Explanipedia

Optimization of Oxygen Supply Conditions for Hypothermic Oxygenated Machine Perfusion Open

Tomoki Abe, M. Furukawa, Naoto Matsuno, Mureo Kasahara, Hiromichi OBARA · 2025

Hypothermic oxygenated machine perfusion (MP) contributes to the reduction of delayed graft function and ischemic reperfusion injury in donated after circulatory death donors. In this study, we focused on perfusate temperature, oxygen cons…

Featured Cover Open

Mureo Kasahara, Jean de Ville de Goyet, João Seda Neto · 2025

Global Trends in Perioperative Imaging Workups and Records for Hepatobiliary and Pancreatic Surgery: Reports From 103 Facilities Worldwide Open

Yoshihiro Sakamoto, Goro Honda, Taizo Hibi, Tomoyuki Yoshida, Shohei Kudo , et al. · 2025

Background Perioperative imaging workups and records, including preoperative schema‐drawing, three‐dimensional tomographic image simulation, intraoperative ultrasonography, fluorescence imaging in hepatectomy, and postoperative record with…

Intrauterine high-dose intravenous immunoglobulin therapy during pregnancy for women with a history of pregnancy ending in documented neonatal haemochromatosis (NH001): study protocol Open

A. Sasaki, Akihiro Yachie, Koichi Mizuta, Hironori Takahashi, Noriki Okada , et al. · 2025

Introduction Neonatal haemochromatosis, considered to be a gestational alloimmune liver disease (NH-GALD), is a rare but serious disease that results in fulminant hepatic failure. The recurrence rate of NH-GALD in a subsequent infant of a …

Phenotypic Divergence of JAG1 ‐ and NOTCH2 ‐Associated Alagille Syndrome & Disease‐Specific NOTCH2 Variant Classification Guidelines Open

Shannon M. Vandriel, Liting Li, Huiyu She, Jian‐She Wang, Kathleen M. Loomes , et al. · 2025

Background & Aims Alagille syndrome (ALGS) is a rare, autosomal dominant disorder with high phenotypic heterogeneity. Disease‐causing variants are primarily identified in Jagged1 ( JAG1 ), with fewer reported in NOTCH2 . JAG1 variants caus…

Chronic Pain Following Pediatric Liver Transplantation: A Single‐Center Prospective Observational Study Open

Kaoru Tsuboi, Hajime Uchida, Norihiko Tsuboi, Seisuke Sakamoto, Mureo Kasahara , et al. · 2025

Background Chronic postsurgical pain (CPSP) has been reported in approximately 20% of children across all surgery types 12 months after surgery and is associated with poorer functional outcomes and health‐related quality of life. However, …

Portal Vein Obstruction in Pediatric Liver Transplant Patients: An Evaluation of Self‐Reported Management Practices Open

Lydia Sieben, Bader A Alfares, Ruben H. de Kleine, Barbara E. Wildhaber, Thomas Casswall , et al. · 2025

Background and Aims Portal vein obstruction (PVO) is a known complication after pediatric liver transplantation (pLT). Effective management strategies are crucial in improving patient outcomes. This study investigated the various practice …

Nationwide survey of patients with acute liver failure and late‐onset hepatic failure in Japan seen between 2016 and 2021 Open

Nobuaki Nakayama, Masamitsu Nakao, Yoshihito Uchida, Akio Ido, Yasuhiro Takikawa , et al. · 2025

Aim A nationwide survey was conducted to clarify the recent status of patients with acute liver failure (ALF) and late‐onset fulminant hepatitis (LOHF) in Japan. Methods Two‐step surveys were performed annually targeting 782 hospitals, foc…

Recurrence of Primary Sclerosing Cholangitis After Pediatric Liver Transplantation: A Single‐Center, Retrospective Study in Japan Open

Athaya Vorasittha, Seisuke Sakamoto, Yusuke Yanagi, Kazuki Degawa, Hirotaka Kato , et al. · 2025

Aim Liver transplantation (LT) is a well‐accepted treatment for primary sclerosing cholangitis (PSC) with generally good outcomes, although recurrent PSC (rPSC) poses significant challenges. This study aimed to describe patient characteris…

Progress of pediatric liver transplantation: In Japan and beyond Open

Mureo Kasahara, Seisuke Sakamoto · 2025

Organ transplantation, particularly pediatric liver transplantation (LT), has transformed medical practice over the past six decades, providing life-saving interventions for children with end-stage liver disease. This review demonstrated t…

First-in-human clinical study of an embryonic stem cell product for urea cycle disorders Open

Akihiro Umezawa, Akinari Fukuda, Reiko Horikawa, Hajime Uchida, Shin Enosawa , et al. · 2025

Background This study assesses the safety and efficacy of hepatocyte-like cell (HLC) infusion therapy derived from human embryonic stem cells as bridging therapy for neonatal-onset urea cycle disorders (UCD). The research includes both pre…

Successful living donor kidney transplantation in a 14-year-old boy with Fontan circulation Open

Satoshi Okada, Koichi Kamei, Kentaro Nishi, Masao Ogura, Hiroshi Ono , et al. · 2025

We report a successful living donor kidney transplantation (KT) in a 14-year-old boy with Fontan circulation. He was diagnosed with complete transposition of the great arteries (type 3) at birth. After Blalock–Taussig shunt placement, he d…

Neonatal acute liver failure cases with echovirus 11 infections, Japan, August to November 2024 Open

Tatsuki Ikuse, Toshihiro Matsui, Kensuke Shoji, Naoko Kono, Masaki Yamada , et al. · 2025

In 2022–23, several European countries reported paediatric acute liver failure (ALF) with enterovirus infection. In August–November 2024, three neonatal cases of ALF with echovirus 11 (E11) were reported in Tokyo, Japan. All neonates devel…

Successful pregnancy in a patient with OTCD using advanced technologies and liver transplant Open

Sachi Koinuma, A. Sasaki, Kei Murayama, Kou Sueoka, Atsuko Murashima , et al. · 2025

Liver transplantation in late‐onset ornithine transcarbamylase deficiency with acute liver failure: A case report Open

Hibiki Doi, Keisuke Yoshii, Kenichi Kashimada, Hajime Uchida, Akinari Fukuda , et al. · 2025

Assessing Native Liver Post-Kasai Portoenterostomy for Biliary Atresia Through Stool Proteome Analysis Open

Eiichiro Watanabe, Takeshi Saito, Masahito Yoshihara, Ryo Konno, Jun Fujishiro , et al. · 2025

This study highlights distinct differences in stool protein profiles following KPE, particularly in patients with poor clinical outcomes. This suggests that stool proteome analysis has the potential to provide insights into the native live…

Innovative Artificial Intelligence System in the Children's Hospital in Japan Open

Akihiro Umezawa, Kazuaki Nakamura, Mureo Kasahara, Takashi Igarashi · 2025

The evolution of innovative artificial intelligence (AI) systems in pediatric hospitals in Japan promises benefits for patients and healthcare providers. We actively contribute to advancements in groundbreaking medical treatments by levera…

Living‐Donor Liver Transplantation in a Carbamoyl Phosphate Synthetase I Deficiency Recipient With Scoliosis: A Case Report Open

Aoi Ninomiya, Hajime Uchida, Seisuke Sakamoto, Tasuku Kodama, Ryuji Komine , et al. · 2024

Background Neuromuscular scoliosis is associated with cerebral palsy caused by metabolic diseases. Patients with scoliosis require meticulous consideration in abdominal surgery, as scoliosis can reduce the abdominal cavity volume, compress…

The Impact of Early Indication of Living Donor Liver Transplantation on the Outcomes of Patients With Propionic Acidemia: A Single‐Center Experience Open

Toshimasa Nakao, Seisuke Sakamoto, Seiichi Shimizu, Akinari Fukuda, Hajime Uchida , et al. · 2024

Background Liver transplantation has been indicated for propionic acidemia (PA) patients with frequent metabolic decompensation and performed as an enzyme replacement therapy. We retrospectively evaluated the outcomes of patients with PA a…

Optimal transplant strategy of pediatric liver transplantation for fibropolycystic liver disease: Multicenter retrospective study in Japan Open

Hajime Uchida, Ayano Inui, Tatsuya Okamoto, Toshihiro Yasui, Masaki Honda , et al. · 2024

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…

Targeted immune cell therapy for hepatocellular carcinoma using expanded liver mononuclear cell-derived natural killer cells Open

Xin Hu, Yifang Shui, Seiichi Shimizu, Seisuke Sakamoto, Mureo Kasahara , et al. · 2024

Natural killer (NK) cells are a promising cellular therapy for T cell-refractory cancers but are frequently deficient or dysfunctional in patients with hepatocellular carcinoma (HCC). In the present study, we explored a novel therapy for H…

Improved survival of pediatric deceased donor liver transplantation recipients after introduction of the pediatric prioritization system: Analysis of data from a Japanese national survey Open

Yusuke Takemura, Masahiro Shinoda, Mureo Kasahara, Seisuke Sakamoto, Etsuro Hatano , et al. · 2024

Background In Japan, there has never been a national analysis of pediatric deceased donor liver transplantation (pDDLT) based on donor and recipient factors. We constructed a Japanese nationwide database and assessed outcomes of pDDLT focu…

Long‐Term Outcomes of Living Donor Liver Transplantation for Methylmalonic Acidemia Open

Robert C. Minnee, Seisuke Sakamoto, Akinari Fukuda, Hajime Uchida, Kazuya Hirukawa , et al. · 2024

Background Despite early diagnosis and medical interventions, patients with methylmalonic acidemia (MMA) suffer from multi‐organ damage and recurrent metabolic decompensations. Methods We conducted the largest retrospective multi‐center co…

Japanese living donor liver transplantation criteria for hepatocellular carcinoma: nationwide cohort study Open

Masahiro Ohira, Gaku Aoki, Yasushi Orihashi, Kenichi Yoshimura, Takeo Toshima , et al. · 2024

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 …

Impact of gallbladder hypoplasia on hilar hepatic ducts in biliary atresia Open

Nanae Miyazaki, Shohei Takami, Mami Uemura, Hironobu Oiki, Masataka Takahashi , et al. · 2024

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 kn…

Endoplasmic reticulum transporter OAT2 regulates drug metabolism and interaction Open

Hiroshi Arakawa, Naoki Ishida, Tomoki Nakatsuji, Natsumi Matsumoto, Rikako Imamura , et al. · 2024

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. Altho…

Efficiency of machine perfusion in pediatric liver transplantation Open

Alessandro Parente, Mureo Kasahara, Vincent E. de Meijer, Koji Hashimoto, Andrea Schlegel · 2024

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…

Excess mortality in COVID-19-affected solid organ transplant recipients across the pandemic Open

Shigeyoshi Yamanaga, Keita Shimata, Satoko Ohfuji, Mikiko Yoshikawa, Yoichiro Natori , et al. · 2024

Clinical symptoms, biochemistry, and liver histology during the native liver period of progressive familial intrahepatic cholestasis type 2 Open

Hiroki Kondou, Satoshi Nakano, Tadahaya Mizuno, Kazuhiko Bessho, Yasuhiro Hasegawa , et al. · 2024

Acute brainstem dysfunction in neonatal hyperammonemia with ornithine transcarbamylase deficiency: A case report Open

Fang Wang, Yuichi Abe, Mureo Kasahara, Reiko Horikawa, Itaru Hayakawa · 2024

Ornithine transcarbamylase deficiency (OTCD) is the most common urea cycle disorder that causes episodic hyperammonemia. Many newborns with OTCD present with nausea, delirium, lethargy, and seizure during a metabolic attack; however, brain…

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