• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제20권4호
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• pp.259-262
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• 2017

Mitochondria play essential role in eukaryotic cells including in the oxidative phosphorylation and generation of adenosine triphosphate via the electron-transport chain. Therefore, defects in mitochondrial DNA (mtDNA) can result in mitochondrial dysfunction which leads to various mitochondrial disorders that may present with various neurologic and non-neurologic manifestations. Mutations in the nuclear gene polymerase gamma (POLG) are associated with mtDNA depletions, and Alpers-Huttenlocher syndrome is one of the most severe manifestations of POLG mutation characterized by the clinical triad of intractable seizures, psychomotor regression, and liver failure. The hepatic manifestation usually occurs late in the disease's course, but in some references, hepatitis was reportedly the first manifestation. Liver transplantation was considered contraindicated in Alpers-Huttenlocher syndrome due to its poor prognosis. We acknowledged a patient with the first manifestation of the disease being hepatic failure who eventually underwent liver transplantation, and whose neurological outcome improved after cocktail therapy.

• 한국간담췌외과학회지
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• 제28권1호
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• pp.34-41
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• 2024

Backgrounds/Aims: Liver transplantation (LT) provides a favorable outcome for patients with hepatocellular carcinoma (HCC) and was launched in Vietnam in 2004. In this study, we evaluated the short-term and long-term outcomes of LT and its risk factors. Methods: This retrospective study analyzed HCC patients who underwent LT at Viet Duc University hospital, Vietnam, from 01/2012-03/2022. The following data were gathered: demographics, virus infection, tumor characteristics, alpha-fetoprotein (AFP) level, Child-Pugh and MELD scores, selection criteria, type of LT, complications, 30-day mortality, and disease-free and overall survival (DFS and OS). Results: Fifty four patients were included, the mean age was 55.39 ± 8.46 years. Nearly 90% had hepatitis B virus-related HCC. The median (interquartile range) AFP level was 16.2 (88.7) ng/mL. The average MELD score was 10.57 ± 5.95; the rate of Child-Pugh A and B were 70.4% and 18.5%, respectively. Nearly 40% of the patients were within Milan criteria, brain-dead donor was 83.3%. Hepatic and portal vein thrombosis occurred in 0% and 1.9%, respectively; hepatic artery thrombosis 1.9%, biliary leakage 5.6%, and postoperative hemorrhage 3.7%. Ninety-day mortality was 5.6%. Five-year DFS and OS were 79.3% and 81.4%, respectively. MELD score and ChildPugh score were predictive factors for DFS and OS (p < 0.05). In multivariate analysis, Child-Pugh score was the only significant factor (p < 0.05). Conclusions: In Vietnam, LT is an effective therapy for HCC with an acceptable complication rate, mortality rate, and good survival outcomes, and should be further encouraged.

• Korean Journal of Radiology
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• 제22권12호
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• pp.1985-1995
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• 2021

Objective: Although the liver-to-spleen volume ratio (LSVR) based on CT reflects portal hypertension, its prognostic role in cirrhotic patients has not been proven. We evaluated the utility of LSVR, automatically measured from CT images using a deep learning algorithm, as a predictor of hepatic decompensation and transplantation-free survival in patients with hepatitis B viral (HBV)-compensated cirrhosis. Materials and Methods: A deep learning algorithm was used to measure the LSVR in a cohort of 1027 consecutive patients (mean age, 50.5 years; 675 male and 352 female) with HBV-compensated cirrhosis who underwent liver CT (2007-2010). Associations of LSVR with hepatic decompensation and transplantation-free survival were evaluated using multivariable Cox proportional hazards and competing risk analyses, accounting for either the Child-Pugh score (CPS) or Model for End Stage Liver Disease (MELD) score and other variables. The risk of the liver-related events was estimated using Kaplan-Meier analysis and the Aalen-Johansen estimator. Results: After adjustment for either CPS or MELD and other variables, LSVR was identified as a significant independent predictor of hepatic decompensation (hazard ratio for LSVR increase by 1, 0.71 and 0.68 for CPS and MELD models, respectively; p < 0.001) and transplantation-free survival (hazard ratio for LSVR increase by 1, 0.8 and 0.77, respectively; p < 0.001). Patients with an LSVR of < 2.9 (n = 381) had significantly higher 3-year risks of hepatic decompensation (16.7% vs. 2.5%, p < 0.001) and liver-related death or transplantation (10.0% vs. 1.1%, p < 0.001) than those with an LSVR ≥ 2.9 (n = 646). When patients were stratified according to CPS (Child-Pugh A vs. B-C) and MELD (< 10 vs. ≥ 10), an LSVR of < 2.9 was still associated with a higher risk of liver-related events than an LSVR of ≥ 2.9 for all Child-Pugh (p ≤ 0.045) and MELD (p ≤ 0.009) stratifications. Conclusion: The LSVR measured on CT can predict hepatic decompensation and transplantation-free survival in patients with HBV-compensated cirrhosis.

• 한국멀티미디어학회논문지
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• 제13권3호
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• pp.430-437
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• 2010

간이식 수술을 함에 있어서 간 내부의 혈관의 형태를 알고 시작하는 것이 수술의 성공률을 매우 높일 수 있다. 본 논문은 조영제를 투여한 정상 환자의 복부 MDCT를 이용하여 얻어진 영상을 다른 여러 장기부분은 제거하고 간 영상만을 추출한 후 간 내의 혈관들의 기본형태를 파악하여 몇몇 구조단위들을 만들고 Morphological filtering을 이용하여 주요 혈관인 좌, 우, 중간정맥을 찾아낸다. 중간정맥을 기준으로 간 실질을 절단하여 절단된 부분의 크기를 예측하고 수술전에 전체 상황을 파악하기 위한 연구이다. 간의 추출 방법은 명암값의 범위와 분포 샘플링 과정에 의한 명암값 분포비율을 가지고 배경과 근육층을 제거하였다. 간의 대략적인 위치 정보와 몸통의 위치정보를 이용하여 단위 매쉬영상과 일치되는 영상을 찾은 후 결과 영상을 조합하고 8방향 연결성을 이용하여 확장하고 화소간의 채우기 과정을 거쳐 최종적인 간영상을 추출하였다. 추출된 간 영상에서 간 영역의 특징적인 명암값과 다양한 구조단위를 가지고 Morpological Filtering을 수행 한 후 나타난 결과들을 조합하여 만들어진 영상에서 각 슬라이스 별로 크기순으로 큰 부분들을 남겨두어 굵은 혈관만을 추출하였다. 추출된 영상들을 3D로 구성 시 자연스럽게 보여지도록 인터폴레이션을 수행한 후 3D Reconstruction 을 수행하여 3D 형태의 간 혈관을 보고 중간 정맥을 파악하여 간 실질의 절단 위치를 예측하게 된다. 절단되어진 간 실질의 크기를 확인하고 계산에 의하여 수술 성공 가능성을 파악할 수 있다.

• 대한기계학회:학술대회논문집
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• 대한기계학회 2008년도 추계학술대회A
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• pp.1419-1426
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• 2008

Fulminant hepatic failure is a clinical syndrome associated with a high mortality rate. Orthotopic liver transplantation is the only clinically proven effective treatment for patients with end-stage liver disease who do not respond to medical management. A major limitation of this treatment modality is the scarcity of donor organs available, resulting in patients dying while waiting for a donor liver. An extracorporeal bioartificial liver (BAL) device containing viable hepatocytes has the potential to provide temporary hepatic support to liver failure patients, serving as a bridge to transplantation while awaiting a suitable donor. In some patients, providing temporary hepatic support may be sufficient to allow adequate regeneration of the host liver, thereby eliminating the need for a liver transplant. Although the BAL device is a promising technology for the treatment of liver failure, there are several technical challenges that must be overcome in order to develop systems with sufficient processing capacity and of manageable size. In this overview, the authors describe the critical issues involved in developing a BAL device. They also discuss their experiences in hepatocyte culture optimization within the context of a microchannel flat-plate BAL device.

• 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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• 제11권sup2호
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• pp.50-58
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• 2008

급성 간부전은 여러 분야의 전문가 및 이식 센터의 협조가 필요한 질환으로 급성 간염을 보이는 모든 환자에서 급성 간부전으로 진행할 가능성에 대해 고려하여야 한다. 소아에서 급성 간부전은 원인 미상인 경우가 가장 흔하며, 영아기에는 대사성 간질환도 염두에 두어야 한다. 급성 간부전의 예후는 간이식이 도입된 이후 획기적으로 향상되었으나, 간성 뇌증, 혈액응고 장애, 감염, 신부전 등 주요 합병증에 대한 치료는 여전히 예후를 좌우하는 중요한 부분이며, 여기에 더하여 간이식의 여부 및 시기에 대한 결정이 소아과 의사의 중요한 역할 중 하나가 되었다. 원인이 밝혀진 경우에는 예후 예측에 도움이 되고, 원인에 대한 치료를 시도해볼 수 있으나 소아 환자는 원인 미상인 경우가 많아 예후 예측이 어렵고, 주 사망 원인인 간성 뇌증에 대한 평가가 어려워 특히 세심한 모니터가 필요하다.

• Korean Journal of Radiology
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• 제22권12호
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• pp.2052-2061
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• 2021

Objective: The role of preoperative overt hepatic encephalopathy (OHE) in the neurophysiological mechanism of cognitive improvement after liver transplantation (LT) remains elusive. This study aimed to explore changes in sub-regional thalamic functional connectivity (FC) after LT and their relationship with neuropsychological improvement using resting-state functional MRI (rs-fMRI) data in cirrhotic patients with and without a history of OHE. Materials and Methods: A total of 51 cirrhotic patients, divided into the OHE group (n = 21) and no-OHE group (n = 30), and 30 healthy controls were enrolled in this prospective study. Each patient underwent rs-fMRI before and 1 month after LT. Using 16 bilateral thalamic subregions as seeds, we conducted a seed-to-voxel FC analysis to compare the thalamic FC alterations before and after LT between the OHE and no-OHE groups, as well as differences in FC between the two groups of cirrhotic patients and the control group. Correction for multiple comparisons was conducted using the false discovery rate (p < 0.05). Results: We found abnormally increased FC between the thalamic sub-region and prefrontal cortex, as well as an abnormally decreased FC between the bilateral thalamus in both OHE and no-OHE cirrhotic patients before LT, which returned to normal levels after LT. Compared with the no-OHE group, the OHE group exhibited more extensive abnormalities prior to LT, and the increased FC between the right thalamic subregions and right inferior parietal lobe was markedly reduced to normal levels after LT. Conclusion: The renormalization of FC in the cortico-thalamic loop might be a neuro-substrate for the recovery of cognitive function after LT in cirrhotic patients. In addition, hyperconnectivity between thalamic subregions and the inferior parietal lobe might be an important feature of OHE. Changes in FC in the thalamus might be used as potential biomarkers for recovery of cognitive function after LT in cirrhotic patients.

• 한국간담췌외과학회지
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• 제26권1호
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• pp.31-39
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• 2022

In patients with neuroendocrine tumors with liver metastases (NETLMs), complete resection of both the primary and liver metastases is a potentially curative option. When complete resection is not possible, debulking of the tumour burden has been proposed to prolong survival. The objective of this systematic review was to evaluate the effect of curative surgery (R0-R1) and debulking surgery (R2) on overall survival (OS) in NETLMs. For the subgroup of R2 resections, outcomes were compared by the degree of hepatic debulking (≥ 90% or ≥ 70%). A systematic review of the literature was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) guidelines using PubMed, Medline, CINAHL, Cochrane, and Embase databases. Hazard ratios (HRs) were estimated for each study and pooled using a random-effects inverse-variance meta-analysis model. Of 538 articles retrieved, 11 studies (1,729 patients) reported comparisons between curative and debulking surgeries. After pooling these studies, OS was found to be significantly shorter in debulking resections, with an HR of 3.49 (95% confidence interval, 2.70-4.51; p < 0.001). Five studies (654 patients) compared outcomes between ≥ 90% and ≥ 70% hepatic debulking approaches. Whilst these studies reported a tendency for OS and progression-free survival to be shorter in those with a lower degree of debulking, they did not report sufficient data for this to be assessed in a formal meta-analysis. In patients with NETLM, OS following surgical resection is the best to achieve R0-R1 resection. There is also evidence for a progressive reduction in survival benefit with lesser debulking of tumour load.

• 한국간담췌외과학회지
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• 제26권4호
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• pp.298-307
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• 2022

Portal cavernoma cholangiopathy is defined as an obstruction of the biliary system due to distended veins surrounding bile ducts that mainly occur in patients with extrahepatic portal venous obstruction. The periductal venous plexuses encircling the ducts can cause morphological changes which may or may not become symptomatic. Currently, non-invasive techniques such as ultrasonography, computed tomography, magnetic resonance cholangiopancreatography, and dynamic contrast enhanced magnetic resonance images are being used to diagnose this disorder. Only a few patients who have symptoms of biliary obstruction require drainage which might be accomplished using endoscopic stenting, decompression of the portal venous system usually via a lienorenal shunt, a difficult direct hepaticojejunostomy, and rarely a liver transplant.