• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제18권4호
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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.

• 성인간호학회지
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• 제27권2호
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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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• 제32권1호
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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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• 제24권6호
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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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• 제6권1호
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• pp.123-130
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• 2002

이식후 발생하는 림프증식성 질환(PTLD)은 양성 다크론성 B림프구 증식증으로부터 악성의 단크론 림프종에 이르기까끼 다양한 임상상을 보이는 질환이다. PTLD는 Epstein-Barr virus(EBV)와 과도한 면역억제와 밀접한 관계가 있다. 국내에서는 1997년 성인에서 신 이식 후 발생한 예가 처음 보고되었으며, 소아에서는 간 이식 후 발생한 예가 있을 뿐 신 이식 후 발생한 증례의 보고는 없어 이 보고가 국내 최초로 신이식 소아에서 발생한 PTLD 증례이다. 환자는 9세 남아로서 긴이식후 4개월후에 PTLD가 발생하였으며 주된 임상 양상은 발열, 림프절 종대와 혈변이었다. 림프절과 대장의 생검조직 에서 EBV in situ hybridization이 양성이고, 조직학적으로 B 세포계열의 PTLD에 합당한 소견이었다. 모든 증상은 면역억제제 감소와 ganciclovir 치료로 완전 관해되었다.

• Korean Journal of Radiology
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• 제23권12호
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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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• 제46권9호
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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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• 제48권6호
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• pp.660-664
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• 2005

저자들은 신생아 시기에 심부전 증세를 보인 간내 혈관내피종 환아에서 내과적 치료와 간동맥 색전술 후 호전을 보이지 않아 간좌엽 절제술을 시행하여 치료한 1례를 경험하였기에 보고하는 바이다.

• Journal of Korean Biological Nursing Science
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• 제15권4호
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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.

• 대한의용생체공학회:의공학회지
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• 제19권3호
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• pp.215-224
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• 1998

본 논문은 급성 간 부전 환자나 간이식 대기상태에 있는 환자에 이용하기 위한 체외 간 보조장치를 개발하기 위한 기초 실험에 관한 것이다. 밀도 33kg/m, 평균 직경 500um, 60-70%의 window를 가진 폴리우레탄 폼을 15% NCO-프리폴리머로 제조하였다. 폴리우레탄 폼에 접종된 간세포는 3일 후에 거의 모두 100-150um 크기의 구상체를 형성하였다. BSA 코팅을 한 폴리우레탄 폼은 BSA로 코팅하지 않은 폴리우레탄 폼보다 간세포 구상체 형성 시간이 더 짧았다. 폴리우레탄 폼에 형성된 간세포 구상체의 간 기능을 증명하기 위하여 암모니아 제거율과 요소 및 알부민 분비율을 측정하였다. 폴리우레탄 폼은 간세포 구상체 배양에 적합하며, 폴리우레탄 폼 내에서의 간세포 구상체 배양은 체외 간 보조장치로의 개발 가능성이 높다.