442例不明原因ALT、胆红素升高的肝脏疾病患者的临床与病理学分析

目的通过病理学分析不明原因肝脏疾病的病因,为临床诊断及治疗指导方向。方法对2005年1月1日至2008年12月30日4年期间在本院住院的442例不明原因肝功异常的患者行肝脏穿刺术,应用CHISS2004统计学软件对其病例资料进行统计学分析。结果在本研究中,86%的患者通过病理学分析可明确临床诊断。ALT升高的肝脏疾病在各年龄组以及不同性别中的分布存在差异（P〈0.01,P〈0.05）,儿童组以肝脏其他感染性疾病、遗传代谢性疾病为主;少年组以药物性及中毒性肝损伤、自身免疫性肝病为主;青年组以药物性及中毒性肝损伤为主;中老年组以药物性及中毒性肝损伤、脂肪肝、酒精性肝病为主;男性以药物性及中毒性肝损伤、脂肪肝为主;女性以药物性及中毒性肝损伤为主。胆红素升高的肝脏疾病在不同年龄组以及不同性别中的分布存在差异（P〈0.01,P〈0.05）。儿童组以肝脏其他感染性疾病、病毒性肝炎以及药物性及中毒性肝损伤为主;少年组以遗传代谢性疾病为主;青年组以遗传代谢性疾病为主;中老年组以药物性及中毒性肝损伤、脂肪肝为主;男性以遗传代谢性疾病为主,女性以遗传代谢性疾病、药物性及中毒性肝损伤为主。（此两段作者没有翻译）结论病理学分析在不明原因的肝脏疾病的诊断中至关重要,不同原因所致肝脏疾病在不同年龄及性别中的分布存在差异,可进一步进行大样本量调查研究。     

肝胆疾病血清γ—谷氨酰转肽酶活性变化分析

血清中γ-谷氨酰转肽酶(γ-GT)主要来自肝脏,故在肝胆系统疾病中,血清γ-GT活力增高。为探讨其临床上的应用价值,我们对658例肝胆系统疾病和100例其他疾病及100例健康成人的血清进行γ-GT活性检测。现简要报告如下:     

自噬与脂肪性肝病

自噬（ autophagy）是真核细胞所特有的生命现象,是细胞利用溶酶体降解自身受损的细胞器如线粒体和大分子物质的过程,自噬参与细胞废物的清除、结构的重建、生长与分化,是细胞对不良刺激的一种生存防御机制。近年来,自噬与肝脏疾病的关系已成为医学研究的热点,自噬与肝脏缺血性疾病、肝脏肿瘤的发生、发展,以及与某些先天代谢性肝脏疾病都有着密不可分的关系［1］。同时,自噬在脂肪性肝病的发生发展中也起到了举足轻重的作用。     

第58届美国肝病学会年会纪要

第58届美国肝病学会年会于2007年11月1-6日在美国波士顿召开。本次年会报道了近一年来肝脏疾病基础研究与临床治疗新进展。2日下午和3日全天为继续教育的内容；主题是“肝脏疾病：肝脏疾病治疗的病理生理基础”。大会交流分为七个部分，分别是遗传性肝病、病毒性肝炎、脂肪性肝炎、肝脏血管性疾病、门静脉高压、肝移植以及肝胆肿瘤。本次大会对肝脏疾病取得的进展进行了广泛的交流，限于篇幅，仅对肝脏疾病临床治疗新进展进行简要介绍。     

腹腔镜肝胆手术中超声技术的应用

目的：探讨腹腔镜肝胆手术中超声技术的应用价值。方法：对68例患者(23例肝脏疾病、45例术前估计处理困难的胆囊病变)进行腹腔镜超声检查，并在术中超声指导下进行治疗性操作。结果：术中超声检查发现2例胆总管小结石和1例Mirrizi综合征，后者及1例多发性肝囊肿中转开腹。余66例均在术中超声引导下顺利完成腹腔镜手术：对肝囊肿实施囊肿开窗术，对肝脓肿实施脓肿切开置管引流术，对肝脏不明原因的占位病变实施肝肿块切除活检术、肝穿刺活检术，对孤立性肝脏占位性病变实施肝脏肿瘤切除术，对不能切除的肝癌实施毁损(微波和注射无水酒精)和门静脉插管化疗，对急性胆囊炎、慢性胆囊炎伴胆囊结石、胆囊粗蒂息肉行胆囊切除术。结论：腹腔镜术中超声具有快速、可重复、准确性高等特点，在肝胆疾病的腹腔镜诊断和治疗中具有重要的临床应用价值。     

急性肝衰竭最新认识

在美国生物化学、影像学和生物工程学研究所（NIBIB）及国立卫生研究院（NIH）罕见疾病部门的支持下,美国糖尿病、消化病和肾脏病研究所（NIDDK）于2007年12月4～5日在马里兰洲的贝斯达（Bethesda）举行了有关急性肝衰竭（ALF）认识和处置的研讨会。会议认为：ALF是一种少见而难治的临床综合征;其病因多样,在15％的成人患者和50％的儿童患者中不能明确具体病因。ALF病程不定,病死率高。     

193例肝胆疾病患者血清ADA和肝脏常规酶学指标联合检测结果分析

2004年1月～2005年12月,我们对193例肝胆疾病患者行腺苷脱氨酶（ADA）及肝脏常规酶学指标联合检测,现探讨其临床意义.     

MicroRNA与肝脏疾病关系的研究

微小RNA（miRNA）参与调节生物体的生长、发育等多个环节,与多种疾病的发生、发展有关。多种miRNA的表型改变和表达异常可正向或负向调控其靶基因的表达,参与各种肝脏疾病的发生、发展过程。本文对miRNA与肝脏疾病关系的研究进行概述。     

妊娠期高血压疾病并发脑血管疾病21例分析

目的探讨严重妊娠期高血压疾病并发脑血管疾病的病因、发病机制、临床特点、预后及预防。方法对新乡市中心医院院产科1997年1月-2006年12月收治的21例严重妊娠期高血压疾病并发脑血管疾病病人的临床资料进行回顾性分析。结果并发脑血管疾病的发生率为1.44%,且多产、高龄孕妇易发生;妊娠缺血性脑血管疾病9例（9/21）;上矢状窦静脉血栓形成7例（7/21）;妊娠出血性脑血管疾病5例（5/21）。21例病人中15例临床治愈,4例遗留轻偏瘫,死亡2例（为大面积脑梗死、脑出血）。结论并发脑血管疾病的病因为血管痉挛、血压增高和凝血功能障碍;应予以定期监测凝血功能及血栓前状态各指标等,给予预防性治疗。终止妊娠是治疗妊娠期高血压疾病的最根本措施。     

肝静脉超声显象诊断肝病的意义

目的探讨肝脏疾病肝静脉的超声显象特点及肝静脉的超声征象在肝脏疾病诊断中的意义．方法应用Ｂ型超声对１４８例（男９３例，女５５例，年龄２１岁～７６岁．肝癌、肝囊肿、肝血管瘤、肝硬变、脂肪肝、瘀血肝、布加综合征各２０例，多囊肝８例）不同肝脏疾病的肝静脉的声象图表现特征进行了观察研究．结果肝脏肿块（肝癌、肝囊肿和肝血管瘤）常导致肝静脉的受压移位；肝硬变、脂肪肝的肝静脉变细或显示不清；瘀血肝的肝静脉扩张；布加综合征肝静脉扩张、闭塞及相互交通并存；多囊肝的肝静脉结构常分辨不清．结论肝脏疾病肝静脉的超声征象具有显著的特征性，它对于临床肝脏疾病的病情分析、诊断与鉴别诊断均具有重要的临床价值和意义．     

Association of congenital cystic dilatation of the common bile duct and congenital diverticulum of the hepatic duct with concomitant ascariasis.

A case with association of congenital cystic dilatation of the common bile duct and congenital diverticulum of the hepatic duct is reported. The etiology and classification are briefly discussed and a radiological approach is recommended.     

成人先天性胆管扩张症68例临床研究

目的 探讨先天性胆管扩张症的临床特点,了解胆总管扩张的分型及其并发症的发生.方法 回顾性研究2000年1月至2009年12月在天津南开医院确诊的成人先天性胆管扩张病例68例,采用Alonso分类法分型,比较各型的临床表现、合并疾病、病情进展.结果 共收集先天性胆管扩张病例68例.其中Ⅰ型占47.1%,Ⅳa型占35.3%,Ⅳb型17.6%,未见Ⅱ、Ⅲ、Ⅴ型.67.6%患者合并胆囊炎,70.6%患者合并胆道结石,胆管狭窄占26.5%,胆囊萎缩占17.6%,胰腺炎占8.8%,胆汁性肝硬变占5.9%,脂肪肝占11.8%,胆囊息肉占17.6%,脾轻度肿大14.7%,肝囊肿8.8%,肾囊肿2.9%.癌变占23.5%.结论 先天性胆管扩张症Ⅰ型最多见,其次为Ⅳa型,未见Ⅱ、Ⅲ、Ⅴ型.常见并发症为慢性胆囊炎、胆道结石,约1/4发生癌变.

Abstract：

Objective To investigate the clinical characteristics of congenital cholangiectasis,the classification of choledochectasia and its complications. Methods Sixty-eight patients with congenital cholangiectasis admitted to Nankai hospital of Tianjin from Jan. 2000 to Dec. 2009 were retrospectively studied. The patients were classified using Todani method. The clinical presentation,complications and development were compared among patients with different types. Results In 68patients, type Ⅰ , Ⅳa and Ⅳb were accounted for 47. 1%, 35.3%, 17.6%,respectively. Wheraes type Ⅱ , Ⅲ and Ⅴ were not found. The complications included cholecystitis (67.6%) and bile duct stone (70. 6%). Biliary stricture and gallbladder atrophy were accounted for 26.5% and 17.6%,respectively. Pancreatitis was found in 8.8% patients, biliary cirrhosis in 5.9% patients,carcinogenesis in 23.5 % patients, fatty liver steatosis in 11.8 % patients, cholecystic polyps in 17.6 %patients, slight spleen enlargement in 14.7% patients, hepatic cyst in 8. 8% patients, renal cyst in 2.9% patients. Conclusions Tyep Ⅰ is commonly found in congenital cholangiectasis, and the next is the type of Ⅳa, but no type of Ⅱ , Ⅲ or Ⅴ is found. The common complications are cholecystitis and bile duct stone. Carcinogenesis is found in 25 % patients.     

Heritable disorders of the bile ducts

Diseases of the bile ducts encompass a wide range of disorders. These include those disorders primarily affecting extra and intrahepatic bile ducts and those that may be classified as panbiliary. The major heritable bile duct disorders are those affecting the intrahepatic ducts, namely syndromic bile duct paucity, or Alagille syndrome, and the fibrocystic cholangiopathies autosomal recessive polycystic kidney disease/congenital hepatic fibrosis, and autosomal dominant polycystic kidney disease. This discussion focuses on heritable disorders of the bile ducts.     

Anomalous arrangement of pancreaticobiliary ductal system associated with cholangiocellular carcinoma

A 66-year-old man with congenital cystic dilatation of the common bile duct (CDB; Alonso-Lej Type I) and anomalous arrangement of the pancreaticobiliary ductal system (AAPB) associated with intrahepatic bile duct cancer (cholangiocellular carcinoma; CCC) underwent an extended right hepatic lobectomy. In the resected specimen, the CCC was located in Couinaud’s segment V, with invasion to segment IV and the right hepatic duct and right portal vein. However, there was no cancer involvement of the dilated extrahepatic bile ducts, except for histologic findings of chronic inflammatory cellular infiltration and intestinal metaplasia. It is presumed that the probable mechanisms underlying carcinogenesis in CCC developing from the epithelium of intrahepatic bile ducts are the same mechanisms as those operating in carcinoma of the extrahepatic bile duct in patients with AAPB, although AAPB associated with CCC is uncommon. AAPB appeared to be related to the development of the CCC.     

Cystic diseases of the liver. From embryology to malformations

Cystic diseases of the liver which are in most cases hereditary, are related to an embryonic disorder know as ductal plate malformation. These diseases correspond to partial or total arrest of remodeling of the ductal plate, leading to more or less complete persistence of the excess of embryonic biliary structures. The ductal plate malformation may concern different segments of the intrahepatic biliary tree (segmental bile ducts, interlobular bile ducts and the smallest bile duct ramifications) leading to various pathoclinical entities. Caroli's disease is characterized by persistent dilated large intrahepatic bile ducts and appears to be the result of a factor acting during the early period of bile duct embryogenesis. Congenital hepatic fibrosis is characterized by ductal plate malformations of more distal, interlobular, bile ducts, and could be due to a factor that acts later on during bile duct development. This disorder may be isolated or associated with malformations of large, segmental, intrahepatic bile ducts. Von Meyenburg complexes and autosomal dominant polycystic liver disease are related to ductal plate malformation of more peripheral interlobular bile ducts and caused by a factor intervening in the later phase of bile duct embryogenesis. The genetic or non genetic factors leading to these ductal plate malformations are unknown.     

Epithelial cell proliferation activity of the biliary ductal system with congenital biliary malformations

Congenital biliary malformations such as anomalous arrangement of the pancreaticobiliary ductal system (AAPB), congenital cystic dilatation of the common bile duct (CCDB), and congenital biliary strictures at the hepatic hilum (CBSH) are newly designated disease entities and are frequently found in adult patients with biliary malignancy such as gallbladder carcinoma, common bile duct carcinoma, and intrahepatic bile duct carcinoma. In the present study, the relationship of these malformations and biliary malignancy was investigated. We studied 61 gallbladders of patients with AAPB and 56 gallbladders of patients without AAPB; 16 common bile ducts of patients with CCDB (12 with AAPB and 4 without AAPB) and 11 gallbladders of patients without CCDB; and 17 intrahepatic bile ducts of patients with CBSH and 6 intrahepatic bile ducts of patients without CBSH. Tissue sections from the mucosa of the gallbladder, common bile duct, and intrahepatic bile duct were stained for proliferating cell nuclear antigen (PCNA). The PCNA labeling indexes of patients with these malformations were significantly higher than those of patients without these malformations (P < 0.05). Cell proliferation of the epithelia in the biliary ductal system in patients with these congenital biliary malformations was accelerated. Consequently, these congenital malformations appear to be an important risk factor for the occurrence of biliary malignancy.     

Angeborene Anomalien und Malformationen der Gallenwege

Congenital anomalies and malformations may affect the intra- and/or extrahepatic bile duct system and present with different symptoms and impact depending on the specific type. Mere anomalies of the bile duct system are common and are generally asymptomatic but may cause substantial problems during hepatobiliary surgery. Severe malformations such as complete or partial biliary atresia are rare and present with prolonged and progressive icterus in the neonatal period requiring surgical reconstruction of the bile duct system or even liver transplantation. Fibrocystic diseases affecting the liver are caused by ductal plate malformations and are characterized by dilation of the intrahepatic bile duct system and focal liver fibrosis. Autosomal recessive and dominant polycystic kidney disease, progressive destructive cholangitis, congenital hepatic fibrosis, Caroli disease and biliary (von Meyenburg complex) as well as mesenchymal hamartomas belong to this heterogeneous group of disorders. Choledochal cysts are usually diagnosed in childhood; however, in about 20?% of patients they are detected in adulthood. According to Todani’s classification they are graduated into 5 categories. Choledochal cysts are common in Asia and show a high prevalence in Japan. An anomalous long common orifice of the extrahepatic bile and pancreatic duct is considered an important pathoanatomical substrate. Adults mainly suffer from symptoms due to cholelithiasis, pancreatitis, biliary cirrhosis and carry a high risk for developing cholangiocarcinoma. Therefore, early and complete excision of the choledochal cysts and long-term surveillance are warranted.     

Imaging in congenital fibrocystic diseases of the liver

The identification of dilatations of the intrahepatic bile ducts in the absence of bile duct obstruction is rare. Imaging techniques, especially MR cholangio-pancreaticography, generally permit the distinction between fibrocystic liver diseases and polycystic liver diseases. The presence of dilated sacciform or tubular bile ducts on cholangio-pancreaticography associated with a centrally located fibrovascular bundle (central dot sign) suggests Caroli's syndrome. The presence of associated signs of liver dysmorphia including right lobe atrophy and hypertrophy of segment IV suggests associated congenital hepatic fibrosis. The findings on cholangio-pancreaticography, computerized tomography or Doppler ultrasonography correlate well with the pathogenesis of fibrocystic liver diseases, linked to an embryologic malformation of the ductal plate.     

Clinical aspects of congenital cystic dilatation of the common bile duct in adults

We reviewed the data for 155 patients with congenital cystic dilatation of the common bile duct (CCDB; Alonso-Lej Type I) treated at our institute in the past 12 years. Our definition of CCDB was segmental enlargement of the common bile duct or enlargement involving the proximal bile duct, visualized on cholangiograms. CCDB was subclassified as infant or adult type cyst, based on the extent of the enlargement and the clinicopathologic findings. The former is typically an enlargement of the common bile duct or an enlargement involving the common hepatic duct, while the latter is an enlargement of the entirety of the extrahepatic ducts or involves the intrahepatic bile ducts. Of the 155 patients, 5 (including 1 child) had infant type cysts and 150 (including 1 child) had adult type cysts; 125 patients had benign CCDB (3 with infant type cyst) and 30 exhibited CCDB biliary malignancy (2 with infant type). Anomalous arrangement of the pancreaticobiliary ductal system (AAPB) and congenital biliary stricture were frequently associated with CCDB and these features played an important role in the presenting symptoms of CCDB: AAPB was detected in 100 of the 155 patients, and stricture in 51. AAPB caused disturbance of the passage of bile from the terminal bile duct to the duodenum and cholestasis in the enlargement, with reciprocal regurgitation of bile into the pancreas and pancreatic juice into the bile duct, and the AAPB appeared to precede obstructive jaundice, acute pancreatitis, or biliary malignancies. Stricture was observed at one to all of six sites in the hepatic hilum. Intrahepatic stones were seen in 30 patients, and intrahepatic bile duct cancer was seen in 8 patients; in all these 8 patients, the cancer was associated with primary intrahepatic stones.     

Mirizzi syndrome in an anomalous cystic duct： A case report

Mirizzi syndrome is a rare complication of gallstone disease, and results in partial obstruction of the common bile duct or a cholecystobiliary fistula. Moreover, congenital anatomical variants of the cystic duct are common, occurring in 18%-23% of cases, but Mirizzi syndrome underlying an anomalous cystic duct is an important clinical consideration. Here, we present an unusual case of type Ⅰ Mirizzi syndrome with an uncommon anomalous cystic duct, namely, a low lateral insertion of the cystic duct with a common sheath of cystic duct and common bile duct.