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

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

297
Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...
297
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

228
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...
228
Chronic Obstructive Pulmonary Disease-II: Pathophysiology01:20

Chronic Obstructive Pulmonary Disease-II: Pathophysiology

3.1K
Chronic Obstructive Pulmonary Disease (COPD) pathophysiology is intricate and multifaceted, involving a complex interplay of physiological processes. Understanding these mechanisms is crucial for effectively managing and treating COPD. Here is an in-depth look at the critical elements in the pathophysiology of COPD:
Chronic Inflammation
3.1K
Chronic Obstructive Pulmonary Disease-V: Management01:29

Chronic Obstructive Pulmonary Disease-V: Management

2.7K
Managing Chronic Obstructive Pulmonary Disease (COPD) involves a multifaceted approach to reduce symptoms, prevent exacerbations, improve overall health status, and slow disease progression. Key strategies include lifestyle modifications, pharmacotherapy, supportive therapies, and, in some cases, surgery. Here is an overview of the primary COPD management strategies:
Smoking Cessation
2.7K
Other Pulmonary Disorders01:17

Other Pulmonary Disorders

914
Respiratory disorders encompass a range of conditions with varying levels of severity. Asthma, marked by chronic airway inflammation and hypersensitivity, is one such condition. It can lead to airway obstruction due to factors like bronchial spasms, mucosal edema, increased mucus secretion, or epithelial damage. Asthma triggers are diverse, ranging from allergens to emotional upset, and treatment focuses on both immediate relief through bronchodilators and long-term inflammation suppression.
914
Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

369
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,...
369

You might also read

Related Articles

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

Sort by
Same author

The invisible footprint: why planetary health is a pediatric radiologist's obligation.

Pediatric radiology·2026
Same author

Clinical Correlation for Imaging of Left Ventricular Assist Devices.

Radiographics : a review publication of the Radiological Society of North America, Inc·2026
Same author

Cervical paraspinal sarcopenia affects ability to achieve PROMIS minimal clinically important difference in patients undergoing anterior cervical discectomy and fusion.

Journal of craniovertebral junction & spine·2026
Same author

A Global Perspective on Viral Pneumonia.

Journal of thoracic imaging·2026
Same author

Utility of Computed Tomography Surveillance of Asymptomatic Infection in Children and Young Adults Before Allogeneic Hematopoietic Stem Cell Transplant.

Pediatric blood & cancer·2026
Same author

A Comprehensive Guide to Selecting and (Potentially) Replacing PACS: Navigating the Decision-Making Processes.

Journal of imaging informatics in medicine·2025
Same journal

MRI of Lesions Growing Along the Pituitary Stalk.

Radiographics : a review publication of the Radiological Society of North America, Inc·2026
Same journal

Invited Commentary: Early Detection of Pancreatic Cancer: Are We Up for the Challenge?

Radiographics : a review publication of the Radiological Society of North America, Inc·2026
Same journal

Radiology Board Examinations: A Fundamental Shift.

Radiographics : a review publication of the Radiological Society of North America, Inc·2026
Same journal

Early Pancreatic Cancer: Clinical Implications, Workup, and Imaging Findings with Histopathologic Correlation for Personalized Surveillance.

Radiographics : a review publication of the Radiological Society of North America, Inc·2026
Same journal

Comprehensive Approach to Prostate Cancer Metastasis Mimics at Prostate-Specific Membrane Antigen PET/CT.

Radiographics : a review publication of the Radiological Society of North America, Inc·2026
Same journal

Invited Commentary: Postdeployment Monitoring of AI in Radiology: Beyond the Test Set.

Radiographics : a review publication of the Radiological Society of North America, Inc·2026
See all related articles

Related Experiment Video

Updated: Sep 18, 2025

Unilateral Lung Volume Analysis Using Micro-CT for Enhanced Assessment of Pulmonary Fibrosis in Preclinical Models
03:38

Unilateral Lung Volume Analysis Using Micro-CT for Enhanced Assessment of Pulmonary Fibrosis in Preclinical Models

Published on: June 20, 2025

343

Multidisciplinary Approach to Familial Pulmonary Fibrosis.

Katherine A Cheng1, Rose McNulty1, Holly Nichols1

  • 1From the Department of Radiology (K.A.C., R.M., H.N., H.P.M., T.S.H.), Department of Pathology (J.C.), and Division of Pulmonary, Allergy, and Critical Care Medicine (A.S.), Duke University Medical Center, 2301 Erwin Rd, Box 3808, Durham, NC 27710; Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio (A.T., E.C.); and Department of Radiology and Biomedical Imaging, University of California San Francisco, San Francisco, Calif (M.V.).

Radiographics : a Review Publication of the Radiological Society of North America, Inc
|June 26, 2025
PubMed
Summary
This summary is machine-generated.

Familial pulmonary fibrosis (FPF) presents diagnostic challenges due to variable presentation and overlapping symptoms. Early diagnosis is crucial for better outcomes in FPF patients compared to sporadic cases.

More Related Videos

Oropharyngeal Administration of Bleomycin in the Murine Model of Pulmonary Fibrosis
06:03

Oropharyngeal Administration of Bleomycin in the Murine Model of Pulmonary Fibrosis

Published on: May 9, 2025

868
A Multimodal Imaging Approach Based on Micro-CT and Fluorescence Molecular Tomography for Longitudinal Assessment of Bleomycin-Induced Lung Fibrosis in Mice
07:38

A Multimodal Imaging Approach Based on Micro-CT and Fluorescence Molecular Tomography for Longitudinal Assessment of Bleomycin-Induced Lung Fibrosis in Mice

Published on: April 13, 2018

11.6K

Related Experiment Videos

Last Updated: Sep 18, 2025

Unilateral Lung Volume Analysis Using Micro-CT for Enhanced Assessment of Pulmonary Fibrosis in Preclinical Models
03:38

Unilateral Lung Volume Analysis Using Micro-CT for Enhanced Assessment of Pulmonary Fibrosis in Preclinical Models

Published on: June 20, 2025

343
Oropharyngeal Administration of Bleomycin in the Murine Model of Pulmonary Fibrosis
06:03

Oropharyngeal Administration of Bleomycin in the Murine Model of Pulmonary Fibrosis

Published on: May 9, 2025

868
A Multimodal Imaging Approach Based on Micro-CT and Fluorescence Molecular Tomography for Longitudinal Assessment of Bleomycin-Induced Lung Fibrosis in Mice
07:38

A Multimodal Imaging Approach Based on Micro-CT and Fluorescence Molecular Tomography for Longitudinal Assessment of Bleomycin-Induced Lung Fibrosis in Mice

Published on: April 13, 2018

11.6K

Area of Science:

  • Pulmonology
  • Genetics
  • Radiology

Background:

  • Familial pulmonary fibrosis (FPF) is a subset of interstitial lung disease with diagnostic complexities.
  • Variability in age of presentation, penetrance, and extrapulmonary symptoms complicates diagnosis.
  • Recent advances in gene sequencing have identified new genetic causes of FPF.

Purpose of the Study:

  • To review general concepts of FPF.
  • To highlight key genetic mutations and syndromes associated with FPF.
  • To raise awareness among radiologists regarding FPF clinical and imaging findings.

Main Methods:

  • Review of current literature on FPF.
  • Identification of key genetic mutations (surfactant dysfunction, telomere shortening).
  • Discussion of associated genetic syndromes (e.g., dyskeratosis congenita, Hermansky-Pudlak syndrome).

Main Results:

  • FPF diagnosis is challenging due to variable clinical and imaging presentations.
  • Key genetic factors include abnormal surfactant function and telomere shortening.
  • Extrapulmonary manifestations can further obscure diagnosis.

Conclusions:

  • Accurate and early diagnosis of FPF is pivotal due to a worse prognosis compared to sporadic forms.
  • Radiologists play a key role in identifying potential FPF cases.
  • Awareness of FPF genetic underpinnings and clinical features is essential for improved patient management.