PURPOSE: The possible mechanisms of increased arylamine N-methyl- transferase (AMT) activity in cholestatic rat livers and serum were studied. METHODS: Rats were divided into eight groups: rats receiving a sham operation, rats with a bile duct obstruction (BDO) alone (BDO group), rats with a BDO plus taurocholic acid (TCA) injection (BDO plus TCA group), rats with a BDO plus tauroursode oxycholic acid (TUDCA) injection (BDO plus TUDCA group), rats receiving a choledocho-caval shunt (CCS) operation (CCS groups), rats receiving a CCS operation plus TCA injection (CCS plus TCA group), and rats receiving a CCS operation plus TUDCA injection (CCS plus TUDCA group). The AMT activities in the serum and in the hepatic subcellular fractions isolated from the above experimental rats were determined. The values of Km and Vmax in this hepatic enzyme were measured. RESULTS: The activities of liver mitochondrial and microsomal AMTs as well as the Vmax values of AMT, were found to be increased significantly in both the CCS plus TCA group and the BDO plus TCA group compared with the CCS and BDO groups. On the other hand, the values of Km of hepatic subcellular AMT was the same in all experimental groups. The serum AMT activity increased significantly in both the CCS plus TCA group and the BDO plus TCA group compared with control the CCS and BDO group. However, these serum and hepatic enzyme activities were the same in both the CCS plus TUDCA group and the BDO plus TUDCA group. CONCLUSION: The above results suggest that TCA stimulates the biosynthesis of AMT in the liver. Also, the elevated AMT activity in the serum is thought to be caused by an increase in the membrane permeability of hepatocytes from liver cell necrosis caused by TCA.
Animals ; Cholestasis ; Cholestasis, Extrahepatic ; Hand ; Hepatocytes ; Liver ; Membranes ; Necrosis ; Permeability ; Rats* ; Subcellular Fractions ; Taurocholic Acid* ; Transferases

Jaundice is one of the poor prognostic factors in the patient with hepatocellular carcinoma (HCC). In HCC patients, the most common cause of jaundice is liver parenchymal dysfunction and jaundice due to biliary obstruction is relatively rare. However, it is clinically important because biliary obstruction can be treated effectively with biliary drainage procedure and by that quality of life and survival of the patient can be improved. It is important to identify the mechanism and location of the bile duct obstruction for an appropriate management of the biliary obstruction. Endoscopic retrograde biliary drainage (ERBD) has commonly been selected as the first-line treatment. However, percutaneous transhepatic biliary drainage or endoscopic ultrasound guided biliary drainage also can be used when the endoscopic approach is impossible or when ERBD fails. Between two types of stents - plastic or self-expandable metal, there is no definitive evidence about which one is superior. Stent type should be selected according to the characteristics of obstruction and expected survival of patient.
Carcinoma, Hepatocellular* ; Cholestasis ; Cholestasis, Extrahepatic ; Disease Management ; Drainage ; Humans ; Jaundice ; Jaundice, Obstructive* ; Liver ; Plastics ; Quality of Life ; Stents ; Ultrasonography

Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal recessive inherited disorder characterized by multiple recurrent episodes of severe cholestatic jaundice without obstruction of extrahepatic bile duct. We present the case of a 7-year-old boy with BRIC confirmed by mutation analysis in the ATP8B1 gene and typical clinical manifestation. Despite inheritance of BRIC, we detected a mutation on only one allele. To our knowledge, this is the first report of BRIC with a confirmed single heterozygote novel mutation in the ATP8B1 gene in Korea.
Alleles ; Bile Ducts, Extrahepatic ; Child ; Cholestasis, Intrahepatic ; Heterozygote ; Humans ; Jaundice, Obstructive ; Korea ; Wills

Choledochal cysts in neonates and young infants take two forms: the choledochal cyst associated with biliary atresia (CCBA) and the choledochal cyst in the absence of biliary atresia (CC). Infants in both groups usually have similar clinical symptoms at presentation, but management and prognosis are different. While early portoenterostomy is required for CCBA, cyst excision with hepaticojejunostomy or choledochojejunostomy is usually performed for CC. The former shows a relatively poor prognosis, and the latter shows a better prognosis. Two infants who presented with clinical features of neonatal cholestasis, and clinically suspected to have choledochal cysts, were found on imaging studies and surgery to have extrahepatic bile duct atresia in association with choledochal cysts.
Bile Ducts, Extrahepatic ; Biliary Atresia* ; Choledochal Cyst* ; Choledochostomy ; Cholestasis ; Humans ; Infant ; Infant, Newborn ; Prognosis

Nonsurgical endoscopic or percutaneous dilatation and insertion of an endoprosthesis is the treatment of choice in the majority of patiens with incurabie malignant biliary obsturction. But these palliative treatment of extrahepatic cholestasis with an endoscopic or percutaneous biliary endoprosthesis is limited by clogging. One of the factors thought to be of importance is the diameter of the stent. So in order to avoid being limited by the size of the instrumentation channel of the endoscope, expandable stents have been developed. Wallstent is braided in the form of a tubular mesh from surgical grade stainless alloy. This prosthesis is geometrically stable, pliable and self expanding. Its elastic properties are such that its diameter can be substantially reduced by moderate elongation. The stent is constrainded on a small diameter delivery catheter(total outside diameter: 9 French). During the implantation procedure the final position of the partially released endoprosthesis can be adjusted by gradual removal of the delivery catheter. If full expansion to 30 French occurs, the stent will be shortened by approximately 30% to their normal length range between 34-102mm. Now in this article we report a new method for endoscopic retrograde placement of biliary Wallstent in a patient with obsturctive jaundice due to periampullary choangiocacrcinoma.
Alloys ; Catheters ; Cholestasis, Extrahepatic ; Dilatation ; Endoscopes ; Humans ; Jaundice ; Palliative Care ; Prostheses and Implants ; Stents

Benign recurrent intrahepatic cholestasis (BRIC) is a rare desease, which usually manifests between the age of 10 and 20. Its main clinical feature is multiple recurrent episodes of cholestasis without extrahepatic bile duct obstruction. We report here a case of nonfamilial benign recurrent intrahepatic cholestasis. The patient has experienced recurrent jaundice with pruritus since childhood. Main bile duct obstrution was excluded by abdominal CT and endoscopic retrograde cholangiopancreatography. Other causes of cholestasis were not found. Hepatic histology revealed bile plug which were mainly concentrated in the centrilobular region, and increased number of mononuclear cells in the portal triad, but hepatic parenchyma showed no inflammation and necrosis. In the last anicteric period, she was healthy and the liver function test and biopsy specimen were normal.
Bile ; Bile Ducts ; Bile Ducts, Extrahepatic ; Biopsy ; Cholangiopancreatography, Endoscopic Retrograde ; Cholestasis ; Cholestasis, Intrahepatic* ; Humans ; Inflammation ; Jaundice ; Liver Function Tests ; Necrosis ; Pruritus ; Tomography, X-Ray Computed

Benign recurrent intrahepatic cholestasis (BRIC) is a rare desease, which usually manifests between the age of 10 and 20. Its main clinical feature is multiple recurrent episodes of cholestasis without extrahepatic bile duct obstruction. We report here a case of nonfamilial benign recurrent intrahepatic cholestasis. The patient has experienced recurrent jaundice with pruritus since childhood. Main bile duct obstrution was excluded by abdominal CT and endoscopic retrograde cholangiopancreatography. Other causes of cholestasis were not found. Hepatic histology revealed bile plug which were mainly concentrated in the centrilobular region, and increased number of mononuclear cells in the portal triad, but hepatic parenchyma showed no inflammation and necrosis. In the last anicteric period, she was healthy and the liver function test and biopsy specimen were normal.
Bile ; Bile Ducts ; Bile Ducts, Extrahepatic ; Biopsy ; Cholangiopancreatography, Endoscopic Retrograde ; Cholestasis ; Cholestasis, Intrahepatic* ; Humans ; Inflammation ; Jaundice ; Liver Function Tests ; Necrosis ; Pruritus ; Tomography, X-Ray Computed

BACKGROUND/AIMS: The covered self-expandable metal stent (CMS) was developed to prevent tumor ingrowth-induced stent occlusion during the treatment of malignant biliary obstruction. However, complications such as cholecystitis, pancreatitis, and stent migration can occur after the endoscopic insertion of CMSs. The aim of the present study was to assess the efficacy and safety of a double-layered CMS (DCMS) for the management of malignant bile duct obstruction. METHODS: DCMSs were endoscopically introduced into 59 patients with unresectable malignant extrahepatic biliary obstruction at four tertiary referral centers, and the patient medical records were retrospectively reviewed. RESULTS: Both the technical and functional success rates were 100%. Procedure-related complications including pancreatitis, cholangitis, stent migration, and liver abscess occurred in five patients (8.5%). The median follow-up period was 265 days (range, 31 to 752 days). Cumulative stent patency rates were 68.2% and 40.8% at 6 and 12 months, respectively. At the final follow-up, the rate of stent occlusion was 33.9% (20/59), and the median stent patency period was 276 days (range, 2 to 706 days). CONCLUSIONS: The clinical outcomes of DCMSs were comparable to the outcomes previously reported for CMSs with respect to stent patency period and complication rates.
Cholangitis ; Cholecystitis ; Cholestasis ; Cholestasis, Extrahepatic ; Common Bile Duct Neoplasms ; Feasibility Studies* ; Follow-Up Studies ; Humans ; Liver Abscess ; Medical Records ; Pancreatitis ; Retrospective Studies ; Stents* ; Tertiary Care Centers

The authors report here on a case of focal stricture in the common hepatic duct that was caused by ischemic bile duct injury after repeat TACE procedures for hepatocellular carcinoma, and the patient was successfully treated with a covered self-expandable nitinol stent.
Alloys ; Bile ; Bile Ducts ; Carcinoma, Hepatocellular ; Chemoembolization, Therapeutic ; Cholestasis, Extrahepatic ; Constriction, Pathologic ; Hepatic Duct, Common ; Humans ; Liver Neoplasms ; Stents

PURPOSE: The purpose of this study was to evaluate the morphologic changes of the injured hepatic acini following ligation of common bile duct and to investigate the pathophysiologic process of hepatic failure and biliary liver cirrhosis in the extrahepatic cholestasis. MATERIALS AND METHODS: The common bile ducts of 18 rabbits were ligated partially. The rabbits were killed and selective microangiography was carried out with infusion of barium suspension via portal vein 4 to 24 weeks after ligation. Selective microangiography was also carried out in two normal rabbits. The microangiographic findings were evaluated and correlated with histopathologic features. RESULTS: The sinusolds of the liver acinus showed distortion, varying degrees of luminal widening, and irregularities in architecture. Terminal branches of the portal vein (TPV) showed increased number of branches, luminal narrowing, tortuosity, distortion, and beaded appearance. Peribiliary plexi were found as thin curvilinear, barium-filled structures along the wall of the dilated bile duct. The microangiographic findings were well correlated with histopathologic findings. The grades of microangiographic and histopathologic findings were poorly correlated with the duration of the ligation of CBD. CONCLUSION: Changes in microvasculature of the liver acinus following partial ligation of common bile duct were demonstrated by microangiography. Although the microvascular changes were evoked secondary to the injury, they might have some active roles in the pathophysiologic process in the liver.
Barium ; Bile Ducts ; Cholestasis, Extrahepatic ; Common Bile Duct* ; Ligation* ; Liver Cirrhosis ; Liver Failure ; Liver* ; Microvessels ; Phenobarbital ; Portal Vein ; Rabbits