• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.18 no.4
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• pp.268-275
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• 2015

Purpose: Primary sclerosing cholangitis (PSC) is a rare condition that can be associated with inflammatory bowel disease (IBD). The aim of this study was to evaluate PSC and its association with IBD in children. Methods: We retrospectively enrolled 13 pediatric patients (<18 years) with PSC treated at Asan Medical Center between June 1989 and December 2013. Clinical findings and long-term outcomes were investigated. During the same period, the incidence of PSC among IBD patients was evaluated among 600 Crohn disease (CD) and 210 ulcerative colitis (UC) patients. Results: All 13 study patients diagnosed with PSC also presented with IBD. Eleven boys and two girls with a median age of 15.0 years old (9.0-17.8 years) were included. The cumulative incidence of PSC for UC was 5.7% (12 of 210) and 0.2% for CD (1 of 600), respectively. PSC occurred during follow-up for IBD for five patients (38.5%) whereas, IBD developed during follow-up for PSC for two patients (15.4%), and was diagnosed during the initial work-up for PSC for 6 patients (46.2%). For the 77.3 month median follow-up period, 9/13 patients (69.2%), neither the clinical symptoms nor blood test results worsened. Two cases (15.4%) developed liver cirrhosis and underwent liver transplantation. Among 13 PSC patients with IBD, two (15.4%) developed colorectal cancer, and no one developed cholangiocarcinoma. Conclusion: All patients with PSC in this study had associated IBD. The incidence of PSC was not rare compared to reports in adults. PSC should be considered during the management of IBD and vice versa in children.

• Korean Journal of Adult Nursing
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• v.27 no.2
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• pp.170-179
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• 2015

Purpose: To examine the relationships between self-care, social support, and biological markers in liver transplant recipients. Methods: The participants included 118 liver transplant recipients who visited outpatient clinic at Y University Hospital in Seoul from April to May, 2013. Questionnaires consisted of self-care and social support scales. The biological markers were collected by reviewing electronic medical records. Data were analyzed with descriptive statistics, t-test, ANOVA with Scheff$\acute{e}$ post-hoc test, and Pearson's correlation. Results: The self-care score was significantly higher in a patient group within 6 months post-transplant when compared to a patient group post-transplant 3 to 5 years (F=3.10, p=.018). The self-care showed positive correlation with social support with statistical significance (r=.36, p<.001). Conclusion: As the self-care in liver transplant recipients had a positive correlation with social support from family and healthcare providers, the development of comprehensive long-term nursing intervention systems including counseling, education, and support in consideration of progress of time period after transplantation is necessary to enhance self-care behaviors among this population.

• Toxicological Research
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• v.32 no.1
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• pp.35-46
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• 2016

No-flow ischemia occurs during cardiac arrest, hemorrhagic shock, liver resection and transplantation. Recovery of blood flow and normal physiological pH, however, irreversibly injures the liver and other tissues. Although the liver has the powerful machinery for mitochondrial quality control, a process called mitophagy, mitochondrial dysfunction and subsequent cell death occur after reperfusion. Growing evidence indicates that reperfusion impairs mitophagy, leading to mitochondrial dysfunction, defective oxidative phosphorylation, accumulation of toxic metabolites, energy loss and ultimately cell death. The importance of acetylation/deacetylation cycle in the mitochondria and mitophagy has recently gained attention. Emerging data suggest that sirtuins, enzymes deacetylating a variety of target proteins in cellular metabolism, survival and longevity, may also act as an autophagy modulator. This review highlights recent advances of our understanding of a mechanistic correlation between sirtuin 1, mitophagy and ischemic liver injury.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.6
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• pp.528-534
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• 2021

Purpose: Living-donor liver transplant emerged as an alternative treatment for end stage liver disease due to the lack of cadaveric organs availability that met the demand. In Portugal, pediatric living-donor liver transplant (P-LDLT) was initiated in 2001 in Portugal in order to compensate for the scarcity of cadaveric organs for such cases. The aim of this study was to retrospectively analyze the morbi-mortality of the 28 donors included in P-LDLT program performed at Coimbra's Pediatric Hospital (CHUC), a Portuguese reference center. Methods: We retrospectively collected pertinent donor data and stratified complications according to Clavien's scoring system. Results: In total, 28.6% (n=8) of the donors had surgical complications. According to Clavien-Dindo's classification, two donors had major complications (Clavien grade ≥3), four donors had grade 2 complications, and two donors had grade 1 complications. There were no P-LDLT-related mortalities in the present case series. The most common verified complications were biliary tract injuries and superficial incisional infections, which are consistent with the complications reported in worldwide series. Conclusion: These patients from CHUC shows that donor hepatectomy in P-LDLT is a safe procedure, with low morbidity and without mortality.

• Childhood Kidney Diseases
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• v.6 no.1
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• pp.123-130
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• 2002

Posttransplant lymphoproliferative disease (PTLD) represents a diverse lymphoproliferative disorder ranging front nonspecific reactive hyperplasia to malignant immunoblastic sarcoma developed in a setting of immunosuppression following organ or cellular transplantation. It is often associated with Epstein-Barr virus (EBV) infection and high dose immunosuppression. PTLD after renal transplantation was reported at first in adult in Korea in 1997. In children there have been several cases of PTLD after liver transplantation but PTLD after renal transplantation has not been reported. This is a case report of PTLD developed 4 months after renal transplantation in a 9-year-old boy. The major clinical manifestations were fever, multiple lymph nodes enlargement and blood-tinged stool. EBV was detected by in-situ hybridization in the enlarged cervical lymph node and the colonic tissue. Histological examination revealed B-cell lineage. Use of ganciclovir and reduction of the immunosuppression level resulted in complete remission of PTLD. This is the first pediatric case report of PTLD following renal transplantation in Korea. (J Korean Soc Pediatr Nephrol 2002 ; 6 : 123-30)

• Korean Journal of Radiology
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• v.23 no.12
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• pp.1260-1268
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• 2022

Objective: To propose standardized MRI-proton density fat fraction (PDFF) cutoff values for diagnosing hepatic steatosis, evaluated using contemporary PDFF measuring methods in a large population of healthy adults, using histologic fat fraction (HFF) as the reference standard. Materials and Methods: A retrospective search of electronic medical records between 2015 and 2018 identified 1063 adult donor candidates for liver transplantation who had undergone liver MRI and liver biopsy within a 7-day interval. Patients with a history of liver disease or significant alcohol consumption were excluded. Chemical shift imaging-based MRI (CS-MRI) PDFF and high-speed T2-corrected multi-echo MR spectroscopy (HISTO-MRS) PDFF data were obtained. By temporal splitting, the total population was divided into development and validation sets. Receiver operating characteristic (ROC) analysis was performed to evaluate the diagnostic performance of the MRI-PDFF method. Two cutoff values with sensitivity > 90% and specificity > 90% were selected to rule-out and rule-in, respectively, hepatic steatosis with reference to HFF ≥ 5% in the development set. The diagnostic performance was assessed using the validation set. Results: Of 921 final participants (624 male; mean age ± standard deviation, 31.5 ± 9.0 years), the development and validation sets comprised 497 and 424 patients, respectively. In the development set, the areas under the ROC curve for diagnosing hepatic steatosis were 0.920 for CS-MRI-PDFF and 0.915 for HISTO-MRS-PDFF. For ruling-out hepatic steatosis, the CS-MRI-PDFF cutoff was 2.3% (sensitivity, 92.4%; specificity, 63.0%) and the HISTO-MRI-PDFF cutoff was 2.6% (sensitivity, 88.8%; specificity, 70.1%). For ruling-in hepatic steatosis, the CS-MRI-PDFF cutoff was 3.5% (sensitivity, 73.5%; specificity, 88.6%) and the HISTO-MRI-PDFF cutoff was 4.0% (sensitivity, 74.7%; specificity, 90.6%). Conclusion: In a large population of healthy adults, our study suggests diagnostic thresholds for ruling-out and ruling-in hepatic steatosis defined as HFF ≥ 5% by contemporary PDFF measurement methods.

• Molecules and Cells
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• v.46 no.9
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• pp.527-534
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• 2023

Liver ischemia-reperfusion injury (IRI) is the main cause of organ dysfunction and failure after liver surgeries including organ transplantation. The mechanism of liver IRI is complex and numerous signals are involved but cellular metabolic disturbances, oxidative stress, and inflammation are considered the major contributors to liver IRI. In addition, the activation of inflammatory signals exacerbates liver IRI by recruiting macrophages, dendritic cells, and neutrophils, and activating NK cells, NKT cells, and cytotoxic T cells. Technological advances enable us to understand the role of specific immune cells during liver IRI. Accordingly, therapeutic strategies to prevent or treat liver IRI have been proposed but no definitive and effective therapies exist yet. This review summarizes the current update on the immune cell functions and discusses therapeutic potentials in liver IRI. A better understanding of this complex and highly dynamic process may allow for the development of innovative therapeutic approaches and optimize patient outcomes.

• Clinical and Experimental Pediatrics
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• v.48 no.6
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• pp.660-664
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• 2005

Infantile hemangioendothelioma(IHE) of the liver is the most common vascular tumor in infants before the age of 6 months. It is a histologically benign tumor with potentially life-threatening complications. The clinical manifestations are variable, ranging from asymptomatic forms to intractable high-output heart failure. In addition, abdominal mass, intraperitoneal hemorrhage due to rupture of mass, respiratory distress, hematologic abnormalities and jaundice can occur. Diagnostic work-up is through doppler ultrasound sonography, computed tomography scan, magnetic resonance imaging and angiography. Treatment consists of medical treatment, interventional therapy, surgical resection and liver transplantation. We experienced symptomatic IHE in a premature neonate who presented with high output heart failure and respiratory distress. Initial medical treatment and steroid therapy failed to improve his condition. Coil embolization of left hepatic artery resulted in improvement of respiratory symptoms. However, a left lobectomy was performed because the mass size was not decreased with development of collateral vessels. The infant was well, after a successful discharge from the hospital.

• Journal of Korean Biological Nursing Science
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• v.15 no.4
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• pp.193-201
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• 2013

Purpose: The purpose of the study was to evaluate the effect of the immediate postoperative nutritional status and calorie adequacy on clinical outcomes in liver transplant recipients. Methods: A total number of 99 patients who received liver transplants were recruited from a tertiary university hospital. Demography, subjective global assessment, clinical outcomes and calorie adequacy were evaluated through personal interviews, electronic medical records and dietary records. Anthropometric measures, body mass index and percent of ideal body weight were also obtained. Results: At admission to the Surgical Intensive Care Unit (SICU), the triceps skinfold thickness and mid-arm muscle circumference were significantly lower in the malnourished group than in the well-nourished group (p<.05, respectively). In the clinical outcomes, transfusion of red blood cells, mechanically ventilated hours, length of stay in the Intensive Care Unit (ICU), length of stay in the hospital, and prothrombin time were significantly higher in the malnourished group than in the well-nourished group (p<.05, respectively). The mechanically ventilated hour was significantly higher in the group with less than 50% of their required energy intake (p<.05). Conclusion: Therefore, it is important to assess the nutritional status of immediate postoperative patients. Furthermore, studies on nutritional interventions are urgently needed to provide adequate nutritional care for patients in ICUs.

• Journal of Biomedical Engineering Research
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• v.19 no.3
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• pp.215-224
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• 1998

This paper is fundamental study to develope the extracorporeal liver support system for patient with fulminant hepatic failure(FHF) or being expected for orthotopic liver transplantation. The polyurethane foam, which is composed of the density of 33kg/m3, the average pore diameter of 500${\mu}{\textrm}{m}$, the closed window of 60-70%, was manufactured with the prepolymer of 15% NCO-, Hepatocytes were inoculated to form spheroids in polyurethane foam. The time of spheroid formation in BSA(Bovine Serum Albumin) coated polyurethane foam was shorter than that in raw polyurethane foam. To verify the function of hepatocyte spheroids, we measured ammonia removal rate, urea and albumin secretion rate. Polyurethane foam was suitable for culture of hepatocyte spheroids. And culture of hepatocyte spheroids in polyurethane foam has high possibility in using as an extracorporeal liver support system.