酰胆碱联合谷胱甘肽治疗ICP患者不仅能明显改善其临床症状,降低肝酶指标与胆汁酸水平,而且能降低发生不良围产结局,有利于母婴安全。     

Potential of ileal bile acid transporter inhibition as a therapeutic target in Alagille syndrome and progressive familial intrahepatic cholestasis

Alagille syndrome (ALGS) and progressive familial intrahepatic cholestasis (PFIC) are rare, inherited cholestatic liver disorders that manifest in infants and children and are associated with impaired bile flow (ie cholestasis), pruritus and potentially fatal liver disease. There are no effective or approved pharmacologic treatments for these diseases (standard medical treatments are supportive only), and new, noninvasive options would be valuable. Typically, bile acids undergo biliary secretion and intestinal reabsorption (ie enterohepatic circulation). However, in these diseases, disrupted secretion of bile acids leads to their accumulation in the liver, which is thought to underlie pruritus and liver‐damaging inflammation. One approach to reducing pathologic bile acid accumulation in the body is surgical biliary diversion, which interrupts the enterohepatic circulation (eg by diverting bile acids to an external stoma). These procedures can normalize serum bile acids, reduce pruritus and liver injury and improve quality of life. A novel, nonsurgical approach to interrupting the enterohepatic circulation is inhibition of the ileal bile acid transporter (IBAT), a key molecule in the enterohepatic circulation that reabsorbs bile acids from the intestine. IBAT inhibition has been shown to reduce serum bile acids and pruritus in trials of paediatric cholestatic liver diseases. This review explores the rationale of inhibition of the IBAT as a therapeutic target, describes IBAT inhibitors in development and summarizes the current data on interrupting the enterohepatic circulation as treatment for cholestatic liver diseases including ALGS and PFIC.     

丁二磺酸腺苷蛋氨酸对胆汁淤积性肝炎的疗效观察

[目的]评估丁二磺酸腺苷蛋氨酸(SAMe)治疗胆汁淤积性肝炎的效果及不良反应。[方法]68例胆汁淤积性肝炎患者被随机分为治疗组(38例)和对照组(30例)。2组均予常规保肝治疗,治疗组加用SAMe针剂1.0g/d静脉滴注,对照组加用苦黄针剂30ml/d静脉滴注,2组疗程均为4周。观察2组治疗前后临床症状与体征、生化指标[总胆红素(TBil)、直接胆红素(DBil)、谷丙转氨酶(ALT)、谷草转氨酶(AST)、γ-谷氨酸转肽酶("-GT)、总胆汁酸(TBA)]、疗效及不良反应。[结果]治疗后治疗组TBil、DBil、"-GT及TBA下降程度明显高于对照组(P0.05或P0.01);其灰白便和皮肤瘙痒改善率也明显高于对照组(P0.05或P0.01);治疗组总有效率为84.2%,明显高于对照组的60.0%(P0.05)。2组患者耐受性良好,均无严重不良反应发生。[结论]SAMe治疗胆汁淤积性肝炎有确切的疗效,且安全性好。     

Cavernous Transformation of the Portal Vein Causing Jaundice,Presenting in the Form of Wilson’s Disease

The following is a case review of portal vein cavernous malformation presenting with intermittent cholestasis and jaundice in a 4 year old child. Correct assessment was supported by radiology, later laparoscopy, yet hindered by histopathology representative Wilson’s disease and elevated urinary copper excretion. During surgical procedure the stenosis of the common bile duct secondary to extremely dilated portal vein reticulation was solved by Roux-en-Y choledochojejunostomy. After a one-year follow up the child remains asymptomatic.     

肝内大胆管旁射频消融治疗的安全性研究

目的 评估于犬肝内大胆管旁进行射频消融(radiofrequency ablation,RFA)治疗的安全性,为临床预防RFA造成胆管损伤提供实验依据.方法 健康成年杂种犬40只,随机分为4组,每组10只,雌雄不限.RFA射频针统一张开直径为(20.0±0.3)mm.RFA治疗时,能量由小到大序贯使用.开始能量为5W,以后每1 min升高5W.于距肝内大胆管不同距离(1.0～2.9 mm、3.0～4.9 mm、5.0～7.9 mm、8.0～10.0 mm)肝组织行RFA,观察治疗后不良反应、并发症、胆红素变化及胆管的病理学改变.结果 距肝内大胆管1.0～2.9 mm肝组织行RFA,术后一般情况差,胆红素升高明显,大部分发生严重并发症,胆管病理见明显坏死;距离3.0～4.9 mm行RFA,术后一般情况差,胆红素升高,可发生严重并发症,胆管病理可见坏死和空泡样变性;5.0～10.0 mm时,术后一般情况好,胆红素轻微升高,未见严重并发症发生,大部分胆管病理改变仅为上皮细胞空泡样变性或正常.结论 于肝内大胆管旁进行RFA时,射频针尖距离肝内大胆管≥5 mm时有较好的安全性.     

熊去氧胆酸胶囊联合地塞米松治疗老年肝内胆汁淤积性肝炎的疗效观察

目的探讨熊去氧胆酸胶囊联合地塞米松磷酸钠注射液治疗老年肝内胆汁淤积性肝炎的临床疗效。方法选取2014年12月—2016年12月重庆市大足区人民医院收治的老年肝内胆汁淤积性肝炎患者70例,按随机数字法将所有患者分为对照组和治疗组,每组各35例。对照组口服熊去氧胆酸胶囊,1粒/次,3次/d;治疗组在对照组的基础上静脉滴注地塞米松磷酸钠注射液,5 mg加入到10%葡萄糖500 m L中,1次/d。两组连续治疗2周。观察两组的临床疗效,比较两组的肝功能和生活质量评分情况。结果治疗后,对照组和治疗组的总有效率分别为71.43%、94.29%,两组比较差异具有统计学意义(P0.05)。治疗后,两组总胆汁酸(TBA)、直接胆红素(DBIL)、天冬氨酸氨基转氨酶(AST)和丙氨酸氨基转移酶(ALT)均显著下降,同组治疗前后比较差异有统计学意义(P0.05);且治疗组这些观察指标明显低于对照组,两组比较差异具有统计学意义(P0.05)。治疗后,两组腹部症状、全身症状、乏力、活动、焦虑和情感功能评分均显著升高,同组治疗前后比较差异有统计学意义(P0.05);且治疗组这些观察指标明显高于对照组,两组比较差异具有统计学意义(P0.05)。结论熊去氧胆酸胶囊联合地塞米松磷酸钠注射液治疗老年肝内胆汁淤积性肝炎具有较好的临床效果,可改善肝功能,提高生活质量,安全性较好,具有一定的临床推广应用价值。     

肝内胆管囊腺瘤再次手术临床分析

目的探讨肝内胆管囊腺瘤再次手术的诊治特点。方法回顾性分析总结我院4例肝内胆管囊腺瘤根治性切除术后再次手术的诊治特点。结果肝内胆管囊腺瘤再次手术2例腹胀2例无明显症状,B超、CT、MRI可发现肝内占位难以确诊,再次手术均行根治性切除术,术后病理提示:囊腺瘤复发1例,囊腺癌1例,原发性肝癌1例,肝恶性纤维细胞瘤1例,术后均无明显并发症,随访2例死亡,生存时间为7个月与21个月,1例囊腺瘤痊愈至今未复发,1例肝内胆管囊腺癌随访18个月健在。结论肝内胆管囊腺瘤术后再发肝占位性质难以术前确诊,首选手术治疗,严格术前评估能够获得较好的治疗效果。     

胆宁片中3种游离蒽醌类成分对急性胆汁淤积大鼠的防治作用

目的:研究胆宁片中大黄素、大黄素甲醚和大黄酚对急性胆汁淤积大鼠的防治作用。方法:采用ɑ-异硫氰酸萘酯(ANIT)复制胆汁淤积模型,实验分为正常对照组、ANIT模型组(60 mg·kg^-1)、胆宁片(3 g·kg^-1)、大黄素(40 mg·kg^-1)、大黄素甲醚(7 mg·kg^-1)和大黄酚(4 mg·kg^-1)保护组。除正常对照组和模型组其它各组大鼠均灌胃给予保护药,1次/天,连续9 d;第7次给保护药12 h后,模型组和其它各保护组动物均灌胃给予ANIT造模。造模48 h后观察各组大鼠胆汁流量变化,测定血清生化指标和检查肝脏病理组织形态。结果:胆宁片和大黄素能显著增加ANIT所抑制的大鼠胆汁流量;能显著降低ANIT导致的大鼠血清中谷丙转氨酶(ALT)、谷草转氨酶(AST)、γ-谷氨酰转氨酶(γ-GTP)、碱性磷酸酶(ALP)活性升高,以及逆转总胆红素(TBil)、直接胆红素(DBil)和总胆汁酸(TBA)的水平;而且胆宁片和大黄素能显著改善大鼠肝脏的组织形态。结论:胆宁片和大黄素对ANIT所致的大鼠急性胆汁淤积具有明显的防治作用,其中大黄素可能为胆宁片发挥利胆药效的有效成分之一。