Publications by authors named "Mureo Kasahara"

Article Synopsis
  • * A 13-year-old girl with carbamoyl phosphate synthetase I (CPS1) deficiency underwent liver transplantation from her mother, facing significant challenges due to her severe scoliosis which led to portal vein complications necessitating multiple surgical interventions.
  • * The case highlights the importance of meticulous preoperative assessments and tailored surgical strategies for patients with structural abnormalities to prevent complications and ensure successful outcomes.
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Article Synopsis
  • Liver transplantation (LDLT) can effectively treat children with propionic acidemia (PA), showing a 100% survival rate in a study of 12 patients.
  • The study compared outcomes based on the timing of LDLT, dividing patients into groups based on whether the surgery occurred within a year of their first metabolic decompensation or later.
  • Early LDLT may prevent complications like cardiomyopathy and neurological issues, despite later patients showing some pre-existing conditions and post-surgery developmental challenges.
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Aim: To assess the preoperative disease characteristics and indications for living donor liver transplantation (LDLT), complications, patient survival, and prognosis after LDLT for fibropolycystic liver disease (FLD) in children.

Methods: We undertook a cross-sectional survey of patients who underwent LDLT for FLD between January 2002 and December 2020.

Results: A total of 35 patients (22 male and 13 female individuals) with FLD were included in this study, of whom 19 (54.

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  • Natural killer (NK) cells show potential as therapy for hard-to-treat cancers like hepatocellular carcinoma (HCC), but are often dysfunctional in HCC patients.
  • This study introduces LMNC-NK cells, derived from liver graft perfusate, which are more plentiful and exhibit enhanced cytotoxic properties compared to traditional NK cells from peripheral blood.
  • Through extensive gene analysis, the research highlights significant differences in gene expression that contribute to LMNC-NK cells’ superior effectiveness against HCC, suggesting their use as a promising treatment option.
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  • The text discusses the limited information on the use of daptomycin for treating persistent bacteremia in pediatric patients, especially those who have undergone solid organ transplantation.
  • It presents a case study of an eight-year-old girl whose persistent bacteremia, caused by Staphylococcus epidermidis, was successfully treated with daptomycin after adjusting the dosage due to her renal dysfunction.
  • A literature review revealed very few cases of daptomycin use in children with persistent bacteremia, emphasizing the need for more research on its clinical efficacy and pharmacokinetics in this special patient group.
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  • Collaboration among international pediatric oncology groups has led to improved survival rates for children undergoing liver transplantation (LT) for unresectable liver tumors, establishing LT as the standard care method.
  • Hepatoblastoma and hepatocellular carcinoma (HCC) are the primary types of liver cancers treated with LT, showing over 80% long-term survival rates for hepatoblastoma and better outcomes for HCC when compared to liver resection.
  • The development of living donor liver transplantation has enhanced waitlist outcomes, graft quality, and timing with chemotherapy, while ongoing research and a multidisciplinary approach are essential for advancing treatments for these rare pediatric malignancies.
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Article Synopsis
  • - A nationwide study in Japan analyzed pediatric deceased donor liver transplantation (pDDLT) outcomes, focusing on the pediatric prioritization system implemented in 2018 to improve organ allocation.
  • - Data from 1999 to 2021 revealed a 1-year graft survival rate of 86.6% and identified four key risk factors affecting survival, including donor characteristics and recipient conditions.
  • - The new prioritization system led to better allocation of pediatric donor livers to pediatric recipients, increasing the number of pDDLTs and significantly enhancing survival rates and overall transplant conditions.
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Background: Although the outcomes of living donor liver transplantation (LDLT) for pediatric acute liver failure (PALF) have improved, patient survival remains lower than in patients with chronic liver disease. We investigated whether the poor outcomes of LDLT for PALF persisted in the contemporary transplant era.

Methods: We analyzed 193 patients who underwent LDLT between December 2000 and December 2020.

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  • Scientists studied patients with a rare disease called methylmalonic acidemia (MMA) who had liver transplants to see how well they did over time.
  • They found that after 10 years, most of the patients were still alive and doing well, especially if they got their transplant within two years of getting sick.
  • However, some patients still had health issues like learning difficulties and kidney problems, but their MMA levels went down a lot after the transplant.
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Background: Validating the expanded criteria for living donor liver transplantation for hepatocellular carcinoma using national data is highly significant. The aim of this study was to evaluate the validity of the new Japanese criteria for living donor liver transplantation for hepatocellular carcinoma patients and identify factors associated with a poor prognosis using the Japanese national data set.

Methods: The study population comprised patients who underwent living donor liver transplantation for hepatocellular carcinoma at 37 centres in Japan between 2010 and 2018.

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Decreasing the graft size in living donor liver transplantation (LDLT) increases the risk of early allograft dysfunction. Graft-to-recipient weight ratio (GRWR) of 0.8 is considered the threshold.

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Background: Biliary atresia (BA) is an intractable disease of unknown cause that develops in the neonatal period. It causes jaundice and liver damage due to the destruction of extrahepatic biliary tracts,. We have found that heterozygous knockout mice of the SRY related HMG-box 17 (Sox17) gene, a master regulator of stem/progenitor cells in the gallbladder wall, exhibit a condition like BA.

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  • Congenital portosystemic shunts (CPSSs) vary in phenotype and pose challenges in determining the best treatment and prognosis, with a study analyzing 122 cases in Japan from 2000-2019 to gather insights.
  • The majority of patients (61.5%) had portal vein (PV) visibility on imaging; however, complications like hyperammonemia and liver masses were notably more prevalent in those without PV visualization, indicating a link between imaging results and health outcomes.
  • While most patients received treatment, including surgical options, those without PV visualization were more likely to experience complications, suggesting the need for early intervention even in asymptomatic cases.
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Xenobiotic metabolic reactions in the hepatocyte endoplasmic reticulum (ER) including UDP-glucuronosyltransferase and carboxylesterase play central roles in the detoxification of medical agents with small- and medium-sized molecules. Although the catalytic sites of these enzymes exist inside of ER, the molecular mechanism for membrane permeation in the ER remains enigmatic. Here, we investigated that organic anion transporter 2 (OAT2) regulates the detoxification reactions of xenobiotic agents including anti-cancer capecitabine and antiviral zidovudine, via the permeation process across the ER membrane in the liver.

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Liver transplantation is the only life-saving procedure for children with end-stage liver disease. The field is however heterogenic with various graft types, recipient age, weight, and underlying diseases. Despite recently improved overall outcomes and the expanded use of living donors, waiting list mortality remains unacceptable, particularly in small children and infants.

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Background: Methylmalonic acidemia (MMA) is an autosomal recessive disorder caused by defects in propionyl-CoA (P-CoA) catabolism; of note, liver neoplasms rarely occur as a long-term complication of the disorder. Herein, we report the case of a patient with MMA and hepatocellular carcinoma (HCC) who was successfully treated with a living-donor liver transplant (LDLT) following prior kidney transplantation.

Case Report: A 25-year-old male patient with MMA underwent LDLT with a left lobe graft because of metabolic instability and liver neoplasms.

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Article Synopsis
  • Liver transplantation (LT) is essential for treating children with severe liver disease, particularly using left lateral segment (LLS) grafts in split and living donor procedures.
  • Small infants face challenges with LLS grafts due to size mismatches, prompting investigations into reducing graft thickness to improve suitability.
  • Successful outcomes in a study from Tokyo show a 15-year graft survival rate of 89.9% for small infants receiving reduced-size LLS grafts, highlighting the importance of tailoring grafts to recipient size for better survival rates.
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Background: Progressive familial intrahepatic cholestasis type 2 (PFIC2) is an ultra-rare disease caused by mutations in the ABCB11 gene. This study aimed to understand the course of PFIC2 during the native liver period.

Methods: From November 2014 to October 2015, a survey to identify PFIC2 patients was conducted in 207 hospitals registered with the Japanese Society of Pediatric Gastroenterology, Hepatology, and Nutrition.

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Background: Living donor liver transplantation (LDLT) remains the only curative treatment for children with end-stage liver disease; however, complications of the procedure are associated with indications for early relaparotomy. Several risk factors associated with early relaparotomy after liver transplantation include pediatric end-stage liver disease (PELD) score, warm ischemia time (WIT), and cold ischemia time (CIT). Our study investigated the incidence and indications of early relaparotomy in postoperative pediatric LDLT recipients and compared the outcomes with patients who did not require relaparotomy.

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Introduction: The management of Budd-Chiari syndrome is determined on the basis of the severity of the disease. There are no standard guidelines regarding the management of Budd-Chiari syndrome in children, particularly in cases of liver transplantation. Therefore, we present a case of a pediatric patient with Budd-Chiari syndrome treated with liver transplantation.

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Background And Aim: Domino liver transplantation (DLT) utilizes otherwise discarded livers as donor grafts for another recipients. It is unclear whether DLT has less favorable outcomes compared to deceased donor liver transplantation (DDLT). We aimed to assess the outcomes of DLT compared to DDLT.

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Objective: To analyze 10,000 cases of living donor liver transplantation (LDLT) recipient data to elucidate outcomes with special reference to the graft-versus-recipient weight ratio (GRWR), based on the Japanese Liver Transplantation Society (JLTS) registry.

Background: The JLTS registry has been accurate and complete in characterizing and following trends in patient characteristics and survival of all patients with LDLT.

Methods: Between November 1989 and August 2021, 10,000 patients underwent LDLT in Japan.

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Background: Venous thromboembolic complications are an uncommon but significant cause of morbidity & mortality after live donor hepatectomy . The precise incidence of these events and the current practices of centers performing living donor liver transplantation worldwide are unknown.

Methods: An online survey was shared amongst living donor liver transplantation centers containing questions regarding center activity, center protocols for donor screening, peri-operative thromboembolic prophylaxis and an audit of -perioperative venous thromboembolic events after live donor hepatectomy in the previous five years (2016-2020).

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