自身免疫性肝炎的肝移植

尽管自身免疫性肝炎 (ＡＩＨ )的治疗已有很大进展 ,但很多患者仍然会发展至终末期肝病 ,或出现其他相关的严重并发症。此时 ,肝移植术仍然是唯一的治疗手段。本文对ＡＩＨ患者的移植时机等问题进行讨论。一、移植时机及适应证在脑死亡立法的发达国家 ,肝移植患者移植的时机尚应取决于取得合适供体的时间。 2 0 0 2年在美国有 190 0 0多例患者在美国器官移植分配小组 (ＵＮＯＳ)登记移植 ,显然 ,这些患者要等待几个月甚至几年。 2 0 0 0年在等待移植的患者中有 1686名死亡。目前活体肝移植开展还不够普遍 ,因此移植时机的掌握尚有很大的制约…     

肝移植后新发自身免疫性肝炎

在因各种原因而行肝移植的患者中,部分会于术后出现典型的自身免疫性肝炎(AIH)表现.因非AIH原因行肝移植后发生的AIH称为肝移植后新发自身免疫性肝炎(de novo AIH).de novo AIH在成人和儿童中均可发生,其临床表现与典型的AIH相似.自身抗体阳性是目前唯一对预测de novo AIH发生较有价值的指标.该病发病机制目前尚不十分明确.同种异体抗原导致的免疫反应触发自身免疫反应可能是其发生的机制之一.de novo AIH的诊断依赖于排除其他疾病及临床综合判断,诊断难点在于与术后各种排异反应的鉴别.其治疗包括强化激素治疗、增加或更换免疫抑制剂.早期诊断和治疗的预后较好.     

自身免疫性肝炎的治疗

单用强的松或低剂量联合运用强的松与硫唑嘌呤是所有自身免疫性肝炎 (ＡＩＨ)的主要治疗方法 ,联合疗法更优。肝移植对终末期病人是有效的手段。由于新的免疫调节剂的出现 ,可能有更有效的治疗机会 ,点特异性疗法是未来发展方向。一、药物作用机制皮质激素具有亲脂性 ,能弥散达胞质中与糖皮质激素受体相结合 ,再至细胞核与糖皮质激素反应基因作用 ,制约ＩＬ 2、ＩＬ 4、ＩＬ 5、ＩＬ 6、ＩＬ 8、ＩＬ 12、γ干扰素和ＴＮＦα等细胞因子表达。另外 ,它可抑制核因子 κＢ (ＮＦ κＢ)活性、缩短细胞因子ｍＲＮＡ的半衰期和阻碍细胞因子对靶细…     

中药治疗自身免疫性肝炎1例报告

1　病历摘要阮某 ,女性 ,5 2岁。因反复乏力、纳差、上腹部饱胀伴尿黄 1年余 ,于 2 0 0 1年 3月 10日入院。既往无肝炎病史 ,无吸毒、酗酒、服损肝药物及输血制品史。查体全身皮肤及巩膜重度黄染 ,未见肝掌 ,可见血管痣 ,腹平软 ,肝脾肋弓下未触及 ,肝区叩痛阳性。实验室检查 :肝功能示TBil13 5 5 μmol/L ,DBil 83 8μmol/L ,ALT 2 76U/L ,AST 43 9U /L ,TP 76g/L ,Alb 41g/L ,Glb 3 5 g/L ,γ GT 13 4U/L ,ALP 173 7U /L ,γ 球蛋白 2 2 5g/L ,IgG 2 4 9g/L ,AFP 2 1ng/ml,PT正常 ,血常规示Hb 80 g/L ,RBC 2 2 0…     

自身免疫性肝炎1例

患者，男，24岁，因“纳差、尿黄3个月”于2004年10月12日入院。3个月前，无明显诱因出现纳差、尿黄，曾在外院检验肝功能：丙氨酸氨基转移酶104u／L，HBsAg阴性，抗-HCV阴性，未诊治。于2004年10月12日复查肝功能：丙氨酸氨基转移酶187u／L，以“病毒性肝炎”收入院，无输血及注射血制品应用史、无饮酒及药物应用史。     

儿童自身免疫性肝炎1例

<正>自身免疫性肝炎(autoimmune hepatitis,AIH)可出现高丙种球蛋白血症和自身抗体阳性,临床表现缺乏特异性。多见于女性,且40岁以后发病率高~([1]),最小发病年龄为6个月~([2-4])。目前,儿童AIH的治疗仍以泼尼松联合或不联合硫唑嘌呤为一线用药~([5-7])。如不治疗,其5 a和10 a生存率分别为50%和10%~([8])。1病例摘要患儿女,5岁5个月,藏族。因"皮肤黄、眼黄、尿黄10     

自身免疫性肝炎的诊断—附一例报告

为加强对自身免疫性肝炎的认识，报道一例除外其它肝损害病因，经血清生化学（肝功能、免疫球蛋白，等）、血身抗体和肝组织病理学证实的儿童自身免疫性肝炎，不明原因的肝脏损害，自身免疫性肝炎应作为鉴别诊断之一，及时检测血清生化学、自身抗体、肝组织病理并除外其它肝损害病因将有助于诊断。     

自身免疫性肝病肝移植术后的治疗

自身免疫性肝病(autoimmune liver disease,ALD)是一组免疫介导的肝脏损伤的自身免疫性疾病,包括以肝炎为主的自身免疫性肝炎(autoimmune hepatitis.AIH),以胆管病变为主的原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)和原发性硬化性胆管炎(primary sclerosing cholangitis,PSC).ALD最终可以进展为肝硬化,导致肝功能衰竭,肝移植术是终末期ALD唯一有效的治疗方法.     

Clinical analysis of liver transplantation in autoimmune liver diseases

Background: Autoimmune liver diseases(ALDs) consist of autoimmune hepatitis(AIH), primary biliary cirrhosis(PBC), primary sclerosing cholangitis(PSC), Ig G4-associated cholangitis and overlap syndromes.Patients with these diseases may gradually progress to end-stage liver diseases and need liver transplantation. The present study aimed to explore the prognosis of patients with ALDs after liver transplantation.Methods: The clinical data of 80 patients with ALD(24 cases of AIH, 35 of PBC, 15 of PSC and 6 of AIHPBC overlap syndromes) who underwent liver transplantation in Renji Hospital, Shanghai Jiao Tong University School of Medicine from June 2004 to September 2016 were collected retrospectively. The causes of death were analyzed and the postoperative cumulative survival rate was estimated by the Kaplan–Meier method. Recurrence and other complications were also analyzed.Results: Of the 80 patients, 18 were males and 62 were females. The average age was 50.5 years and the average Model for End-stage Liver Disease(MELD) score was 14.1. After a median follow-up of 19.8 months, 8 patients died. The 1-, 3-and 5-year cumulative survival rates were all 89.0%. Three cases of recurrent ALDs were diagnosed(3.8%) but they were not totally consistent with primary diseases. Biliary tract complication occurred in 10 patients(12.5%). The new onset of tumor was observed in 1 patient(1.3%). De novo HBV/CMV/EBV infection was found in 3, 8 and 3 patients, respectively.Conclusion: Liver transplantation is an effective and safe treatment for end-stage ALD.     

Recurrence of autoimmune hepatitis after liver transplantation without elevation of alanine aminotransferase

It is controversial whether steroid therapy should be continued to prevent the recurrence of autoimmune hepatitis(AIH)in patients who have undergone liver transplantation(LTx)due to AIH.We report a case of recurrent autoimmune hepatitis after LTx despite a persistently normal range of alanine aminotransferase(ALT).A 50-year-old woman was admitted to our hospital because of jaundice and severe liver dysfunction,where she was diagnosed with liver failure due to AIH.Steroid therapy was not effective enough and the patient received living-donor LTx in 1999.Following the operation,the level of ALT was maintained within a normal range and anti-nuclear antibody(ANA)became negative,however,the serum level of IgG gradually elevated and ANA became positive,while platelets decreased.A liver biopsy performed 6 years after LTx showed histological findings of AIH and she was diagnosed with recurrent AIH.A recurrence of AIH may occur after LTx even if the level of ALT remains within a normal range.We consider that a protocol liver biopsy should be performed in patients who undergo LTx due to AIH to decide the indication for steroid therapy.     

人工肝联合肝移植治疗重型肝炎的初步研究

目的 观察并评价混合人工肝支持系统联合肝移植在救治重型病毒性肝炎（重型肝炎）中的作用与疗效。方法 采用自行研制的体外混合人工肝支持系统对8例中晚期重型肝炎患者进行人工肝过渡支持治疗,3－14d后行原位肝移植术。结果 8例重型肝炎患者经混合人工肝支持,肝衰竭得到有效控制,均成功等到肝移植。肝移植后4例存活,存活率为50％,另4例因肺部感染、肝肾综合征等死亡。结论 人工肝支持系统与肝移植联合可作为中晚期重型肝炎肝衰竭患者治疗的有效手段。     

Autoimmune hepatitis-primary biliary cirrhosis concurrent with biliary stricture after liver transplantation

Although the development of de novo autoimmune liver disease after liver transplantation(LT)has been described in both children and adults,autoimmune hepatitis(AIH)-primary biliary cirrhosis(PBC)overlap syndrome has rarely been seen in liver transplant recipients.Here,we report a 50-year-old man who underwent LT for decompensated liver disease secondary to alcoholic steatohepatitis.His liver function tests became markedly abnormal 8 years after LT.Standard autoimmune serological tests were positive for anti-nuclear and antimitochondrial antibodies,and a marked biochemical response was observed to a regimen consisting of prednisone and ursodeoxycholic acid added to maintain immunosuppressant tacrolimus.Liver biopsy showed moderate bile duct lesions and periportal lymphocytes infiltrating along with light fibrosis,which confirmed the diagnosis of AIH-PBC overlap syndrome.We believe that this may be a case of post-LT de novo AIH-PBC overlap syndrome;a novel type of autoimmune overlap syndrome.     

Celecoxib-induced cholestatic liver failure requiring orthotopic liver transplantation

Selective cyclooxygenase-2 （COX-2） inhibitors are widely used due to their efficacy and good safety profile. However, recent case reports have described varying degrees of liver injudes associated with the use of COX-2 inhibitors. We report the case of a patient who developed acute cholestatic hepatitis progressing to hepatic failure requiring liver transplantation, following a 3-d course of celecoxib for treatment of generalized muscle aches and pains. The clinical presentation, the laboratory data, as well as the liver histopathology were supportive of the putative diagnosis of drug induced liver injury.     

Optimizing management in autoimmune hepatitis with liver failure at initial presentation

Autoimmune hepatitis (AIH) is a disease of unknown etiology,its hallmark being ongoing hepatic inflammation.By its very nature,it is a chronic condition,although increasingly,we are becoming aware of patients with acute presentations,some of whom may have liver failure.There are very limited published data on patients with AIH with liver failure at initial diagnosis,which consist mostly of small retrospective studies.As a consequence,the clinical features and optimal management of this cohort remain poorly ...     

Liver transplantation in autoimmune liver diseases

Liver transplantation is indicated for terminal phases of autoimmune hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis. Indications for transplantation in autoimmune liver diseases are similar to those used in other acute or chronic liver diseases. Therapeutic advances have reduced the need for transplantation for autoimmune hepatitis and primary biliary cirrhosis but not for primary sclerosing cholangitis. Overall, outcomes of transplantation for autoimmune liver diseases are excellent. However, recurrence of autoimmune liver diseases in the allograft has variable impacts on graft and patient survivals. Treatment of recurrent diseases requires changes in immunosuppression or addition of ursodeoxycholic acid. Among autoimmune liver diseases, only autoimmune hepatitis occurs de novo in recipients transplanted for other diseases. Patients transplanted for autoimmune hepatitis or primary sclerosing cholangitis are at risk for reactivation or de novo onset of ulcerative colitis. Better understanding of the pathogenesis of recurrent autoimmune liver diseases is needed to devise effective means of prevention and treatment.     

受体门静脉血栓形成与肝移植

在肝硬化、门静脉高压的患者中门静脉血栓（PVT）形成并不少见,据文献报道,其发生率为0.6%～64.1%,其中包括门静脉部分血栓形成和门静脉完全血栓形成造成管腔完全阻塞.门静脉血栓形成以男性病例为多,多数患者除了肝硬化门静脉高压本身的临床表现外,并无其他特殊表现,常不被发现,也不需特殊处理.但若此类患者需行肝移植,作为肝移植受体其门静脉血栓形成就有特别的临床意义.受体门静脉血栓形成对肝移植的不利影响主要是指肝外门静脉和（或）肠系膜上静脉主干因血栓形成引起门静脉血流量减少或消失,在重建供肝门静脉时,供肝得不到足够的门静脉血流灌注,没有充足的门静脉血供,移植肝就不能存活.以往认为,受体门静脉血栓形成是肝移植的禁忌证,近年随着肝移植技术及相关处理的进步,门静脉血栓形成已不作为肝移植的禁忌证了,但是此类患者的肝移植手术处理更为复杂,而且术后并发症也常有增加.     

重症酒精性肝炎与肝移植

饮酒相关性肝病包括酒精性脂肪肝（alcoholic steatosis,AS）、酒精性肝炎（alcoholic hepatitis,AH）和酒精性肝硬化（alcoholic liver cirrhosis,ALC）等一组疾病[1]。重症酒精性肝炎（severe alcoholic hepatitis, SAH）是AH的严重类型,可发生于无肝病史的人群（急性酒精中毒）,也可发生在AS或ALC患者。主要表现为发热、肝脏肿大伴触痛、血清胆红素和外周血白细胞显著升高,可伴有慢性肝损伤和门脉高压的表现。 SAH 患者Maddrey 判别函数（Maddrey discrimi-nant function,MDF）［MDF=4.6x（PT-正常对照）＋TBIL （mg/dl）］通常≥32分,极易并发感染和多器官功能衰竭[2]。即使积极治疗,SAH 患者近期病死率仍可高达35%至50%[3]。