• Advances in pediatric surgery
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• v.13 no.1
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• pp.1-12
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• 2007

Biliary atresia (BA) is the result of fibrosing destructive inflammatory process affecting intrahepatic and extrahepatic bile ducts, which lead to cirrhosis and portal hypertension. Kasai portoenterostomy has been the standard operative procedure in biliary atresia. Recently, there has been remarkable increase in the survival rate in cases of BA. However, long-term survivors are not clearly evaluated in Korea. To define long-term prognosis factors of patients who underwent surgery for BA, a retrospective study was undertaken of 10 (37 %) patients surviving more than 10 years among 27 patients who underwent one of Kasai procedures between 1981 and 1995. Hepatomegaly was present in 4 and splenomegaly in 7 patients. Serum bilirubin was normalized at 1 year after operation. Aspartate aminotransferase (AST, GOT), Alanine aminotransferase(ALT, GPT) were normalized at 12 years and alkaline phosphatase(ALP) was normalized at 13.5 years. Cholangitis developed mainly within 5 years after operation so close follow up is needed. Life long follow-up is needed because of progressive deterioration of liver function even after 10 years.

• 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.

• Advances in pediatric surgery
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• v.8 no.2
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• pp.156-160
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• 2002

When jaundice persists for more than 14 days postnatally, the early diagnosis of surgical jaundice is important for the prognosis in extrahepatic biliary atresia after draining procedure. The role of diagnostic laparoscopy to differenctiate medical causes of jaundice from biliary atresia is evaluated in this report. Four patients with prolonged jaundice have been included in this study. When the gallbladder was not visualized we proceeded to laparotomy. In patients with enlarged gallbladder visualized at laparoscopy, laparoscopic guided cholangiogram was performed, and laparoscopic liver biopsy was done for those who had a patent biliary tree. Two patients had small atretic gallbladder and underwent a Kasai hepato-portoenterostomy. One patients showed a patent gallbladder and common bile duct with atresia of the common hepatic and intrahepatic ducts, and they underwent a Kasai hepatic-portoenterostomy. One patient showed an enlarged gallbladder and laparoscopic-guided cholangiogram were normal. Laparoscopic liver biopsy was performed. There were no complications. Laparoscopy with laparoscopic-guided cholangiogram may be a valuable method in accurate and earlier diagnosis in an infant with prolonged jaundice.

• Parasites, Hosts and Diseases
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• v.54 no.5
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• pp.679-684
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• 2016

Clonorchiasis, caused by direct contact with Clonorchis sinensis worms and their excretory-secretory products (ESPs), is associated with chronic inflammation, malignant changes in bile ducts, and even cholangiocarcinogenesis. Our previous report revealed that intracellular free radicals enzymatically generated by C. sinensis ESPs cause NF-${\kappa}B$-mediated inflammation in human cholangiocarcinoma cells (HuCCT1). Therefore, the present study was conducted to examine the role of upstream Toll-like receptors (TLRs) on the initial host innate immune responses to infection. We found that treatment of HuCCT1 cells with native ESPs induced changes in TLR mRNA levels in a time-dependent manner, concomitant with the generation of free radicals. ESP-mediated free radical generation was markedly attenuated by preincubation of the cells with TLR1-4-neutralizing antibodies, indicating that at least TLR1 through 4 participate in stimulation of the host innate immune responses. These findings indicate that free radicals triggered by ESPs are critically involved in TLR signal transduction. Continuous signaling by this pathway may function in initiating C. sinensis infection-associated inflammation cascades, a detrimental event leading to progression to more severe hepatobiliary diseases.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.1
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• pp.101-107
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• 2002

We experienced two cases of Rotor syndrome in brothers who were a 13 year-old boy and an 11 year-old boy, respectively. They presented with icteric scleras for a few months. Their common laboratory characteristics were as follows: Direct bilirubin was more increased than indirect bilirubin, but aminotransferases were normal. Plasma indocyanine green (ICG) test revealed hepatic excretory defect: plasma ICG concentrations 15 minutes after intravenous injection were 80.45% and 78.28%, respectively. 99mTc-DISIDA Hepatobiliary scan showed that severely decreased hepatic extraction with mild cardiac blood pool, markedly delayed biliary excretion in both intra- & extra- hepatic bile ducts, delayed visualization of gall bladder, and markedly delayed intestinal biliary passage. Needle liver biopsy showed normal hepatic histology without pigmentation.

• Journal of Trauma and Injury
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• v.36 no.3
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• pp.310-314
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• 2023

Pancreatic trauma from a blunt injury is fairly uncommon in the pediatric population. Furthermore, such trauma with associated disruption of the pancreatic duct (PD) is even less prevalent and is associated with high morbidity and mortality. Pancreatic injuries in the pediatric population are often missed and hence require a thorough workup in children presenting with any form of abdominal injury. This case report describes a young boy who presented with abdominal pain and did not initially inform medical staff about any injury. For this reason, his initial provisional diagnosis was appendicitis, but he was later found to have transection of the pancreas with injury to the PD on imaging. The management of such injuries in pediatric patients often poses a challenge due to a lack of pediatric physicians trained to perform interventions such as endoscopic retrograde cholangiopancreatography. Furthermore, such interventions carry a higher risk when performed on children due to the smaller size of their pancreatic ducts. As a result, our patient had to be transferred to an adult center to undergo this procedure. Thus, maintaining a high degree of suspicion, along with a detailed history and examination, is crucial for the early diagnosis and management of pancreatic injuries.

• Biomolecules & Therapeutics
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• v.25 no.5
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• pp.545-552
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• 2017

Increasing concern is being given to the association between risk of cancer and exposure to low-dose bisphenol A (BPA), especially in young-aged population. In this study, we investigated the effects of repeated oral treatment of low to high dose BPA in juvenile Sprague-Dawley rats. Exposing juvenile rats to BPA (0, 0.5, 5, 50, and 250 mg/kg oral gavage) from post-natal day 9 for 90 days resulted in higher food intakes and increased body weights in biphasic dose-effect relationship. Male mammary glands were atrophied at high dose, which coincided with sexual pre-maturation of females. Notably, proliferative changes with altered cell foci and focal inflammation were observed around bile ducts in the liver of all BPA-dosed groups in males, which achieved statistical significance from 0.5 mg/kg (ANOVA, Dunnett's test, p<0.05). Toxicokinetic analysis revealed that systemic exposure to BPA was greater at early age (e.g., 210-fold in $C_{max}$, and 26-fold in AUC at 50 mg/kg in male on day 1 over day 90) and in females (e.g., 4-fold in $C_{max}$ and 1.6-fold in AUC at 50 mg/kg vs. male on day 1), which might have stemmed from either age- or gender-dependent differences in metabolic capacity. These results may serve as evidence for the association between risk of cancer and exposure to low-dose BPA, especially in young children, as well as for varying toxicity of xenobiotics in different age and gender groups.

• Journal of Yeungnam Medical Science
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• v.18 no.1
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• pp.51-58
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• 2001

Background: Cholestatic hepatitis is failure of bile to reach the duodenum with hepatocellular damage and no demonstable obstruction of the major bile ducts. The prognosis is usually good with recovery in less than 4 weeks after withdrawal of the offending drug. However, a prolonged course lasting over 3 months is possible and, in rare cases, progression to ductopenia with development of a vanishing bile duct syndrome occurs. A differential diagnosis with other causes of Chronic liver disease is needed. Materials and Methods: From January 1991 through January 2000, 14 patients diagnosed as cholestatic hepatitis by liver biopsy were included. The possible causative drug, clinical features, laboratory findings, and progression of cholestatic hepatitis were evaluated. The semiquantitative study of liver lesions was performed by two independent observers. Results: Causes of cholestatic hepatitis are 5 cases of oriental medicine, 3 cases of anti-tuberculosis medication, 1 case of ticlopidine and antibiotics and 4 cases of unknown causes. The clinical features of cholestatic hepatitis were jaundice, itching, urine color change, and general weakness. During 6 to 30 months, LFT of 5 patients showed prolonged elevation. Elevated total cholesterol ${\geq}$250 mg/dL in 6 patients, pheripheral blood eosinophilia in 5 patients, auto-antibody positive in 6 patients were observed respectively. The biopsies showed intralobular bilirubinostasis with a mixed portal inflammatory infiltration. Conclusion: In cholestatic hepatitis, durations of abnormal LFT are variable regardless of causative drugs. If cholestatic hepatitis progresses toward chronic course, viral hepatitis, primary biliary cirrhosis, and autoimmune hepatitis should be differentially diagnosed and sequential liver biopsies are needed.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.26 no.1
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• pp.76-83
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• 2022

Backgrounds/Aims: The aim of this study was to evaluate longitudinal changes of post-liver transplantation (LT) biliary anatomy and to assess the association of increased laboratory values after LT with the development of post-LT anastomotic biliary stricture (ABS). Methods: Adult deceased donor LT recipients from 2008 and 2019 were evaluated. ABS was defined after blinded review of endoscopic cholangiograms. Controls were patients who underwent LT for hepatocellular carcinoma who did not have any clinical or biochemical concerns for ABS. Results: Of 534 patients who underwent LT, 57 patients had ABS and 57 patients served as controls. On MRI, ABS patients had a narrower anastomosis (2.47 ± 1.32 mm vs. 3.38 ± 1.05 mm; p < 0.01) and wider bile duct at 1-cm proximal to the anastomosis (6.73 ± 2.45 mm vs. 5.66 ± 1.95 mm; p = 0.01) than controls. Association between labs at day 7 and ABS formation was as follows: aspartate aminotransferase hazard ratio (HR): 1.014; 95% confidence interval (CI): 1.008-1.020, p = 0.001; total bilirubin HR: 1.292, 95% CI: 1.100-1.517, p = 0.002; and conjugated bilirubin HR: 1.467, 95% CI: 1.216-1.768, p = 0.001. Corresponding analysis results for day 28 were alanine aminotransferase HR: 1.004, 95% CI: 1.002-1.006, p = 0.001; alkaline phosphatase HR: 1.005, 95% CI: 1.003-1.007, p = 0.001; total bilirubin HR: 1.233, 95% CI: 1.110-1.369, p = 0.001; and conjugated bilirubin HR: 1.272, 95% CI: 1.126-1.437, p = 0.001. Conclusions: Elevation of laboratory values early after LT is associated with ABS formation.

• Journal of the Korean Society of Radiology
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• v.17 no.3
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• pp.385-392
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• 2023

Caroli's disease is a fibrocystic liver disease. Autosomal recessive disorder is characterized by congenital multiple dilatation of the bile duct. Computerized tomography, magnetic resonance imaging, cholangiography and ultrasound are among the methods for diagnosing caroli disease. Computerized tomography is essential for detecting and distinguishing fibroplastic liver disease and is useful for determining intrahepatic bile duct dilatation. However, awareness of the possible side effects of using contrast mediums is necessary. A typical method of magnetic resonance cholangiography is used for magnetic resonance imaging. A non-invasive examination can reduce the pain of the patient, and the anatomical structure of the bile pancreatic duct and the presence or absence of lesions can be easily and quickly observed. Biliary contrast is an effective diagnostic method that can directly visualize various cystic dilatations throughout the enlarged bile duct. However, since this procedure is also an invasive procedure, it is recommended not for diagnosis but for treatment purposes. Ultrasonography can confirm similar findings to computerized tomography. The hepatic artery root is difficult to prove with conventional grayscale ultrasound. However, it is of clinical value in that it can not only describe dilated bile ducts with vascular roots in the tube but also easily identify color Doppler signals in the tube. With the development of video diagnostics, early diagnosis has become possible through computerized tomography, magnetic resonance imaging, cholangiography, and ultrasound. In order to further contribute to the development of video diagnostics so that long-term prognosis can be improved after treatment through early diagnosis, we examined what aspects of each test's caroli disease appear.