Surgical Strategies for Hepatoblastoma Involving Four Liver Sectors, Look Back to Look Forward: Lessons From a Single-Center Experience.

Background: Liver transplantation (LT) has been recommended for hepatoblastoma (HB) involving all four liver sectors at diagnosis (PRETEXT IV). However, recent studies question the universal indication for primary LT in PRETEXT IV HB.

Methods: We reviewed 32 PRETEXT IV HB patients treated at our center from February 2007 to July 2024.

Living Donor Liver Transplantation for Symptomatic Liver Involvement in Infant With Hereditary Hemorrhagic Telangiectasia.

Aim: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder that can cause vascular malformations in multiple organs, including the liver. Although liver involvement typically remains asymptomatic until adulthood, severe cases can progress to high-output cardiac failure (HOCF). Although liver transplantation (LT) is a well-established curative option for adult patients with HHT, pediatric data are limited.

Chronic Pain Following Pediatric Liver Transplantation: A Single-Center Prospective Observational Study.

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, data on pediatric liver transplantation recipients are lacking.

Methods: Among all patients who visited our organ transplantation center outpatient clinic between April 2023 and January 2024, those who met the following criteria were included: (1) had undergone liver transplantation under 18 years of age, (2) older than 6 years on the day of their visit, and (3) more than three months had passed since their last liver transplantation.

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

We report 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 developed acute kidney injury, which recovered to stage 4 chronic kidney disease.

Diagnoses and Timelines in Pediatric Liver Transplant Recipients With Suspected Acute Infections.

Infections are a leading cause of morbidity after pediatric liver transplantation (LT), yet data beyond 3 months post-LT are limited. Among 315 LT recipients, 135 (42.9%) accounted for 342 unscheduled visits for suspected acute infections.

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

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 patterns related to PVO management to clarify the degree of consensus on the diverse facets of care involved in addressing PVO after pLT.

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

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 characteristics and identify potential risk factors of rPSC in pediatric LT recipients.

Methods: This retrospective study analyzed 13 pediatric patients who underwent LT for PSC at a single center.

Progress of pediatric liver transplantation: In Japan and beyond.

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 the historical milestones of pediatric organ transplantation, emphasizing Japan's contributions, mainly through the National Center for Child Health and Development. While early transplantation efforts in the 1950s and 1960s faced significant challenges, breakthroughs in preservation methods, immunosuppressive therapies, surgical techniques, and innovations such as living donor LT in Asia have greatly improved success rates.

Feasibility of peritoneal dialysis and survival outcomes following laparotomy in children with kidney failure: a single-center, retrospective, observational study in Japan.

Background: Children with kidney failure may have congenital or acquired gastrointestinal comorbidities requiring laparotomy, which can complicate the initiation/continuation of peritoneal dialysis (PD) because of peritoneal adhesions.

Methods: This retrospective observational study included patients younger than 18 years who initiated PD after laparotomy or underwent laparotomy after PD initiation between February 1, 2006, and July 31, 2024. The primary endpoint was post-laparotomy PD failure, and the secondary endpoint was death.

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

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 preclinical and clinical evaluations to determine the feasibility of HLC infusion as a therapeutic option for safer pediatric liver transplantation.

Methods: Preclinical studies were conducted to validate the safety, biodistribution, and ammonia metabolism capabilities of HLCs using SCID mice models of UCD and extensive animal studies.

Pattern of Infection After Pediatric Liver Transplant and Its Associated Risk Factors.

Background: Liver transplantation has improved survival rates in pediatric patients with end-stage liver failure. However, post-liver transplant infection remains a threat to patient recovery. This study reported the frequency and patterns of infections after liver transplantation and identified factors related to the accuracy of fever source investigation.

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

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 developed irreversible multiple-organ failure and died.

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

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.

Living-donor liver transplantation for methylmalonic acidemia patient with hepatocellular carcinoma: A case report and literature review.

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.