• Journal of Yeungnam Medical Science
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• v.39 no.3
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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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• v.5 no.1
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• pp.51-61
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• 2002

Purpose: The most common causes of neonatal cholestasis are neonatal hepatitis (NH) and extrahepatic biliary atresia (EHBA). Since neonatal cholestasis presents with variable expression of same pathologic process and has similar clinical, biochemical, and histologic features between EHBA and idiopathic neonatal hepatitis (NH), differential diagnosis is often difficult. We reviewed the differences of clinical characteristics and laboratory data to find out any correlation between the results of $Tc^{99m}$ DISIDA scan and presence of acholic stool. Methods: Between June 1993 and January 2001, total 29 infants younger than 4 month-old underwent $Tc^{99m}$ DISIDA scan. Their biochemical tests and clinical course were reviewed retrospectively. Results: Patients who had negative intestinal activity on $Tc^{99m}$ DISIDA scan showed acholic stool and revealed higher serum direct bilirubin and urine bilirubin level. 18.2% of patients with acholic stool showed intestinal activity on $Tc^{99m}$ DISIDA scan and 81.8% of them did not. All the patients without acholic stool showed positive intestinal activity on $Tc^{99m}$ DISIDA scan. The result of $Tc^{99m}$ DISIDA scan and the presence of acholic stool showed high negative correlation (r :-0.858). Patients with acholic stool and negative intestinal activity on $Tc^{99m}$ DISIDA scan showed higher serum total bilirubin level. Patients without acholic stool and positive intestinal activity on $Tc^{99m}$ DISIDA scan showed higher serum level of ALT. Conclusion: Patients with acholic stool and negative intestinal activity showed high correlation, but 18.2% of patients with acholic stool showed positive intestinal activity. So operative cholangiogram or transcutaneous liver biopsy should be performed for confirmation.

• Journal of Physiology & Pathology in Korean Medicine
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• v.23 no.1
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• pp.7-14
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• 2009

Bile juice prevents deposition of cholesterol in the blood vessel, digests fat, and absorbs fatty acid and vitamins, and it plays a great role on metabolism. Recently, emotional stimulus and mentally over-depression cause a person to come to illness, and westernization of way of life makes more patients with cholelithiasis, resulting into without bile secretion after cholelithotomy. Ageing, and gastrectomy and kidney transplantation are also the causes of more cholelithiasis occurrences. To solve these medical problems, we studied how Fel Sus Scrofa, which is not different from human bile juice, was used in the traditional Korean medicine. We I researched flavor, property, efficacy of Fel Sus Scrofa and how it was used by folk medicine, and we studied the usage examples of Fel Sus Scrofa in Sanghanlon and Dongeuibogam. The property of Sus Scrofa is bitter and cold. Its efficacy is to deposit glycogen. So it is known that it has been widely used, with many edible forms, without any humoral loss, for the inflammatory disease from various fever, problems of urine and feces, cutaneous disease, pulmonary disease, opthalmopathy, fever, thirst from diabetes, hepatocystic duct disorder. Fel Sus Scrofa can be used internally and externally to prevent humoral loss, and to control cutaneous disease among various pediatric disorder full of fever. And as we have in mind that it also can be used to treat patients with cholestasis after cholecystectomy, it is expected that post study of it must be done.

• Biomedical Science Letters
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• v.19 no.2
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• pp.158-163
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• 2013

Cholestatic liver is associated with hepatic inflammation and elevated proinflammatory cytokines. Recent studies indicate that certain cytokines can modulate bile secretion. In the present study, we have examined the role of interleukin (IL-2) on the bile secretion by a combination of study models. To examine the relevance of IL-2 on bile secretion, the expression of IL-2 and IL-2 receptor (IL-2R) of isolated normal and bile duct ligated (BDL) rats cholangiocytes was first measured by RT-PCR. In BDL rats, the expression of IL-2 and IL-2R was significantly increased compared with normal rats. To study the effect of IL-2 on bile secretion, bile flow was measured in normal and BDL rats. At the level of cholangiocytes, secretory responses of isolated bile duct unit (IBDU)s were quantified by videomicroscopy. The administrations of IL-2 had no significant effect on basal bile secretion in normal and BDL rats. There was no significant effect of IL-2 on basal bile ductular secretion as evidenced by no significant difference in luminal area of the IBDUs perfusedwith 100 pM of IL-2 from those of albumin carrier control. However, the secretin-stimulated bile ductular secretion was significantly (P < 0.01) inhibited by $34{\pm}4%$ (normal, n = 12), $21{\pm}5.3%$ (BDL 2 wk, n = 12) and $15{\pm}5.2%$ (BDL 4 wk, n = 12) with the co-administration of IL-2. As with other cytokines, physiologically relevant concentration of IL-2 can significantly inhibit secretin-stimulated bile ductular secretion. These findings support the important roles of cytokines in modulating bile secretion and may contribute to the cholestasis seen in cholestatic liver diseases.

• Korean Journal of Veterinary Research
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• v.32 no.3
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• pp.357-364
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• 1992

Artemisia Iwayomogi Compositae) has been used clinically for jaundice, hepatitis, liver cirrhosis etc. The purposes of present study were to examine pharmacological effects of Artemisia Iwayomogi water extract(AIWE) on biochemical parameters (activities of ALP and LAP, contents of glucose, total bilirubin, total protein and albumin in serum, A/G ratio, and levels of hepatic glycogen) against hepatic injury by carbon tetrachloride($CCl_4$) in rats. The results were as follows ; 1. Increased ALP activities by $CCl_4$ were very significantly(p<0.001) decreased in AIWE posttreatment groups at 72 hours and significantly(p<0.05) decreased in AIWE pretreatment groups at 72 hours. Increased LAP activities by $CCl_4$ were significantly (p<0.05) decreased in AIWE posttreatment groups at 72 hours. A little increased total bilirubin contents by $CCl_4$ were very significantly (p<0.001) decreased in AIWE posttreatment groups at 24, 48 and 72 hours. 2. Increased glucose contents by $CCl_4$ were decreased in AIWE posttreatment groups. Decreased hepatic glycogen levels by $CCl_4$, were significantly (p<0.05) increased in AIWE posttreatment groups at 48 and 72 hours. 3. Decreased total protein contents by $CCl_4$ were significantly (p<0.05) increased in AIWE posttreatment groups at 48, 72 hours. Decreased albumin contents by $CCl_4$ were increased in proportion to numbers of AIWE treatments in AIWE pre- and posttreatement groups. Decreased A/G ratios by $CCl_4$ were significantly (p<0.05) increased in AIWE posttreatment groups at 48 hours. In conclusion, AIWE did not affect normal liver function and had hepatoprotective effects rather than direct preventive effects to $CCl_4$-induced cholestasis, damages in metabolisms of glucose, protein and bilirubin.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.104-107
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• 2005

Choledochal cyst is considered to be congenital anomalies of the biliary tract, characterized by varying degrees of cystic dilatation at various segments of the biliary tract. A 20-month-old girl was admitted to Eul-Ji general hospital because of abdominal distension. Physical examination revealed marked splenomegaly and hepatomegaly with nodular surface and hard consistency. Laboratory examination showed elevated transaminase level, alkaline phosphatase level and gamma glutamyltranspeptidase level without evidence of cholestasis. Diagnostic imaging study revealed choledochal cyst with Todani classification type 1. Cholecystectomy and Roux-en-Y choledochojejunostomy was performed, and wedge liver biopsy showed diffuse periportal fibrosis with cirrhotic change and ductular proliferation in the portal area. After operation, hepatosplenomegaly and abnormal laboratory examinations improved rapidly, and in 9 months, the liver and spleen became not palpable. We experienced a case of choledochal cyst complicated by liver cirrhosis on pathology in a 20 month-old girl, and removal of choledochal cyst improved clinical manifestations rapidly.

• Clinical and Experimental Pediatrics
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• v.51 no.5
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• pp.538-541
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• 2008

Menkes disease is an X-linked recessive copper transport disorder characterized by neurological deterioration, connective-tissue damage, and abnormal hair growth. It is caused by the mutation of the ATP7A gene. This report describes a four-month-old boy with neurological symptoms typical of Menkes disease plus unusual liver involvement. He developed seizures at three months of age and exhibited hypotonia, cephalhematoma, a sagging face, redundant and hypopigmented skin, and abnormal hair growth. In addition, he had unexplained hepatomegaly and high hepatic transaminase. We confirmed the diagnosis of Menkes disease by mutation analysis of the ATP7A gene. To exclude other possible causes for the hepatic abnormalities, a liver biopsy was performed, revealing intracytoplasmic cholestasis, focal spotty necrosis, and minimal lobular activity. The patient's liver involvement may be an underestimated complication of Menkes disease.

• Molecular & Cellular Toxicology
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• v.2 no.2
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• pp.141-149
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• 2006

Tamoxifen (TAM), a non-steroidal anti estrogen anticancer drug and chemopreventive agent for breast cancer, have caused cholestasis in liver. The potent hepatocarcinogenicity of this drug has been reported. Methotrexate (MTX) is dihydrofolate reductase inhibitor which interfaces with the synthesis for urine nucleotide and dTMP. And it may cause atrophy, necrosis and steatosis in liver. These two anticancer drug have well-known hepatotoxicity. So, in this study we compare the gene expression pattern of antitumor agent TAM and MTX, using the cDNA microarray. We have used 4.8 K cDNA microarray to identify hepatotoxicity-related genes in 5-week-old male Sprague-Dawley (SD) rats. Confirm the pattern of gene expression, we have used Real time PCR for targeted gene. In the case of MTX, Protease related gene (Ctse, Ctsk) and Protein kinase (Pctk 1) have shown specific expression pattern. And in the case of TAM, apoptosis related gene (Pdcd 8) and signal transduction related gene (kdr) have significantly up regulated during treatment time. Gene related with growth factor, lipid synthesis, chemokins were significantly changed. From the result of this study, the information about influence of TAM and MTX to hepatoxicity will provide.

• Biomolecules & Therapeutics
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• v.18 no.1
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• pp.83-91
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• 2010

Bear bile has been used as a therapeutic for cerebral and coronary thrombosis, convulsion, hepatitis, jaundice, and abscess in traditional oriental medicine. In recent decades, the effects of bile acids on cancer, cholestasis, and liver injury have been investigated in many studies. In this study, we investigated the anti-inflammatory effects of whole bear bile (WBB) and its two major components, chenodeoxycholic acid (CDCA) and ursodeoxycholic acid (UDCA), on rectal inflammation in rats. Bile acids in WBB were quantitatively analyzed by HPLC. Rectal inflammation was induced in male Sprague-Dawley rats by insertion of croton oil-saturated cotton tips. WBB, UDCA or CDCA solution was orally administered to rats one hour after induction of rectal inflammation. Rats were sacrificed 4 or 24 hours after induction of rectal inflammation. The evaluation included measurement of weight and thickness of rectum and histopathologic examination of rectal tissue. Furthermore, we examined the inhibitory effect of WBB, UDCA or CDCA against NO production in LPS-stimulated RAW 264.7 cells. The contents of UDCA and CDCA in WBB were $39.26{\mu}g/mg$ and $47.11{\mu}g/mg$, respectively. WBB treatment significantly reduced the weight and thickness of rectum compared with UDCA or CDCA treatment. The inhibition of NO production by WBB, UDCA and CDCA in LPS-stimulated RAW 264.7 cells was much higher than that by the control. And, WBB treatment suppressed the induction of NO synthase in rectum homogenates. These results suggest that the anti-inflammatory effect of WBB is related to the suppression of NO synthase induction and the inhibition of NO production by UDCA, CDCA and other bile acids of WBB.

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.86-90
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• 2003

Alagille syndrome is a autosomal dominant disorder characterized by intrahepatic bile duct paucity and resultant chronic cholestasis in combination with cardiac(mainly peripheral pulmonary stenosis), skeletal, ocular, and facial abnormalities. In addition to the pulmonary stenosis, in large series, anecdotal reports of vascular lesions have concerned the renal artery, aorta, hepatic artery, carotid artery, celiac artery or subclavian artery. Theses diffuse vascular abnormalities, which appear to be a feature of Alagille syndrome, suggest Notch signaling pathway defects affect angiogenesis. The associations of Alagille syndrome with moyamoya disease, the chronic cerebrovascular occlusive disease, were reported and suggested as additional evidence of vasculopathy of Alagille syndrome. We report another 25 month-old Alagille syndrome girl who presented with acute left hemiparesis and was diagnosed with moyamoya disease through the cerebral angiographic study.