Congenital Hepatic Fibrosis

We present four pediatric patients with histologically proven congenital hepatic fibrosis. The patients had diverse manifestations. The first two patients were seven-year old identical twins who presented with hepatosplenomegaly and were found to have portal hypertension with esophageal varices. The third patient was a newborn who had intractable ascites, secondary to portal hypertension. The fourth patient was a seven-year old with adult type polycystic disease of the kidney but no evidence of portal hypertension. Contrary to what has been reported in the literature of the appearance of portal hypertension in the late childhood period, congenital hepatic fibrosis may present in any age group with portal hypertension.     

酒精性肝纤维化的病理研究

本文观察了66例酒精性肝病(ALD)的肝纤维化特点。酒精性肝纤维化(AHF)主要有三种形成方式:①窦周纤维化,最具特征;②静脉周围纤维化;③汇管区及汇管区周围纤维化,后者包括汇管区胶原增多,扩大,汇管区周围星芒状纤维化及界板侵蚀。重度AHF时以Ⅰ型胶原沉积为主。AHF的形成与Ito细胞增殖密切相关。图像定量分析表明,酒精性肝病肝纤维化程度重于慢性病毒性肝炎者。     

肝纤维化的临床研究进展

肝纤维化是所有慢性肝病的共同特征，多种因素参与其发生和发展。各种因素所致的肝纤维化的进程不同，但目前对其自然史的认识尚不全面。肝纤维化的非创伤性诊断临床实际应用尚有一定距离。对临床、生化和影像等指标进行综合评估应是今后努力的方向。肝纤维化和肝硬化可出现逆转和消失完全改变了对其治疗的认识。目前尚无有效的肝纤维化治疗药物，最有效的治疗方法仍是病因治疗。肝纤维化药物治疗开发存在不少困难，但前景广阔。     

Multimodality Imaging Assessment of Myocardial Fibrosis

Myocardial fibrosis, seen in ischemic and nonischemic cardiomyopathies, is associated with adverse cardiac outcomes. Noninvasive imaging plays a key role in early identification and quantification of myocardial fibrosis with the use of an expanding array of techniques including cardiac magnetic resonance, computed tomography, and nuclear imaging. This review discusses currently available noninvasive imaging techniques, provides insights into their strengths and limitations, and examines novel developments that will affect the future of noninvasive imaging of myocardial fibrosis.     

New Developments in Therapy for Hepatic Fibrosis

Over the past 30 years, methods have been developed for isolating the principal cell types of the liver. These have made it possible to analyze quantitatively and in detail the injury response of the liver, which underlies the scarring process known as fibrosis. Attention has focused in particular on hepatic stellate cells because of their dynamic response to injury, termed activation. Metabolically quiescent in the normal liver, stellate cells in injury rapidly display properties of myofibroblasts, including expression de novo of smooth muscle proteins,     

乙肝3号冲剂抗肝纤维化的临床研究

目的：观察乙肝3号冲剂抗肝纤维化的疗效。方法 选择慢性乙肝型肝炎肝纤维化指标异常的患者120例,随机分成两组;治疗组乙肝3号冲剂,对照组皮下注射α-2b干扰素300万U,观察治疗后肝功能和肝纤维化指标的变化。结果：经治疗后治疗组患者丙氨酸转氨酶,γ球蛋白、血清透明质酸、层粘蛋白和Ⅳ胶原均明显降低,与对照组比较,有显著性差异（P＜0．05或0．01）。结论：乙肝3号具有良好的抗肝纤维化作用。     

双甲五灵胶囊治疗肝纤维化的临床研究

目的 :观察中药双甲五灵胶囊治疗肝纤维化的疗效。方法 :将 96例患者随机分为双甲五灵胶囊治疗组(A组 )和基础保肝对症治疗对照组 (B组 ) ,观察两组治疗前后临床症状、体征、肝功能 [总胆红素 (TBi L)、丙氨酸转氨酶 (AL T)、白蛋白 (AL B) ]、血清透明质酸 (HA)、 型前胶原 (PC )、转化生长因子 β1 (TGF- β1 ) ,并进行统计学处理。结果 :双甲五灵胶囊对改善乏力、纳差、腹胀、肝区不适等症状及脾大回缩、恢复肝功能疗效明显 ,并能降低血清 HA、PC 、TGF- β1 浓度 (P <0 .0 5 )。结论 :双甲五灵胶囊治疗肝纤维化效果较好 ,未发现副作用。     

中药复方肝欣合剂抗肝纤维化作用的临床研究

目的:观察中药复方肝欣合剂对慢性乙型肝炎及肝纤维化的疗效。方法:选择慢性乙型肝炎肝功能及肝纤维化指标异常的患者80例,随机分成两组,治疗组患者口服中药复方肝欣合剂,对照组患者口服强肝丸,观察治疗后肝功能和肝纤维化指标的变化。结果:两组患者血清ALT、TBil、HA、LM和PCⅢ均有明显降低,Alb亦有明显提高,两者相比较有统计学意义(P<0.05或P<0.01)。结论:中药复方肝欣合剂具有明显的抗肝纤维化作用。     

小鼠血吸虫病肝纤维化的超微结构动态观察

目的　研究血吸虫病小鼠肝纤维化过程中几种相关细胞和肝组织超微结构动态变化 ,以探讨血吸虫病肝纤维化的可能机制。　方法　日本血吸虫尾蚴经皮肤感染小鼠建立血吸虫病肝纤维化模型。常规方法制作肝组织透射电镜标本并观察。常规 HE染色观察其病理变化。　结果　 HE染色显示小鼠血吸虫病肝纤维化模型建立成功。电镜观察显示小鼠感染后 6 wk,急性肉芽肿周围的肝细胞发生坏死 ,肝窦内皮细胞窗孔减少 ,贮脂细胞 (FSC)脂滴减少 ,枯否细胞胞浆出现大吞噬体和粗面内质网。 8wk时部分肝细胞发生脂肪变性 ,少数肝细胞间隙增宽 ,间面出现微绒毛。肝窦周隙内充满大量胶原纤维 ,并形成肝窦毛细血管化。FSC胞浆出现含胶原原纤维的分泌泡 ,周围见大量胶原纤维。枯否细胞粗面内质网增加。 10 wk时 FSC转变为肌成纤维细胞。 12 wk时肌成纤维细胞减少 ,成纤维细胞和纤维细胞增加。　结论　 FSC被激活转化为肌成纤维细胞是血吸虫病肝纤维化发生的关键环节 ,激活的枯否细胞、损伤的肝细胞和肝窦内皮细胞与 FSC的活化密切相关 ,肝窦毛细血管化可能加速肝纤维化的发展。     

非创伤性检查诊断肝纤维化的进展

肝纤维化的诊断依赖肝活检,但由于其创伤性而难以被患者接受,因此非创伤性检查诊断肝纤维化始终是肝病领域中的研究热点。以往仅靠几项血清学指标预测肝纤维化并不足够准确,近年影像学技术的革新为诊断肝纤维化提供了良好的前景,血清学联合影像学可进一步提高诊断准确性。本文就非创伤性检查诊断肝纤维化的进展作一综述。     

排钱草对大鼠实验性肝纤维化的影响

目的:观察排钱草水、醇提取物对肝纤维化的影响。方法:在给大鼠皮下注射CCl_4进行肝纤维化模型制作的同时给予排钱草水、醇提取物(相当于每日原生药5g/ke、3g/kg),给药6周后检测大鼠肝组织羟脯氨酸(Hyp)及血清中谷丙转氨酶(ALT)和碱性磷酸酶(ALP)活性,血清蛋白电泳测定γ-球蛋白,并观察肝脏的组织病理改变,与联苯双酯进行药效对照。结果:排钱草水、醇提取物的高、低剂量组和联苯双酯组均能显著降低肝脏Hyp含量及血清ALT、ALP的活性,降低γ-球蛋白以维持白/球(A/G)比值,对大鼠肝细胞坏死和胶原纤维增生亦有显著减轻作用。结论:排钱草水、醇提取物能减轻CCl_4致大鼠的肝脏损害,有利于减轻肝纤维化。     

Noninvasive Markers of Hepatic Fibrosis in Chronic Hepatitis B

A serum biomarker (FibroTest; Biopredictive, Paris, France; FibroSure; LabCorp, Burlington, USA) and liver stiffness measurement (LSM) by Fibroscan (Echosens, Paris, France) have been extensively validated in chronic hepatitis C. This review updates the clinical validation of serum biomarkers and LSM in patients with chronic hepatitis B (CHB). One meta-analysis combined all published studies and another used a database combining FibroTest individual data. Sensitivity analysis assessed the impact of several factors, including authors’ independence, length of biopsy, ethnicity, hepatitis B early antigen status, viral load, and alanine aminotransferase value. Only two biomarkers had several validations: FibroTest (8 studies, 1,842 patients), and Fibroscan (5 studies, 618 patients). For the diagnosis of advanced fibrosis, the standardized area under the receiver operating curve was 0.84 (0.79–0.86) for FibroTest and 0.89 (0.83–0.96) for LSM, without significant difference. No significant factors of variability were identified for FibroTest’s performance. In conclusion, FibroTest and LSM were the most validated biomarkers of fibrosis in CHB. However, the reliability of Fibroscan must be better assessed.