• YAKHAK HOEJI
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• v.40 no.5
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• pp.550-557
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• 1996

One of therapeutics in liver disease (morbus wilson) is D-penicillamin (D-pen: D-3-mercapto-valin). Especially the cross-linking of collagen molecules could be inhibited by D-pe n in extracellular space. In this study we investigated the antifibrotic effects of D-pen in rats that were induced the liver fibrosis by bile duct ligation and scission (BDL/S). Rats were treated for 4 weeks with D-pen after BDL/S operation or sham operation. The balance between fibrogenesis-marker (PNIIIP) and the fibrolysis-maker (PNIVP) were observed in sera by RIA (radioimmunoassay), and the parameter of collagen deposition in liver tissue (hydroxyproline: HYP) was measured by colorimetry. The weight of liver in BDL/S operated group was increased significantly in compared with sham operation group (15.2g${\pm}$1.1, vs 11.9g${\pm}$3.9: p<0.005, p<0.05). The rats group treated by D-pen showed the lower level of PNIIIP (6.7ng/ml${\pm}$1.5, vs 9.5ng/ml${\pm}$2.8) and the higher value of PIVCP (14.0ng/ml${\pm}$1.9, vs 7.9ng/ml${\pm}$1.5) in sera that compared to untreated rats. The content of HYP was decreased by 141% in BDL/S with D-pen treated group than that of it in BDL/S group. No correlation was revealed between collagen parameters in sera and HYP in liver tissue of BDL/S operated and D-pen treated rats. The group treated with D-pen showed the lower value of clinical biochemistry parameters (GOT: glutamate oxalacetate transaminase, Total-Bilirubin) in compared with only BDL/S operated rats, but the value of GPT (glutamate pyruvate transaminase) and Alkaline phosphatase in two BDL/S groups was nearly same. In the histological finding, we observed mild bile duct proliferation, weak inflammation and fibrosis in BDL/S with D-pen treated group, but BDL/S operated group showed the formation of septum (island of hepatocytes), massive bile duct proliferation. This result represents that the BDL/S operation induces liver fibrosis (cirrhosis) in 4 weeks, and D-pen inhibits the synthesis of collagen weakly and stimulates the degradation of collagen in the extracellular space. We conclude that the monitoring of PNIIIP, PIVCP in sera is useful parameter for screening of antifibrotic effect, and D-pen delay the liver fibrosis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.5
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• pp.353-375
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• 2022

No systematic review to date has examined histopathological parameters in relation to native liver survival in children who undergo the Kasai operation for biliary atresia (BA). A systematic review and meta-analysis is presented, comparing the frequency of native liver survival in peri-operative severe vs. non-severe liver fibrosis cases, in addition to other reported histopathology parameters. Records were sourced from MEDLINE, Embase, and CENTRAL databases. Studies followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines and compared native liver survival frequencies in pediatric patients with evidence of severe vs. non-severe liver fibrosis, bile duct proliferation, cholestasis, lobular inflammation, portal inflammation, and giant cell transformation on peri-operative biopsies. The primary outcome was the frequency of native liver survival. A random effects meta-analysis was used. Twenty-eight observational studies were included, 1,171 pediatric patients with BA of whom 631 survived with their native liver. Lower odds of native liver survival in the severe liver fibrosis vs. non-severe liver fibrosis groups were reported (odds ratio [OR], 0.16; 95% confidence interval [CI], 0.08-0.33; I²=46%). No difference in the odds of native liver survival in the severe bile duct destruction vs. non-severe bile duct destruction groups were reported (OR, 0.17; 95% CI, 0.00-63.63; I²=96%). Lower odds of native liver survival were documented in the severe cholestasis vs. non-severe cholestasis (OR, 0.10; 95% CI, 0.01-0.73; I²=80%) and severe lobular inflammation vs. non-severe lobular inflammation groups (OR, 0.02; 95% CI, 0.00-0.62; I²=69%). There was no difference in the odds of native liver survival in the severe portal inflammation vs. non-severe portal inflammation groups (OR, 0.03; 95% CI, 0.00-3.22; I²=86%) or between the severe giant cell transformation vs. non-severe giant cell transformation groups (OR, 0.15; 95% CI, 0.00-175.21; I²=94%). The meta-analysis loosely suggests that the presence of severe liver fibrosis, cholestasis, and lobular inflammation are associated with lower odds of native liver survival in pediatric patients after Kasai.

• 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.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.28 no.1
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• pp.42-47
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• 2024

Backgrounds/Aims: Hepatolithiasis and choledocholithiasis are frequent pathologies and unfortunately, with the current treatment strategies, the recurrence incidence is still high. This study aimed to assess the outcomes of laparoscopic choledochotomy using cholangioscopy via the percutaneous-choledochal tube for the treatment of hepatolithiasis and choledocholithiasis in Vietnamese patients. Methods: A cross-sectional study of patients with hepatolithiasis and/or choledocholithiasis who underwent laparoscopic choledochotomy using intraoperative cholangioscopy via percutaneous-choledochal tube at the Department of Hepatopancreatobiliary Surgery, 108 Military Central Hospital, from June 2017 to March 2020. Results: A total of 84 patients were analyzed. Most patients were females (56.0%) with a median age of 55.56 years. Among them, 41.8% of patients had previous abdominal operations, with 33.4% having choledochotomy. All patients underwent successful laparoscopic common bile duct exploration followed by T-tube drainage without needing to convert to open surgery. Most patients (64.3%) had both intrahepatic and extrahepatic stones. The rate of stones ≥ 10 mm in diameter was 64.3%. Biliary strictures were observed in 19.1% of patients during cholangioscopy. Complete removal of stones was achieved in 54.8% of patients. Intraoperative complications were encountered in two patients, but there was no need to change the strategy. The mean operating time was 121.85 ± 30.47 minutes. The early postoperative complication rate was 9.6%, and all patients were managed conservatively. The residual stones were removed through the T-tube tract by subsequent choledochoscopy in 34/38 patients, so the total success rate was 95.2%. Conclusions: Laparoscopic choledochotomy combined with cholangioscopy through the percutaneous-choledochal tube is a safe and effective strategy for hepatolithiasis and/or choledocholithiasis, even in patients with a previous choledochotomy.

• Journal of Yeungnam Medical Science
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• v.7 no.2
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• pp.197-202
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• 1990

Mechanical obstruction of the common hepatic duct includes the following causes ; choledocholithiasis, sclerosis, cholangitis, pancreatic carcinoma, cholangiocarcinoma, postoperative stricture, primary hepatic duct carcinoma, enlarged cystic duct lymph nodes, and metastatic nodal involvement of the porta hepatis. Partial mechanical obstruction of the common hepatic duct caused by impaction of stones and inflammation surrounding the vicinity of the neck of the gallbladder had been reported on the "syndrome del conducto hepatico" in 1948 by Mirizzi. Nowadays, this disease was named by Mirizzi syndrome. Mirizzi syndrome is a rare entity of common hepatic duct obstruction that results from an inflammatory response secondary to a gallstone impacted in the cystic duct or neck of the gallbladder. It results from an almost parallel course and low insertion of the cystic duct into the common hepatic duct. In a varient of Mirizzi's syndrome, the cause of the common hepatic duct obstruction was a primary cystic duct carcinoma rather than gallstone disease. A 71-year-old man was admitted with a four-day history of right upper quadrant abdominal pain. Past medical history was unremarkable. On physical examination, the patient had a temperature of $38^{\circ}C$, icteric sclera and right upper quadrant tenderness. Pertinent laboratory findings included WBC 18,000/$cm^2$;albumin 2.6g/dl(normal 3.9-5.1) ; SGOT 183u/L(normal 0-50) ; SGPT167u/L(normal 0-65) ; bilirubin, 8.2mg/dl(normal 0-1) with the direct bilirubin, 4.4mg/dl(normal 0-0.4). Ultrasonography revealed a dilated extrahepatic biliary tree. ERCP showed that the superior margin was angular and more consistent with a calculus causing partial CHD obstruction(Mirizzi syndrome). At surgery a diseased gallbladder containing calculi was found. In addition, there was two calculi partially eroding through the proximal portion of the cystic duct and compressing the common hepatic duct. A cholecystectomy and excision of common bile duct was performed, with Roux-en-Y hepaticojejunostomy. The postoperative course was uneventful.

• Clinical and Experimental Pediatrics
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• v.59 no.5
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• pp.239-241
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• 2016

Choledochal cyst is a dilation that encloses the intrahepatic or both extra- and intrahepatic portions of the biliary ducts. Postnatally, ultrasonography is the initial diagnostic modality of choice, allowing for precise measurements of intra- or extrahepatic duct dilatation and identification of stones and sludge. Symptoms depend on the age at presentation. Common bile duct malformations should be considered as a differential diagnosis of a cystic mass regardless of the cyst's size or patient's age, especially in children presenting with abdominal pain, jaundice, and palpable mass. To the best of our knowledge, we report the largest choledochal cyst in infancy.

• 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.

• Clinical Endoscopy
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• v.57 no.2
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• pp.175-180
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• 2024

Therapeutic endoscopic ultrasonography (EUS) procedures using the forward-viewing convex EUS (FV-EUS) have been reviewed based on the articles reported to date. The earliest reported procedure is the drainage of pancreatic pseudocysts using FV-EUS. However, the study on drainage of pancreatic pseudocysts focused on showing that drainage is possible with FV-EUS rather than leveraging its features. Subsequently, studies describing the characteristics of FV-EUS have been reported. By using FV-EUS in EUS-guided choledochoduodenostomy, double punctures in the gastrointestinal tract can be avoided. In postoperative modified anatomical cases, using the endoscopic function of FV-EUS, procedures such as bile duct drainage from anastomosis, pancreatic duct drainage from the afferent limb, and abscess drainage from the digestive tract have been reported. When a perpendicular puncture to the gastrointestinal tract is required or when there is a need to insert the endoscope deep into the gastrointestinal tract, FV-EUS is considered among the options.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.3
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• pp.304-309
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• 2020

An 18-year-old woman with annular pancreas and duodenal duplication presented with recurrent acute pancreatitis and underwent a resection of duodenal duplication. However, the patient experienced recurrent abdominal pain after resection. Abdominal computed tomography and magnetic resonance imaging showed a dilatation of the peripheral pancreatic duct and stenosis and malformation of both the Wirsung's and Santorini's duct due to multiple stones. The modified puestow procedure was performed. The main pancreatic ducts in the body and tail were opened, and the intrapancreatic common bile duct was preserved. A Roux-en-Y pancreatico-jejunostomy was performed for reconstructing the pancreaticobiliary system after removing the ductal protein plug. The patient experienced no abdominal pain, no significant elevation of the serum amylase and lipase levels, and no stone formation during the 2 years of follow-up. This procedure is considered to be beneficial for pediatric patients with chronic pancreatitis due to annular pancreas and duodenal duplication.

• Proceedings of the Korea Environmental Mutagen Society Conference
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• 2002.11a
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• pp.108-114
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• 2002