• 대한이식학회지
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• 제31권4호
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• pp.193-199
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• 2017

Background: Although there more than 1,000 liver transplantations (LTs) are performed in Korea annually, their immense cost remains a great hurdle. Hence, in an attempt to reduce the medical costs of LT, a program was initiated at a public hospital affiliated with the Seoul National University Hospital. Methods: A total of 11 LTs have been successfully executed since the first LT performed at Seoul Metropolitan Government Seoul National University Boramae Medical Center in July 2011 through December 2014. Results: Nine patients (81.8%) were male and two (18.2%) were female. The mean age of patients was 53.4±11.4 years. Hepatitis B virus-related liver disease (n=6, 54.5%) was the most common causative disease, followed by alcoholic liver disease (ALD) (n=4, 36.4%). The actuarial 3-year survival rate was 90.9%. The median total medical cost of LTs was US $41,583 (calculated from operation to discharge), but only $11,860 was actually charged for patients with health insurance coverage. One female patient who had undergone deceased donor LT for alcoholic liver cirrhosis died during follow-up. This patient was non-compliant with the medical instructions after discharge, and finally expired due to septic shock at 10 months post-LT. Conclusions: In the public hospital, LT was successfully performed at a much lower cost. However, LT guidelines and peritransplant management protocols for patients with ALD must be established before escalating LT at public hospitals since ALD with poor compliance is one of the most common causes of complications at public hospitals.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제18권3호
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• pp.175-179
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• 2015

Purpose: Infants with Alagille syndrome (AGS) are occasionally misdiagnosed as biliary atresia and subsequently undergo Kasai operation. The purpose of this study was to investigate the outcome of patients with AGS who had previously received Kasai operation during infancy. Methods: This retrospective study was conducted at the Department of Pediatrics, Samsung Medical Center. We compared the prognosis and mortality between those who had undergone Kasai operation during infancy (Kasai group) and those who had not (non-Kasai group). Results: Among the 15 children with AGS, five had received Kasai operation, while 10 had not. All subjects in the Kasai group revealed neonatal cholestasis, while 70% of the non-Kasai group showed neonatal cholestasis. Liver transplantation was performed in 100% (5/5) among the Kasai group, and 20.0% (2/10) among the non-Kasai group (p=0.007). Mortality was observed in 60.0% (3/5) among the Kasai group, and 10.0% (1/10) among the non-Kasai group (p=0.077). Conclusion: Although overall mortality rate did not significantly differ between the two groups, the proportion of patients receiving liver transplantation was significantly higher in the non-Kasai group. The relatively worse outcome in AGS patients who had received Kasai operation may be due to the unfavorable influences of Kasai operation on the clinical course of AGS, or maybe due to neonatal cholestasis, irrespective of the Kasai operation.

• 대한자기공명의과학회:학술대회논문집
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• 대한자기공명의과학회 2001년도 제6차 학술대회 초록집
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• pp.135-135
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• 2001

Purpose： To know the differences of the proton MR spectroscopic features of the liver between th patients with graft-versus-host disease (GVHD) and without GVHD (non-GVHD) after to marrow transplantation (BMT), and to evaluate the possibility to discriminate GVHD fro non-GVHD by analysis of the in vivo proton MR spectra. Method： We evaluated the in vivo proton MR spectra from the livers of 37 patients wh underwent BMT. Our series included 14 cases with GVHD and 23 without GVHD in the liver. Nineteen men and 18 women were included in our series. All cases of GVHD and 2 o non-GVHD were confirmed by liver biopsy and remaining of non-GVHD by evaluation clinical follow up. Proton MR spectroscopy (1H-MRS) was performed at 1.5T GE Sign Horizon (GE Medical System, Milwaukee, USA) system using localized proton STEAM sequence and body coil in all cases with subjects were located in supine position. N respiratory interruption was required during the spectroscopic signal acquisition. Paramete using in MRS were： TR = over 3000ms, TE = 30ms, number of scans = 128, voxel size = ($2{\times}2{\times}2$)$cm^3$, and one NEX. We evaluated the spectra with an attention to the differences o patterns of the peaks between GVHD and non-GVHD groups. The ratio of peak area of peaks at 1.6-4.1ppm to lipid (0.9-1.6ppm) [P(1.6-4.1ppm)/P(0.9-1.6ppm)] was calculated in GVHD and non-GVHD group, and compared the results between these groups. We als evaluated the sensitivity and specificity for discriminating GVHD from non-GVHD by anal of 1H-MRS.

• Clinical and Experimental Pediatrics
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• 제56권5호
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• pp.224-226
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• 2013

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is characterized by a severe idiosyncratic reaction including rash and fever, often with associated hepatitis, arthralgias, lymph node enlargement, or hematologic abnormalities. The mortality rate is approximately 10%, primarily owing to liver failure with massive or multiple disseminated focal necrosis. Here, we report a case of a 14-year-old girl treated with vancomycin because of a wound infection by methicillin-resistant Staphylococcus aureus, who presented with non-specific symptoms, which progressed to acute liver failure, displaying the hallmarks of DRESS syndrome. With the presence of aggravated hepatic encephalopathy and azotemia, the patient was refractory to medical treatments, she received a living-donor liver transplantation, and a cure was achieved without any sign of recurrence. Vancomycin can be a cause of DRESS syndrome. A high index of suspicion and rapid diagnosis are necessary not to miss this potentially lethal disease.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제6권2호
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• pp.161-166
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• 2003

목 적: 상염색체 열성 유전 질환인 윌슨병에서 이형 접합 보인자인 부모를 공여자로 생체 부분 간이식을 시행 받은 윌슨병 환아의 치료 경험을 통해 이의 치료 효과를 알아보고자 본 연구를 실시하였다. 방 법: 1994년 12월부터 2002년 3월까지 서울아산병원에서 간이식을 시행받은 75명의 소아 중 윌슨병으로 간이식을 시행 받은 7명을 대상으로 수술 전후의 간 기능과 Kayser-Fleischer ring 소실 여부 및 구리 대사에 관련된 검사 소견 등을 중점으로 의무 기록을 통해 후향적으로 분석하였다. 결 과: 전체 7명의 환자 가운데 5명은 전격성 간염, 2명은 치료에 반응하지 않는 간경변으로 간이식을 받았으며, 공여자는 모두 혈연간 즉 이형 접합 보인자인 부모들이었다. 이식 후 추적 관찰 동안 환자의 생존율은 100%이었으며, 이는 같은 기간 동안 소아 간이식 전체 환자와 대사성 간 질환으로 간이식을 받은 환아의 각각의 5년 생존율 87%, 84%보다 유의하게 높았다(p<0.05). 간이식을 받은 모든 환자는 penicillamine과 구리 제한 식이를 시행하지 않고 이들의 AST치, 총 빌리루빈치 및 프로트롬빈 시간이 정상으로 회복되었고, 추적 관찰 기간 동안 윌슨병 재발의 소견은 없었다. 그리고 혈중 ceruloplasmin치와 혈중 구리 농도는 정상으로 회복되었으며, 24시간 소변 구리 배설양도수술 후 모든 환아에서 현저한 감소를 보였다. Kayser-Fleischer ring은 추적 관찰된 5명 중 4명에서 사라졌고, 추적 관찰이 3개월로 짧았던 한 명에서는 감소되었으나 남아 있었다. 결 론: 윌슨병이 내과적인 약물 치료에 반응하지 않는 간경변으로 진행하거나 전격성 간염으로 발현하여 간이식이 필요한 경우 윌슨병의 이형 접합 보인자인 부모를 이용한 혈연간 생체 부분 간이식은 효과적인 치료법임을 확인할 수 있었다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제27권3호
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• pp.158-167
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• 2024

Purpose: To evaluate the prevalence of vertebral fracture (VF) in children with chronic liver disease (CLD) with and without liver transplantation (LT) and to determine the associated factors. Methods: A cross-sectional study was conducted. Patients aged 3-21 years with CLD both before and after LT were enrolled in the study. Lateral thoracolumbar spine radiographs were obtained and assessed for VF using Mäkitie's method. Clinical and biochemical data were collected. Results: We enrolled 147 patients (80 females; median age 8.8 years [interquartile range 6.0-11.8]; 110 [74.8%] in the LT group and 37 [25.2%] in the non-LT group). VF was identified in 21 patients (14.3%): 17/110 (15.5%) in the LT group and 4/37 (10.8%) in the non-LT group (p=0.54). Back pain was noted in only three patients with VF. In the univariate analysis, a height z-score below -2.0 (p=0.010), pre-LT hepatopulmonary syndrome (p=0.014), elevated serum direct and total bilirubin levels (p=0.037 and p=0.049, respectively), and vitamin D deficiency at 1-year post-LT (p=0.048) were associated with VF in the LT group. In multivariate analysis, height z-score below -2.0 was the only significant associated factor (odds ratio, 5.94; 95% confidence interval, 1.49-23.76; p=0.012) for VF. All VFs in the non-LT group were reported in males. Conclusion: In children with CLD, VF is common before and after LT. Most patients with VF are asymptomatic. Screening for VF should be considered in patients with a height z-score below -2.0 after LT.

• IMMUNE NETWORK
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• 제15권3호
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• pp.125-134
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• 2015

Acute graft-versus-host-disease (GVHD) is characterized by selective damage to the liver, the skin, and the gastrointestinal tract. Following allogeneic hematopoietic stem cell transplantation, donor bone marrow (BM) cells repopulate the immune system of the recipient. We previously demonstrated that the acute intestinal GVHD (iGVHD) mortality rate was higher in MyD88-deficient BM recipients than that in the control BM recipients. In the present study, the role of MyD88 (expressed by donor BM) in the pathophysiology of hepatic GVHD (hGVHD) was examined. Unlike iGVHD, transplantation with MyD88-deficient T-cell depleted (TCD) BM attenuated hGVHD severity and was associated with low infiltration of T cells into the liver of the recipients. Moreover, GVHD hosts, transplanted with MyD88-deficient TCD BM, exhibited markedly reduced expansion of $CD11b^+Gr-1^+$ myeloidderived suppressor cells (MDSC) in the liver. Adoptive injection of the MDSC from wild type mice, but not MyD88-deficient mice, enhanced hepatic T cell infiltration in the MyD88-deficient TCD BM recipients. Pre-treatment of BM donors with LPS increased MDSC levels in the liver of allogeneic wild type BM recipients. In conclusion, hGVHD and iGVHD may occur through various mechanisms based on the presence of MyD88 in the non-T cell compartment of the allograft.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제25권5호
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• pp.353-375
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• 2022

No systematic review to date has examined histopathological parameters in relation to native liver survival in children who undergo the Kasai operation for biliary atresia (BA). A systematic review and meta-analysis is presented, comparing the frequency of native liver survival in peri-operative severe vs. non-severe liver fibrosis cases, in addition to other reported histopathology parameters. Records were sourced from MEDLINE, Embase, and CENTRAL databases. Studies followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines and compared native liver survival frequencies in pediatric patients with evidence of severe vs. non-severe liver fibrosis, bile duct proliferation, cholestasis, lobular inflammation, portal inflammation, and giant cell transformation on peri-operative biopsies. The primary outcome was the frequency of native liver survival. A random effects meta-analysis was used. Twenty-eight observational studies were included, 1,171 pediatric patients with BA of whom 631 survived with their native liver. Lower odds of native liver survival in the severe liver fibrosis vs. non-severe liver fibrosis groups were reported (odds ratio [OR], 0.16; 95% confidence interval [CI], 0.08-0.33; I²=46%). No difference in the odds of native liver survival in the severe bile duct destruction vs. non-severe bile duct destruction groups were reported (OR, 0.17; 95% CI, 0.00-63.63; I²=96%). Lower odds of native liver survival were documented in the severe cholestasis vs. non-severe cholestasis (OR, 0.10; 95% CI, 0.01-0.73; I²=80%) and severe lobular inflammation vs. non-severe lobular inflammation groups (OR, 0.02; 95% CI, 0.00-0.62; I²=69%). There was no difference in the odds of native liver survival in the severe portal inflammation vs. non-severe portal inflammation groups (OR, 0.03; 95% CI, 0.00-3.22; I²=86%) or between the severe giant cell transformation vs. non-severe giant cell transformation groups (OR, 0.15; 95% CI, 0.00-175.21; I²=94%). The meta-analysis loosely suggests that the presence of severe liver fibrosis, cholestasis, and lobular inflammation are associated with lower odds of native liver survival in pediatric patients after Kasai.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제10권2호
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• pp.166-172
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• 2007

목 적: 최근 HBsAb 및 HBcAb 양성인 공여자의 간이 식편을 이식 받은 수혜자에서 신생 B형 간염이 발생하는 것이 보고 되고 있으며, 저자들도 약 40%에서 신생B형 간염이 발생하는 것을 보고하였다. 한국인에서의 HBcAb 양성률은 50%가 넘는 것으로 보고하고 있는데, 이는 임상 간이식의 걸림돌이 될 수 있으며 저자들은 이를 예방하기 위한 일환으로써 본 연구를 시행하였다. 방 법: 1997년 11월부터 1998년 11월까지 12개월 동안 서울 아산병원에서 생체 간이식 공여자가 과거 B형간염과 C형 간염 감염의 증거가 없으면서 HBsAg 음성이면서 HBsAb 양성, HBcAb 양성인 성인 공여자 6명을 대상으로 하였다. 간이식 수술 시 동결 생검을 위하여 채취한 절편의 일부를 보관하여 실험에 사용하였다. 동결 절편 조직에서 DNA를 분리하여, HBV DNA의 표면 구역과 핵심 구역에 대한 시발체를 이용하여 이중 중합효소 연쇄 반응을 시행하여 검사를 시행하였다. 결 과: 공여자 6명의 조직에서 표면 구역이 모두 양성으로 관찰되었으며, 핵심 구역은 4명에서 양성으로 관찰되었다. 그 중 4명의 간을 이식받은 소아 수혜자는 모두 예방법을 시행하면서, 신생 B형 간염의 발생은 관찰되지 않고 있다. 결 론: 본 결과는 간이식 후 발생하는 신생 B형 간염의 원인으로 HBcAb 양성이 위험 인자임을 지지하고 있다. HBcAb 양성 공여자의 간이식편에서 핵심구역은 66%에서 양성으로 보여 이식 후 잠재 HBV 감염 혹은 신생 B형 간염의 발생을 막기 위해 예방적 치료가 필요할 것으로 사료된다.

• Korean Journal of Radiology
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• 제23권12호
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• pp.1269-1280
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• 2022

Objective: This study aimed to evaluate the usefulness of quantitative indices obtained from deep learning analysis of gadoxetic acid-enhanced hepatobiliary phase (HBP) MRI and their longitudinal changes in predicting decompensation and death in patients with advanced chronic liver disease (ACLD). Materials and Methods: We included patients who underwent baseline and 1-year follow-up MRI from a prospective cohort that underwent gadoxetic acid-enhanced MRI for hepatocellular carcinoma surveillance between November 2011 and August 2012 at a tertiary medical center. Baseline liver condition was categorized as non-ACLD, compensated ACLD, and decompensated ACLD. The liver-to-spleen signal intensity ratio (LS-SIR) and liver-to-spleen volume ratio (LS-VR) were automatically measured on the HBP images using a deep learning algorithm, and their percentage changes at the 1-year follow-up (ΔLS-SIR and ΔLS-VR) were calculated. The associations of the MRI indices with hepatic decompensation and a composite endpoint of liver-related death or transplantation were evaluated using a competing risk analysis with multivariable Fine and Gray regression models, including baseline parameters alone and both baseline and follow-up parameters. Results: Our study included 280 patients (153 male; mean age ± standard deviation, 57 ± 7.95 years) with non-ACLD, compensated ACLD, and decompensated ACLD in 32, 186, and 62 patients, respectively. Patients were followed for 11-117 months (median, 104 months). In patients with compensated ACLD, baseline LS-SIR (sub-distribution hazard ratio [sHR], 0.81; p = 0.034) and LS-VR (sHR, 0.71; p = 0.01) were independently associated with hepatic decompensation. The ΔLS-VR (sHR, 0.54; p = 0.002) was predictive of hepatic decompensation after adjusting for baseline variables. ΔLS-VR was an independent predictor of liver-related death or transplantation in patients with compensated ACLD (sHR, 0.46; p = 0.026) and decompensated ACLD (sHR, 0.61; p = 0.023). Conclusion: MRI indices automatically derived from the deep learning analysis of gadoxetic acid-enhanced HBP MRI can be used as prognostic markers in patients with ACLD.