• Advances in pediatric surgery
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• v.14 no.2
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• pp.125-133
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• 2008

To evaluate the long-term prognosis of biliary atresia after Kasai operation, a total of 14 patients (of the 41 patients operated upon from 1982 to 1997), who had been followed up for more than 10 years, were included in this retrospective study. Eleven out of 14 patients survived with their native livers, and their data analyzed for age at operation, clearing time of jaundice, histological outcome, postoperative complications, effectiveness after the application of an intussusception anti-reflex valve, and quality of life. Average age at surgery was 62.8 days. Serum bilirubin was normalized within three months in all patients. Six among the eleven long-term survivors had ascending cholangitis as one of the postoperative complications. The application of an intussusception anti-reflux valve did not show any statistical significance in long-term survival. Most of long-term survivors appeared to enjoy good quality of life. Kasai operation might not be the definitive treatment for biliary atresia; however, Kasai operation made it possible to achieve long-term survival for patients with biliary atresia when the patients were detected and treated as early as possible.

• Advances in pediatric surgery
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• v.6 no.1
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• pp.19-26
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• 2000

This paper reports our 9-year experience treating 34 infants with biliary atresia utilizing a new non-invasive diagnostic method, ultrasonographic "triangular cord"(TC) sign. The TC sign is present when there is visualization of a triangular or a band-like echogenicity just cranial to the portal vein. The ultrasonographic TC sign appears to be a simple, non-invasive, time-saving and useful tool in the diagnosis of biliary atresia. Sensitivity is 84 %. Active bile excretion was restored in 90 % of the patients who were treated between 31-60days, 78 % of those between 61-90 days, and 33 % of those being 91days or older. The incidence of postoperative cholangitis was 36 %. Construction of an antireflux valve in the Roux-en-Y loop did not affect the incidence of postoperative cholangitis(p=0.18). Among 34 infants with biliary atresia, 23(68 %) are alive for 2-102 months after operation, and 12 are alive for more than 5 years. Five-year estimate survival by Kaplan-Meier method was 66 %.

• Advances in pediatric surgery
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• v.3 no.1
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• pp.6-14
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• 1997

To differentiate biliary atresia from intraheaptic cholestasis, Tc-99m DlSIDA hepatobiliary scintigraphies and percutaneous needle biopsies of 60 consecutive infants were evaluated retrospectively. Twenty three patients had biliary atresia and 37 patients intraheaptic cholestasis(neonatal hepatitis 34, TPN induced jaundice 2 and Dubin-Johnson syndrome 1). All sixty patients underwent Tc-99m DlSIDA hepatobiliary scintigraphy with phenobarbital pretreatment. Of 23 patients with biliary atresia, 22 were correctly interpreted showing 96% sensitivity while of 37 patients with intraheaptic cholestasis, only 12 had intestinal excretion of radionuclide showing 32% specificity. Among the forty needle biopsies, 17 of biliary atresia and 23 of intrahepatic cholestasis, 37 were correctly interpreted as either having biliary atresia or intrahepatic cholestasis. The overall diagnostic accuracy was 93%. Of 3 misdiagnosed cases, the histologic findings of two patients with biliary atresia(aged 43 days and 54 days at the first needle biopsy) were essentially the same as those of neonatal hepatitis. Follow-up biopsies, however, showed findings consistent with biliary atresia. The third one(VLBW premie with history of 8 weeks of TPN) showed mild ductal proliferation and portal fibrosis. This was interpreted as suspicious for biliary atresia. Jaundice resolved gradually. In summary, patients who have intestinal excretion of radionuclide on Tc-99m DlSIDA hepatobiliary scintigraphy, biliary atresia can be ruled out. But the patients who do not have intestinal excretion of radionuclide should have further investigation by needle biopsy. Judicious use of Tc-99m DISIDA hepatobiliary scintigraphy and percutaneous needle biopsy can give a diagnostic accuracy of 95% or more in cases of infantile cholestasis.

• Journal of the Korean Society of Radiology
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• v.85 no.3
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• pp.581-595
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• 2024

Purpose The present study aimed to investigate the frequency and extent of compensatory common bile duct (CBD) dilatation after cholecystectomy, assess the time between cholecystectomy and CBD dilatation, and identify potentially useful CT findings suggestive of obstructive CBD dilatation. Materials and Methods This retrospective study included 121 patients without biliary obstruction who underwent multiple CT scans before and after cholecystectomy at a single center between 2009 and 2011. The maximum short-axis diameters of the CBD and intrahepatic duct (IHD) were measured on each CT scan. In addition, the clinical and CT findings of 11 patients who were initially excluded from the study because of CBD stones or periampullary tumors were examined to identify distinguishing features between obstructive and non-obstructive CBD dilatation after cholecystectomy. Results The mean (standard deviation) short-axis maximum CBD diameter of 121 patients was 5.6 (± 1.9) mm in the axial plane before cholecystectomy but increased to 7.9 (± 2.6) mm after cholecystectomy (p < 0.001). Of the 106 patients with a pre-cholecystectomy axial CBD diameter of < 8 mm, 39 (36.8%) showed CBD dilatation of ≥ 8 mm after cholecystectomy. Six of the 17 patients with long-term (> 2 years) serial follow-up CT scans (35.3%) eventually showed a significant (> 1.5-fold) increase in the axial CBD diameter, all within two years after cholecystectomy. Of the 121 patients without obstruction or related symptoms, only one patient (0.1%) showed IHD dilatation > 3 mm after cholecystectomy. In contrast, all 11 patients with CBD obstruction had abdominal pain and abnormal laboratory indices, and 81.8% (9/11) had significant dilatation of the IHD and CBD. Conclusion Compensatory non-obstructive CBD dilatation commonly occurs after cholecystectomy to a similar extent as obstructive dilatation. However, the presence of relevant symptoms, significant IHD dilatation, or further CBD dilatation 2-3 years after cholecystectomy should raise suspicion of CBD obstruction.

• Toxicological Research
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• v.17 no.3
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• pp.187-194
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• 2001

The oxidative stress causes the cell damage and death and thereby, stimulates membrane lipid peroxidation. In this study, the correlation between the lipid peroxidation product and the parameter of liver fibrosis (cirrhosis) was investigated in cholestasis induced rats. The Sprague-Dawley rats were divided into 3 groups (sham: sham operation, BDL/S-I and BDL/S-II : bile duct ligation/scission) and were observed for 2 or 4 weeks. After observation period, the organs were weighed and the ratio of organ weight/body weight was calculated. Sera and liver tissue were used for the measurement of malondealdehyde (MDA), parameter of clinical biochemistry, total collagen content and the staining. The ratio of organ weight/body weight in BDL/S-I and BDL/S-II was significantly increased compared to sham operated group. Serological parameters (Alanine transaminase, Aspartate transaminase, Alkaline phosphatase and Total bilirubin) in BDL/S-I and BDL/S-II group were significantly higher than those in sham operated group. Concentration of MDA in BDL/S-I (261%) and BDL/S-II(790%) was significantly increased compared to MDA in sham operated group. And the content of hydroxyproline (hyp) in BDL/S-I and BDL/S-II group was significantly increased 2~4 times than in sham operated group. The good correlations between hyp in liver tissue and MDA in sera of sham operated group and all operated group were found (r=0.825). The significantly higher value of MDA, hyp and serological parameters in BDL/S-I and BDL/S-II group suggests the stimulation of lipid peroxidation and chronic liver damage. Especially the activation of lipid peroxidation and the stimulation of liver fibrosis was stronger in BDL/S-II group than in BDL/S-I group. The stronger fibrosis, portal-portal septum formation, the more massive bile duct proliferation in portal triads and stroma, and hepatocytes swelling were observed in liver tissue of and BDL/S-II group compared to BDL/S-I group. Conclusively, a good correlation between MDA as a lipid peroxidation marker and hyp as a liver fibrotic parameter could be connected with the process of liver fibrosis. Moreover, cholestasis condition may cause jaundice, activation of lipid peroxidation, and collagen accumulation in liver. Additionally, optimal observation period of bile duct obstruction for the screening of antioxidant and antifibrotic effect in rats would be four weeks.

• Radiation Oncology Journal
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• v.13 no.4
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• pp.339-348
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• 1995

Purpose : Since 1983, authors have conducted a study to evaluate the effect of external radiation therapy and to determine affected factors in management of the patients with malignant obstructive jaundice due to porta hepatis metastasis from stomach cancer. Materials and Methods : Thirty two patients with malignant obstructive jaundice due to porta hepatis metastasis from gastric cancer were presented. We have analysed 23 patients who were treated with external radiation therapy of more than 3000cGy. The radiation dose, disease extent at developement of jaundice, total bilirubin levels before radiation therapy, differentiation of histology, combind treatment, intent of primary surgery, initial stage of gastric cancer were analyzed to determine affected factors in radiation therapy. External radiation therapy was delivered with a daily dose of 180-300cGy, 5 times a week fractionation using 4 MeV linear accelerator. The radiation field included the porta hepatis with tumor mass by the abdominal ultrasonography or CT scan. In twenty three patients received more than 3000cGy, total irradiation dose was ranged from 3000cGy to 5480cGy, median 3770cGy. Among 23 patients, 13 patients were delivered more than equivalant dose of TDF 65(4140cGy/23fx). Results : Among 23 patients, complete, partial and no response were observed in 13, 5, 5 patients, respectively. The median survival for all patients was 5 momths. The significant prolongation of median survival was observed in complete responders(11 months) as compared to partial and no responders(5 months, 5 months respectively) Out of 13 patients with complete response, 6 patients lived more than a year Among 13 patients receiving more than 4140cGy equivalent dose, complete, partial and no response were observed in 10, 2 and 1 patients, respectively. The median survival for all these patients was 9.5 months. The median survival for complete responders(10/13) was 11.5 months. Among 10 patients receiving less than 4140cGy equivalent dose, complete, partial and no response were observed in 3, 3, 4 patients, respetively. The median survival for all these patients was 4.3 months Therefore, the radiation dose affected the results of treatment. For the complete response with prolongation of survival duration, at least 4140cGy equivalant dose should be delivered to porta hepatis. In evaluation of the disease extent, 7 patients of 13 complete responders showed localized disease in porta hepatis or peripancreatic area, but all patients with partial and no response showed wide extensive disease or persistant disease of primary gastric cancer. Therefore. the patients with the localized disease were the higher probability of complete response and long term survival. This study suggested that the radiation dose and the disease extent at developement of jaundice affected in radiation therapy for malignant obstructive jaundice. There were no serious complications related to external radiation therapy. Conclusion : External radiation therapy only could achieve the palliative effect in the patients with malignant obstructive jaundice due to porta hepatis metastasis from stomach cancer. This study suggested that the prolongation of survival duration could be achived in complete responders and radiation dose, extent of disease affected the results of treatment of malignant obstructive jaundice.

• Advances in pediatric surgery
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• v.4 no.2
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• pp.125-130
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• 1998

To assess the clinical and nutritional status of long-term survivors of biliary atresia, history taking, medical record review, physical examination (height, weight, MAC, TSF), blood tests (LFT, prothrombin time, platelet count, prealbumin, calcium) and liver needle biopsy were performed in 12 patients in whom Kasai procedure were performed more than 10 years ago at Department of Pediatric Surgery in Seoul National University Hospital. None were below the 5th percentile in height and weight. TSF was above the 75th percentile in all patients and showed good subcutaneous fat deposition. MAC was above the 5th percentile in all patients. Serum prealbumin level was abnormal in 2 patients with abnormal liver function and revealed visceral protein malnutrition. Serum calcium level was decreased below normal range in 4 patients with abnormal liver function. One patient had mild ascites. Five patients had abnormal liver function and 7 patients showed clinical manifestation of portal hypertension. Liver needle biopsy was performed in 5 patients and no cirrhotic change was observed. Although some patients who have survived for more than 10 years after Kasai procedure developed protein malnutrition and vitamin deficiencies, growth and development and nutritional status were generally satisfactory. Five patients(42%) showed normal liver function and no portal hypertension. In conclusion, Kasai procedure is satisfactory as a primary treatment in biliary atresia but significant portion of long-term survivors had abnormal liver function and portal hypertension. Continuous and careful follow-up is necessary to determine when liver transplantation may be indicated.

• Advances in pediatric surgery
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• v.12 no.2
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• pp.202-212
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• 2006

The prognostic factors for extrahepatic biliary atresia (EHBA) after Kasai portoenterostomy include the patient's age at portoenterostomy (age), size of bile duct in theporta hepatis (size), clearance of jaundice after operation (clearance) and the surgeon's experience. The aim of this study is to examine the most significant prognostic factor of EHBA after Kasai portoenterostomy. This retrospective study was done in 51 cases of EHBA that received Kasai portoenterostomy by one pediatric surgeon. For the statistical analysis, Kaplan-Meier method, Logrank test and Cox regression test were used. A p value of less than 0.05 was considered to be significant. Fifteen patients were regarded as dead in this study, including nine cases of liver transplantation. There was no significant difference of survival to age. The age is also not a significant risk factor for survival in this study (Cox Regression test; p = 0.63). There was no significant difference in survival in relation to the size of bile duct. However, bile duct size was a significant risk factor for survival (Cox Regression test; p = 0.002). There was a significant difference in relation to survival and clearance (Kaplan-Meier method; p = 0.02). The clearing was also a significant risk factor for survival (Cox Regression test; p = 0.001). The clearance of jaundice is the most significant prognostic factor of EHBA after Kasai portoenterostomy.

• Journal of the Korean Society of Radiology
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• v.83 no.5
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• pp.966-978
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• 2022

Jaundice in children have various etiologies. Among them, physiological jaundice is a very common disease observed in more than half of full-term neonates. When jaundice persists or develops after 2 weeks of age, the total/direct bilirubin is measured in consideration of the possibility of cholestasis. In case of cholestasis, imaging studies differentiate biliary atresia and other disorders of the extrahepatic bile ducts. In this review, we briefly presented the major differential diseases of cholestasis in children and introduced diagnostic imaging techniques, including normal findings.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.12 no.1
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• pp.16-22
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• 2009

Purpose: To update the epidemiologic information of hepatobiliary diseases in pediatric inpatients using cross-sectional survey data throughout the Republic of Korea. Methods: Nationwide cross-sectional survey was obtained from the 85 residency training hospitals in Korea to gather the final diagnosis on discharge. The surveyed periods were from 2004 to 2006. All the reports regarding the diagnosis were based on ICD-10 system. In this study, we focused on hepatobiliary diseases. Results: A total of 826,896 cases with discharge data were collected, of which 4,151 (5.0%) hepatobiliary cases were identified; 2,385 cases (57.4%) of hepatobiliary disease were hepatitis, which was the most common hepatobiliary disease. Other diseases included congenital hepatobiliary diseases (524 cases [12.6%]) and biliary diseases (315 cases [7.6%]). The prevalence of hepatobiliary disease according to age differed. Biliary atresia was the most common hepatobiliary disease in the neonatal period, whereas the prevalence of hepatitis increased in adolescents. The total number of hepatobiliary operations was 416 cases. With the comparison of annual data, there was no definite difference in the total number of hepatobiliary cases. The average duration of hospital stay appeared to decrease gradually. Conclusion: In this study, we have summarized the recent epidemiology of hepatobiliary disorders in Korean children based on discharge data. Hepatobiliary disorders in pediatric inpatient units consisted of diverse disorders with a low prevalence, so multi-center approaches should be considered to enhance the clinical and public health outcomes. To improve this nationwide survey, a new data collecting system should be developed.