肝紫癜病的诊断与治疗

肝紫癜病是一种少见的良性肝血管病变.因其发病率低,缺乏特异临床表现,术前诊断困难.2014年4月天津市第三中心医院收治1例因间断肝区胀痛就医患者.术前CT和超声检查发现肝右叶占位性病变,肝细胞性或血管源性恶性肿瘤待查,肝不典型局灶性结节状增生待查.患者行肝切除联合胆囊切除术治疗.术后病理学检查证实为肝紫癜病伴肝细胞腺瘤样增生.术后随访至2014年10月20日,未见复发.     

Spontaneous hepatic rupture in a patient with peliosis hepatis: A report of one case

INTRODUCTIONLiver rupture is a serious event that is most commonly due to blunt abdominal trauma. We present a case of peliosis hepatis in a patient admitted for acute pyelonephritis who developed hemoperitoneum due to spontaneous hepatic rupture from this rare liver condition.PRESENTATION OF CASEWe report a 44 year-old woman who presented to our hospital with acute pyelonephrititis and hemoperitoneum due to spontaneous hepatic rupture from peliosis hepatis. Physicians should be aware of this rare condition in patients who present with non-traumatic hepatic rupture with hemoperitoneum.DISCUSSIONPH should be considered in all patients with known risk factors who present with typical morphological changes or a hepatic mass, especially when the cause of sudden intraperitoneal hemorrhage is obscure.CONCLUSIONPeliosis hepatis is most often asymptomatic and an incidental finding at autopsy. In symptomatic patients, surgery should be reserved for those patients whose hemorrhage is-life-threatening. Familiarity with the imaging characteristics can help in earlier diagnosis of peliosis hepatis.     

Hepatocellular carcinoma with extensive peliotic change

Peliosis hepatis is a rare lesion histologically characterized by multiple cavities representing dilated sinusoids filled with blood in the liver. Although it has been observed in the liver parenchyma in association with several diseases and medications, there are few reports of nodules of hepatocellular carcinoma (HCC) showing extensive peliotic change. We describe a case of HCC showing extensive peliotic change in the cancer nodule. A 73-year-old man with a liver tumor was referred to our hospital for further investigation. Abdominal ultrasonography revealed an 8-cm hyperechoic lesion with a halo and mosaic pattern in segment 8 (S8) of the liver. Dynamic magnetic resonance imaging of the liver showed early irregular enhancement of the peripheral part of the lesion, and the effect persisted into the late phase, spreading into the central part of the nodule. Hepatic arteriography showed the “cotton–wool” sign, usually observed in cavernous hemangiomas. Fine-needle aspiration biopsy revealed the diagnosis of HCC. Anterior sectionectomy of the liver was conducted. Histological examination of the resected specimen showed that the tumor was a well-differentiated HCC with extensive dilated sinusoid-like structures in the main portion of the nodule, suggestive of peliotic change.     

原发性肝癌与非肝癌病人行肝移植治疗的比较研究

目的：探讨我国目前肝癌与非肝癌病人行肝移植治疗的风险及长期生存效果。方法：回顾性总结21例晚期肝癌病人行肝移植手术治疗风险及长期生存情况，并与同期所行另外19例非肝癌病人的肝移植进行比较。结果：晚期肝癌病人的手术前凝血状态好于因其它非肝癌原因而接受肝移植的病人，与此相应的手术中出血量、需要输血量、术中输液总量均少于非肝癌病人，手术中因出血而导致的低血压时间短，手术后较恢复顺利，围手术期病死率低。虽然肿瘤复发所致的远期死亡率明显高于非肝癌病人，但是，总生存率与非肝癌病人无明显区别，部分病人可长期无瘤生存。结论：现阶段肝移植仍是失去根治性切除机会的肝癌病人的有效治疗手段，术后部分病人有无瘤长期生存的可能性。     

第一肝门区小肝癌的经皮肝穿刺射频消融治疗

目的探讨对于位于第一肝门区的小肝癌行B超引导经皮肝穿刺射频消融(PRFA)治疗的可行性、疗效和应注意的问题。方法2000年4月至2002年10月选择肿瘤位于第一肝门区、≤5cm、病理或临床证实为原发性或继发性肝癌的21例患者进行PRFA治疗。治疗前甲胎蛋白(AFP)阳性者治疗后定期复查AFP。治疗后1个月复查MRI或CT确定肿瘤是否完全坏死,以后每3个月定期复查。Kaplan-Meier法计算“无瘤”生存率和累积生存率。结果AFP转阴率约为78％,MRI或CT显示第一肝门区肿瘤完全凝固坏死率为90．5％(19／21)。0．5、1、1．5、2年无原位复发生存率均为94．7％;0．5、1、1.5、2年无“瘤旁复发”生存率分别为90．0％、77．1％、77．1％和77．1％;0．5、1、1．5、2年总生存率分别为89．2％、82．8％、82．8％和55．2％。胆管狭窄发生率为4．8％。结论第一肝门区小肝癌并非PRFA治疗的禁忌证,只要治疗时穿刺点选择恰当、穿刺路径合理、超声监测下电极展开确切、热凝范围控制恰当,对于第一肝门区小肝癌PRFA是一种行之有效的治疗方法。     

Heart transplantation in patients with liver cirrhosis

OBJECTIVE: Liver cirrhosis is considered to be a contraindication to heart transplantation. However, the clinical outcome of heart transplantation in patients with liver cirrhosis has not been reported. Here, we sought to evaluate the clinical outcome of heart transplantation in cirrhotic patients. METHODS: Data were collected by retrospective chart review. Patients with liver cirrhosis at the time of transplantation were included. RESULTS: Between 1987 and 2007, 12 patients with liver cirrhosis underwent heart transplantation at the authors' hospital. Diagnosis of liver cirrhosis was based on preoperative abdominal sonography in five, autopsy in five, and laparotomy in two patients. Causes of heart failure were dilated cardiomyopathy in four, coronary artery disease in three, congenital heart disease in three and valvular heart disease in two patients. Causes of liver cirrhosis were alcoholism in two, cardiac in seven, and unknown in three patients. The Child classification was class A in three, class B in five and class C in four patients. Overall, the hospital mortality rate was 50% and major in-hospital complications occurred in nine patients (75%). Patients with non-cardiomyopathy diagnosis, previous sternotomy, and massive ascites had a high hospital mortality rate. The median follow-up duration was 33.5 months. There was no late death. Late post-transplant complications occurred in four patients and there was no event of liver dysfunction. All survivors were in Child class A at outpatient follow-up. CONCLUSIONS: Although there was high mortality and morbidity, patients with end-stage heart failure and liver cirrhosis can be considered for heart transplantation with careful case selection.     

体外静脉转流原位肝移植一例

目的 采用肝脏移植治疗直硬化的原发性肝癌。方法 在动物实验的基础上,供肝发取,４℃ＵＷ液灌注和保存。手术采用体外静脉转流肝脏移植技术。结果 手术历时１２小时,无肝期１００分钟术中血流动力学平稳,术后肾功能正常。结论肝移植治疗肝硬化肝癌本外静脉转汉是十分重要的。     

肝硬化病人肝移植术中脑氧代谢的变化

目的观察肝硬化病人原位肝移植术中脑氧代谢的变化。方法16例拟行原位肝移植术的终末期肝硬化病人，年龄25～67岁，体重45—80kg，Child分级B级3例、C级13例，心功能I或Ⅱ级，ASAⅢ或Ⅳ级。持续监测心输出量（CO）、平均动脉血压和体温；分别在术前（基础值）、切肝前10min、无肝期20min、新肝期30min和术毕采集桡动脉、肺动脉（混合静脉血）和左颈内静脉血，进行血气分析，记录血红蛋白、动脉、颈静脉的血氧饱和度（SaO2、SivO2）、血氧分压（PaO2、PjvO2），计算脑氧代谢和机体氧供指标：动脉血氧含量（CaO2）、颈静脉血氧含量（CjvO2）、动．颈静脉血氧含量差（Ca-jvO2）、氧供（DO2）、氧耗（VO2）、脑氧摄取率（CERO2）、脑血流／脑氧代谢率比值（CBF／CMRO2）。结果与基础值比较，切肝前、新肝期及术毕CO升高，无肝期CO降低；术中Hb、CaO2、Ca-jvO2、CERO2均降低，Sjv02、CBF／CMR02升高；无肝期DO2、VO2、CjvO2降低，其余时期DO2升高，术毕VO2升高；新肝期PaCO：升高，pHa降低；无肝期和新肝期pHv和pHjv均降低，术中及术毕BEa、B瞄和BEjv降低（P＜0．05）。结论肝硬化病人在肝移植围术期不存脑缺氧，脑氧摄取率降低。     

供受体血型不合肝移植可行性探讨

目的 探讨供受体血型不合肝移植治疗重症肝炎的可行性。方法 回顾性分析2004年7月上海交通大学医学院附属新华医院收治的1例O型血重症肝炎病人接受B型血供肝的临床病例资料。采用四联免疫抑制药物治疗，激素减量延迟。结果病人术后恢复顺利，未发生排斥反应，已健康存活17个月。结论 供受体ABO血型不合肝移植可以作为抢救重症肝炎病人的选择，尤其对O型血受体疗效较好。     

肝硬化失代偿期并发意外肝癌患者的肝移植及其临床特点

目的探讨肝硬化失代偿期并发意外肝癌患者的肝移植效果及其临床特点。方法11例肝硬化失代偿期患者接受原位肝移植,在术后的病肝病理检查中意外发现癌灶(称之为"意外肝癌"),其原发病,8例为乙型肝炎后肝硬化,丙型肝炎后肝硬化、酒精性肝硬化和原发性硬化性胆管炎各1例。11例术后采用他克莫司、霉酚酸酯及甲泼尼龙预防排斥反应,3个月后撤除霉酚酸酯,6个月后撤除甲泼尼龙。病肝沿水平面作间隔1 cm连续平行切片,对于直径大于1 cm或有灰白色结节者,完整取材。结果11例中,术前仅4例(36.4%,4/11)的甲胎蛋白(AFP)水平超过正常值。除1例肿瘤为多发外,其余均为单发,肿瘤直径最大为2.0 cm,平均直径为1.18 cm;肿瘤TNM分期,Ⅰ期占72.7%,Ⅲ期占27.3%;存在微血管侵犯2例,包膜完整、无血管及淋巴侵犯2例;病理诊断为胆管细胞癌1例,肝细胞癌10例。术后随访12～50个月,1例因肿瘤复发于术后10个月死亡。结论意外肝癌的病理学特点为肿瘤分期早,多为单发,组织分化程度高,肝移植后的1年存活率与良性肝病患者肝移植类似,肿瘤复发率低。     

同种原位肝移植治疗晚期肝硬变一例

对１例乙肝炎所致的肝硬变患者施行同种异体原位肝移植术，获得成功，患者对今已存活６个月，生存情况良好。术中经门静脉主干侧壁及肾静脉下方的下腔静脉侧壁分别插管，经Ｙ型管汇合后连接转流泵，再经右腋静脉的插管构成回路进行静脉转流。整个手术历时１１小时３５分，术中输血９０００ｍｌ。术后应用甲基强的松龙和环孢素Ａ进行免疫抑制。术后为改善患者的生理紊乱状况，进行了３次血浆转换，效果良好。     

肝细胞、胰岛细胞肝内移植对肝硬变影响的研究

目的避免脾切除、门腔分流术后所致向肝血流减少造成的肝功能损害。方法白蛋白将分离纯化的肝细胞、胰岛细胞经肝动脉灌注肝内移植。结果细胞组与对照组比较血总蛋白、白蛋白、胆红素改善程度,差异有极显著意义（Ｐ＜０．０１）。肝纤维化光镜检查及Ⅰ、Ⅲ型胶原免疫组织化学染色及图象分析结果,差异有显著意义（Ｐ＜０．０５）。结论肝细胞、胰岛细胞经肝动脉灌注肝内移植,不但能改善肝功能,还能促进肝脏胶原纤维降解,逆转肝纤维化、肝硬变的程度。     

背驮式同种原位肝移植一例

对１例患原发性胆汁性肝硬变合并门静脉高压征的终末期患者作了背驮式同种原位肝移植术，历时８小时３０分钟，手术获得成功，患者术后恢复良好。认为该术式由于保留受者的肝后下腔静脉，具有术中血流动力学稳定，肾功能不受影响，术后恢复快等优点，但是对手术前一般情况差或者估计无肝期时间长的患者，采用背驮式手术，加作门静脉－腋静脉（或颈静脉）转流是必要的。采用经胆囊管插管引流，可以减少胆总管的损伤和术后胆管狭窄的机会。     

肝移植治疗原发性胆汁性肝硬化的远期效果观察

目的 观察肝移植治疗原发性胆汁性肝硬化(PBC)的远期效果.方法 对15例接受了肝移植的终末期PBC患者进行长期随访,中位随访时间为70个月(38～86个月),记录并总结随访期间患者的相关资料,分析肝移植术后患者的预后、新发疾病以及术后远期存活率.结果 15例PBC患者共接受原位肝移植16例次,其中1例因原发性移植肝无功能进行了2次肝移植.术后患者均采用环孢素A(或他克莫司)+霉酚酸酯+激素的三联免疫抑制方案,部分患者使用了熊去氧胆酸.术后2周时,患者肝功能指标基本恢复正常,乏力、皮肤瘙痒及黄疸等症状缓解,患者的生存质量明显改善.术后有4例患者出现新发疾病(26.7%),1例为乙型肝炎病毒感染,2例为自身免疫性肝炎,1例为结肠癌,经治疗后2例好转,2例治疗无效死亡.肝移植术后,患者无PBC复发,1、2和5年的存活率分别为100%、100%和86.7%.结论 肝移植可提高终末期PBC患者的生存质量和存活率,但一些少见的新发疾病对患者的远期存活率影响较大.     

Clinical significance of recurrent primary biliary cirrhosis after liver transplantation

Abstract  Primary biliary cirrhosis (PBC) represents a classic indication for orthotopic liver transplantation (OLT); as an autoimmune disease, however, the existence and incidence of recurrent PBC is a matter of significant controversy. Between September 1988 and September 1994 a total of 544 OLTs was performed at our institution. Forty-nine patients (40 female) with a median age of 50.5 years and previous surgery in 36.4 %, received a liver graft for PBC. The mean serum bi-lirubin level was 8.9 mg/dl (range 0.7–29.7). Immunosuppression was commenced as a cyclosporine A-based quadruple therapy or with FK 506 and prednisolone. Protocol liver biopsies were taken at defined intervals posttransplant. Two patients died due to Legionella pneumonia and hypoxic brain damage 2 and 8 weeks after OLT, resulting in an actuarial 5-year survival rate of 95 % with 47/49 patients being alive compared to 83.5 % of all other liver recipients. Evidence of recurrence of PBC, as defined by elevated chole-static parameters and histological features of PBC, was found in four patients, another five patients showed only histological signs. Recurrence of PBC, which might compromise the long-term outcome after OLT, was suspected in 4/47 patients (8.5 %). This evidence of recurrent PBC is in conflict with findings of other groups that did not report recurrent PBC. OLT is still the optimal therapy for advanced PBC, with an excellent prognosis.     

肝移植治疗肝内胆管结石和肝包虫病

目的探讨肝移植治疗合并胆汁性肝硬化的广泛肝内胆管结石和不能手术治疗的肝包虫病的效果。方法对2例广泛肝内胆管结石伴胆汁性肝硬化和4例不能手术切除的肝包虫病(泡球蚴病)患者施行原位肝移植，术后定期随访，评价疗效。结果2例胆管结石患者已存活2年多：4例肝包虫病患者，除1例术后3个月死于心力衰竭外，其余3例已分别存活9个月、15个月及2年；存活患者均恢复了正常生活和工作。结论肝移植可作为治疗广泛肝内胆管结石和不能手术治疗的肝包虫病的有效手段。     

超声造影对患者肝移植术前肝脏局灶性病变的鉴别诊断价值

目的利用超声造影技术鉴别诊断患者肝移植术前肝硬化并发局灶性病变的价值。方法对104例患者肝移植术前的158个病灶进行超声造影检查。结果33个硬化结节以门脉期整体等增强为主要特点；2个不典型增生结节表现为门脉期中央低增强、周围等增强；106个原发性肝细胞癌（HCC）病灶表现为动脉期高增强、门脉期及延迟期低或等增强，HCC低分化组较高分化组病灶内造影剂到达时间及达峰时间比自身肝实质明显提前（P〈0．05）；6个卫星病灶表现为三期低增强；3个肝脓肿病灶表现为动脉期及门脉期周边厚环状高增强；8个血管瘤病灶表现为三期整体或结节状向心性高增强。结论超声造影技术有助于肝移植术前肝硬化并发局灶性病变的鉴别诊断，为肝移植术前制定治疗方案提供帮助。     

78例肝硬化患者肠道细菌易位及其相关性研究

目的了解肝硬化患者肠道细菌易位（BT）的发生率及其相关危险因素,分析BT与术后感染的关系。方法对77例肝移植和1例未行肝移植的肝硬化患者进行术中采样,取外周血、门脉血及肠系膜淋巴结（MLN）,分别进行需氧及厌氧培养,了解BT的发生率。结合术前、术后各种临床资料分析发生BT的危险因素及BT的临床意义。结果78例肝硬化患者中BT的发生率为10．3％（8／78）;细菌易位的部位以MLN为主,占5／8,发生BT的细菌主要是肠道G-兼性厌氧杆菌（55．6％）,其次为G^＋兼性厌氧球菌（22．2％）。BT组患者术前胆红素总量显著高于无BT组（P=0．022）;发生BT的患者其术后感染的风险是无BT患者的1．3倍。结论高胆红素血症是促发BT的独立危险因素,发生BT的肝移植患者术后感染的风险明显升高。     

Primary liver disease as a determinant for acute rejection after liver transplantation

Background: Graft rejection and infection remain major problems following liver transplantation; both are heavily influenced by the immunosuppressive regimen. Despite the disparity in the primary disease leading to transplantation, all patients receive the same post-transplant immunosuppressive treatment in a given center. The aim of this study is to detect a possible effect of the underlying disease on the incidence of early acute rejection episodes after orthotopic liver transplantation (OLT). Patients and Methods: Retrospective analysis on all 101 consecutive liver transplants performed in 95 patients between 1983 and March 1998; five of these patients, surviving less than 30 days, were not included. The immunosuppressive regimen was based on conventional triple therapy during the whole study period. The diagnosis and treatment of acute rejection within the first 30 days post-OLT was uniform throughout the whole study period. Results: Though there were no differences with respect to patients' characteristics [age, child classification, number of HLA-mismatches or cytomegalovirus (CMV)-serocompatibility], patients with primary biliary cirrhosis (PBC) showed a significant increase of acute rejection after OLT compared with the other patients transplanted for other liver diseases (P = 0.024). The incidence of infection was not elevated in patients transplanted for PBC when compared with other diagnoses. Conclusion: Our results indicate that primary liver disease may be a determinant for acute graft rejection in PBC. Furthermore, these results suggest that immunosuppressive regimens based on the underlying disease should be considered. Received: 19 October 1998 Accepted: 28 January 1999     

门静脉血栓对终末期肝硬化病人行肝移植手术的影响

目的探讨合并门静脉血栓(portal vein thrombosis,PVT)的终末期肝硬化病人行肝移植手术的处理方法及其疗效。方法回顾性分析2010年1月至2015年12月在中山大学附属第一医院器官移植中心接受肝移植手术的152例终末期肝硬化病人的临床资料。32例合并PVT的病人作为PVT组,其中Ⅰ级10例、Ⅱ级13例、Ⅲ级8例、Ⅳ级1例。其余120例无PVT的病人作为对照组。结果PV/T组术前脾切除史的比例明显高于对照组(46.8%比18.3%,P0.05),差异有统计学意义。PVT组较对照组明显延长无肝期时间[(72.5±25.3)min比(57.6±18.4)min,P0.05]和总手术时间[(622.4±183.5)min比(503.2±123.6)min],差异均有统计学意义。2组病人在术中出血量、ICU住院时间、术后并发症发生率、围手术期病死率、1年及3年生存率的比较上差异均无统计学意义(P0.05)。PVT组术后再栓塞率高于对照组(9.4%比1.7%,P0.05)。结论门静脉血栓一定程度上增加了终末期肝硬化病人肝移植手术的难度,Ⅰ~Ⅲ级PVT不影响病人的预后,仍可通过肝移植手术取得良好的疗效。Ⅳ级PVT肝移植手术的难度和风险会明显增加,应谨慎对待。