血浆纤维连接蛋白水平与肝脏疾病的关系

对114例不同肝脏病患者和30例正常人血浆纤维连接蛋白（Fn）进行了测定，同时检测肝功能各项指标。结果，正常对照组血浆Fn水平为2．94±0．60g／L，与对照组相比，急性肝炎（2．71±0．92g／L）、慢性活动性肝炎（2．82±0．94g／L）无显著差异（P＞0．05）；重症肝炎（1．64±0．68g／L、肝硬化（1．53±0．57g／L）显著降低（P＜0．001）。丙氨酸转氨酶（ALT）在30u以下者，Fn浓度在正常范围，随ALT升高，Fn水平降低。血浆Fn与肝功能各指标相关分析表明，血浆Fn与ALT、血清总胆红素（TBil）、直接胆红素（DBil）和麝香草酚浊度（TFT）呈负相关，与总蛋白（TP）和白蛋白（Alb）呈正相关，其中与ALT（r＝－0．8393）和Alb（r＝0．9377）相关性有显著意义（P＜0．05，P＜0．01）。上述结果提示血浆Fn水平与肝脏病的严重性及肝功能的损害程度有一定关系。     

优思弗治疗胆汁淤积性肝病64例临床观察

目的观察优思弗对胆汁淤积性肝病的疗效。方法128例胆汁淤积性肝病病人随机分为治疗组和对照组，每组各64例，对照组给予甘利欣及常规护肝治疗，治疗组在与对照组相同治疗的基础上再给予优思弗250mg／次，3次，d，po，疗程均为8wk。结果治疗后两组在症状恢复方面比较：纳差、乏力症状有明显改善，但差异无显著性（P〉0．05）；而皮肤瘙痒差异有显著性（P〈0．05）。体征复常方面比较：肝、脾肿大差异无显著性（P〉0．05）；而胆囊壁增厚、水肿差异有显著性（P〈0．05）。治疗组治疗后肝功能指标下降幅度均明显优于对照组，差异有显著性，其中总胆红素（TBi1）、直接胆红素（DBi1）、胆汁酸（TB）与对照组比较，P〈0．01；丙氨酸氨基转移酶（ALT）、碱性磷酸酶（ALP）、γ-谷氨酰转移酶（T-GT）与对照组比较P〈0．05。根据疗效判定标准，总有效率为93．75％，与对照组比较P〈0．01。结论优思弗治疗胆汁淤积性肝病疗效显著，可作为胆汁淤积性肝病的首选药物。     

肝病患者Bactec 9120全自动血培养仪培养标本分析

目的分析肝病患者Bactec 9120全自动血培养仪培养标本分离菌及阳性报警时间分布情况。方法培养3471瓶肝病患者标本,使用Phoenix100自动细菌鉴定仪进行细菌生化鉴定和药物敏感性分析。结果共分离出356株,42种病原菌,革兰阴性杆菌197株(55.3%,197/356),阳性球菌132株(37.1%,132/356),念珠菌24株(7.6%,24/356)。总阳性率10.3%,其中血培养阳性率11.2%,腹水培养阳性率4.3%,胆汁培养阳性率92.9%。分离率居前三位的病原菌依次为大肠埃希菌(20.2%),凝固酶阴性葡萄球菌(17.1%),肺炎及产酸克雷伯氏菌(12.9%)。在全部356例阳性瓶中,8h阳性报告率为17.1%,8h～24h阳性报告率为62.5%,24h～48h及72小时阳性率分别为14.0%、4.0%,>72h为2.4%。结论肝病患者的培养标本分离菌以革兰氏阴性杆菌为主,其中大肠埃希菌占首位,其次为凝固酶阴性葡萄球菌、肺炎及产酸克雷伯氏菌。培养标本在8h～24h阳性报告率最高。     

Sickle cell hepatopathy: clinical presentation, treatment, and outcome in pediatric and adult patients

BACKGROUND: Standard diagnostic criteria and therapy are lacking for sickle cell hepatopathy, an uncommon complication of sickle cell disease. Here we propose diagnostic and therapeutic guidelines based on our experience and on reported cases. METHODS: We defined sickle hepatopathy by a total serum bilirubin concentration >13 mg/dl not explained by severe acute hemolysis, viral hepatitis, extrahepatic obstruction, or hepatic sequestration. We reviewed the records of all children with sickle hepatopathy seen at our institution during the past 20 years and the reported cases from the literature. Patients were categorized into two groups based on whether hepatic dysfunction at presentation was mild (Group I) or severe (Group II). RESULTS: Seven patients were identified from our institution and 37 patients from the literature. The 44 patients were evenly divided between the two groups. Group I patients had a significantly lower mean age (11.8 years vs. 24.5 years, P = 0.0001), maximum bilirubin level (36.2 mg/dl vs. 76.8 mg/dl, P = 0.0008), and frequency of treatment with exchange transfusions (P = 0.03). Overall, mortality was 4% in Group I and 64% in Group II (P = 0.0001). Gender and recurrence rate did not differ. Among Group II patients, only two of nine who received exchange transfusion died, whereas 12 of 13 who did not receive exchange transfusion died (P = 0.0015). CONCLUSIONS: Sickle cell hepatopathy is an uncommon complication characterized by extreme hyperbilirubinemia and either mild or severe hepatic dysfunction. Children and adults can present with either form; however, adults have a higher frequency of the severe form. Exchange transfusion may be the only effective management for initial episodes of severe sickle cell hepatopathy.     

线粒体m-AST在肝病诊治中的应用

目的:探讨不同肝病患者血清线粒体天门冬氨酸氨基转移酶同工酶(m-A ST)的变化及其临床意义。方法:用全自动生化分析仪测定了130例不同肝病患者血清m-A ST活性。结果:肝病患者m-A ST变化与ALT、A ST变化呈正相关,且因病情不同而有不同程度的升高。急性肝炎组m-A ST明显升高,与对照组相比,升高13.3倍,其次是慢性活动性肝炎和肝硬化失代偿期,升高10.9倍和9.9倍,慢性迁延性肝炎、肝硬化代偿期、阻塞性黄疸、肝癌组m-A ST轻度升高,升高倍数分别为1.3~3.2。不同肝病患者经住院治疗2周后(第3周)随着肝功能的恢复,急性肝炎m-A ST明显下降,与入院时相比差异有极显著性,P<0.01,慢性活动性肝炎虽有下降,但与入院时相比无明显差异,P>0.05,迁延性肝炎变化不明显。肝硬化代偿期,m-A ST变化不明显,失代偿期,m-A ST持续不降,且有升高。阻塞性黄疸和肝癌治疗前后m-A ST基本无变化。结论:检测并动态观察m-A ST活性,可作为诊断急慢性肝炎,评价疗效,判断肝病预后的一项新的生化指标。     

Mitochondrial hepatopathy: Respiratory chain disorders- ‘breathing in and out of the liver’

Mitochondria, the powerhouse of a cell, are closely linked to the pathophysiology of various common as well as not so uncommon disorders of the liver and beyond. Evolution supports a prokaryotic descent, and, unsurprisingly, the organelle is worthy of being labeled an organism in itself. Since highly metabolically active organs require a continuous feed of energy, any dysfunction in the structure and function of mitochondria can have variable impact, with the worse end of the spectrum producing catastrophic consequences with a multisystem predisposition. Though categorized a hepatopathy, mitochondrial respiratory chain defects are not limited to the liver in time and space. The liver involvement is also variable in clinical presentation as well as in age of onset, from acute liver failure, cholestasis, or chronic liver disease. Other organs like eye, muscle, central and peripheral nervous system, gastrointestinal tract, hematological, endocrine, and renal systems are also variably involved. Diagnosis hinges on recognition of subtle clinical clues, screening metabolic investigations, evaluation of the extra-hepatic involvement, and role of genetics and tissue diagnosis. Treatment is aimed at both circumventing the acute metabolic crisis and long-term management including nutritional rehabilitation. This review lists and discusses the burden of mitochondrial respiratory chain defects, including various settings when to suspect, their evolution with time, including certain specific disorders, their tiered evaluation with diagnostic algorithms, management dilemmas, role of liver transplantation, and the future research tools.     

血浆置换联合血液灌流治疗重度药物性肝病12例临床分析

目的探讨血浆置换和血液灌流在重度药物性肝损害治疗中的作用。方法对我院2003年~2004年收治的12例重度药物性肝功能损害患者的治疗情况进行回顾性分析。结果12例患者(包括4例急性肝功能衰竭)患者经过内科综合治疗联合血浆置换和血液灌流治疗,均痊愈出院。结论血浆置换联合血液灌流是治疗重度药物性肝损害有效的重要手段。     

丹参注射液合大黄（庶/虫）虫丸治疗慢性肝病纤维化的临床研究

为观察丹参注射液合大黄Zhe虫丸配合西药治疗慢性乙型肝炎、肝硬化纤维化的临床疗效，选择肝硬化患者46例，设健康对照组40例比较，结果显示在总有效率、临床症状、体征、肝功能、门舒脉主干血流动力学参数、肝纤维化指标上，治疗组疗效优于对照组(P＜0．05)，说明丹参注射液合大黄Zhe虫丸是一种安全、有效、经济的治疗侵性肝病纤维化的药物。     

老年肝病患者医院感染临床分析

目的　探讨老年肝病患者医院感染状况及对转归的影响。方法　回顾性分析、比较老年组和非老年组 ,老年各类肝病组医院感染的发生率和病死率。结果　老年组医院感染发生率和病死率分别为 33.93%和2 5 .2 6 % ,明显高于非老年组的 1 4 .6 6 %和 5 .95 % (P <0 .0 1 ) ;老年急慢性肝炎、重型肝炎、肝硬化各组间医院感染率和病死率的差异显著 (P <0 .0 1 ) ,以重型肝炎组感染率和病死率较高 ;感染部位以呼吸系统最多见 ;病原菌以革兰阴性菌及真菌为主。结论　真菌已成为老年肝病患者医院感染的重要病原菌。老年肝病患者 ,尤其是重型肝炎患者 ,易发生各种医院感染 ,并严重影响其预后