Related Experiment Video

Updated: Dec 30, 2025

Author Spotlight: Evaluating Therapeutic Strategies to Enhance Liver Regeneration
05:25

Author Spotlight: Evaluating Therapeutic Strategies to Enhance Liver Regeneration

Published on: May 24, 2024

3.3K

Etiology of liver dysfunction after liver transplantation in children with metabolic disorders

Rie Irie1,2, Atsuko Nakazawa3, Seisuke Sakamoto4

  • 1Department of Pathology, National Center for Child Health and Development, Tokyo, Japan.

Hepatology Research : the Official Journal of the Japan Society of Hepatology
|January 21, 2020
PubMed

Insights

Liver dysfunction after liver transplantation (LT) in children with metabolic disorders is often caused by steatosis, not rejection. Prompt liver biopsy is crucial for accurate diagnosis and appropriate treatment, improving patient outcomes.

Area of Science:

  • Pediatric Hepatology
  • Transplantation Medicine
  • Metabolic Disorders

Background:

  • Liver transplantation (LT) offers excellent long-term outcomes for children with metabolic disorders.
  • Early post-LT liver dysfunction is a concern in this patient population.
  • Investigating the causes of early liver dysfunction is critical for optimizing care.

Purpose of the Study:

  • To examine the pathogenesis of early liver dysfunction after LT in pediatric recipients with metabolic disorders.
  • To identify the primary causes of liver dysfunction in this specific cohort.
  • To guide appropriate therapeutic strategies and avoid excessive immunosuppression.

Main Methods:

  • Retrospective analysis of 36 children (<18 years) with metabolic disorders who underwent LT and liver biopsy within 60 days.
  • Underlying metabolic disorders included urea cycle disorders, methylmalonic acidemia, Wilson's disease, and mitochondrial hepatopathy.
  • Biopsies were performed due to liver dysfunction or ascites.

Main Results:

  • The most common finding on graft liver biopsy was diffuse steatosis (21/36 patients).
  • Rejection was identified in 8 patients and infection in 3 patients.
  • Liver function improved post-biopsy in 18 of 21 patients with steatosis, even when donor livers had no or mild steatosis.

Conclusions:

  • Steatosis is the primary cause of early liver dysfunction post-LT in pediatric metabolic disorder patients.
  • The risk of rejection appears low in this cohort.
  • Liver biopsy is essential for diagnosing the cause of dysfunction, guiding treatment, and preventing over-immunosuppression.
Abstract

Keywords:
childrenliver transplantationmetabolic disorderssteatosis

More Related Videos

Reduced Complications after Arterial Reconnection in a Rat Model of Orthotopic Liver Transplantation08:39

Reduced Complications after Arterial Reconnection in a Rat Model of Orthotopic Liver Transplantation

Published on: November 7, 2020

8.2K
Technique of Subnormothermic Ex Vivo Liver Perfusion for the Storage, Assessment, and Repair of Marginal Liver Grafts09:29

Technique of Subnormothermic Ex Vivo Liver Perfusion for the Storage, Assessment, and Repair of Marginal Liver Grafts

Published on: August 13, 2014

15.5K

Related Concept Videos

Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
649
Pharmacokinetics in Pediatric Patients: Drug Metabolism01:24

Pharmacokinetics in Pediatric Patients: Drug Metabolism

In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses...
151
Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test01:22

Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test

In clinical practice, the direct measurement of hepatic blood flow to evaluate liver function presents significant challenges due to the intricate and specialized nature of the necessary techniques. Consequently, healthcare professionals often rely on empirical estimates derived from thorough patient examinations and liver function tests to gauge liver health. Among the tools at their disposal, the Child–Pugh and MELD scoring systems stand out for their ability to categorize and assess...
137
Kidney Transplant I: Introduction01:28

Kidney Transplant I: Introduction

A kidney transplant is a surgical approach that involves replacing a non-functioning kidney with a healthy one from a donor. This procedure is often a treatment option for end-stage renal disease (ESRD) patients. The method requires careful recipient selection, including evaluating various medical and psychosocial factors. These criteria vary between transplant centers but generally include assessments of the patient's overall health, adherence to medical recommendations, and lifestyle...
279
Effect of Hepatic Disease on Pharmacokinetics: Drug Dosing and Hepatic Blood Flow01:26

Effect of Hepatic Disease on Pharmacokinetics: Drug Dosing and Hepatic Blood Flow

Chronic liver disease significantly impacts drug metabolism due to alterations in hepatic blood flow and enzyme accessibility. This disruption affects the body's pharmacokinetics—the movement and processing of drugs within the system. Key enzymes crucial for metabolizing medications become less accessible, changing how drugs are processed and utilized. Furthermore, liver disease influences the synthesis of plasma proteins, such as albumin and globulins, which play critical roles in drug...
172
Kidney Transplant II: Surgical Procedure01:26

Kidney Transplant II: Surgical Procedure

Preoperative ManagementThe primary goals of preoperative management in kidney transplantation are to optimize the patient’s metabolic state and prepare them for surgery through diet adjustments, necessary dialysis, and tailored medical treatment. This phase also involves comprehensive infection screening and patient education about the surgical procedure and postoperative care to improve outcomes and adherence.Medical ManagementA comprehensive evaluation is required for both the living...
242