肝内胆管癌的诊断与治疗(附12例报告)

目的探讨肝内胆管癌发病的诊治方法。方法对我院1990年1月至2003年3月经手术和病理证实的12例肝内胆管癌病例资料进行回顾性分析。结果早期症状无特异性，AFP检查均为阴性，血CEA检查升高显著3例，4例术前诊断为肝内胆管癌，8例误诊，本组行根治性切除5例(41．6％)，姑息性手术7例。结论肝内胆管癌误诊率高，根治性手术率低，熟悉本病的临床特征，B超检查仍是首选方法。术中病理学检查是避免误诊漏诊的重要方法，根治性手术切除是本病最有效的治疗方法。     

Liver resection without hilus preparation and with selective intrahepatic hilus stapling for benign tumors and liver metastasis

Background Nowadays, liver resection is a routine operative procedure in surgical centers, and strategies must be aimed at avoiding additional risk factors. Extrahepatic isolation of portal vein, hepatic artery and hepatic duct, as well as lymphadenectomy of the liver hilum are generally accepted steps of liver resection, even for metastatic and benign indications. Our primary aim was to analyze the feasibility, blood loss, blood transfusion requirements, incidence of complications, and outcome using the approach for intrahepatic devascularization leaving the extrahepatic hilus untouched. Materials and methods Thirty-eight consecutive patients with resection for metastases and benign liver tumors were selected. After hilar examination, the extrahepatic structures remain intact, and during parenchyma dissection, the whole right or left or the appropriate bi-segmental pedicle is isolated intrahepatically and then transected using a stapler device. Results The used technique was feasible in all cases, and no intra- or postoperative surgical complications were observed. To date, no tumor recurrence was found in the hilum during the follow-up period. Conclusion The intrahepatic pedicle stapling technique appears to be feasible and safe in liver resection. Hilar dissection can, thus, be avoided in liver metastasis and benign liver tumors.     

Fatal familial cholestatic syndrome in Greenland Eskimo children

Summary We report the first detailed study of hepatic morphlogy in 28 biopsies from 16 Greenland Eskimo children with fatal familial cholestatic syndrome. The changes were categorized as early, intermediate and late. In the early stage, until 5 months of age, changes were restricted to zone 3, consisting of cholestasis and rosette formation without fibrosis. In the intermediate stage, from 5 to 14 months, cholestasis persisted and rosette formation increased, both with further extension into zone 2. Perisinusoidal fibrosis developed, first in zone 3 and later in zone 1. The late stage, from 17 to 60 months, showed a further increase in cholestasis and rosette formation, and fibrosis of zones 3 and 1 in nearly all biopsies. Portal to portal and portal to central fibrosis was evident with resulting cirrhosis in 2 of 7 patients. The morphological features can be summarized as pure cholestasis with prominent rosette formation followed by zone 3 fibrosis, zone 1 fibrosis, and, cirrhosis. Other characteristics are the virtual absence of inflammation and the lack of anatomical abnormalities such as paucity of bile ducts. The changes and their progression resemble those of Byler disease. Clinical and biochemical features are also largely similar, except for the presence of thrombocytosis in many of the Eskimo patients.     

Ultrastructural changes of bile duct epithelium in primary biliary cirrhosis in relation to progression of bile duct loss

Summary Using wedge liver biopsies from patients with primary biliary cirrhosis (PBC), ultrastructural features of the intrahepatic bile ducts in livers with slight or no bile duct loss were compared with those in livers with advanced bile duct loss and in extrahepatic cholestasis (EHC).Most changes in the biliary epithelium in PBC were similar to those in EHC. Microvillous loss and bleb formation, mitochondrial damage and increase in endoplasmic reticulum and ribosomes were found in PBC irrespective of the degree of bile duct loss, and also in EHC. These changes were present almost equally at any level of the biliary tree, and are presumed to represent a variety of non-specific lesions of biliary epithelial cells. As the loss of bile ducts in PBC progressed, cytoskeletal filaments and cytophagosomes increased in number and basement membranes were more thickened and reduplicated. These changes were more or less conspicuous in smaller branches of the biliary tree, and were also prominent in EHC. They might be causally related to the bile flow disturbance in the liver. Lateral intercellular spaces were irregularly dilated and contained osmiophilic membranous and/or granular material, similar to that found in duct lumena, within and without the basement membrane, and in the cytoplasm of periductal macrophages. Furthermore, pinocytotic vesicles were increased in the biliary cytoplasm facing periphery. These findings suggest possible alteration of the permeability of biliary epithelial cells, probably in the direction from the lumena to the periductal tissue. Such changes were found in PBC livers with virtual absence of bile duct loss, and the significance of this phenomenon is discussed.     

Histopathology of mixed anaerobic intraabdominal infection in mice

Summary This study characterized acute peritonitis and chronic abscess formation resulting from experimental mixed anaerobic infection withBacteroides melaninogenicus andFusobacterium necrophorum. At intervals after infection liver and spleen samples were obtained, fixed, and processed for histological examination. An acute to chronic infection progressed in mice infected with this mixture of anaerobic bacteria, whereas, no infection resulted when either organism was injected alone. Acute inflammatory cell infiltrates were noted in tissue samples at 12 h postinfection. Small, discrete areas of liver cell necrosis with neutrophilic infiltrates were observed as early as 24 h. By 48 h after infection the liver parenchyma was infiltrated with both acute and chronic inflammatory cells, with moderate to severe hepatocyte degeneration recognized at 72 h. Large intrahepatic abscesses were present in the subphrenic (upper lobe) area 2 to 6 weeks after experimental infection.     

Peripheral hepatolithiasis incidentally found at autopsy

Summary Hepatolithiasis is a common disease in East Asia though very rare in the West. Four cases of hepatolithiasis in which calculi were incidentally found in the peripheral branches of the intrahepatic biliary tree at autopsy are described and compared with hepatolithiasis involving the major branches of the intrahepatic biliary tree. These four cases were all elderly, three patients were male and one female. The calculi were brown pigment stones in each case, as seen in the major branch type. The stone-containing ducts showed mild fibrosis and glandular proliferation with inflammatory changes in three cases; these changes were marked in the fourth case. The hepatic parenchyma around the stone-containing ducts was atrophic or collapsed in all four cases. The major branches of the intrahepatic biliary tree as well as the extrahepatic tree failed to show findings suggestive of bacterial infections or biliary anomalies. These data suggest that brown pigment stones develop primarily in the peripheral ducts in the liver. It remains uncertain whether the peripheral type eventually progresses to the major type or not.     

Surgical resection for non-Asian intrahepatic lithiasis: The Brazilian experience

BackgroundIntrahepatic lithiasis (IHL) is a rare disease in the western world. Complications associated with IHL include acute cholangitis, liver atrophy, secondary biliary cirrhosis, and risk for intrahepatic cholangiocarcinoma. Liver resection is considered the treatment of choice for IHL. The objective of this study was to analyze patients who underwent liver resection for non-Asian hepatolithiasis.Methods127 patients with symptomatic non-Asian hepatolithiasis underwent resection in six institutions. Demographic data, clinical presentation, diagnosis, classification according to stone location, presence of atrophy, bile duct stricture, biliary cirrhosis, incidence of cholangiocarcinoma, treatment and postoperative course were evaluated.Results52 patients (40.9%) were male and the mean age was 46.1 years. Sixty-six patients (51.9%) presented with history of cholangitis. Stones were located in the left lobe in 63 (49.6%), and right lobe in 28 patients (22.0%). Atrophy was observed in 31 patients (24.4%) and biliary stenosis in 18 patients (14.1%). The most common procedure performed was left lateral sectionectomy in 63 (49.6%) patients, followed by left hepatectomy in 36 (28.3%), right hepatectomy in 19 (15.0%), and associated hepaticojejunostomy in 28 (22.0%). Forty-two patients (33.0%) presented postoperative complications and the most common were biliary fistula (13.3%) and surgical site infection (7.0%). Postoperative mortality was 0.7%. Intrahepatic cholangiocarcinoma was observed in 2 patients (1.5%). Recurrence was identified in 10 patients (7.8%), mostly with bilateral stones and/or hepaticojejunostomy.ConclusionLiver resection is the standard treatment for symptomatic unilateral or complicated IHL with good operative results. Risk of cholangiocarcinoma was low in non-Asian patients.     

孕妇外周血NKT细胞水平与妊娠期肝内胆汁淤积症发病及病情关系

目的:探究孕妇外周血自然杀伤T(NTK)细胞水平与妊娠期肝内胆汁淤积症(ICP)发病及病情严重程度相关性。方法:选择2016年3月1日-2018年3月1日本院就诊的ICP患者52例(ICP患者组),根据ICP严重程度分为轻度ICP组(n=28)和重度ICP组(n=24),同时随机选择孕周相当的健康产前检查孕妇52例(健康孕妇组),比较各组外周血单个核细胞(PBMC)中NKT细胞水平,分析NKT细胞水平与ICP发生发展相关性。结果:ICP患者组外周血PBMC中NKT细胞含量高于健康孕妇组(P<0.05)。对ICP患者组和健康孕妇组外周血PBMC给予不同刺激物培养后,均显示出NKT细胞含量在正常组与空白组无差异(P>0.05),而在植物血凝素(PHA)组、ICP组、正常组依次降低(P<0.05)。重度ICP组外周血PBMC中NKT细胞含量高于轻度ICP组(P<0.05),NKT细胞含量与ICP病情严重程度呈正相关关系(P<0.05)。结论:ICP发生及进展与孕妇外周血NKT细胞水平呈正相关关系,监测NKT细胞水平可为诊断ICP提供新思路。     

妊娠肝内胆汁淤积症孕妇血及脐血中一氧化氮水平与胎儿脐血流的关系

目的　了解妊娠肝内胆汁淤积症 (ICP)时 ,孕妇血及脐血中一氧化氮 (NO)水平与胎儿脐动脉血流的关系 ,以进一步探讨ICP时胎盘循环阻力增高的原因。方法　对 35例ICP孕妇 (ICP)组 )及 32例正常孕妇 (对照组 ) ,用镉还原显色法测定母血及脐血NO水平 ;超声多普勒测定胎儿脐血流S/D值。结果　ICP组母血、脐血NO水平均明显低于对照组 (P <0 .0 1,P <0 .0 1) ;两组母血、脐血NO水平均有相关关系 (r =0 .5 481,r =0 .82 0 6 ,P<0 .0 1,P <0 .0 1) ,ICP组脐动脉血流S/D值明显高于对照组 (P <0 .0 1) ,母血、脐血NO水平与S/D值呈负相关(r =- 0 .34 15 ,r=- 0 .42 6 1,P <0 .0 5 ,P <0 .0 1。结论　ICP时母血、脐血NO水平降低对胎儿—胎盘循坏阻力增加有重要的作用