Related Experiment Video

Updated: May 21, 2026

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
05:56

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis

Published on: August 29, 2025

Optimizing approach to liver biochemistry abnormalities in cystic fibrosis pediatrics: Balancing efficiency and

Sarah B Lowry Adamitis1, Wikrom Karnsakul1

  • 1Johns Hopkins University, Baltimore, MD, United States.

Clinics and Research in Hepatology and Gastroenterology
|May 19, 2026
PubMed

Insights

This review outlines a structured approach to diagnosing liver issues in pediatric cystic fibrosis (CF) patients. It helps differentiate temporary changes from chronic conditions, focusing on drug-induced liver injury (DILI) and fatty liver disease.

Area of Science:

  • Pediatric Gastroenterology
  • Hepatology
  • Pharmacology

Background:

  • Liver biochemistry abnormalities are frequent in children with cystic fibrosis (CF).
  • These abnormalities can range from transient changes to indicators of serious underlying liver disease.
  • A systematic diagnostic strategy is needed to improve efficiency and reduce unnecessary tests.

Purpose of the Study:

  • To present a structured, evidence-based framework for evaluating liver biochemistry abnormalities in pediatric CF.
  • To differentiate between transient (<6 months) and chronic (>6 months) abnormalities.
  • To address drug-induced liver injury (DILI), including CFTR modulator hepatotoxicity, and metabolic dysfunction-associated steatotic liver disease (MASLD).

Main Methods:

  • The review synthesizes current evidence to propose a stepwise diagnostic approach.
  • It emphasizes risk stratification to guide further investigations, such as imaging or invasive diagnostics.
  • Special attention is given to high-risk CF populations with complications like portal hypertension or cirrhosis.

Main Results:

  • A structured framework allows for optimized diagnostic efficiency in pediatric CF liver abnormalities.
  • The approach aids in distinguishing transient changes from chronic liver disease, including DILI and MASLD.
  • Risk stratification effectively guides the escalation of diagnostic workup.

Conclusions:

  • Implementing a structured, stepwise approach improves the diagnostic evaluation of liver biochemistry abnormalities in pediatric CF.
  • This framework aids in managing DILI, CFTR modulator-associated hepatotoxicity, and emerging MASLD.
  • Effective clinical management is informed by considering the specific risks within the CF population.

More Related Videos

Optimized LC-MS/MS Method for the High-throughput Analysis of Clinical Samples of Ivacaftor, Its Major Metabolites, and Lumacaftor in Biological Fluids of Cystic Fibrosis Patients06:14

Optimized LC-MS/MS Method for the High-throughput Analysis of Clinical Samples of Ivacaftor, Its Major Metabolites, and Lumacaftor in Biological Fluids of Cystic Fibrosis Patients

Published on: October 15, 2017

Murine Precision-Cut Liver Slices as an Ex Vivo Model of Liver Biology12:36

Murine Precision-Cut Liver Slices as an Ex Vivo Model of Liver Biology

Published on: March 14, 2020

Related Concept Videos

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 binding...
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 the...
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 a challenge in...
Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption01:23

Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption

Understanding the physiological differences in the pediatric population is crucial for effective pharmacotherapy. Neonates, infants, and children exhibit significant variations in gastric pH, gastric emptying time, intestinal transit time, and biliary function. These variations profoundly affect oral drug absorption, necessitating a nuanced approach to pediatric dosing.Neonates present with a unique physiological profile, having a gastric pH greater than 4 and faster and more irregular gastric...
Pharmacokinetics in Pediatric Patients: Drug Excretion01:26

Pharmacokinetics in Pediatric Patients: Drug Excretion

In pediatric medicine, understanding the renal function and drug elimination nuances is crucial for administering safe and effective treatments. Newborns, in particular, display markedly slower renal functions than adults, profoundly affecting how drugs are cleared from their bodies. This slower drug clearance requires clinicians to extend the dosing intervals for many medications to prevent drug accumulation and toxicity while ensuring therapeutic efficacy.One key area where these adjustments...
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic sinusitis...