Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

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...
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...
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...
Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features01:24

Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features

Chronic bronchitis is a key phenotype of chronic obstructive pulmonary disease (COPD), characterized by airway-centered inflammation and mucus overproduction. It develops from long-term exposure to harmful particles or gases, most commonly cigarette smoke, which triggers a persistent inflammatory response.Cellular and Structural ChangesInflammation initially affects the large bronchi and later the smaller airways, with infiltration by immune cells, including neutrophils, macrophages, and...
Chronic Pancreatitis I: Introduction01:25

Chronic Pancreatitis I: Introduction

Chronic pancreatitis is a long-standing, relapsing inflammation of the pancreas, characterized by irreversible damage to the gland. It results in progressive destruction of the pancreatic parenchyma, fibrosis, and eventual loss of both exocrine and endocrine function. The disease may evolve gradually after multiple episodes of acute pancreatitis or develop independently.EtiologyChronic pancreatitis can arise from a variety of causes:Alcohol use is the leading cause, accounting for 70–80% of...
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...

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

A multi-center study of non-invasive colorectal cancer evaluation in cystic fibrosis (NICE-CF).

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society·2026
Same author

Study Design Considerations for the Evaluation of Pancreatic Enzyme Replacement Therapy (PERT) Products in People with Cystic Fibrosis: An Update.

Clinical therapeutics·2026
Same author

Increased gastrointestinal cancer risk in cystic fibrosis: Screening, prevention, and future directions.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society·2026
Same author

Longitudinal changes in bone mineral density after initiation of elexacaftor-tezacaftor-ivacaftor in youth and adults with cystic fibrosis: PROMISE-ENDO.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society·2026
Same author

Expansion of variant panels in newborn screening algorithms to identify cystic fibrosis: A retrospective analysis of 25 years of genotypes and implications on diagnosis.

Genetics in medicine : official journal of the American College of Medical Genetics·2025
Same author

Results of a clinical trial of ANG003, a non-porcine pancreatic enzyme replacement therapy, in people with cystic fibrosis.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society·2025
Same journal

Safety and efficacy of mRNA vaccines: a mechanistic and public health perspective.

Lancet (London, England)·2026
Same journal

Efficacy and safety of tirofiban after successful endovascular reperfusion in acute ischaemic stroke (ATTRACTION) in China: a multicentre, double-blind, randomised controlled trial.

Lancet (London, England)·2026
See all related articles

Related Experiment Video

Updated: Jun 23, 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

Cystic fibrosis.

Brian P O'Sullivan1, Steven D Freedman

  • 1Department of Pediatrics, University of Massachusetts Medical School, Worcester, MA 01655, USA. osullivb@ummhc.org

Lancet (London, England)
|May 1, 2009
PubMed
Summary
This summary is machine-generated.

Cystic fibrosis (CF) care has improved due to earlier diagnosis and advanced therapies targeting the basic defect. New treatments are emerging, offering hope for patients with this common lethal genetic disease.

More Related Videos

Cystic Fibrosis Aggregate Biofilm Model to Study Infection-relevant Gene Expression
08:58

Cystic Fibrosis Aggregate Biofilm Model to Study Infection-relevant Gene Expression

Published on: April 18, 2025

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
08:00

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study

Published on: April 11, 2018

Related Experiment Videos

Last Updated: Jun 23, 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

Cystic Fibrosis Aggregate Biofilm Model to Study Infection-relevant Gene Expression
08:58

Cystic Fibrosis Aggregate Biofilm Model to Study Infection-relevant Gene Expression

Published on: April 18, 2025

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
08:00

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study

Published on: April 11, 2018

Area of Science:

  • Genetics
  • Pulmonology
  • Molecular Biology

Background:

  • Cystic fibrosis is a prevalent lethal genetic disorder in Caucasian populations.
  • Significant improvements in patient outcomes are attributed to early diagnosis, intensive treatments, and specialized care centers.
  • Advances in understanding the molecular basis of CF are paving the way for novel therapeutic strategies.

Purpose of the Study:

  • To review current clinical care standards for cystic fibrosis.
  • To discuss recent breakthroughs in understanding CF pathogenesis.
  • To highlight advancements in neonatal screening and the development of therapies targeting the underlying molecular defect.

Main Methods:

  • Literature review of clinical care practices.
  • Analysis of recent research on cystic fibrosis pathogenesis.
  • Examination of emerging neonatal screening protocols.
  • Assessment of novel therapeutic approaches, including hypertonic saline and treatments for the basic defect.

Main Results:

  • Patient outlook for cystic fibrosis has shown consistent improvement over time.
  • A deeper comprehension of the molecular defect driving CF is facilitating new treatment avenues.
  • Hypertonic saline is an established treatment, with other novel therapies in advanced development.

Conclusions:

  • Continued progress in understanding cystic fibrosis pathogenesis is crucial.
  • Enhanced neonatal screening and targeted therapies are transforming CF management.
  • The development of treatments addressing the fundamental molecular defect holds significant promise for improving the lives of individuals with cystic fibrosis.