Related Experiment Video

Updated: Aug 7, 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

Liver disease in cystic fibrosis

Carla Colombo1, Maria Chiara Russo, Laura Zazzeron

  • 1Department of Pediatrics, CF Center, Fondazione IRCCS, Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, University of Milan, Milan, Italy. carla.colombo@unimi.it

Journal of Pediatric Gastroenterology and Nutrition
|July 5, 2006
PubMed

Insights

Cystic Fibrosis (CF) liver disease (LD) affects one-third of patients and is increasingly important. Identifying genetic modifiers and evaluating bile acid therapy and liver transplantation are key for managing CF-associated LD.

Area of Science:

  • Hepatology
  • Genetics
  • Pulmonology

Background:

  • Liver disease (LD) affects one-third of Cystic Fibrosis (CF) patients, becoming a significant clinical issue due to reduced mortality from other causes.
  • Genetic modifiers may influence CF-associated LD, offering potential for early risk identification and prophylactic strategies.
  • Current management for CF-LD includes oral bile acid therapy, though its long-term impact requires further investigation.

Purpose of the Study:

  • To highlight the growing clinical relevance of liver disease in Cystic Fibrosis.
  • To emphasize the need for identifying genetic modifiers for early risk stratification.
  • To review current therapeutic approaches and the role of liver transplantation in advanced CF-LD.

Main Methods:

  • Review of current literature on CF-associated liver disease.
  • Discussion of genetic factors influencing disease expression.
  • Analysis of therapeutic strategies including bile acid therapy and liver transplantation.

Main Results:

  • CF-associated LD is a relevant clinical issue, influenced by potential genetic modifiers.
  • Oral bile acid therapy is the current treatment, but its long-term effectiveness needs more study.
  • Liver transplantation offers an option for severe CF-LD, with approximately 80% 1-year survival and improved quality of life.

Conclusions:

  • Early identification of patients at risk for CF-associated LD through genetic modifiers is crucial.
  • Further research is needed to define the long-term impact of bile acid therapy on CF-LD.
  • Liver transplantation is a viable option for select CF patients with end-stage liver disease, improving survival and quality of life.

More Related Videos

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

The Murine Choline-Deficient, Ethionine-Supplemented (CDE) Diet Model of Chronic Liver Injury07:27

The Murine Choline-Deficient, Ethionine-Supplemented (CDE) Diet Model of Chronic Liver Injury

Published on: October 21, 2017

Related Concept Videos

Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
Cirrhosis II: Pathophysiology01:24

Cirrhosis II: Pathophysiology

Cirrhosis is a progressive chronic liver injury caused by prolonged inflammation, excessive fibrotic remodeling, and impaired regeneration. Over time, repeated hepatic insults disrupt the liver’s architecture and function, leading to reduced blood flow, impaired bile drainage, and diminished metabolic capacity.Pathophysiology of cirrhosisCirrhosis arises from three main responses to chronic liver damage: inflammation, immune activation, and hepatocyte death. These processes lead to structural...
Diseases of the Liver and Gallbladder01:26

Diseases of the Liver and Gallbladder

Liver and gallbladder diseases are a significant health concern, with prominent conditions including cirrhosis, hepatitis, non-alcoholic fatty liver disease (NAFLD), and gallstones. Jaundice is a common manifestation of liver and biliary disease.
Cirrhosis is characterized by the scarring of hepatic lobules in the liver, which are replaced by fibrous tissue, affecting the liver's normal functioning. NAFLD, on the other hand, is caused by an excessive build-up of fat in the liver, not related to...
Cirrhosis I: Introduction01:23

Cirrhosis I: Introduction

Cirrhosis is a chronic, irreversible liver disease characterized by the widespread replacement of healthy liver tissue with fibrotic scar tissue and the formation of regenerative nodules.Etiology of cirrhosisCirrhosis results from sustained liver injury that triggers progressive fibrosis and structural remodeling. The underlying causes are diverse, encompassing common and less frequent clinical conditions. Regardless of the origin, all causes lead to chronic inflammation, hepatocyte loss, and...
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...
Chronic Pancreatitis II: Pathophysiology01:21

Chronic Pancreatitis II: Pathophysiology

Chronic pancreatitis is a progressive and irreversible inflammation of the pancreas, most often caused by long-term alcohol abuse, but it can also be related to ductal obstruction, smoking, or genetic factors.Chronic pancreatitis occurs when the pancreas is repeatedly exposed to harmful agents like alcohol, smoking, ductal obstruction, or genetic predisposition. These factors lead to the release of toxic metabolites and inflammatory cytokines, sustaining chronic inflammation in the pancreatic...