以亚急性肝衰竭为首发表现的自身免疫性肝炎1例报告

<正>1病例资料患者男性,76岁,退休,病程9个月,反复乏力、纳差、尿黄,先后入住本院3次。患者首次住院于2017年2月8日主诉"乏力、纳差、尿黄10余天",无畏寒、发热,无鼻衄及牙龈出血,无恶心、呕吐、腹痛、腹泻。既往史、个人史无特殊。查体:皮肤、巩膜黄染,未见其他阳性体征。肝功能:TBil 218. 9μmol/L,DBil 187. 6μmol/L,ALT 496 U/L,AST 723 U/L,GGT 268 U/L;     

亚急性重型与慢性重型肝炎预后评分探讨

亚急性重型和慢性重型肝炎病情严重,病死率高,如能及早准确地判断患者的预后,对于及时采取有针对性的治疗措施,十分重要。目前临床上根据患者的病情轻重将患者分为早期、中期和晚期,对于判断预后、指导治疗,具有重要的意义。但此分期方法比较笼统,缺少综合量化指标。为此,在分析与预后相关诸因素的基础上,设计了一个简便的评分方法,以替代现行的分期方法。 1 资料与方法 1.1 临床资料 本院1996-2002年住院的亚急性重型和慢性重型肝炎患者73例,其中男60例,女13例;年龄18～72     

151例亚急性重型肝炎患者临床特征与预后分析

目的分析151例亚急性重型肝炎的临床特征及其预后。方法应用SPSS 10.0及STATA软件对151例亚急性重型肝炎病人的临床特征进行回顾性分析。结果(1)亚急性重型肝炎男性发病高于女性,好发年龄>40岁;(2)亚急性重型肝炎病原仍以乙型肝炎病毒为主,其次为戊型肝炎病毒及不明原因,药物性以抗结核药物为主;(3)实验室指标TB、PT、PTR、WBC及其分类、BLA、CRE与预后成正相关,与PTA、TC、ALB、CHE、TG、PLT成负相关,与ALT、AST无相关性;(4)并发症发生率最高前3位为电解质紊乱、肝性脑病、自发性腹膜炎,病死率最高前三位为脑疝、肝肾综合征和脑水肿。结论亚急性重型肝炎好发于男性,好发年龄段为40岁以上;仍以乙型肝炎病毒为主要病原;电解质紊乱是最主要的并发症,肝性脑病发病不足50%;PT、TB、PLT、TC、ALB等可作为肝衰竭(亚急性重型肝炎)预后判断的独立指标。对于无肝性脑病的亚急性重型肝炎患者,严重的凝血功能异常是一个重要的灵敏和特异性指标。     

亚急性重型肝炎140例临床分型分期

为了对亚急性重型肝炎临床诊断标准中的分型、分期进行探讨 ,特将我院 1991年至 2 0 0 0年间收治的符合 2 0 0 0年 9月 (西安 )修订的《病毒性肝炎防治方案》[1] 中亚急性重型肝炎临床诊断标准 ,并按住院期间的临床表现进行分型、分期的 14 0例亚急性重型肝炎进行分析。根据其临床转归 ,探讨现行的分型、分期的合理性。临床资料一、一般资料14 0例亚急性重型肝炎均为我院患者。男 88例 ,女 5 2例 ;年龄 <10岁 2例 ,11～ 15岁 2 0例 ,2 6～ 4 0岁 71例 ,4 1～ 6 0岁 4 0例 ,≥ 6 1岁 7例 ;住院天数最短 2d ,最长 2 19d ,平均 39.5d ;血清胆红…     

以亚急性肝衰竭为首发表现的IgG4相关性自身免疫性肝炎1例报告

<正>自身免疫性肝炎(AIH)是一种自身免疫反应介导的慢性进行性肝脏炎症性疾病,其临床特征为不同程度的血清转氨酶升高,IgG球蛋白增高、自身抗体阳性,组织学特征为以淋巴细胞、浆细胞浸润为主的界面性肝炎。IgG4相关性自身免疫性肝炎(immunoglobulin G4 -associated autoimmune hepatitis, IgG4-AIH)除了符合经典AIH的表现,     

高压氧治疗亚急性重型肝炎的临床疗效分析

目的观察在基础治疗的同时加用高压氧治疗亚急性重型肝炎的临床效果。方法对102例亚急性重型肝炎患者在综合治疗的基础上，52例加用高压氧治疗20天，观察两组患者血生化指标和免疫球蛋白的变化。结果高压氧治疗组患者血清胆红素明显下降，凝血酶原活动度显著提高，血IgA、IgG、IgM和C1、C。水平明显降低，白蛋白明显升高；高压氧治疗组总有效率为84．6％，对照组为28．0％，两组差异显著。结论高压氧治疗对亚急性重型肝炎患者的体液免疫功能有抑制作用，从而减轻肝脏损伤，缩短退黄时间和病程，改善病情，降低病死率。     

10例亚急性重型肝炎救治体会

从1995年1月～1995年12月我们共收治10例亚急性重型肝炎患者,取得较好治疗效果,现报道如下。 1 一般资料 10例病人中男8例,女2例。年龄最小者23岁,最大者67岁,平均39岁。病原学分型:乙型肝炎病毒感染者7例(早期内毒素血症3例),乙、戊型肝炎病毒重叠感染者1例,戊型肝炎病毒感染者1例,病原未明者1例。血清胆红素定量171～375.8μmol/,凝血酶原活动度<40％者7例、41％～50％者3例。有其他并发症者4例,其中乙、戊型肝炎病毒感染的1例病人合并糖尿病及自发性腹膜炎,乙型肝炎病毒感染的病人中,1例合并自发性腹膜炎,2例合并肝性脑病。10例患者的诊断均符合1995年北京第5次全国传染病寄生虫病学术会议修订的病毒性肝炎诊断标准。     

副流感病毒引起亚急性重型肝炎一例

患者 ,女 ,35岁 ,因发热、乏力、纳差 5d ,尿黄 2d ,肝功能异常 ,于 2 0 0 0年 12月 5日以“急性黄疸型肝炎”入院。入院后经保肝对症治疗 ,4 5d后自觉症状消失 ,肝功能恢复正常。 2 0 0 1年 1月 18日突然出现持续高热、频繁呕吐、身目黄染再次出现并急剧加深 ,同时伴全身极度衰竭、精神错乱、昏睡、腹胀、尿少、鼻齿出血。体检 :体温 39.2°C ,脉搏 12 0次 /min ,血压 90 / 6 0mmHg。神志不清 ,处于浅昏迷状态 ,但对各种刺激有反应。巩膜皮肤深度黄染 ,手背及上肢可见大片状暗紫色皮下瘀斑。咽部充血。颈无抵抗。心肺正常。腹部膨隆 ,肝浊…     

肝豆状核变性误诊为亚急性重型肝炎一例报道

患者女性 ,6岁 ,因乏力、纳差、尿色加深 9ｄ在当地接“病毒性肝炎甲型急性黄疸型”给予保肝、抗生素等综合治疗 ,3ｄ后患者出现精神萎靡、少尿 ,又给予抗肝性脑病、利尿和白蛋白等治疗 1ｄ ,病情渐加重于 2 0 0 0年 3月 7日转来我院。入院时查体 :体温 3 5 .8°Ｃ ,脉搏 84次     

三环疗法佐治治愈亚急性重型肝炎1例

男。35岁。因发热、纳差恶心、全身乏力、尿色加深7天于2000-04-02收入院。查体：神清，精神差。全身皮肤巩膜呈深度黄染，肝掌及蜘蛛痣(-)。心肺(-)。肝剑下3cm。质韧，触叩痛。脾未及。实验实检查ALT2037u／L、AST1042u/L，A/G=34／35，SB492μmol/L，SB’300μmol/L，抗-HAV-IgM( )，乙肝三系(-)。抗-HCV(-)。入院后诊为病毒性     

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 ...     

Overlap syndrome of primary biliary cirrhosis and autoimmune hepatitis with unusual initial presentation as fulminant hepatic failure

Autoimmune hepatitis and primary biliary cirrhosis are generally easy to discriminate on the basis of clinical, laboratory, and histological findings. The presence of anti-mitocondrial antibodies seropositivity and cholestatic clinical, laboratory, and/or histological features in patients with autoimmune hepatitis indicates the overlap syndrome of autoimmune hepatitis and primary biliary cirrhosis. Fulminant hepatic failure is an unusual initial form of presentation of autoimmune hepatitis and primary biliary cirrhosis overlap syndrome. We report the case of a 50-year-old woman with autoimmune hepatitis and primary biliary cirrhosis overlap syndrome who presented with fulminant hepatic failure. Fulminant hepatic failure has a high mortality rate and may require liver transplant. Our patient revealed a good response to corticosteroid and ursodeoxycholic acid therapy. It is important to identify and distinguish autoimmune hepatitis and variant syndromes from other forms of liver disease because of response to corticosteroid therapy.     

Uveitis in autoimmune hepatitis： A case report

In this case report we describe for the first time an association between autoimmune hepatitis (AIH) and uveitis, without any doubts about other possible etiologies, such as HCV, since all the old reports describe the association of AIH with iridocyclitis before tests for HCV-related hepatitis could be available. A 38-year-old businessman with abnormal liver function tests and hyperemia of the bulbar conjunctiva was admitted to the hospital. Six years before admission, the patient presented with persistent fever, arthralgias, conjunctival hyperemia, leukocytosis and increased ESR, referred to acute rheumatic fever. The presence of systemic diseases, most commonly associated with uveitis, was investigated without results and the patient was then treated with topical corticosteroids. His symptoms resolved. A test for anti-nuclear antibodies was positive, at a titre of 1:320, with a speckled and nucleolar staining pattern. Liver ultrasound showed mild hepatomegaly with an increased echostructure of the liver. Percutaneous liver biopsy was performed under ultrasound assistance. Histological examination showed necroinflammation over the portal, periportal and lobular areas, fibrotic portal tracts, with periportal fibrosis and occasional portal-to-portal bridgings, but intact hepatic architecture. Some hepatocytes showed barely discernible granules of hemosiderin in the lobular area. Bile ductules had not any significant morphological alterations. METAVIR score was A2-F3, according to the modified HAI grading/fibrosis staging. The patient was diagnosed to have AIH with mild activity and fibrosis and was discharged on 25 mg prednisone, entering clinical and biochemical remission, further confirming diagnosis. After discharge the patient continued to have treatment with corticosteroids as an outpatient at a dose of 5 mg. On January 2002 the patient was readmitted to the hospital. A test for anti-nuclear antibodies was positive, at a titre of 1:320, with a speckled and nucleolar staining pattern. Anti-smooth muscle antibody test was also positive (1:160), while anti-LKM antibodies were negative. Ophthalmologic examination revealed inflammatory cells and proteinaceous flare in the anterior chamber of the left eye, and a stromal lesion in the cornea. He was maintained on immunosuppressive therapy (5 mg prednisone plus topical antibiotic therapy for two weeks) and then discharged. A complete remission of the symptoms was registered on follow-up. At present (July 2005), the patient is on prednisone (5 mg) and has no symptoms. Liver function tests are also within the normal range.     

亚急性重型肝炎的肝脏血流动力学改变

目的探讨亚急性重型肝炎时肝固有动脉(PHA)、门静脉(PV)的血流动力学改变.方法应用彩色多谱勒超声检测25例亚急性重型肝炎患者的PHA、PV的管径、时间平均血流速度(Vmean)、血流量及PHA的阻力指数(RI),并对21例进行了随访;设30例为正常对照组.结果亚急性重型肝炎时,PHA内径增宽,Vmean增加,血流量增多;PV内径增宽,Vmean降低,血流量减少;PHA血流量与TBIL呈正相关,PV血流量与TBIL呈负相关.治疗后PV血流量增加,PHA血流量减少.结论亚急性重型肝炎时,肝脏血流动力学检测有助于提示病情程度,并可为治疗提供参考依据.     

Juvenile autoimmune hepatitis: Spectrum of the disease

Juvenile autoimmune hepatitis(JAIH) is a progressive inflammatory liver disease, affecting mainly young girls, from infancy to late adolescence, characterized by active liver damage, as shown by high serum activity of aminotransferases, by elevated immunoglobulin G levels, high titers of serum non organ-specific andorgan-specific autoantibodies, and by interface hepatitis on liver biopsy. It is a multifactorial disease of unknown etiology in which environmental factors act as a trigger in genetically predisposed individuals. Two types of JAIH are identified according to the autoan-tibody panel detected at diagnosis: AIH-1, characterized by the presence of anti-smooth muscle antibody and/or antinuclear antibody and AIH-2, by anti-liver-kidney microsomal antibody type 1 and/or by the presence of anti-liver cytosol type 1 antibody. Epidemiological distribution, genetic markers, clinical presentation and pattern of serum cytokines differentiate the two types of AIH suggesting possible pathogenetic mechanisms. The most effective therapy for AIH is pharmacological suppression of the immune response. Treatment should be started as soon as the diagnosis is made to avoid severe liver damage and progression of fibrosis. The aim of this review is to outline the most significant and peculiar features of JAIH, based largely on our own personal database and on a review of current literature.     

血清免疫抑酸性蛋白对亚急性重型肝炎的诊断意义

目的　探讨血清免疫抑酸性蛋白 (IAP)对亚急性重型肝炎的诊断价值。方法　收集亚急性重型肝炎 (5 6例 )和急性肝炎 (95例 )患者血清 ,用单向免疫扩散法检测IAP水平。亚急性重型肝炎与急性肝炎血清IAP水平间比较采用t检验 ,血清IAP对亚急性重型肝炎与急性肝炎的鉴别价值分析采用ROC曲线法。结果　亚急性重型肝炎患者血清IAP水平为 (2 2 2 .14± 15 7.47) μg/ml ,急性肝炎患者为 (3 43 .60± 15 8.2 1) μg/ml ,两者间差异有显著性 (t =1.976,P =0 .0 0 0 )。两组血清IAP水平的ROC曲线下面积为 0 .741,显著大于标准参考线下面积 (P =0 .0 0 0 )。以血清IAP≥2 2 5 μg/ml诊断急性肝炎而排除亚急性重型肝炎的灵敏度、特异度和准确度分别为0 .789、0 .679和 0 .748,阳性和阴性预测值分别为 0 .80 6和 0 .65 5 ,阳性和阴性似然比分别为 2 .45 8和 0 .3 11。结论　血清IAP对亚急性重型肝炎有一定的诊断价值。     

52例自身免疫性肝炎的临床特点及其治疗

目的 研究自身免疫性肝炎(AIH)患者的临床特点和治疗.方法 收集复旦大学附属中山医院2003至2010年之间收治入院并诊断AIH患者的临床资料,分析其特点.结果 8年间共收治AIH患者52例,平均年龄(55.5±12.9)岁,男、女之比为1:6.4.入院时病程中位数6个月,最长者已达20年.入院时患者的临床分期如下:急性肝功能衰竭1例,失代偿性肝硬化6例,代偿性肝硬化6例,复发1例,症状期24例,缓解期2例,无症状期6例,不能明确6例.有肝活检病理诊断者30例,占57.7％,其余患者系综合临床症状、生化检查、自身抗体检查及激素疗效诊断.其中23例合并原发性胆汁性肝硬化(PBC),占44％.除PBC之外,合并桥本氏甲状腺炎4例,各类肾炎3例,类风湿性关节炎2例,干燥综合征1例,红斑狼疮1例.常见症状依次排列有乏力(48％),纳差(40％),中上腹不适(38％),黄疸(33％),恶心(27％),体质量下降(21％),皮肤瘙痒(13％),关节酸痛(13％),口干(12％),其他(＜10％).23例接受糖皮质激素治疗,其中14例同时接受熊去氧胆酸治疗.12例仅接受熊去氧胆酸治疗.2例因单用糖皮质激素2周反应不佳而加用硫唑嘌呤.另有1例单纯AIH失代偿性肝硬化患者同时接受这三种药物治疗.12例除“保肝药物”之外,不接受任何针对性治疗.这些患者随访7年,存活47例,1例死亡(死因为“急性粒细胞性白血病”),4例失访.结论 本组AIH患者以中老年人居多,女性大大超过男性,临床表现多样,无特异性指标,但症状较为明显.对不愿接受病理活检者,综合临床表现、肝功能、自身免疫抗体,通过非典型AIH积分系统的计算和对激素治疗的反应也可获得诊断.AIH通常并发其他自身免疫性疾病.其中AIH-PBC重叠综合征较为多见.常用药物为糖皮质激素、硫唑嘌呤.当出现重叠综合征、肝损伤较重或出现毛细胆管受损征象时倾向于糖皮质激素和UDCA联合应用.部分患者不用激素肝功能也保持在正常值或2倍正常值上限以内.对这部分患者需要严密监测.     

急性重症自身免疫性肝炎的临床特征和预后分析

目的:分析急性重症自身免疫性肝炎（AIH）的临床特征及预后。方法:回顾性分析2008-2019年入住空军军医大学第一附属医院的急性重症AIH患者临床资料,根据有无肝病基础分为急性AIH（A-AIH）和慢加急性AIH（AC-AIH）,分析患者一般情况、肝生物化学、免疫学、肝组织学特征、激素治疗预后及相关因素。结果:共收集...