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Related Concept Videos

Pulmonary Function Tests01:25

Pulmonary Function Tests

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Pulmonary Function Tests (PFTs)
Pulmonary Function Tests are crucial diagnostic tools for assessing respiratory function, particularly in patients with chronic respiratory disorders. They comprehensively evaluate lung volumes, ventilatory function, breathing mechanics, diffusion, and gas exchange. These tests help diagnose pulmonary diseases and play a significant role in monitoring disease progression, evaluating disability, and assessing response to therapy.
PFTs involve using a spirometer, a...
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Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care01:29

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Diagnosing Pulmonary EmbolismDiagnosing pulmonary embolism (PE) involves clinical assessment and advanced imaging tests. The preferred diagnostic tool is the spiral (helical) CT scan or CT angiography (CTA), which uses intravenous contrast media to visualize the pulmonary vasculature and identify emboli.A ventilation-perfusion (V/Q) scan is an alternative for patients unable to receive contrast media. This scan includes both perfusion and ventilation scanning. Perfusion scanning involves...
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Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies01:27

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Assessing and diagnosing Chronic Obstructive Pulmonary Disease (COPD) involves a detailed approach that includes a comprehensive review of medical history, physical examination, and a variety of diagnostic tests. This thorough evaluation is essential to ensure an accurate diagnosis and guide effective management strategies.
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Serum Studies: Renal Function Tests01:24

Serum Studies: Renal Function Tests

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Renal function tests are crucial for assessing kidney health, monitoring disease progression, and evaluating the kidneys' efficiency in waste elimination, fluid balance, and electrolyte regulation. These tests offer critical insights into kidney function, even though routine measurements may appear normal until there is a significant decline in the glomerular filtration rate or GFR. Typically, signs of kidney impairment only become evident when the GFR falls to about 50% of its normal level.
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Pulmonary Tuberculosis I01:29

Pulmonary Tuberculosis I

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Tuberculosis, often called TB, is a contagious illness primarily caused by Mycobacterium tuberculosis. It mainly affects the lung parenchyma but can also impact other body parts.
Causative Organism
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Pulmonary Tuberculosis II01:28

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Tuberculosis, or TB, is a bacterial infectious disease caused by Mycobacterium tuberculosis. While its primary impact is on the lungs, leading to pulmonary tuberculosis, it can also affect various other organs, a condition referred to as extrapulmonary tuberculosis.
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Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension
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Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension

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A multicenter study to evaluate pulmonary function in osteogenesis imperfecta.

Allison Tam1, Shan Chen1, Evan Schauer1

  • 1Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas.

Clinical Genetics
|August 29, 2018
PubMed
Summary
This summary is machine-generated.

Osteogenesis imperfecta (OI) patients, especially type III, show significantly reduced lung function (FVC, FEV1). Standard "percent predicted" values mask the true extent of pulmonary issues in OI patients.

Keywords:
lung diseaseosteogenesis imperfectapulmonary functionspirometry

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Area of Science:

  • Pulmonary Medicine
  • Genetics
  • Orthopedics

Background:

  • Pulmonary complications are a major cause of illness and death in osteogenesis imperfecta (OI).
  • Systematic evaluation of pulmonary function in OI patients is limited.
  • Osteogenesis imperfecta is a group of genetic disorders characterized by fragile bones.

Purpose of the Study:

  • To systematically evaluate pulmonary function in a large cohort of individuals with OI.
  • To assess the accuracy of standard pulmonary function metrics in OI.
  • To identify correlations between lung function and clinical variables in OI.

Main Methods:

  • Analysis of spirometry measurements (FVC, FEV1) in 217 individuals with OI.
  • Comparison of OI pulmonary function to normal population trends.
  • Use of linear mixed models to correlate lung function with clinical factors (mobility, bisphosphonate use, scoliosis).

Main Results:

  • Individuals with severe OI (type III) exhibit significantly reduced FVC and FEV1.
  • Standard "percent predicted" values underestimate pulmonary impairment in OI.
  • Correlations found between FEV1/FVC and mobility, bisphosphonate use, and scoliosis within OI subtypes.

Conclusions:

  • Pulmonary function is significantly impaired in OI, particularly in severe forms.
  • Current methods for assessing lung function in OI may not fully capture the disease burden.
  • Findings support the development of specific pulmonary endpoints for OI patient care and research.