拉米夫定治疗慢性乙型肝炎病毒感染的近期疗效

目的评价拉米夫定治疗不同临床类型慢性乙型肝炎病毒（HBV）感染的近期疗效。方法口服拉米夫定150mg,每日1次,连服6个月,治疗慢性乙型肝炎病人40例,肝炎肝硬化18例,慢性重型肝炎10例。观察其临床、生物化学、血清学和病毒学改变。结果（1）慢性乙型肝炎病情缓解。对照组病毒血症持续,27．5％病人于随访期内肝炎复发（P＜0．001）。同时观察拉米夫定联用干扰素治疗病人20例,未见提高疗效。（2）肝炎肝硬化病情渐趋稳定,肝功能好转,Child—Pugh积分下降。（3）慢性重型肝炎除2例服药不足3个月死亡外,余8例病情缓解,随着肝功能改善,生活质量显著好转。结论拉米夫定适用于治疗慢性乙型肝炎,对处于HBV复制状态的肝硬化和重型肝炎也有效。     

799例重型肝炎患者的临床病原学与实验室分析

目的探讨乙型重型肝炎患者乙型肝炎病毒(HBV)DNA定量、e抗原表达与病死率的相关性,为重型肝炎临床治疗提供参考。方法统计我院2000-2004年各型重型肝炎的发病率,进一步应用荧光定量多聚酶链反应方法检测乙型重型肝炎患者血清HBV DNA,应用微粒子方法检测乙型肝炎e抗原表达情况,并分析其与病死率及抗病毒治疗的临床疗效间的关系。结果(1)重型肝炎中乙型肝炎占83．50％,慢性重型肝炎中乙型肝炎占96．77％;(2)5年间慢性乙型重型肝炎患者HBV DNA定量大于1×10,拷贝／ml组,总病死率为53．25%,小于1×105拷贝／ml组,病死率为34．50％,差异有统计学意义(P<0．01);e抗原表达对病死率无影响;(3)2004年,慢性乙型重型肝炎患者HBV DNA定量大于1×105拷贝／ml病例加用拉米夫定抗病毒治疗,病死率由2000年的54．64%下降至2004年的30．38%,差异有统计学意义(P<0．01)。结论重型肝炎以慢性乙型重型肝炎为主,病毒载量高是高病死率关键因素之一,抗病毒治疗可以降低患者的病死率。     

抗病毒基础上应用糖皮质激素治疗慢性重型乙型肝炎临床分析

目的 评价在抗病毒治疗的基础上应用糖皮质激素治疗早期慢性重型乙型肝炎的临床疗效.方法 选取63例早期慢性重型乙型肝炎患者,随机分为治疗组33例,对照组30例,其中治疗组在综合治疗的基础上应用拉米夫定或恩替卡韦抗病毒及地塞米松等糖皮质激素治疗.对照组30例采用拉米夫定或恩替卡韦抗病毒及常规综合治疗.观察两组治疗前后的临床症状、血清总胆红素、凝血酶原活动度、HBVDNA及并发症等情况.结果 8周后与对照组比较,治疗组患者血清总胆红素明显降低(P＜0.05),凝血酶原活动度提升幅度大(P＜0.05),肝性脑病、肝肾综合征等并发症减少(P＜0.05),临床症状明显好转.4周后血清PCRHBVDNA阴转率为87.1%与对照组的85.1%相比,差异无统计学意义(P＞0.05),治疗组的治疗失败率为15.2%(5/33)明显低于对照组的40.0%(12/30)(P＜0.05).结论 在抗病毒治疗的基础上应用糖皮质激素对早期慢性重型乙型肝炎疗效显著,安全性好.     

替比夫定治疗慢性乙型重型肝炎近期疗效观察

目的观察替比夫定治疗慢性乙型重型肝炎12周的疗效。方法对照组24例慢性乙型重型肝炎患者采用常规内科综合治疗,治疗组30例在常规综合治疗基础上加用替比夫定600mg/d抗病毒治疗,观察3个月。观察两组治疗前后肝功能、凝血酶原活动度、吲哚菁绿15分钟排泄率（ICGR15）和HBV DNA定量的变化。结果在治疗3个月时,对照组有2例患者发生肝性脑病,经处理恢复,治疗组有1例患者发生自发性细菌性腹膜炎,治愈。两组均无患者死亡;治疗组TBIL下降、PTA上升和IC-GR15下降均优于对照组（P〈0.05）;治疗组HBV DNA阴转率为83.5%,对照组HBV DNA阴转率为12.5%,两组差异有统计学意义（P〈0.01）;治疗组HBeAg阴转率和HBeAg血清学转换率分别为25%和20%,与对照组（0.0%,0.0%）比较差异有统计学意义（P值均〈0.05）。结论 LdT治疗伴有病毒复制的乙型重型肝炎,能快速抑制HBV DNA复制、起效快e,抗原血清转换率高,改善肝功能。     

拉米夫定和干扰素α及苦参碱防治乙型肝炎肝衰竭生存者的肝纤维化进展

目的 探讨防止乙型肝炎肝衰竭生存患者肝纤维化进展的方法.方法 在多中心选择亚急性或慢加亚急性乙型肝炎肝衰竭生存者(A组)100例随机分为3组,在度过肝衰竭后分别给予干扰素α、拉米夫定和拉米夫定联合苦参碱治疗6个月,选择慢性乙型肝炎肝衰竭生存者(B组)100例随机分为2组,分别给予拉米夫定和拉米夫定联合苦参碱治疗6个月.全部患者均随访6个月,观察血生物化学、血清学、病毒学和肝组织学应答情况.根据资料不同采用t检验和χ2检验进行统计学分析.结果 全部A组患者生存,B组接受拉米夫定治疗的患者死亡3例(6.1%),接受拉米夫定联合苦参碱治疗的患者死亡4例(7.8%,P＞0.05);B组患者在治疗结束时血清总胆红素水平未降至正常,拉米夫定治疗使超过90%患者血清HBV DNA阴转,A组接受干扰素α或拉米夫定联合苦参碱治疗的患者HBeAg/抗-HBe血清转换率分别为52.9%(9/17)和31.3%(5/16),均高于拉米夫定治疗者(1/17,5.9%);在A组接受拉米夫定治疗的患者在随访结束时肝组织Knodell HAI计分(7.2±0.8)低于干扰素α治疗者(8.2±1.3),P＜0.05;联合苦参碱治疗可使HAI计分进一步降低(6.9±0.7),差异有统计学意义(P＜0.01).拉米夫定或拉米夫定联合苦参碱治疗对B组患者能抑制肝内炎症病变,但对已形成的肝纤维化无明显的逆转作用. 结论 乙型肝炎肝衰竭生存患者长期口服核苷类抗病毒药对阻断肝硬化的形成有重要作用,适时联合苦参碱可提高HBeAg/抗-HBe血清转换率和抑制肝内纤维组织增生.     

血清总胆固醇水平对重型肝炎诊断及预后判断价值

探讨血清总胆固醇(serum total cholesterol简称sTC)水平对重型病毒性肝炎(重肝)的临床诊断及预后判断价值.方法:对经临床和病理双重确诊的234例慢性乙型病毒性肝炎(慢乙肝)及54例乙型重型肝炎患者sTC进行定量观察,并与肝组织病变程度进行对照分析.结果:sTC水平与慢性乙肝及重型肝炎患者肝组织病变程度Ishak计分呈明显负相关(P＜0.01,r=-0.56).sTC水平均值＜2.07mmol/L者重型肝炎组为77.8%(42/54),重度慢性乙肝组为12.5%(6/48),两组差异有显著性意义(x2=43.46,P＜0.01).重型肝炎组存活率与sTC水平呈明显正相关r=0.65,P＜0.01),经治疗1周,存活组sTC水平稳步回升,死亡组持续下降.结论:sTC水平变化反映了肝脏的合成功能,与肝组织的病理损害程度密切相关.     

慢性乙型肝炎肝组织中HLA-DR和HSP70的表达意义

目的探讨HLA-DR抗原和热休克蛋白70(HSP70)在慢性乙型肝炎肝组织中的表达及其意义.方法采用免疫组化技术对36例慢性乙型肝炎和20例正常肝组织中HLA-DR抗原和HSP70的表达进行检测.结果正常肝组织中肝细胞未见HLA-DR表达.慢性乙型肝炎肝细胞HLA-DR表达阳性率为28%,其中,中重度肝炎HLA-DR阳性率明显高于轻度肝炎(阳性率分别为38%和20%,χ2=13.6,P＜0.01).慢性乙型肝炎和正常肝组织中肝细胞HSP70表达阳性率分别为45%和15%,两者相比差异显著(χ2=6.3,P＜0.05).中度和重度肝炎HSP70阳性率明显高于轻度肝炎(阳性率分别为63%和30%,χ2=3.9,P＜0.05).结论肝细胞HLA-DR和HSP70的异常表达在慢性乙型肝炎免疫损伤和免疫保护中起重要作用.     

e 抗原阴性慢性乙型肝炎患者核苷类停药后的临床和病毒学复发评估

目的：探讨 e 抗原阴性慢性乙型肝炎患者核苷类停药后复发的临床特点和复发情况。方法对我院自2010年5月至2013年11月治疗的80例 e 抗原阴性慢性乙型肝炎患者入院资料进行分析,医护人员分析患者临床症状、使用的药物类型、疗程、患者的停药原因、停药复发后生化及病毒、血清学等指标变化。结果49例患者服用拉米夫定（LMV）,患者用药时间为3个月～3年;14例患者服用阿德福韦酯（ADV）患者用药时间为3个月～2年;11例患者短期服用拉米夫定后再用 ADV,患者用药时间为6个月～2年;6例患者服用恩替卡韦（ETV）,患者用药时间为6个月～1年;患者复发后,有35例患者慢性中度肝炎,23例患者慢性重度肝炎,12例患者慢性重型肝炎,6例患者代偿性肝硬化,4例患者失代偿性肝硬化。结论e 抗原阴性慢性乙型肝炎患者核苷类停药后多在2年以内复发,且停药复发后肝损害更加严重。     

拉米夫定治疗慢性重型乙型肝炎疗效观察

观察拉米夫定治疗慢性重型乙型肝炎的疗效和安全性。98例慢性重型乙型肝炎患者随机分为2组。对照组36例,给予常规的综合治疗;治疗组62例,在常规综合治疗的基础上,加拉米夫定100mg,每日1次口服。分别观察2组治疗前后肝功能和凝血酶原活动度(PTA)、血清HBV DNA水平变化及疗效情况。结果表明,治疗组的总有效率为79．0%,对照组为52．8%,(P＜0．01)。治疗后4周,与对照组比较,治疗组存活患者的总胆红素(TBil)降低(P＜0．001)、凝血酶原活动度(PTA)升高(P＜0．001)、血清HBV DNA水平降低得更明显(P＜0．001)。拉米夫定治疗期间未发现明显的毒副作用,治疗安全性良好。拉米夫定治疗慢性重型乙型肝炎有较好的疗效和安全性。     

阿德福韦酯联合拉米夫定治疗YMDD变异慢性乙型肝炎的临床研究

目的:对经拉米夫定治疗出现YMDD变异的慢性乙型肝炎患者采用阿德福韦酯联合拉米夫定治疗48周,观察其疗效及不良反应,探讨慢性乙型肝炎YMDD变异后的治疗方案.方法:将72例患者随机分为3组,A组单用阿德福韦酯治疗48周;B组采用阿德福韦酯与拉米夫定联合治疗12周,后单用阿德福韦酯治疗36周;C组采用阿德福韦酯与拉米夫定联合治疗48周.结果:3组治疗12周内ALT水平进一步反弹升高的患者比例分别为29.2%(7/24)、12.5%(3/24)、4.2%(1/24)(P＜0.05),A组1例患者出现重型肝炎;治疗12周3组患者YMDD变异株检测阳性率分别有17.4%、0、0.治疗48周与治疗前比较3组患者血清ALT水平均显著下降(P＜0.05);但3组之间比较差异均无显著性意义(P＞0.05),HBV DNA水平较基线值显著下降(P＜0.05),均未检测出YMDD变异株;治疗48周3组间e抗原阳性患者血清转换率比较,差异均无显著性意义(P＞0.05);C组患者HBV DNA转阴率与A组比较,差异有显著性意义(P＜0.05),3组患者的HBV DNA反弹率分别为8.3%(2/24)、4.2%(1/24)、0.A组2例耐药患者测序结果为rtN236T变异,B组rtA181V+rtN236变异和rtN236T变异各1例.结论:YMDD变异后采用阿德福韦酯与拉米夫定联合治疗更安全有效.     

糖皮质激素阻断慢性乙型肝炎肝衰竭发生的临床对照观察

目的应用糖皮质激素治疗慢性乙型肝炎重度试图阻断重型肝炎的发生。方法 120例慢性乙型肝炎重度患者随机分为常规治疗组(58例)和在常规治疗基础上加用糖皮质激素治疗组(62例)。结果在激素治疗组,只有22％(14/62)的患者发展至慢性重型肝炎,显著低于常规治疗组的48％(28/58,X2=7.60,P<0.01)。在常规治疗组53.6％(15/28)的慢性重型肝炎患者死亡,而在激素治疗组,仅28.6％(4/14)的患者死亡(X2=0.02,P>0.05)。除在激素治疗组出现一些能控制的特殊感染外,两组的出血、感染发生率差异无显著性。结论在基础治疗的支持下,及时应用皮质激素能阻断部分慢性乙型肝炎重度患者发展到重型肝炎。激素治疗的成败取决于强有力的支持疗法、科学的给药方法和严格的管理。     

Acute fulminant hepatitis B in a patient with diabetic nephropathy treated successfully with concomitant lamivudine and molecular adsorbents recirculating system

A 36-year-old man with type 2 diabetes and diabetic nephropathy treated with hemodialysis developed hepatitis B virus (HBV)-induced acute fulminant hepatic failure (FHF). Despite supportive treatment, the condition rapidly progressed as manifested by severe jaundice, coagulopathy and hepatic coma. He was placed on the waiting list for liver transplantation and was treated with lamivudine and extracoporeal liver support with the molecular adsorbent recirculating system (MARS). After three 8-h sessions of MARS treatment in 1 week, he had remarkable improvement in clinical symptoms and serum biochemistry. On the 14th hospital day, surface antigen seroconversion was noted with undetectable hepatitis B virus surface antigen (HBs Ag) and low titre of anti-HBs antibody, indicating a complete recovery from acute fulminant hepatitis B. MARS treatment has been reported to benefit patients with liver failure from different causes including acute exacerbation of chronic hepatitis B, poisoning, post transplantation and Wilson's disease. The present case suggests its potential benefit when combined with lamivudine in treating uremic patients with acute fulminant hepatitis B.     

The role of lamivudine and predictors of mortality in severe flare-up of chronic hepatitis B with jaundice

summary . Patients with chronic hepatitis B (CHB) may develop severe disease exacerbations (flare) with jaundice, and some may progress to fulminant hepatic failure. Whether early administration of lamivudine can prevent liver failure and mortality is uncertain. We investigated the role of lamivudine treatment in severe hepatitis B virus (HBV) exacerbations. Consecutive patients presented with severe flare-up of HBV (new onset of jaundice plus alanine aminotransferase greater than five times upper limit of normal) treated with lamivudine and historical controls who did not receive lamivudine were studied. All patients had no hepatic encephalopathy on admission. Univariate analysis and multivariate logistic regression were performed on various clinical and laboratory factors for the prediction of mortality. Twenty-eight patients treated with lamivudine and 18 controls were identified. Overall, nine patients died and two other received liver transplants for fulminant hepatic failure. Six of 28 (21.4%) lamivudine-treated patients vs five of 18 (27.8%) controls died or received a liver transplant ( P =0.62). On multivariate analysis, platelet ≤143 × 10E9/L (odds ratio 22.4, 95% CI 1.8–281.6) and bilirubin > 172 μmol/L (odds ratio 18.4, 95% CI 1.5–228.5) were independent predictors of liver-related mortality. The mortality of patients who had thrombocytopenia and high bilirubin, thrombocytopenia, high bilirubin, and no risk factor were 69.2%, 11.1%, 12.5% and 0% respectively. Hence lamivudine confers no survival benefit to conventional treatment in severe exacerbations of CHB. Patients with thrombocytopenia and high bilirubin should be considered for liver transplantation.     

拉米夫定耐药突变相关慢性重型乙型肝炎27例分析

目的 研究在拉米夫定治疗慢性乙型肝炎的过程中出现拉米夫定耐药突变相关的慢性重型乙型肝炎的临床特点.方法 回顾性分析27例在拉米夫定治疗过程中出现耐药突变的慢性重型乙型肝炎病例,分析其临床特征,用基因芯片法或基因测序法检测YMDD突变类型,对8例施行肝移植术后的离体肝组织分析其病理特点.采用χ2检验进行统计学分析.结果 27例拉米夫定耐药患者的YMDD突变类型分别为:YVDD突变5例,YVDD突变+L180M突变2例,YIDD突变13例,YIDD+L180M突变4例,YVDD突变+YIDD突变1例,YVDD突变+Y1DD突变+L180M突变2例,单独L180M突变未检出.根据治疗前是否存在肝硬化,分为肝硬化组和非肝硬化组.与肝硬化组相比,非肝硬化组慢性重型乙型肝炎的发生率低,预后好,年龄小,HBeAg阳性率高.8例离体肝组织病理分析显示两种主要病理类型,一种主要表现为活动性肝硬化,另一种为大块或亚大块坏死,肝脏显著萎缩.结论 肝硬化为拉米夫定耐药突变相关的慢性重型乙型肝炎的高危因素.拉米夫定耐药突变相关的慢性重型乙型肝炎可能存在两种发病机制.     

人工肝支持系统治疗重型肝炎的疗效评价

目的 探讨应用人工肝支持系统（ＡＬＳＳ）治疗重型肝炎肝衰竭合并肝性脑病,研究其机制及评价疗效。方法 设治疗组和对照组,治疗组５１例重型肝炎（重肝）患者,急性、亚急性重肝１７例,慢性重肝３０例,酒精性重肝２例,钩体病黄疸型重肝１例,肝豆状核病肝衰竭１例。在综合护肝治疗基础上同时给予ＡＬＳＳ治疗。对照组３９例重肝患者,急性、亚急性重肝８例、急性重肝３１例,仅给予基础上同时给予ＡＬＳＳ治疗。对照组３９例     

Visual and auditory evoked responses in acute severe hepatitis

Evoked responses have not been studied in patients with acute severe hepatitis (ASH) with or without hepatic encephalopathy. This prospective study was undertaken to find out diagnostic as well as prognostic value of visual evoked responses (VER), and brain stem auditory evoked responses (BAER) in patients with ASH with or without encephalopathy. Visual evoked responses and BAER were studied in 20 patients (14 males and six females) with ASH. The patients were diagnosed as having severe hepatitis if acute hepatitis was associated with raised serum bilirubin and serum transaminases, and if they had a prothrombin time index of < 50%. After a detailed neuropsychiatric examination of each patient, the study sample was divided into two groups of 10 patients: ASH without encephalopathy (ASH-WOE), and ASH with encephalopathy (fulminant hepatic failure, FHF). The median P100 latencies of FHF patients were significantly increased compared with controls and patients in the ASH-WOE group. Abnormal P100 latencies, exceeding 95th percentile values of the controls, were present in one patient in the ASH-WOE group and six patients in the FHF group. The median interpeak latencies I-III, III-V and I-V were significantly prolonged in the FHF group. Interpeak latencies III-V were also increased significantly in patients in the ASH-WOE group. While abnormal BAER were seen frequently in both groups, VER abnormalities were largely confined to patients in the FHF group. In the FHF group, six out of 10 patients survived and exhibited clinical improvement in the status of hepatic encephalopathy. Evoked responses were repeated after 2–3 weeks of recovery in these patients and VER abnormalities showed a tendency to normalize, thereby suggesting a prognostic implication. The incidence of abnormal VER in hepatic encephalopathy complicating ASH far exceeded that of abnormal BAER. Markedly prolonged P100 latencies in FHF patients indicate poor prognosis.     

拉米夫定联合抗结核药物治疗肺结核合并乙型肝炎病毒携带者的临床分析

目的探讨在抗结核治疗的基础上联合应用拉米夫定治疗肺结核合并乙型肝炎病毒（HBV）携带者肝损伤发生情况,及拉米夫定使用的疗程和疗效。方法将江西省胸科医院治疗前肝功能正常的初治肺结核合并慢性HBV携带者142例,通过随机数字表法分为拉米夫定治疗组73例（失访4例,69例获得随访）和对照组69例（失访6例,63例获得随访）。对照组抗结核治疗至满疗程后停药,拉米夫定治疗组在使用抗结核治疗的同时,应用拉米夫定治疗至18个月后停药,两组患者均随访24个月,定期检测肝功能,对两组患者不同时期肝损伤率、总肝损伤率、死亡率、HBV-DNA阴转率、结核病治愈率及失败率进行分析。使用SPSS13．0软件进行统计学处理,以7。检验或Fisher确切概率法对计数资料进行统计分析,P〈0．05为差异有统计学意义。结果拉米夫定治疗组患者4周内肝损伤率、总肝损伤率、死亡率分别为4．3％（3／69）、20．3％（14／69）、0．0%（0／69）,均低于对照组[19．0％（12／63）、49．2％（31／63）、7．9％（5／63）],差异均有统计学意义（X2=7．064,P=0．007;X2=15．176,P=0．0001;Fisher确切概率法,P=0．023）。抗结核治疗结束时和随访结束时,拉米夫定治疗组患者HBVDNA阴转率E24．6％（17／69）、21．7％（15／69）]均高于对照组[3．2％（2／63）、3．2％（2／63）],差异均有统计学意义（X2=12．311,P=0．0005;X2=10．115,P=0．001）。拉米夫定治疗组患者结核病治愈率（89．9％,62／69）高于对照组（73．0％,46／63）,差异有统计学意义（X2=6．277,P=0．012）;治疗失败率（4．3％,3／69）低于对照组（17．5％,11／63）,差异亦有统计学意义（X2=5．972,P=0．015）。结论应用拉米夫定可降低慢性HBV携带者眼用抗结核药物所导致的肝损伤,使用18个月后停用相对安全,可为肺结核合并慢性HBV携带者应用拉米夫定治疗提供一定的临床依据。     

Lamivudine monotherapy for spontaneous severe acute exacerbation of chronic hepatitis B

BACKGROUND: Severe acute exacerbations of chronic hepatitis B virus (HBV) infection can spontaneously occur and rapidly progress to fatal hepatic failure. The purpose of the present paper was to identify factors that could influence the rapid progression of liver disease to hepatic failure, and assess the effects of lamivudine on serious disease. METHODS: Twenty-five patients with spontaneous severe acute exacerbation (accompanied by jaundice and coagulopathy) were consecutively treated with lamivudine. Their clinical outcomes were compared with those of 25 lamivudine-untreated patients, as historical controls. RESULTS: Six lamivudine-treated patients (24%)and seven controls (28%) rapidly developed hepatic failure. Lamivudine monotherapy did not significantly prevent progression to hepatic failure. Multivariate analysis identified baseline serum bilirubin >/=6 mg/dL (odds ratio [OR]: 5.61; 95% confidence interval [CI]: 1.66-21.61; P = 0.018), pre-existing cirrhosis (OR: 4.52; 95%CI: 1.26-30.42; P = 0.034), and baseline prothrombin time <40% (OR: 3.75; 95%CI: 1.03-43.86; P = 0.045) as independent determinants of the event. Of the aforementioned patients with hepatic failure, three lamivudine-treated patients (50%) and two controls (29%) survived (P > 0.15). However, lamivudine induced a sustained normalization of liver function and inhibited the development of cirrhosis in survivors. CONCLUSIONS: Lamivudine monotherapy conferred no significant protection against rapid progression of the disease to hepatic failure, but it resulted in long-term benefits. Lamivudine combined with other drugs could be more beneficial for patients with the aforementioned risk factors.     

Safety and efficacy of lamivudine in patients with severe acute or fulminant hepatitis B, a multicenter experience

Acute hepatitis B progresses to liver failure with the need of liver transplantation in about 1% of cases. We treated patients with severe acute or fulminant hepatitis B with lamivudine in an attempt to prevent hepatitis B virus (HBV) reinfection after potential liver transplantation. Since September 2000, 17 patients with severe acute or fulminant HBV infection were treated with 100 or 150 mg lamivudine daily once we had evidence for a severe course as indicated by an INR >2.0. These were compared to a historic control from our unit and to external patients. Fourteen of the 17 patients (82.4%) survived with full recovery without liver transplantation. All these 14 individuals cleared HBsAg on lamivudine within less than 6 months. Twelve patients recovered quickly as indicated by a normalized prothrombin time within 1 week while two patients had a more prolonged course. None of the patients showed an adverse event. Three patients requiring transplantation despite lamivudine therapy had more advanced disease on admission, of whom one had additionally ingested paracetamol (acetaminophen) while the second was already HBV-DNA negative by polymerase chain reaction on admission. The lamivudine treated patients had significant higher frequency of survival without liver transplantation 82.4 vs 20% (4/20) in the historic control (P < 0.001). Similar data were derived from external centres using lamivudine (15/20, 75%). Lamivudine is safe in patients with severe acute or fulminant hepatitis B, leading to fast recovery with the potential to prevent liver failure and liver transplantation when administered early enough.     

Presentation, Diagnosis and Outcome of Predominantly Hepatic Wilson's Disease in Adult Saudi Patients: A Single Centre Experience

Background/Aim:

To evaluate the clinical manifestations, diagnostic features, disease course and response to treatment among Saudi adults with predominantly hepatic Wilson''s disease. A retrospective cohort study of 40 adult patients diagnosed with predominantly hepatic Wilson''s disease between 1994 and 2008 at King Abdulaziz Medical City, Riyadh was carried out.

Patients and Methods:

The diagnosis was based on varying combinations of clinical and laboratory evidence of liver disease, presence of Kayser Fleisher rings, low serum ceruloplasmin levels, elevated 24 hour urinary copper excretion and histopathological findings on liver biopsy.

Results:

The most frequent clinical presentation was decompensated chronic liver disease in 19 (47.5%), followed by chronic hepatitis in 15 (37.5%) and fulminant hepatic failure (FHF) in 5 (12.5%) patients. Eight (20%) patients with end-stage liver disease had liver transplantation, while 24 (60%) patients followed up on medical treatment for a variable period of 1-12 years showed clinical and laboratory improvement. One patient was lost early in follow up. Eight (20%) patients died during the study period, 5 with FHF, and 2 with advanced hepatic and neurological disease and one seven years after liver transplantation. Mortality rate was 100% in FHF without liver transplantation.

Conclusion:

A predominantly hepatic Wilson''s disease has varied clinical presentations with decompensated chronic liver disease being the most common among adult patients. Majority of the patients show stabilization of the disease on medical treatment. FHF in Wilson''s disease has a grave prognosis without liver transplantation, the later remains a definitive treatment option for decompensated cirrhotics and patients with FHF.