Related Experiment Video

Updated: Jan 4, 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

402

Gastrointestinal and Hepatobiliary Disease in Cystic Fibrosis

Megan E Gabel1, Gary J Galante2, Steven D Freedman3

  • 1Division of Pediatric Gastroenterology, Golisano Children's Hospital at Strong, University of Rochester, Rochester, New York.

Seminars in Respiratory and Critical Care Medicine
|October 30, 2019
PubMed

Insights

Cystic fibrosis (CF) significantly impacts the gastrointestinal (GI) system, affecting most patients. Advances in CF treatment are increasing life expectancy, making GI health a priority for improved quality of life.

Area of Science:

  • Gastroenterology
  • Pulmonology
  • Genetics

Background:

  • Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, with significant gastrointestinal (GI) involvement.
  • Up to 85% of individuals with CF experience GI symptoms, impacting morbidity and mortality.
  • Increased life expectancy due to CFTR modulators shifts focus to GI health for quality of life.

Purpose of the Study:

  • To provide an updated overview of GI and hepatic manifestations in CF.
  • To highlight the importance of managing GI issues in the context of improved CF survival.
  • To inform healthcare providers about the spectrum of GI diseases in CF patients.

Main Methods:

  • Literature review of current research on GI and hepatic diseases in CF.
  • Synthesis of information regarding common and emerging GI complications.
  • Focus on the impact of CFTR modulator therapies on GI health.

Main Results:

  • GI symptoms are highly prevalent in CF, contributing to significant patient burden.
  • Common GI issues include GERD, dysbiosis, and chronic abdominal pain.
  • Hepatobiliary disease and colorectal cancer screening are critical considerations for long-lived CF patients.

Conclusions:

  • Optimizing GI health is crucial for enhancing the well-being of individuals with CF.
  • Awareness of diverse GI manifestations is essential for comprehensive CF care.
  • Proactive management of GI and hepatic conditions improves outcomes in the growing CF population.

More Related Videos

The WinCF Model - An Inexpensive and Tractable Microcosm of a Mucus Plugged Bronchiole to Study the Microbiology of Lung Infections06:57

The WinCF Model - An Inexpensive and Tractable Microcosm of a Mucus Plugged Bronchiole to Study the Microbiology of Lung Infections

Published on: May 8, 2017

9.5K
Using Multi-fluorinated Bile Acids and In Vivo Magnetic Resonance Imaging to Measure Bile Acid Transport08:42

Using Multi-fluorinated Bile Acids and In Vivo Magnetic Resonance Imaging to Measure Bile Acid Transport

Published on: November 27, 2016

11.6K

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,...
673
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...
422
Chronic Bowel Disorders: Introduction01:17

Chronic Bowel Disorders: Introduction

Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
712
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...
1.8K
Chronic Pancreatitis I: Introduction01:24

Chronic Pancreatitis I: Introduction

The pancreas, an elongated and flat gland situated behind the stomach, serves a vital function in digesting food and managing blood sugar levels.
Pancreatitis is the inflammation of the pancreas, which occurs when the immune system becomes active and causes swelling, pain, and disruptions in organ function. Pancreatitis can manifest as either an acute or chronic condition.
Acute pancreatitis arises suddenly and lasts for a brief duration, while chronic pancreatitis is a long-term affliction...
639
Chronic Pancreatitis II: Collaborative Care01:29

Chronic Pancreatitis II: Collaborative Care

The management of chronic pancreatitis is multifaceted, involving a comprehensive approach that includes thorough assessment, diagnostic testing, and a variety of management strategies.
Assessment:
274