Evidence for microfilament involvement in budded Autographa californica nuclear polyhedrosis virus production

Autographa californica nuclear polyhedrosis virus (AcNPV) is a large, double-stranded DNA virus that infects lepidopteran insects. In the course of infection two different forms of the virus are produced; one that serves to spread the infection from insect to insect and another that spreads the infection within the insect from cell to cell. The latter form matures by budding from the plasma membrane of infected cells. We have found that cytochalasins B and D prevent the production of infectious budded AcNPV, not by inhibiting budding but by inhibiting the synthesis of complete virions. In the presence of cytochalasins B and D, particles lacking nucleocapsids bud from the plasma membrane of AcNPV infected cells, implicating microfilaments in the synthesis, transport and/or assembly of critical virus components.     

Biochemical features of intrahepatic cholestasis.

A pattern of results is reported which was found to be common among patients who had intrahepatic cholestasis (IHC) which was rarely found in patients with other hepatic conditions. The pattern was recognized from over 1000 cases suspected of hepatobiliary disease. 29 were diagnosed with IHC, and excluding 4, 25 revealed the following etiological pattern: chlorpromazine (12 patients); pregnancy and oral contraceptive use (8); and other (5). As opposed to patients with acute and chronic hepatic disease, IHC sufferers had relatively normal values for immunoglobulins and antibody titers. A disproportionate elevation of serum bilirubin vis-a-vis serum enzymatic activities separated potential IHC cases into intra- and extrahepatic cholestasis. The following factorial evaluations were useful in distinguishing hepatic disease states: 1) when the sum of the activities of serum alkaline phosphatase, 5'-nucleotidase, aspartate and alanine amiotransferases, and isocitrate dehydrogenase was divided by the serum bilirubin concentration, there was good resolution of the distinction between patients with IHC and those with primary biliary cirrhosis, early and late viral hepatitis, cholelithiasis, and pancreatic and bile duct cancers. 2) Resolution was also achieved when the numerator included alkaline phosphatase, 5'-nucleotidase, and aspartate aminotransferase, but not when alkaline phosphatase alone, or alkaline phosphatase combined with 5'-nucleotidase, was used. The essential lesion in IHC is an excretory defect.     

良性复发性肝内胆汁淤积

目的 对国内外良性复发性肝内胆汁淤积（BRIC）病例进行系统性的回顾、归纳及总结。方法 报道北京协和医院的1例BRIC,在国内外文献中检索符合条件的BRIC病例,并对该病的临床特点进行总结。结果 国内有6例符合检索条件,国外21例。BRIC表现为反复发作性的黄疸和皮肤瘙痒,在发作期呈肝内胆汁淤积性黄疸表现,发作间期临床表现,生化指标,影像学表现完全正常。本病预后良好,极少数病例转变为预后不佳的进展性家族性肝内淤积性黄疸（PFIC）。     

Primary amyloidosis presenting as intrahepatic cholestasis

Hepatic involvement in primary amyloidosis is not rare but is often clinically silent. A mild elevation of the serum alkaline phosphatase level and hepatomegaly are the most common findings. We report a case of primary amyloidosis in an adult male presenting with intrahepatic cholestasis where viral, drug, alcohol, and autoimmune etiologies were all excluded and the definite diagnosis was established by liver biopsy with Congo red staining. Subsequently the patient was found to have monoclonal light chain disorder.     

Recurrent benign intrahepatic cholestasis. Description of 3 cases]

Benign recurrent cholestasis. Report of three cases. 3 cases of benign intrahepatic recurrent cholestasis are reported. The disease is very rare and both clinical and pathological features are necessary for the right diagnosis. The value of liver biopsy in excluding other icteric diseases is stressed; clinical and serological findings are also important for the diagnosis and for the management of the patient.     

Autosomal-recessive inheritance of benign recurrent intrahepatic cholestasis

Benign recurrent intrahepatic cholestasis BRIO is a rare disorder characterized by recurrent episodes of cholestasis without permanent liver damage. Familial and sporadic cases have been described. Based on existing evidence, both autosomal-recessive and autosomal-dominant inheritance have been considered. We describe a large Dutch pedigree with 4 patients, strongly suggesting autosomal-recessive inheritance. © 1995 Wiley-Liss, Inc.     

孕鼠肝内胆汁淤积症致胎鼠心肌细胞凋亡

妊娠肝内胆汁淤积症(intrahepatic cholestasis of pregnan-cy,ICP)是妊娠期特有的并发症,临床表现为妊娠中、晚期出现皮肤瘙痒和/或黄疸;孕妇血液中胆汁酸水平明显增加;并可经胎盘转运至胎儿,致胎儿脐静脉血和羊水中胆汁酸浓度也异常增高。大量临床研究显示,ICP孕妇血液中胆汁酸水平愈高,胎儿窘迫发生率亦愈高[1]。药理学和毒理学基础研究表明,胆汁酸对动物组织和细胞具有明显的毒性作用,可以引起细胞凋亡和/或死亡。本研究应用雌、孕激素建立ICP孕鼠模型,探讨ICP时胎鼠心肌细胞凋亡及凋亡调控基因BCL2-和BAX的表达。1材料与方法1…     

Efficiency of enzyme-inducing agents in rats with intrahepatic cholestasis

Inductors of the monooxygenase system benzonal, halonal, and halodif prevented the development of intrahepatic cholestasis induced by a-naphthylisothiocyanate and stimulated detoxifying function of the liver in rats. These agents increased the content of microsomal protein and cytochrome P-450 and accelerated metabolism of types I and II substrates. This was accompanied by a decrease of serum concentrations of total and free bilirubin and activity of liver-specific enzymes. Phenobarbital did not prevent the development of hepatocyte cytolysis.     

Inheritance of intrahepatic cholestasis of pregnancy in one kindred

Hirvioja M-L, Kivinen S. Inheritance of intrahepatic cholestasis of pregnancy in one kindred
Clin Genet 1993: 43: 315–317. © Munksgaard, 1993
We report three sisters with intrahepatic cholestasis of pregnancy (ICP) and the pedigree of the family, including six: generations. ICP was observed in five successive generations; most of the patients also had cholelithiasis. The uniform expression, the complete penetrance of the trait and the direct parent-to-child transmission support the Mendelian dominant mode of inheritance. Determination of HLA A, B and C haplotype was made in five ICP patients, without any findings of HLA type common to everyone. X-linked inheritance cannot be excluded in this study.     

Acute intrahepatic cholestasis accompanied with Chlamydophila pneumoniae infection

We report a case of Chlamydophila (C.) pneumoniae infection presenting with fever and rapid intrahepatic cholestasis. A 63-year-old man had a week-long history of intermittent high fever and rapidly progressive jaundice with atypical erythema. The results of liver function tests were recorded. The results of all serological tests were negative; the IgM, IgG, and IgA titers for C. pneumoniae had increased, which indicates a C. pneumoniae infection. The patient's fever and liver dysfunction improved upon administration of minocycline. Light microscopic findings showed the presence of enlarged liver cells with clear cytoplasm, a few mitotic figures, multinucleated cells, and bile cholestasis. The electron microscopic appearance of liver biopsy showed that bile canaliculi exhibited intrahepatic forms of cholestasis. From the results of light and electron microscopy, we inferred atypical intrahepatic cholestasis, probably resulting from the C. pneumoniae infection.     

Immaturity of the biliary excretory system predisposes neonates to intrahepatic cholestasis.

Intrahepatic cholestasis associated with both gram-negative bacterial infections and total parenteral nutrition (TPN) is observed more frequently in neonates than in older children or adults. Factors involved in the pathogenesis of this syndrome are uncertain. The cholestatic effects of gram-negative bacterial infections appear to result from the inhibitory effects of endotoxin on bile flow. Since the adverse effects of both endotoxin and TPN on bile flow involve primarily the bile acid-independent portion, the immaturity of the neonatal hepatic excretory system which an inadequate bile acid-dependent fraction of bile would explain the increased susceptibility of the neonate to endotoxin- and, perhaps, to TPN-induced cholestasis.