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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.
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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Diffuse Cystic Lung Disease. Part I.

Nishant Gupta1,2, Robert Vassallo3, Kathryn A Wikenheiser-Brokamp4,5,6

  • 11 Division of Pulmonary, Critical Care, and Sleep Medicine and.

American Journal of Respiratory and Critical Care Medicine
|April 24, 2015
PubMed
Summary
This summary is machine-generated.

Diffuse cystic lung diseases (DCLDs) involve multiple air-filled spaces in the lungs. This review covers DCLDs caused by neoplasms, infections, smoking, and interstitial lung diseases, focusing on specific conditions.

Keywords:
high-resolution computed tomographylung cystslymphangioleiomyomatosispulmonary Langerhans cell histiocytosistuberous sclerosis

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Area of Science:

  • Pulmonology
  • Pathology

Background:

  • Diffuse cystic lung diseases (DCLDs) are characterized by multiple air-filled spaces in lung tissue.
  • Cyst formation often results from lung remodeling due to inflammation or infiltration, damaging lung structures.

Purpose of the Study:

  • To provide an overview of DCLDs.
  • To classify DCLDs based on underlying causes.

Main Methods:

  • Literature review of DCLDs.
  • Classification of DCLDs by etiology.

Main Results:

  • DCLDs are pathologically heterogeneous.
  • Etiologies include neoplasms, infections, smoking, interstitial lung diseases, lymphoproliferative disorders, and congenital defects.
  • This article focuses on DCLDs from neoplasms, infections, smoking, and interstitial lung diseases, including lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis.

Conclusions:

  • Understanding DCLD mechanisms is crucial.
  • Classification aids in diagnosis and management of these diverse lung conditions.