• Korean Journal of Veterinary Research
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• v.58 no.4
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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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.1
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• pp.1-6
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• 2021

Next-generation sequencing (NGS) technologies have changed the process of genetic diagnosis from a gene-by-gene approach to syndrome-based diagnostic gene panel sequencing (DPS), diagnostic exome sequencing (DES), and diagnostic genome sequencing (DGS). A priori information on the causative genes that might underlie a genetic condition is a prerequisite for genetic diagnosis before conducting clinical NGS tests. Theoretically, DPS, DES, and DGS do not require any information on specific candidate genes. Therefore, clinical NGS tests sometimes detect disease-related pathogenic variants in genes underlying different conditions from the initial diagnosis. These clinical NGS tests are expensive, but they can be a cost-effective approach for the rapid diagnosis of rare disorders with genetic heterogeneity, such as the glycogen storage disease, familial intrahepatic cholestasis, lysosomal storage disease, and primary immunodeficiency. In addition, DES or DGS may find novel genes that that were previously not linked to human diseases.

• Journal of Applied Biological Chemistry
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• v.66
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• pp.138-143
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• 2023

Liver fibrosis is caused by metabolic problems such as cholestasis, genetic problems, or viral infections. Inhibiting hepatic stellate cell (HSC) activation or inducing selective apoptosis of activated HSCs is used as a treatment strategy for liver fibrosis. It has been reported that when HSCs are activated, their apoptosis sensitivity to tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) is enhanced because the expression of death receptor 5 is elevated. Finding a natural compound that can enhance the apoptotic effect of TRAIL on HSCs is a necessary strategy for liver fibrosis treatment. It was confirmed here that mangosteen-derived gartanin increased the effect of TRAIL-induced apoptosis by increasing the expression of DR5 in a p38-dependent manner in the hepatic stellate cell line LX-2. Combined treatment with gartanin and TRAIL accelerated DNA cleavage through caspase-3 activation and enhanced antifibrotic effects in LX-2 cells.

• Clinical Endoscopy
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• v.56 no.2
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• pp.135-142
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• 2023

Endoscopic biliary drainage strategies for managing unresectable malignant hilar biliary obstruction differ in terms of stent type, drainage area, and deployment method. However, the optimal endoscopic drainage strategy remains unclear. Uncovered self-expandable metal stents (SEMS) are the preferred type because of their higher functional success rate, longer time to recurrent biliary obstruction (RBO), and fewer cases of reintervention than plastic stents (PS). Other PS subtypes and covered SEMS, which feature a longer time to RBO than PS, can be removed during reintervention for RBO. Bilateral SEMS placement is associated with a longer time to RBO and a longer survival time than unilateral SEMS placement. Unilateral drainage is acceptable if a drainage volume of greater than 50% of the total liver volume can be achieved. In terms of deployment method, no differences were observed in clinical outcomes between side-by-side (SBS) and stent-in-stent deployment. Simultaneous SBS boasts a shorter procedure time and higher technical success rate than sequential SBS. This review of previous studies aimed to clarify the optimal endoscopic biliary drainage strategy for unresectable malignant hilar biliary obstruction.

• Clinical and Experimental Pediatrics
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• v.49 no.9
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• pp.972-976
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• 2006

Purpose : The purpose of this study was to evaluate the effect of amino acid mixtures on incidence and severity of total parenteral nutrition associated-cholestasis(PNAC) in very low birth weight infants. Methods : Retrospective review of 63 very low birth weight infants(birth weight ${\leq}1,500g$) who received total parenteral nutrition(TPN) in our neonatal intensive care unit from January 2000 to December 2004 was performed. Patients were divided into 2 groups : Group I(n=32, Jan 2000-Jun 2002) and Group II(n=31, Jul 2002-Dec 2004), where infants in Group II received taurine and glutamic acid-rich amino acid mixtures. PNAC was defined as serum direct bilirubin(DB) level greater than 2.0 mg/dL. The incidence and severity of PNAC were compared between these groups. Results : The incidence of PNAC was significantly lower in Group II than in Group I(21.9% vs 6.5%, P<0.148). Maximum and mean DB levels were also significantly lower in Group II(P<0.05). Conclusion : The incidence and severity of PNAC in very low birth weight infants may be reduced with different composition of amino acid mixtures in TPN. Further prospective randomized controlled studies are needed to determine an ideal composition of acid mixtures to prevent the development of PNAC.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.1
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• pp.61-73
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• 2004

Purpose: Liver function test abnormalities have been reported frequently in patients receiving total parenteral nutrition (TPN). In adults, it is known that liver complications decrease with the use of cyclic parenteral nutrition (CPN), especially if the shift to cycling was not too late. However, there are few studies about the effects of cycling on liver injury in children beyond the neonatal period. The aim of this study is to evaluate the effect of the early use of CPN on total parenteral nutrition induced hepatic dysfunction. Methods: Twelve sets of CPN in 11 children (2 months to 17 years) were included in this study. Data on underlying diseases, age, length of time on TPN, macronutrient intake, complications, and biochemical parameters were collected from clinical records. All children had received CPN in the early period of persistent transaminase elevation or cholestasis complicated by previous continuous PN. The duration of infusion off-time in CPN was 2 hours in patients less than 3 months of age and 4 hours in the older children. Results: All 12 cases showed elevated aminotransferase and 5 of them also showed cholestasis. Serum total bilirubin concentration was normalized in all 5 cases with median periods of 8 days (p<0.05) after initiation of CPN. ALT either decreased significantly or was normalized in all cases with median periods of 30 days (p<0.05) on CPN. The CPN was well tolerated without significant complication except for one case of hyperglycemia. Conclusion: The early use of cyclic parenteral nutrition had a beneficial effect in improving hepatic dysfunction complicated by TPN in children.

• Korean Journal of Clinical Pharmacy
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• v.15 no.1
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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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• v.4 no.1
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• pp.71-76
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• 2001

Purpose: Many diagnostic modalities for neonatal cholestasis have shown features that are helpful, however until recently none of them are not pathognomonic of biliary atresia (BA). We carried out a prospective study of infants with cholestatic jaundice with the aim of establishing an efficient method of diagnosing BA. Methods: Twenty-seven consecutive infants with cholestatic jaundice were enrolled in this study and ranged from 7 to 152 days in age (mean; $51.2{\pm}34.2$ days). Gastroduodenoscopy was carried out using a fiberscope (Olympus N30). All the babies were fasted for at least 4 hours before the procedure and 20 ml of 10% dextrose solution was given at the time of endoscopy. The endoscopic examination focused on the 5 minutes observation of the evidence of biliary secretion. If there was lack of the evidence of the biliary secretion, endoscopy was removed and repeated the examination with some pause. Results: There are lack of the evidence of biliary secretion in all infants with BA. In non-BA group, 8 out of the 10 infants showed biliary secretion on the first trial, however one (Alagille syndrome) of the two infants without evidence of biliary secretion, finally exhibited biliary secretion on the second trial. The above observations resulted in the diagnostic accuracy of 96.3% with 100.0% sensitivity and 90.0% specificity. Conclusion: In light of the results from our relatively small study, endoscopy is a convenient, and relative inexpensive procedure. we strongly support the use of endoscopy for the diagnosis of BA in the screening and evaluation of infantile cholestasis.

• 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.

• 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.