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Related Concept Videos

Pleural Effusion I: Introduction01:25

Pleural Effusion I: Introduction

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Pleural effusion is an abnormal fluid accumulation in the pleural cavity, a narrow space between the lungs and the chest wall. It is not a disease per se but rather a symptom or indication of an underlying disease. In normal circumstances, this space contains a small amount of fluid (5 to 15 mL), a lubricant facilitating the non-frictional movement of the pleural surfaces.
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Amyloid Fibrils03:03

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Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
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Flail chest is a severe and potentially life-threatening condition characterized by the fracture of three or more adjacent ribs in multiple places. It is most commonly caused by direct impacts and trauma, such as motor vehicle accidents or injuries from a steering wheel impact. It can also occur due to falls in elderly individuals with osteoporosis, or assaults involving sharp objects.
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Rheumatic Heart Disease I: Introduction01:23

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Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
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Endoscopic Ultrasound (EUS) and FibroScan are valuable diagnostic tools in gastroenterology and hepatology, each with specific applications and techniques.
Endoscopic Ultrasound (EUS):
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Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

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Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
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Related Experiment Video

Updated: Sep 4, 2025

Refined Murine Model of Idiopathic Pulmonary Fibrosis
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Published on: June 17, 2025

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Pleuroparenchymal fibroelastosis.

Vincent Cottin1, Salim Si-Mohamed2, Rémi Diesler1

  • 1Department of Respiratory Medicine, National Reference Centre for Rare Pulmonary Diseases.

Current Opinion in Pulmonary Medicine
|July 20, 2022
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Summary
This summary is machine-generated.

Pleuroparenchymal fibroelastosis (PPFE) is a lung disease with a poor prognosis, often seen in various interstitial lung diseases (ILDs). Further research is needed to determine effective treatments for PPFE.

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Area of Science:

  • Pulmonology
  • Pathology
  • Radiology

Background:

  • Pleuroparenchymal fibroelastosis (PPFE) is a distinct interstitial lung disease (ILD).
  • It is characterized by upper lobe and subpleural fibrosis involving the pleura and lung parenchyma.
  • Histologically, it shows dense fibroelastic changes and elastosis.

Purpose of the Study:

  • To provide a comprehensive review of the current understanding of PPFE.
  • To summarize the latest findings and clinical implications of PPFE.

Main Methods:

  • Review of existing literature and clinical data on PPFE.
  • Analysis of prevalence in various ILD cohorts.
  • Evaluation of prognostic factors and potential therapeutic approaches.

Main Results:

  • PPFE is increasingly recognized across different conditions, with heterogeneous disease courses.
  • Specific subtypes like idiopathic PPFE, or those linked to gene mutations, chemotherapy, or combined with usual interstitial pneumonia, indicate a poor prognosis.
  • PPFE features are found in 10% of idiopathic pulmonary fibrosis, 11% of systemic sclerosis-ILD, 6.5% of rheumatoid arthritis-ILD, and 23% of hypersensitivity pneumonitis cases.
  • Nintedanib may slow progression, but antifibrotic efficacy in PPFE requires further study.

Conclusions:

  • PPFE is associated with a poor prognosis in fibrosing ILDs.
  • Further research into the management and treatment strategies for PPFE is essential.