• Biomedical Science Letters
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• v.10 no.2
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• pp.93-98
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• 2004

The effects of intravenously administered of high concentration of taurocholic acid (TCA) on $\alpha$-D- and $\beta$-D-mannosidase activities in rat liver lysosomes were studied. These liver lysosomal enzymes, and serum lysosomal $\alpha$-D- and $\beta$-D-mannosidase isozymes activities were determined in experimental rats with common bile duct ligation (CBDL). The liver lysosomal $\beta$-D-mannosidase activity as well as the serum lysosomal $\alpha$-D- and $\beta$-D-mannosidase isozymes activities were found to be significantly increased in the CBDL plus TCA injected group than in the control group such as CBDL alone group. However, the liver lysosomal $\alpha$-D-mannosidase activity was found to be significantly decreased in the CBDL plus TCA injected group. The above results suggest that TCA repress the biosynthesis of the lysosomal $\alpha$-D-mannosidase and induce the biosynthesis of the lysosomal $\beta$-D-mannosidase in the liver. And that the elevated serum lysosomal $\alpha$-D- and $\beta$-D-mannosidase isozymes activities are most likely due to increased hepatocyte membrane permeability caused by TCA mediated liver cell necrosis.

• Proceedings of the Korean Society of Veterinary Clinics Conference
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• 2009.10a
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• pp.239-239
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• 2009

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.15 no.2
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• pp.122-126
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• 2012

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.

• Journal of the Korean Society of Radiology
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• v.83 no.5
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• pp.966-978
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• 2022

Jaundice in children have various etiologies. Among them, physiological jaundice is a very common disease observed in more than half of full-term neonates. When jaundice persists or develops after 2 weeks of age, the total/direct bilirubin is measured in consideration of the possibility of cholestasis. In case of cholestasis, imaging studies differentiate biliary atresia and other disorders of the extrahepatic bile ducts. In this review, we briefly presented the major differential diseases of cholestasis in children and introduced diagnostic imaging techniques, including normal findings.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.2
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• pp.262-266
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• 1999

Obstruction of the extrahepatic bile ducts is the most common cause of conjugated hyperbilirubinemia in early infancy. More than 90% of such obstructive lesions are accounted for by extrahepatic biliary atresia. A rare lesion is obstruction of the common duct by impacted, thickened secretions and bile. Bile plug syndrome is defined as extrahepatic obstruction of the bile ducts by bile sludge in term infants without anatomic abnormalities, congenital chemical defects of bile, or hepatocellular lesions. Obstruction of extrahepatic ducts by plugs of biliary material apperas to be due to the inspissation and precipitation of bile and mucus within the lumen of the ducts. Cholestasis and precipitation of bile develop in association with abnormal composition of bile in cystic fibrosis, hepatocellular damage, prolonged erythroblastic jaundice, altered biliary dynamics with total parenteral nutrition, gut dysfunction, diuretic therapy, exchange transfusions and perinatal hemolysis. In those cases, the term inspissated bile syndrome is used. The clinical and laboratory findings in bile plug syndrome are identical to those observed in biliary atresia and choledochal cyst. The diagnosis can be suspected based on the findings of clinical and laboratory examinations together with hepatobiliary imaging, ultrasonography, radionuclide scan and liver biopsy. We experienced a case of spontaneous resolution of bile plug syndrome in a 4-year-old girl. We report this case with brief review related literatures.

• Korean Journal of Radiology
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• v.23 no.4
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• pp.389-401
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• 2022

Objective: This study aimed to determine a factor for predicting suboptimal image quality of the hepatobiliary phase (HBP) of gadoxetic acid-enhanced MRI in patients with extrahepatic bile duct (EHD) cancer before MRI examination. Materials and Methods: We retrospectively evaluated 259 patients (mean age ± standard deviation: 68.0 ± 8.3 years; 162 male and 97 female) with EHD cancer who underwent gadoxetic acid-enhanced MRI between 2011 and 2017. Patients were divided into a primary analysis set (n = 184) and a validation set (n = 75) based on the diagnosis date of January 2014. Two reviewers assigned the functional liver imaging score (FLIS) to reflect the HBP image quality. The FLIS consists of the sum of three HBP features, each scored on a 0-2 scale: liver parenchymal enhancement, biliary excretion, and signal intensity of the portal vein. Patients were classified into low-FLIS (0-3) or high-FLIS (4-6) groups. Multivariable analysis was performed to determine a predictor of low FLIS using serum biochemical and imaging parameters of cholestasis severity. The optimal cutoff value for predicting low FLIS was obtained using receiver operating characteristic analysis, and validation was performed. Results: Of the 259 patients, 140 (54.0%) and 119 (46.0%) were classified into the low-FLIS and high-FLIS groups, respectively. In the primary analysis set, total bilirubin was an independent factor associated with low FLIS (adjusted odds ratio per 1-mg/dL increase, 1.62; 95% confidence interval [CI], 1.32-1.98). The optimal cutoff value of total bilirubin for predicting low FLIS was 2.1 mg/dL with a sensitivity of 95.1% (95% CI: 88.9-98.4) and a specificity of 89.0% (95% CI: 80.2-94.9). In the validation set, the total bilirubin cutoff showed a sensitivity of 92.1% (95% CI: 78.6-98.3) and a specificity of 83.8% (95% CI: 68.0-93.8). Conclusion: Serum total bilirubin before acquisition of gadoxetic acid-enhanced MRI may help predict suboptimal HBP image quality in patients with EHD cancer.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.1
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• pp.60-64
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• 2008

Kabuki syndrome is characterized by peculiar facial features, developmental delay, and mental retardation. Congenital hepatic abnormalities in Kabuki syndrome patients have been sporadically reported in the literature and consist of extrahepatic biliary atresia, neonatal sclerosing cholangitis, and transient neonatal cholestasis. We report here a case of congenital hepatic fibrosis in a patient with Kabuki syndrome. To our knowledge, only one case of congenital hepatic fibrosis has been reported in the setting of Kabuki syndrome.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.19 no.4
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• pp.286-290
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• 2016

Inspissated bile syndrome (IBS) is a rare condition in which thick intraluminal bile, including bile plugs, sludge, or stones, blocks the extrahepatic bile ducts in an infant. A 5-week-old female infant was admitted for evaluation of jaundice and acholic stool. Diagnostic tests, including ultrasound sonography, magnetic resonance cholangiopancreatography, and a hepatobiliary scan, were not conclusive. Although the diagnosis was unclear, the clinical and laboratory findings improved gradually on administration of urodeoxycholic acid and lipid emulsion containing omega-3 polyunsaturated fatty acids (PUFAs) for 3 weeks. However, a liver biopsy was suggestive of biliary atresia. This finding forced us to perform intraoperative cholangiography, which revealed a patent common bile duct with impacted thick bile. We performed normal saline irrigation and the symptom was improved, the final diagnosis was IBS. Thus, we herein report that IBS can be treated with omega-3 PUFAs as an alternative to surgical intervention.

• Toxicological Research
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• v.19 no.1
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• pp.67-72
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• 2003

Cholestatic liver injury results from the accumulation of toxic bile salts within the liver. The aim of the present study is to elucidate the changes in expression and cellular localization of apoptosis related proteins in the liver of bile duct-ligated (BDL) rat. Extrahepatic cholestasis was induced by double ligation of the common bile duct and cut between the ligatures. Animals were sacrificed at day 3 and at week 1, 2, 4, 6, and 8 after BDL. The number of TUNEL positive cells was increased significantly after 3 days of BDL, decreased over 2 weeks and remained constant thereafter. Fas expression was not changed and activation of caspase 8 did not occur. Fas immunoreactivity was exclusively observed in the cytoplasm of hepatocytes, indicating that Fas expressed in rat hepatocytes is a soluble form. Hepatocyte apoptosis was associated with Bax expression, which showed a peak at day 3 and decreased over time gradually. Immnunostaining of Bax was observed in hepatocytes and bile duct epithelial cells (BEC) of control and BDL rats. Bcl-2 was increased over time in BDL rats. These results suggest that apoptosis of hepatocytes in BDL rats is independent of Fas and controlled by Bax expression.

• Applied Microscopy
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• v.22 no.2
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• pp.15-29
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• 1992

The gallbladder is known to have the function of the storage and the concentration of the bile produced by the liver. This function is carried out by the removal of water and inorganic electrolytes. Extrahepatic cholestasis or the impairment of excretion of the bile leads to the distension and loss of the function of the gallbladder. The purpose of this study was to examine the ultrastructural characteristics of the normal gallbladder epithelial cells, and their structural changes induced by the ligation of common bile duct of the rabbit. Common bile duct ligation was performed under ether anesthesia. The rabbits were sacrificed on the 1st, 3rd, 5th, 7th and 14th day, respectively after operations. The tissue blocks of the gallbladder were fixed in 2.5% glutaraldehyde-1.5% paraformaldehyde prior to fixation in 1% osmium tetroxide, and embedded in the araldite mixture, and observed with JEM 100 CX-II electron microscope. The results were as follows: 1. The normal gallbladder epithelium of adult rabbit demonstrated two cell types, the ordinary epthelial cell and the dark cell. The dark cells have electron dense cytoplasm, and were found much infrequently, whereas ordinary epthelial cells were found quite numerous. 2. The ordinary epthelial cells of normal gallbladder were provided with the regular microvilli at the free surface and the images of pinocytotic activities in the apical cytoplasm, and exhibit highly convoluted lateral surfaces with elaborated microfolds. These figures of the cells suggest that they are resorptive in functional activity. 3. In the early stages (1st, 3rd, 5th day groups) following the ligation, the apical cytoplasm of some cells is protruding from the free surface and lost their microvilli. Numerous mucous granules filled in the apical and supranuclear cytoplasm compactly. 4. In the late stages (7th, 14th day groups) following the ligation, many light cells containing mumerous mucous granules are seen, between the ordinary epthelial cells. Mucous granules are fused each other, and are discharged into the lumen from the apical cytoplasm. The lateral membranes are straight or undulating without any interdigitations. From the above results, it was concluded that in the cholestasis induced by the common bile duct ligation, there is a tendency for the mucosal epithelium of the rabbit gallbladder to have secretory rather than an absorptive function.