• BMB Reports
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• 제29권2호
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• pp.142-145
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• 1996

To investigate the cause of increased plasma catecholamine levels in liver disease, catechol-O-methyltransferase (COMT), which provides a major route of catabolism for circulating catecholamines, was studied under the cholestasis induced by mechanical biliary obstruction in rats. Monoamine oxidase (MAO) activity and the $K_m$ and $V_{max}$ values for both enzymes were also measured. Cytosolic, microsomal, and mitochondrial COMT activities in the cholestatic liver were significantly decreased throughout the experiment. Microsomal, and mitochondrial MAO activity in the cholestatic liver were also significantly decreased. Vmax values of COMT and MAO were lower. Serum COMT and MAO activities were detected after CBD ligation. These results indicate that plasma catecholamine levels are increased in liver disease due to decreased hepatic degradation of catecholamines by decreased activities of COMT and MAO. The decreased activity of these enzymes is caused by decreased biosynthesis and by flowage into the blood from the damaged hepatocyte.

• Journal of Yeungnam Medical Science
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• 제18권1호
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• pp.51-58
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• 2001

담즙정체성 간염의 원인과 임상양상, 검사실 소견 및 경과를 알아보고자 1991년에서 2000년까지 영남대학교 의과대학 부속병원에서 혈액검사 및 간생검으로 담즙정체성 간염이 확진된 14명의 환자를 대상으로 후향적 연구를 시행하여 다음과 같은 결과를 얻었다. 담즙정체성 간염에서 비정상 간기능검사의 기간은 1개월에서 30개월까지 다양하게 나타났고, 항결핵제, 항생제에 의한 담즙정체성 간염이외에도 한약제, 건강식품에 의한 경우 검사실 소견과 임상경과가 중하게 나타나는 경우가 있으므로, 이들 약물을 사용한 병력이 있는 경우 정기적인 간기능 검사가 필요하다. 담즙정체성 간염이 만성 간내 담즙정체를 보이는 경우 바이러스에 의한 담즙정체성 간염과 담관소멸 증후군으로의 진행 유무, 원발성 담즙성 간경변증, 자가면역성 간염과의 감별이 필요하며, 지속적인 간기능 검사이상을 보일 경우에는 연속적인 간생검이 필요할 것으로 생각된다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제11권sup2호
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• pp.35-43
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• 2008

Jaundice is common in breast-fed infants. Any infant noted to be jaundiced at 2 weeks of age need to be evaluated for cholestasis with measurement of total and direct serum bilirubin. The most common causes of cholestatic jaundice in infants are biliary atresia and neonatal hepatitis. Genetic causes of the neonatal hepatitis syndrome are increasingly recognized and idiopathic neonatal hepatitis is decreasing. Cholestasis should be investigated using a structured protocol. Early detection and timely, accurate diagnosis is important for successful treatment and a favorable prognosis. In particular, a Kasai portoenterostomy for biliary atresia has the best outcome if performed before the infant is 8 weeks of age. The management of cholestasis is mainly supportive, including nutritional support and alleviation of symptoms to improve the quality of life. Specific treatments are available for some causes of neonatal hepatitis syndrome and should be started as soon as possible. For decompensated liver disease, liver transplantation yields a better outcome.

• 한국임상약학회지
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• 제15권1호
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• pp.9-20
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• 2005

Cholestatic liver disease is a frequent complication of prolonged parenteral nutrition, especially in premature infants. Numerous factors have been cited as contributing to TPN associated cholestasis. However the exact etiology remains obscure. Ursodeoxycholic acid (UDCA) has been reported to be beneficial far children and adults with various chronic cholestatic liver disease. The aim of this prospective, randomized, double-blind, placebo-controlled study was to determine the preventive effects of UDCA administration during TPN. Seventeen pediatric patients (8 boys and 9 girls) undergoing TPN were assigned randomly to two groups, UDCA and placebo group. UDCA group (n=9) received 15 mg/kg/day UDCA and placebo group (n=8) received 15 mg/kg/day placebo enterally during the TPN period. Liver function tests (total bilirubin, aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase) were per-formed before TPN and weekly or three times a week. The patients' weights, complete blood count, composition of TPN, and the infusion rate of TPN and lipid were monitored everyday. Calcium and phosphate were monitored twice a week. Between the UDCA and placebo groups, there were no differences in weight at the onset of TPN, birth weight, duration of TPN, respiratory distress syndrome associated with prematurity, age at the onset of TPN, gestational age, the number of days the patients received antibiotics, the number of patients received enteral nutritions and the composition of TPN. In contrast, there was a significant difference between the UDCA and placebo groups in alanine aminotransferase levels during TPN. It doesn't seem that UDCA administration during TPN correlates directly with improvement of liver function. But the preventive administration of UDCA may be effective in reducing liver enzyme, alanine aminotransferase and has no adverse effects.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제23권2호
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• pp.180-187
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• 2020

Giant cell hepatitis with autoimmune hemolytic anemia (AHA) is a rare disease of infancy characterized by the presence of both Coombs-positive hemolytic anemia and progressive liver disease with giant cell transformation of hepatocytes. Here, we report a case involving a seven-month-old male infant who presented with AHA followed by cholestatic hepatitis. The clinical features included jaundice, pallor, and red urine. Physical examination showed generalized icterus and splenomegaly. The laboratory findings suggested warm-type AHA with cholestatic hepatitis. Liver biopsy revealed giant cell transformation of hepatocytes and moderate lobular inflammation. The patient was successfully treated with four doses of rituximab. Early relapse of hemolytic anemia and hepatitis was observed, which prompted the use of an additional salvage dose of rituximab. He is currently in clinical remission.

• Advances in pediatric surgery
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• 제17권1호
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• pp.1-14
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• 2011

Biliary atresia (BA) is an infantile cholestatic disease of progressive obliterative cholangiopathy with varying degrees of damage to both extra and intrahepatic bile ducts due to unknown causes. The diagnostic studies should be done to diagnose or exclude BA without unnecessary delay. Kasai portoenterostomy is the first choice of treatment for bile drainage from microscopic bile ductules present in the portal fibrous mass. The medical management after Kasai portoenterostomy should be done carefully to maintain bile excretion and prevent and treat complications Including cholangitis, hepatic fibrosis, portal hypertension and nutritional problem. The reported five years-survival rates after Kasai portoenterostomy range from 30 to 60 %. About 20 % of all patients undergoing Kasai portoenterostomy during infancy survive into adulthood with their native liver. Even if Kasai portoenterostomy remains as the first line of treatment In BA, liver transplantation serves as a good salvage treatment when portoenterostomy fails or liver function gradually deteriorates after initially successful establishment of bile flow, Overall 5-year survival rate in BA is about 90 % in recent series.

• Journal of Yeungnam Medical Science
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• 제39권3호
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• pp.256-261
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• 2022

Sarcoidosis often involves the liver. However, primary hepatic sarcoidosis confined to the liver without evidence of systemic involvement is rare. We report the case of a 37-year-old man with hepatic sarcoidosis who initially presented with elevated liver enzymes and suspicious cirrhotic nodules on computed tomography. The patient had cirrhosis but did not have portal hypertension. Based on the initial histopathologic finding of chronic granulomatous inflammation and the common clinical characteristics of sarcoidosis, he was initially diagnosed with primary biliary cholangitis, and his daily dosage of ursodeoxycholic acid was increased to 900 mg. After 14 months of treatment, his total serum bilirubin concentration was 10.9 mg/dL (upper normal limit, 1.2 mg/dL). Additionally, a transjugular liver biopsy revealed multiple noncaseating granulomas. He was diagnosed with primary hepatic sarcoidosis involving the lungs, heart, spleen, kidneys, and skin. Treatment with methylprednisolone was initiated. Two weeks later, he was started on azathioprine, and the dose of steroid was simultaneously reduced. These findings indicate the importance of including hepatic sarcoidosis as a possible diagnosis in patients with elevated liver enzymes or cryptogenic cirrhosis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제20권1호
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• pp.55-60
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• 2017

Intestinal hypoganglionosis is a rare innervation disorder that provides numerous nutritional, medical and surgical challenges. In this case report, we present a case of a newborn with intestinal hypoganglionosis leading to intestinal failure and intestinal failure-associated liver disease who responded to $Omegaven^{TM}$, a fat emulsion comprised of omega-3 fatty acids. $Omegaven^{TM}$ has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with $Omegaven^{TM}$ was seen in this patient with a clear decrease in aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and complete resolution of cholestasis with a direct bilirubin of zero within two weeks of initiation of $Omegaven^{TM}$. No current guidelines for the diagnosis and management of hypoganglionosis are available. We recommend a multidisciplinary approach and the use of novel therapies such as fat emulsions composed of omega-3 fatty acids for improved patient outcomes. Appropriate compassionate use protocols should be obtained from the Food and Drug Administration prior to initiation of $Omegaven^{TM}$.

• BMB Reports
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• 제32권4호
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• pp.331-338
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• 1999

Ethanol catabolism is thought to produce metabolic disorders resulting in alcoholic liver disease. To investigate the mutual effects of ethanol catabolism and cholestasis induced by common bile duct ligation on the activities of carboxylesterase, we have determined the enzyme activities in rat hepatic (cytosolic, mitochondrial, and microsomal) preparations as well as in rat serum using ten animal models: normal rats (group 1), sham-operated rats (group 2), common bile duct-ligated rats (group 3), ethanol-intoxicated rats (group 4), sham-operation plus chronic ethanol-intoxicated rats (group 5), common bile duct-ligated plus chronic ethanol-intoxicated rats at 1.5h and 24h (groups 7A and 7B), and duct-ligated and acute ethanol intoxicated rats at 1.5 h and 24 h (groups 8A and 8B). The $K_m$ and $V_{max}$ values of carboxylesterase from these hepatic preparations of cholestatic rat liver combined with chronic ethanol intoxication were also measured by using ethyl valerate as the substrate from the 14th day post-ligation. Carboxylesterase activities of all hepatic preparations and rat serum (group 3) showed significant decreases compared to the activities from the sham-operated control (group 2). Enzyme kinetic parameters indicated that $V_{max}$ of carboxylesterase from all the hepatic preparations in cholestatic rats (group 3) decreased significantly, although the $K_m$ values were about the same as in the sham-operated control (group 2). When cholestasis was combined with chronic ethanol intoxication (group 6), carboxylesterase activities showed further decrease in all the hepatic preparations and serum compared to the control activity (group 5). The $V_{max}$ also decreased significantly, although $K_m$ values did not change. When common bile duct ligation was combined with acute ethanol intoxication (group 8), the enzyme activities in the rat liver and serum showed significant decrease compared to the activity from acute ethanol-intoxicated rats (group 7). However, quite contrary to this, the activities of serum from acute ethanol intoxication 1.5 h (group 7A) increased significantly compared to the activities in the normal control (group 1). These results, therefore, suggest that the biosynthesis of hepatic carboxyl-esterase seems to decrease when cholestasis is combined with chronic and acute ethanol intoxication, and the decrease in activity is more significant than from cholestasis alone.

• 약학회지
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• 제54권4호
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• pp.270-281
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• 2010

Total parenteral nutrition (TPN) is necessary to neonates in neonatal intensive care unit (NICU) for survival and growth because of impossible of enteral feeding. Long-term TPN can be associated with a broad spectrum of hepatobiliary disorder, ranging from mild hepatic dysfunction to severe end-stage liver disease. Cholestasis developed most commonly in neonate, ursodeoxycholic acid (UDCA) is widely used in adult with cholestatic and non-cholestatic liver diseases but there have been limited data on the effects in neonate with PNAC. This study was performed retrospectively to review all medical histories of the total 30 neonates with was administrated UDCA for treatment to parenteral nutrition associated cholestasis (PNAC) at Chungbuk National University Hospital NICU from April 2002 to December 2008. UDCA was administrated at bilirubin is over 2 mg/dl. The criterias for drug evaluation were included hepatic biochemical marker such as direct bilirubin, total bilirubin, AST, ALT, ALP and GGT, TPN therapy period, cholestasis development period, UDCA treatment period, UDCA dosage and adverse effect. In the results, Post-UDCA treatment significant was decreased direct bilirubin, total bilirubin, AST and ALP (p<0.05), and was decreased GGT (p>0.05) and slightly was increased ALT (p>0.05). Reffective timect biDCA was appear at mean $10.5{\pm}1.3$ days, iDCA administration period was mean $64.4{\pm}5.9$ days, cholestasis period was mean $71.9{\pm}6.4$ days and UDCA dosage was mean $22.9{\pm}0.9$ mg/kg/day. Common adverse effects is diarrhea, 5 patients arised mild diarrhea but it possible also related with increased enteral feeding. In conclusion, iDCA can decrease direct bilirubin that major parameter t bcholestasis and oher hepatic biochemical makers. UDCA is effective on PNAC without any serious side effect and cost-effective. Although no greatly shortening cholestasis period, but can protect to develop into severe liver disease and other complication or death. Based on these result, UDCA is recommended for treatment of cholestasis at direct bilirubin is over 2 mg/dl.