• 대한의생명과학회지
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• 제8권4호
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• pp.203-209
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• 2002

Serum $\alpha$-D-mannosidase isozyme activities were measured in rats with ethanol intoxication combined with extrahepatic cholestasis induced by common bile duct ligation for the manifestation of the biochemical background of drinking hazards under the hepatobiliary disease. When chronic ethanol intoxication was combine with extraheparlc cholestasis, the activities of the rat's serum cytosolic, Iysosomal and Golgi $\alpha$-D-mannosidase isozymes increased at a more significant rate than those of the cholestasis alone. However, when acute ethanol intoxication was combined with extrahepatic cholestasis, the activities of the above isozymes were seen in the cholestasis alone. The results suggested that the elevated activities of these isozymes in chronic ethanol intoxication with cholestasis rather than in cholestasis alone were indications of increased hepatic damages, which caused these isozymes to leak into the blood in great quantity. Accordingly, the resulting data supported the fact that alcoholic drinks were enzymologically harmful to the hepatobiliary disease.

• 대한의생명과학회지
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• 제9권1호
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• pp.21-27
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• 2003

Hepatic subcellular $\alpha$-D-mannosidases activities and its Km and Vmax values were determined in chronic ethanol intoxicated rats with extrahepatic cholestasis induced by common bile duct ligation to manifest the biochemical background of alcohol drinking hazard under the hepatobiliary disease. In case of extrahepatic cholestasis, chronic ethanol intoxication in animals led to the increased activities of liver Golgi and microsomal $\alpha$-D-mannosidase as well as the Vmax values of these enzymes. However, the difference of Km values on hepatic subcellular enzymes were not found between the experimental groups. Therefore, the results indicate that the liver Golgi and microsomal $\alpha$-D-mannosidase may be more induced in chronic ethanol intoxication animals in case of cholestasis. Accordingly, the resulting data supported the fact that alcoholic drinks may led to enhancement of the hepatobiliary liver damage.

• 대한의생명과학회지
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• 제10권2호
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• pp.99-105
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• 2004

Liver and serum rhodanese activities were determined in acute ethanol intoxicated rats with extrahepatic cholestasis induced by common bile duct ligation (CBD) to manifest the biochemical background of alcohol drinking hazard under the hepatobiliary disease. Liver cytosolic and microsomal rhodanese activities and these Vmax values in CBD ligated rats with acute ethanol intoxication were found to be decreased much more than that in CBD ligation alone. However, the difference of Km value on above hepatic enzyme was not found between the experimental groups. On the other hand, serum rhodanese activity in CBD ligated rats with acute ethanol intoxication was greater increased more than that in CBD ligation alone. These results indicate that the biosynthesis of the hepatic rhodanese decreases and the serum rhodanese activity increases in cholestasis combined with acute ethanol intoxication, reflecting damage of aggravated hapatocytic membrane. Accordingly, the resulting data supported the fact that alcoholic drinks were enzymologically harmful to the hepatobiliary disease.

• 대한의생명과학회지
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• 제10권1호
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• pp.35-42
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• 2004

Liver and serum $\beta$-D-mannosidase activities were determined in ethanol intoxicated rats with extrahepatic cholestasis induced by common bile duct ligation (CBD) to manifest the biochemical background of alcohol drinking hazard under the hepatobiliary disease. Liver $\beta$-D-mannosidase activity and its Vmax value in CBD ligated rats with chronic ethanol intoxication were found to be significantly decreased than that in CBD ligation alone. However, the difference of Km value on above hepatic enzyme was not found between the experimental groups. On the other hand, serum $\beta$-D-mannosidase activity in CBD ligated rats with chronic ethanol intoxication was increased more than that in CBD ligation alone. These results indicate that the biosynthesis of the hepatic $\beta$-D-mannosidase decreases and the serum $\beta$-D-mannosidase activity increases in cholestasis combined with chronic ehtanol intoxication, reflecting damage of aggravated hapatocytic membrane. Accordingly, the resulting data supported the fact that alcoholic drinks were enzymologically harmful to the hepatobiliary disease.

• Journal of Pharmaceutical Investigation
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• 제25권3호
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• pp.193-204
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• 1995

In order to examine the effect of extrahepatic cholestasis induced by common bile duct ligation on the hepatic function, the pharmacokinetics of antipyrine and d-propranolol were investigated in rats. In addition, in an attempt to observe the degree of direct hepatic injury, light and electron microscopic observations and conventional pathologic test using serum were performed. Five days after common bile duct ligation, antipyrine(15 mg/kg) and d-propranolol(3 mg/kg) were intravenously administrated to the rats, respectively. The total clearances of antipyrine and d-propranolol were significantly(p<0.05) decreased. Because hepatic clearance of antipyrine poorly extracted by the liver and that of d-propranolol highly extracted by the liver are respectively dependent on the hepatic intrinsic clearance and the hepatic blood flow, it may be concluded that extrahepatic cholestasis following five days after common bile duct ligation decreased the hepatic intrinsic clearance and the hepatic blood flow. SGPT, SGOT, cholesterol, bilirubin(total bilirubin, direct bilirubin) and alkaline phosphatase were significantly increased(p<0.05). The proliferation of bile ducts was prominent, and degeneration and necrosis of hepatocytes were observed by light microscope. Also, ultrastructurally, bile canaliculi were containing the amorphous materials and losing microvilli, and SER and RER in hepatocytes were dilated and vacuolated.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제19권1호
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• pp.1-11
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• 2016

Cholestasis results from impairment in the excretion of bile, which may be due to mechanical obstruction of bile flow or impairment of excretion of bile components into the bile canaliculus. When present, cholestasis warrants prompt diagnosis and treatment. The differential diagnosis of cholestasis beyond the neonatal period is broad and includes congenital and acquired etiologies. It is imperative that the clinician differentiates between intrahepatic and extrahepatic origin of cholestasis. Treatment may be supportive or curative and depends on the etiology. Recent literature shows that optimal nutritional and medical support also plays an integral role in the management of pediatric patients with chronic cholestasis. This review will provide a broad overview of the pathophysiology, diagnostic approach, and management of cholestasis beyond the neonatal and infancy periods.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권6호
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• pp.565-570
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• 2019

Benign biliary strictures are uncommon in children. Classically, these cases are managed surgically, however less invasive approaches with interventional radiology and or endoscopy may have similar results and improved safety profiles While benign biliary strictures have been described in literature on several occasions in young children, (most older than 1 year and once in an infant 3 months of age), all reported cases were managed surgically. We present two cases of benign biliary strictures in infants less than 6 months of age that were managed successfully with novel non-invasive procedures and a review of all current pediatric cases reported in the literature. Furthermore, we describe the use of a Rendezvous procedure, which has not been reported as a treatment approach for benign biliary strictures.

• 대한핵의학회지
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• 제22권2호
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• pp.181-185
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• 1988

Since hepatocyte clearance, leading edge parencymal transit time and biliary excretion can be evaluated separately with hepatobiliary scan using $^{99m}Tc-DISIDA$, hepatobiliary scan may be useful in differentiating intrahepatic cholestasis from extrahepatic cholestasis. Excretory liver function was analysed in 13 healthy subjects and 11 patients with clinically suspected hepatocellular disease and 9 patients with extrahepatic biliary obstruction confirmed by surgery, radiological and clinical evidence. Indices of total liver activity (% TLA), liver parechymal uptake (% LPU), heart pool clearance (% HPC) and liver-heart rate (% LHR) were calculated from time activity curve over heart and liver. Compared with healthy subjects, significant reduction (p<0.05) in total liver activity (% TLA) and liver-heart rate (% LHR) was observed in all patients group. But no useful indices was demonstrated in differentiating hepatocellular disease from extrahepatic biliary obstruction.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제20권2호
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• pp.114-123
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• 2017

Purpose: The goal of this study was the early diagnosis of ABCB11 spectrum liver disorders, especially those focused on benign recurrent intrahepatic cholestasis and progressive familial intrahepatic cholestasis. Methods: Fifty patients presenting neonatal cholestasis were evaluated to identify underlying etiologies. Genetic analysis was performed on patients suspected to have syndromic diseases or ABCB11 spectrum liver disorders. Two families with proven ABCB11 spectrum liver disorders were subjected to genetic analyses to confirm the diagnosis and were provided genetic counseling. Whole exome sequencing and Sanger sequencing were performed on the patients and the family members. Results: Idiopathic or viral hepatitis was diagnosed in 34%, metabolic disease in 20%, total parenteral nutrition induced cholestasis in 16%, extrahepatic biliary atresia in 14%, genetic disease in 10%, neonatal lupus in 2%, congenital syphilis in 2%, and choledochal cyst in 2% of the patients. The patient with progressive familial intrahepatic cholestasis had novel heterozygous mutations of ABCB11 c.11C>G (p.Ser4*) and c.1543A>G (p.Asn515Asp). The patient with benign recurrent intrahepatic cholestasis had homozygous mutations of ABCB11 c.1331T>C (p.Val444Ala) and heterozygous, c.3084A>G (p.Ala1028Ala). Genetic confirmation of ABCB11 spectrum liver disorder led to early liver transplantation in the progressive familial intrahepatic cholestasis patient. In addition, the atypically severe benign recurrent intrahepatic cholestasis patient was able to avoid unnecessary liver transplantation after genetic analysis. Conclusion: ABCB11 spectrum liver disorders can be clinically indistinguishable as they share similar characteristics related to acute episodes. A comprehensive genetic analysis will facilitate optimal diagnosis and treatment.

• 대한수의학회지
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• 제58권4호
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• pp.227-230
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• 2018

A 2-year-old, spayed female, Korean domestic short-hair cat was presented with depression and vomiting. The patient had history of weight loss lasting seven months. Physical examination revealed icterus in the pinna, oral mucosa, and sclera. Based on ultrasonography and computed tomography, tentative diagnosis was extrahepatic biliary tract obstruction with acquired portosystemic shunt (PSS). Tumor or inflammation of hepatobiliary system was suspected as the cause of obstruction of the common bile duct. But it could not be determined without biopsy. The severely dilated cystic duct was considered to cause portal hypertension and secondary multiple PSS. The patient expired without histopathologic examination.