肝硬化患者322例流行病学分析

目的 分析肝硬化患者的病历资料,调查肝硬化的常见病因、并发症和病死率,为进一步防治肝硬化提供依据.方法 采用回顾性调查方法,对2006 年-2011 年5 年间住院确诊为肝硬化的322 例患者的病历资料进行分析.结果 322 例患者中,病毒性肝炎222 例（68.94%）,酒精性肝硬化28 例（8.70%）,非酒精性脂肪性肝炎17 例（5.28%）,胆汁性肝硬化19 例（5.90%）,药物性肝炎20 例（6.21%）,病因不明的有16例（4.97%）.肝硬化最常见的并发症是上消化道出血（152 例,47.20%）,最常见的死亡原因是上消化道出血（25 例,64.10%）.结论 病毒性肝炎和酒精性肝炎是肝硬化的主要病因,防治病毒性肝炎,减低饮酒量是预防肝硬化的重要手段,积极防治肝硬化并发症是提高患者生活质量,延长患者生命最重要的环节.     

生化酶谱检测对原发性胆汁性肝硬化患者病情评估价值的探讨

目的分析雷州地区原发性胆汁性肝硬化患者肝功能结果的临床特点。方法采用Olympuslx10生化分析仪检测390例原发性胆汁性肝硬化病人和412例无肝脏疾病的健康者血清丙氨酸氨基转移酶(ALT),r-谷氨酰转肽酶(r-GT)、碱性磷酸酶(ALP)、总胆红素(TBIL)、直按胆红素(DBIL)、总蛋白(TP)、总胆汁酸(TBA)球蛋白(GLB)、白蛋白(ALB)/球蛋白(A/G)及AST/ALT比值。结果原发性胆汁性肝硬化组ALT、AST、AST/ALT、ALP、TPDBIL、TBIL、TBA、TBA、r-GT和GLB显著高于对照值(P<0.05)、ALB、AIG比值显著低于对照组(P<0.05)。结论肝功能检测结果是原发性胆汁性肝硬化病情严重程度,判断病情发展及预后的重要参考指标。     

不明原因肝功能异常患者的病理结果及临床资料分析

目的 通过对33例经肝组织活体检查术(简称“肝活检”)诊断明确的不明原因肝功能异常患者的病理及临床资料分析,总结导致肝功能异常的病因以及不同病因的临床及病理特点,为今后肝脏损害患者的临床诊断提供思路,减少误诊与漏诊。方法 回顾性分析浙江中医药大学附属第一医院感染科2014年12月至2016年6月以来在B超定位下行1秒肝穿刺的不明原因肝功能异常患者的病理诊断。结果 1.33例不明原因肝功能异常患者通过一般的临床资料及实验室检查分析,均难以得出诊断,通过肝活检大多数可明确诊断,包括自身免疫性肝炎(autoimmune hepatitis,AIH)12例(36.4％)、原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)1例(3%)、药物性肝损伤(drug - induced liver injury,DILI)7例(21.2％)、非酒精性脂肪性肝病(NAFLD)4例(12.1％)、非特异性炎症6例(18.2％)、肝内胆汁淤积1例(3%)、轻度脂肪变性2例(6.1%)。结论 1.肝活检是不明原因肝功能异常患者明确诊断的重要方法;2.自身免疫性肝病和药物性肝损伤是不明原因肝功能异常最主要的原因;3.临床资料特点,自身免疫性肝病(包括自身免疫性肝炎、原发性胆汁性肝硬化)男女比例为1:12,年龄集中分布在(45-60)岁。     

ALP、γ-GT和CHE检测在慢性乙型肝炎、肝硬化、肝癌、胆道疾病中的临床意义

探讨碱性磷酸酶(ALP)、γ-谷氨酰转肽酶(γ-GT)、胆碱酯酶(CHE)3项检测指标对肝胆疾病诊断的临床意义。方法搜集2008年3月至2010年12月住院病例295份,其中慢性乙型肝炎78例,乙型肝炎肝硬化74例,原发性肝癌71例,胆道疾病(包括胆管结石、胆囊炎)72例,检测这些病例的ALP、γ-GT、CHE 3项指标,并随机抽查51例健康体检者作为对照,观察比较ALP、γ-GT、CHE在不同肝胆疾病中的水平。结果各组肝胆疾病患者血清中ALP和γ-GT水平均较正常对照组高(P<0.001,P<0.01);且肝癌组ALP和γ-GT水平较其他组升高更显著;肝硬化组和肝癌组与正常对照组比较CHE明显降低(P<0.001),慢性乙型肝炎组和胆道疾病组与正常对照组比较CHE无显著差异(P>0.05)。结论 ALP、γ-GT、CHE在慢性乙型肝炎、肝硬化、肝癌和胆道疾病中均有不同程度的差异。测定ALP、γ-GT有助于诊断慢性乙型肝炎、肝硬化、肝癌和胆道疾病。测定CHE有助于肝硬化和肝癌的诊断,并可评估病情的严重程度及预后。     

结缔组织病并发早期原发性胆汁性肝硬化临床分析

目的分析结缔组织病（CTD）并发早期原发性胆汁性肝硬化（PBC）的临床特点（类型、肝脏生化指标、免疫学指标）、治疗和转归。方法对11例CTD并发早期PBC的临床资料进行回顾性分析。结果病例CTD以干燥综合征（SS）居多,其次为系统性红斑狼疮（SLE）、类风湿关节炎（RA）、未分化结缔组织病（UCTD）、多发性肌炎（PM）,病程最长8年,最短4个月。多数无PBC症状,仅2例有黄疸、皮肤瘙痒、乏力表现,肝脏生化指标均以胆酶升高为主,AMA和（或）AMA-M2阳性;ANA荧光核型多样,以胞浆颗粒型为主,免疫球蛋白IgM升高较明显;经糖皮质激素GC、免疫抑制剂和熊去氧胆酸（ODCA）治疗,随访的10例患者中,8例病情有改善。结论 CTD并发PBC并不少见,涉及的疾病谱较广,早期症状不典型,胆酶的升高及AMA、AMA-M2阳性是PBC早期诊断的主要依据,ANA的荧光模型、IgM的增高亦有重要的临床意义,糖皮质激素、免疫抑制剂和熊去氧胆酸治疗有效。     

Histopathological profile of childhood thyroid carcinoma in Ibadan,Southwestern Nigeria

BackgroundThyroid carcinoma is a common endocrine malignancy. It is however rare in childhood and often occurs as a result of radiation exposure or inherited genetic mutations. Most childhood thyroid carcinomas are well differentiated. There are very few epidemiological studies of this disease in Nigeria and our study aimed to determine the frequency in a subset of our population. The study aimed to determine the prevalence and histopathological characteristics of childhood thyroid carcinoma in our environment.MethodsThis was a retrospective review of histopathologically diagnosed thyroid carcinoma in children less than 18 years of age in the Department of Pathology, University College Hospital, Ibadan over a 40-year period. Histopathological diagnosis, age, sex and other relevant clinical information were extracted from the hospital records and surgical pathology records of the department. All cases of patients under18 years old had their slides re-examined and reclassified by two pathologists according to the World Health Organization histopathological classification of thyroid tumours.ResultsThere were 25 cases of thyroid carcinomas seen in children within the study period. Papillary thyroid carcinoma was the most common, accounting for 80% of the cases. Follicular carcinoma accounted for 12%, and medullary and anaplastic carcinoma accounted for 4% each. The mean age at presentation was 13 years. There was a female preponderance with females accounting for 60% of cases. Tumours with distant metastasis made up 20% of the cases.ConclusionChildhood thyroid carcinomas are rare in our environment, with a minority of cases presenting with metastases.     

Etiological features of cirrhosis inpatients in Beijing, China

Background The etiological spectrum of cirrhosis has changed over the years,but our knowledge of it is limited.The present study aimed to investigate the etiological features of cirrhosis inpatients an...     

婴儿胆汁淤积症88例临床研究

目的探讨婴儿胆汁淤积症的临床特点和诊断方法。方法收集我科2009-2011年收治的88例胆汁淤积症患儿,对其病因、临床检查、病理及预后进行分析。结果婴儿胆汁淤积症男孩、足月儿多见;2~3月龄发病率高。本组患儿中胆道闭锁组19例;肝内淤胆组69例,其中仅10.1%明确病因。胆道闭锁组γ谷氨酰转肽酶(GGT)水平明显高于肝内淤胆组,其他生化指标差异无统计学意义。血筛瓜氨酸血症1例;尿筛40%患儿高度怀疑Citrin缺陷。肝内淤胆组中23例行LSC25A13基因12种突变位点的检测,结果均为阴性。胆道闭锁组B超确诊率为64.7%。49例肝组织病理提示两组胆汁性肝硬化发生率差异有统计学意义(P<0.01)。两组的病死率和好转痊愈率差异有统计学意义(P<0.01)。结论胆道造影术、内科动态观察是鉴别胆道闭锁的常用方法,前者更可靠。对于有肝内胆汁淤积病史或存在肝功能反复异常者,应积极找病因,注意代谢病检查,并长期随访。     

Anti-mitochondrial antibodies in patients with Graves' disease may not signify primary biliary cirrhosis.

Two patients with Graves'' disease were incidentally found to have anti-mitochondrial antibodies by immunofluorescence in the absence of symptoms, clinical signs or biochemical evidence of liver dysfunction. Anti-mitochondrial antibody titres became undetectable in both patients on follow-up. Screening of the patients'' sera by immunoblotting against the purified antigens of the M2 complex was negative. We conclude that in these cases, anti-mitochondrial antibodies detected by immunofluorescence were directed against antigens other than the primary biliary cirrhosis-associated M2 complex and therefore did not signify subclinical primary biliary cirrhosis.     

炎症性肠病合并原发性胆汁性肝硬化1例报道

 炎症性肠病（inflammatory bowel disease,IBD）是一种慢性非特异性肠道炎性疾病,部分患者具有肠外表现。原发性胆汁性肝硬化（primary biliary cirrhosis,PBC）是一种慢性自身免疫性疾病,发病机制尚不明确。IBD合并PBC的报道较少。本文报道1例IBD合并肝硬化的中年女性,行肝移植后病理学确诊为PBC。     

原发性胆汁性胆管炎临床特征及红细胞分布宽度与血小板计数比值对肝硬化的诊断价值分析

  目的  探究原发性胆汁性胆管炎(primary biliary cholangitis,PBC)的临床特征及红细胞分布宽度与血小板计数比值(RDW-to-platelet ratio, RPR)对PBC肝硬化期的诊断价值,提高临床医生对该病的诊疗水平。  方法  收集2014年1月—2019年12月蚌埠医学院第一附属医院感染科门诊及住院部初次确诊为PBC的患者61例,对入组患者的临床资料进行回顾性分析。依据肝硬化诊断标准分为肝硬化组和非肝硬化组,比较2组的血常规、生化指标、RPR值、免疫球蛋白及补体水平。通过受试者工作特征曲线下面积(AUC)判断RPR对PBC肝硬化期的诊断价值。  结果  本组61例PBC患者中女性47例(77.05%);临床主要表现为黄疸(44.26%)和腹胀(24.59%);干燥综合征是最常合并的肝外自身免疫疾病;56例(91.80%)出现肝功能异常,以谷氨酰转肽酶(GGT)升高为主;抗线粒体抗体(anti-mitochondrial antibody, AMA)和(或)AMA-M2阳性率为93.44%;肝硬化组和非肝硬化组红细胞分布宽度(RDW)、血小板计数(PLT)、RPR及补体C3、C4比较差异均有统计学意义(均P < 0.05)。RPR诊断肝硬化的AUC值为0.705(95% CI:0.567~0.843;P=0.008), 最佳界值0.471, 灵敏度和特异度分别为52.20%和81.60%。  结论  PBC好发于中年女性,黄疸是最常见的临床表现。碱性磷酸酶(ALP)和GGT的升高以及AMA阳性是该病的主要特点。干燥综合征是最常合并的肝外自身免疫疾病。RPR对PBC肝硬化诊断有较高的特异度,可结合其他无创血清学指标协助评估疾病进程。      

Chronic liver disease in Kuala Lumpur, Malaysia: a clinical study

This study was undertaken to analyse the clinical spectrum of chronic liver disease (cirrhosis, and others with portal hypertension) in Kuala Lumpur. Eighty patients were diagnosed over a 6-year period. Twenty-two had biopsy proven cirrhosis while 58 others had portal hypertension with clinical and biochemical evidence of chronic liver disease. The commonest aetiology was alcohol (36%), followed by the idiopathic variety and hepatitis B. The male to female ratio was 4.4:1. Indians had a high prevalence of alcohol-associated chronic liver disease. Overall, ascites was the commonest presentation. Eight patients presented with hepatocellular carcinoma. Spontaneous bacterial peritonitis was diagnosed in 13% of patients undergoing abdominal paracentesis. Gallstones were detected in 37% of patients who underwent ultrasonography. Diabetes mellitus and peptic ulcer disease were noted in 22% and 31% of patients respectively.     

胆道系统疾病的肝脏移植治疗

胆道系统疾病始终是选择肝脏移植治疗的诸多病种中的一个重要适应证,其在病例选择及适应证方面主要包括:先天性胆道闭锁、易恶变的胆道疾病、胆道恶性肿瘤、原发性胆汁性肝硬化和继发性胆汁性肝硬化等病变,且这些疾病各有其适应证.胆道系统疾病行肝脏移植手术的时机包括:当肝脏移植为疾病治疗的唯一手段或唯一有效手段时;肝脏移植为疾病外科治疗的方法之一时.虽然国内外目前尚无一个统一标准用于胆道疾病行肝脏移植术前的评估,但首先应是明确诊断;其次要确定胆道疾病患者目前是否急需行肝脏移植以及急需的程度;再者要患者家属了解肝脏移植的整个过程,知道其存在的危险、移植的价值以及术后终身服用免疫抑制剂等.本文通过笔者的经验和实例分析胆道系统疾病肝脏移植手术的相关问题.     

Liver disease in rural Tanzania--a diagnostic approach

In a prospective study on the aetiology of liver disease and its diagnostic approach in a District hospital in rural Tanzania, 48 consecutive patients with evidence of liver disorders were investigated by physical examination, biochemical tests, laparoscopy and histology. Liver cirrhosis (posthepatic, alcoholic) was found in 31%; non cirrhotic alcoholic liver disease in 15%; viral, bacterial and protozoal liver disorders in 33%, and neoplastic liver changes in 21% of all patients. Clinical impression alone coincided with the final diagnosis in 40% of all cases. This figure was increased to 46%, when haematological and biochemical results were included, and to 71%, when laparoscopy (without histology) was used in addition. Laparoscopy was particularly decisive in the diagnosis and further management of cirrhosis, liver abscess and neoplastic liver disorders. The additional information obtained from histology led to the final diagnosis. Histology was specially useful for the diagnosis of alcoholic liver disease, tropical splenomegaly syndrome and non specific reactive hepatitis. The usefulness of laparoscopy as a diagnostic tool in a district hospital is discussed.     

Hepatic granulomas: a 15-year experience in the Royal Adelaide Hospital

The clinical and pathological associations of hepatic granulomas in patients who presented to the Royal Adelaide Hospital between January 1, 1968 and February 29, 1984 were reviewed retrospectively. Cases of primary biliary cirrhosis were excluded. Of 59 patients with hepatic granulomas, clear associations with diseases were identified in 42 (71%) patients. These were sarcoidosis (seven cases), chronic liver disease (12 cases), biliary tract disease (three cases), tuberculosis (four cases), Q-fever (three cases), other infections (four cases), drug hypersensitivity (four cases) and neoplasms (five cases). Ten patients had multiple associations and five other patients presented without any clearly defined cause for granulomas. Three of these latter patients presented with an acute febrile illness, showed hepatomegaly and had abnormal results of liver function tests. These cases may represent the entity that is labeled "idiopathic granulomatous hepatitis". Two other patients abused alcohol. Granulomas were categorized morphologically as microgranulomas, macrogranulomas and lipogranulomas according to their size, organization and the presence of fat droplets. Microgranulomas were associated with diseases of short duration and less architectural disturbance of the liver parenchyma. The presence of granulomas did not confer any prognostic implication over and above that of the associated disease.     

Primary biliary cirrhosis--experience in University Hospital, Kuala Lumpur

Primary biliary cirrhosis is an uncommon disease amongst Malaysians. Over a 12-year period, between 1979 and 1991, only seven patients with clinical, biochemical and histologic evidence of primary biliary cirrhosis were identified in University Hospital Kuala Lumpur. All were Chinese females between the ages of 30 to 55 years. The presenting complaint was pruritus in 5 patients. All except one patient was jaundiced when the diagnosis was made. These patients were followed up from 1 to 11 years. Three deaths were reported, one from massive hemetemesis and two from liver failure.     

Increased incidence of menstrual abnormalities and hysterectomy preceding primary biliary cirrhosis

A study was performed to assess the incidence of previous hysterectomy and dilatation and curettage among women with primary biliary cirrhosis. In 87 patients with primary biliary cirrhosis hysterectomy or dilatation and curettage had been performed significantly more often than among 100 age matched normal controls and 80 age matched patients with chronic active hepatitis or alcoholic liver disease. Among the 47 patients with primary biliary cirrhosis who had undergone hysterectomy or dilatation and curettage operations had been performed at a mean of 10.7 years and 13.2 years, respectively, before the onset of disease. The main indication for hysterectomy among patients with primary biliary cirrhosis and controls was menorrhagia. These menstrual disorders may be a consequence of high concentrations of oestrogens in patients with primary biliary cirrhosis.     

Management of choledochal cyst with portal hypertension

Portal hypertension (PHT) is a rare complication associated with choledochal cysts. Management issues of PHT patients are inadequately addressed, as its incidence is low and underlying causes variable. We report three cases of choledochal cyst with PHT. All patients had type IVa choledochal cysts, and the causes of PHT were secondary biliary cirrhosis (SBC) (two cases) and alcoholic liver disease (one case). Clinical presentation included jaundice, gastrointestinal bleeding and ascites. One patient with SBC successfully underwent excision with Rouxen-Y hepaticojejunostomy, while the patient with cholangitis was managed with endoscopic retrograde cholangiopancreatography stenting. The last patient with alcoholic liver disease was managed conservatively for seven years and died of liver failure. Management of choledochal cysts depends on the severity of liver disease in cases of cirrhosis of unrelated cause, while those with SBC should be considered for surgical management. Endoscopic stenting may be considered as a temporary measure in high-risk cases.     

上海徐汇区老年人抗M2型线粒体抗体的筛查结果分析

对上海市徐汇区6 008例60岁以上体检的老年人进行抗M2型线粒体抗体（胶体金免疫层析法）筛查,并以酶联免疫吸附法复测,其阳性率为1.31％ （79/6 008）,其中男性0.82％（18/2 186）,女性1.60％ （61/3 822）.经进一步检查显示11例抗M2型线粒体抗体阳性者中,8例确诊为原发性胆汁性肝硬化,3例高度疑诊;11例患者肝功能生化指标和/或临床症状等均有不同程度的异常.     

婴幼儿巨细胞病毒感染138例临床及治疗观察

目的：探讨婴幼儿巨细胞病毒（ CMV）感染的临床特点及治疗。方法：对在我院确诊、住院治疗的138例CMV感染患儿的临床资料进行回顾性分析。结果：138例患儿（男80例,女58例）血清CMV-IgM阳性和（或）血、尿CMV-DNA-PCR阳性,入院年龄9 d～3岁。临床表现为肺炎99例,伴有肺功能改变18例;婴儿肝炎62例,其中肝硬化1例,肝豆状核变性1例,高胆红素血症30例（2例合并核黄疸,3例胆道闭锁,1例胆总管囊肿,3例肝糖原累积症）;溶血性贫血10例,特发性血小板减少症6例;脑发育异常15例,其中脑积水1例、脑结节硬化2例、运动落后3例、智力低下3例、癫痫2例;脑干听觉诱发电位异常16例（双侧听力受损10例、青光眼1例）;先天性心脏病8例,多发畸形3例,迁延性肠炎5例,营养不良7例,喉软骨发育不良3例,选择性IgA缺乏症1例,遗传代谢性疾病1例,同时合并多重感染11例,合并呼吸衰竭、休克2例。治疗后好转114例,放弃治疗19例,死亡5例。结论：婴幼儿CMV感染临床表现多样化,以肺炎最多见;其次是肝胆系统受累。中枢神经系统受损可导致脑发育异常、听神经受损、运动落后等,少数病例可伴发其他畸形甚至多发畸形。绝大部分CMV感染病例预后良好,少数病例预后差甚至死亡。