• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제15권2호
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• pp.111-116
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• 2012

Toxic hepatitis is a rare but devastating disease in children. Herbs are widely used in oriental medicine to treat various symptoms in Korea, however, several herbs have been reported to induce liver injury. We report a case of toxic hepatitis induced by Hovenia dulcis in a 3-year-old boy. He complained of nausea, abdominal discomfort, and jaundice. The patient had consumed water boiled with hovenia dulcis for about 1 year prior to presentation. A diagnosis of toxic hepatitis was made based on his history, laboratory data, viral markers, ultrasonography, and biopsied liver tissue. We administered supportive management for acute fulminant hepatitis but his symptoms and liver function progressed. He was transferred to another hospital for further evaluation and consideration for liver transplantation. Because acute liver failure due to herbs or dietary supplement taken for a long time is often fetal, it is important to make early diagnosis and stop taking the drug as soon as drug induced liver injury is suspected.

• Biomolecules & Therapeutics
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• 제30권5호
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• pp.391-398
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• 2022

Polyploidization is a process by which cells are induced to possess more than two sets of chromosomes. Although polyploidization is not frequent in mammals, it is closely associated with development and differentiation of specific tissues and organs. The liver is one of the mammalian organs that displays ploidy dynamics in physiological homeostasis during its development. The ratio of polyploid hepatocytes increases significantly in response to hepatic injury from aging, viral infection, iron overload, surgical resection, or metabolic overload, such as that from non-alcoholic fatty liver diseases (NAFLDs). One of the unique features of NAFLD is the marked heterogeneity of hepatocyte nuclear size, which is strongly associated with an adverse liver-related outcome, such as hepatocellular carcinoma, liver transplantation, and liver-related death. Thus, hepatic polyploidization has been suggested as a potential driver in the progression of NAFLDs that are involved in the control of the multiple pathogenicity of the diseases. However, the importance of polyploidy in diverse pathophysiological contexts remains elusive. Recently, several studies reported successful improvement of symptoms of NAFLDs by reducing pathological polyploidy or by controlling cell cycle progression in animal models, suggesting that better understanding the mechanisms of pathological hepatic polyploidy may provide insights into the treatment of hepatic disorders.

• 한국간담췌외과학회지
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• 제27권3호
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• pp.313-316
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• 2023

Attenuated portal vein (PV) flow is challenging in pediatric liver transplantation (LT) because it is unsuitable for classic end-to-end jump graft reconstruction from a small superior mesenteric vein (SMV). We thus introduce a novel technique of an end-to-side jump graft from SMV during pediatric LT using an adult partial liver graft. We successfully performed two cases of end-to-side retropancreatic jump graft using an iliac vein graft for PV reconstruction. One patient was a 2-year-old boy with hepatoblastoma and a Yerdel grade 3 PV thrombosis who underwent split LT. Another patient was an 8-month-old girl who had biliary atresia and PV hypoplasia with stenosis on the confluence level of the SMV; she underwent retransplantation because of graft failure related to PV thrombosis. After native PV was resected at the SMV confluence level, an end-to-side reconstruction was done from the proximal SMV to an interposition iliac vein. The interposition vein graft through posterior to the pancreas was obliquely anastomosed to the graft PV. There was no PV related complication during the follow-up period. Using a jump vascular graft in an end-to-side manner to connect the small native SMV and the large graft PV is a feasible treatment option in pediatric recipients with inadequate portal flow due to thrombosis or hypoplasia of the PV.

• Journal of Chest Surgery
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• 제49권3호
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• pp.157-164
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• 2016

Background: Heart-lung transplantation (HLT) has provided hope to patients with end-stage lung disease and irreversible heart dysfunction. We reviewed the clinical outcomes of 10 patients who underwent heart-lung transplantation at Asan Medical Center. Methods: Between July 2010 and August 2014, a total of 11 patients underwent HLT at Asan Medical Center. After excluding one patient who underwent concomitant liver transplantation, 10 patients were enrolled in our study. We reviewed the demographics of the donors and the recipients' baseline information, survival rate, cause of death, and postoperative complications. All patients underwent follow-up, with a mean duration of $26.1{\pm}16.7months$. Results: Early death occurred in two patients (20%) due to septic shock. Late death occurred in three patients (38%) due to bronchiolitis obliterans (n=2) and septic shock (n=1), although these patients survived for 22, 28, and 42 months, respectively. The actuarial survival rates at one year, two years, and three years after HLT were 80%, 67%, and 53%, respectively. Conclusion: HLT is a procedure that is rarely performed in Korea, even in medical centers with large heart and lung transplant programs. In order to achieve acceptable clinical outcomes, it is critical to carefully choose the donor and the recipient and to be certain that all aspects of the transplant procedure are planned in advance with the greatest care.

• Journal of Dental Anesthesia and Pain Medicine
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• 제22권6호
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• pp.451-455
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• 2022

Glycogen storage disease (GSD) is a group of inherited disorders, which result in the deficiency of enzymes involved in glycogen metabolism, leading to an accumulation of glycogen in various organs. Deficiency of amylo-1-6-glicosidase (debranching enzyme) causes glycogen storage disease type III (GSD III). The main problems that anesthesiologists face in patients with GSD III include hypoglycemia, muscle weakness, delayed awakening due to abnormal liver function, possible difficulty in airway, and cardiomyopathy. In the face of these difficulties, airway preparation and appropriate glucose monitoring and support during the fasting period are important. The doses of the drugs to be used should be calculated considering the increased volume of distribution and decreased metabolic activity of the liver. We present the case of a child with GSD IIIa who underwent dental prosedation under general anesthesia. She was also being prepared for liver transplantation. This case was additionally complicated by the patient's serious allergic reaction to eggs and milk.

• Asian Pacific Journal of Cancer Prevention
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• 제17권2호
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• pp.473-478
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• 2016

Hepatocellular cancer is a very common cause of cancer related deaths worldwide. Only 30-40% of patients present with early-stage disease open to curative treatments, such as resection or transplantation, while others can only undergo local therapies or palliative care. Various trans-arterial approaches have been used for treatment of hepatocellular carcinoma in patients who need a down-staging to liver transplantation, and who are not candidates for transplantation or radiofrequency ablation. Transarterial chemoembolization (TACE), transarterial embolization (TAE), drug-eluting beads, and radioembolization have been used for locoregional control, and have been shown to prolong the overall survival when compared with supportive care. In this review, we discuss patient selection, pre- and post-procedure imaging, techniques, safety, and clinical outcomes related to these therapies. Newer advances with future directions in various fields related to trans-arterial therapies are also discussed.

• 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.