Xanthomatous neuropathy of liver

A 36-year-old man with juvenile-onset diabetes mellitus developed cholestatic hepatitis of unknown cause, possibly drug-induced. He remained jaundiced for four months, until he died unexpectedly. At autopsy, severe and macroscopically identifiable xanthomatous neuropathy was found in the liver; it involved the unmyelinated nerves in the hilus and in all portal tracts examined. Microscopic evidence of minimal extrahepatic involvement of unmyelinated nerves was also found. Somatic nerves were affected by diabetic neuropathy but not by xanthomatous neuropathy. The condition seems to be a rare complication of diseases that are characterized by accumulations of lipids.     

Long-term occlusion of the porcine cystic duct by means of endoluminal radio-frequency electrocoagulation

Recurrent cholelithiasis must be expected after gallstone removal without cholecystectomy. Chemical gallbladder ablation may offer prevention but requires preliminary cystic duct occlusion. Radio-frequency (RF) electrocoagulation of the cystic duct was performed in 15 pigs to induce occlusion by a controlled thermal epithelial injury. A flexible coagulation catheter was placed into the cystic duct lumen under fluoroscopic control by means of either subhepatic cholecystostomy or direct, percutaneous transhepatic gallbladder puncture. Complete cystic duct occlusion was proved in 14 animals. Follow-up ranged from 1 to 17 weeks (mean, 11 weeks). Histologically, the RF technique induced an intense chronic inflammatory and fibroblastic reaction, which eventually obliterated the coagulated cystic duct segments. There was no epithelial regeneration or recanalization of the fibrotic cystic duct segments. The adjacent structures, particularly the cystic artery, were intact in all specimens.     

Engineered measles virus as a novel oncolytic viral therapy system for hepatocellular carcinoma

The oncolytic measles virus Edmonston strain (MV-Edm), a nonpathogenic virus targeting cells expressing abundant CD46, selectively destroys neoplastic tissue. Clinical development of MV-Edm would benefit from noninvasive monitoring strategies to determine the speed and extent of the spread of the virus in treated patients and the location of virus-infected cells. We evaluated recombinant MV-Edm expressing carcinoembryonic antigen (CEA) or the human sodium iodide symporter (hNIS) for oncolytic potential in hepatocellular carcinoma (HCC) and efficiency in tracking viruses in vivo by noninvasive monitoring. CD46 expression in human HCC and primary hepatocytes was assessed by flow cytometry and immunohistochemistry. Infectivity, syncytium formation, and cytotoxicity of recombinant MV-Edm in HCC cell lines were evaluated by fluorescence microscopy, crystal violet staining, and the MTS assay. Transgene expression in HCC cell lines after infection with recombinant MV-Edm in vitro and in vivo was assessed by CEA concentration, 125I-uptake, and 123I-imaging studies. Toxicology studies were performed in Ifnar(KO)xCD46 transgenic mice. The CD46 receptor was highly expressed in HCC compared to nonmalignant hepatic tissue. Recombinant MV-Edm efficiently infected HCC cell lines, resulting in extensive syncytium formation followed by cell death. Transduction of HCC cell lines and subcutaneous HCC xenografts with recombinant MV-Edm resulted in high-level expression of transgenes in vitro and in vivo. MV-Edm was nontoxic in susceptible mice. Intratumoral and intravenous therapy with recombinant MV-Edm resulted in inhibition of tumor growth and prolongation of survival with complete tumor regression in up to one third of animals. In conclusion, engineered MV-Edm may be a potent and novel cancer gene therapy system for HCC. MV-Edm expressing CEA or hNIS elicited oncolytic effects in human HCC cell lines in vitro and in vivo, enabling the spread of the virus to be monitored in a noninvasive manner.     

Primary sclerosing cholangitis: a progressive disease?

Patients with PSC should be viewed as having a disease that frequently progresses to liver failure if followed long enough. The rate of disease progression is variable and can occur without changes in symptoms or signs. With the advent of ERCP and widespread use of liver tests on routine examinations, the diagnosis of PSC is being made earlier and survival estimates have lengthened. Varying methods of survival analysis have also been responsible for differing opinions regarding the natural history of this disease. Nevertheless, it is probable that with close prospective, long-term follow-up, evidence of disease progression and increased mortality becomes apparent. Challenges for the future include being able to predict accurately the rate of disease progression and timing of liver failure in the individual patient. Preliminary studies using statistical survival modeling have been developed; however, further refinement of these models will be needed prior to application to clinical trials and liver transplantation.     

Solitary bronchioloalveolar carcinoma: CT criteria

The computed tomographic (CT) scans of 30 patients with solitary bronchioloalveolar carcinoma were reviewed. Common features at CT included the peripheral or subpleural location of a pulmonary mass (25 cases), pseudocavitation (18 cases), heterogeneous attenuation (17 cases), irregular margins forming a star pattern (22 cases), and pleural tags (21 cases). Using these CT criteria, four independent observers attempted to identify cases of bronchioloalveolar carcinoma from a larger sample of lung cancers and benign lesions by categorizing a series of test cases into four probability categories. Although the bronchioloalveolar carcinomas were correctly ranked in the two highest probability categories 75% of the time (in 45 of 60 cases), there was considerable overlap with other lung lesions, particularly with adenocarcinoma and large cell undifferentiated carcinoma. However, even though the typical features of bronchioloalveolar carcinoma are not invariable or highly specific, they are characteristic enough to suggest the diagnosis.     

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胆道系统恶性肿瘤包括胆管癌和胆囊癌 ,前者主要指原发于肝内、肝门部和远端肝外胆管的恶性肿瘤。至今胆系肿瘤仍依ＴＮＭ分类法分为 0～Ⅳ期。 0期 :为原位癌 ;Ⅰ期 :肿瘤局限于粘膜层、肌层 ;Ⅱ期 :出现局部浸润 ;Ⅲ期 :在Ⅰ或Ⅱ期基础上肿瘤累及邻近组织或肝十二指肠韧带上淋巴结 ;Ⅳ期 :出现肝脏等器官受累或远处转移或出现以下之一区域的淋巴结转移 ,如胰周、十二指肠周、肝门周、腹腔及肠系膜。1　诊断胆系恶性肿瘤的诊断 ,主要依靠临床表现、实验室检查以及影象学检查。临床表现特异性不强 ,其中最基本的表现为胆道梗阻症状 ,同时可…