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Cystic Fibrosis: Pathogenesis01:23

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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.
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Cystic Fibrosis Aggregate Biofilm Model to Study Infection-relevant Gene Expression
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Diffuse Cystic Lung Diseases.

Baha Obaidat1, Dina Yazdani1, Kathryn A Wikenheiser-Brokamp2

  • 1Division of Pulmonary, Critical Care and Sleep Medicine, University of Cincinnati Medical Center, Cincinnati, Ohio.

Respiratory Care
|October 17, 2019
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Summary
This summary is machine-generated.

This review covers diffuse cystic lung diseases (DCLDs), focusing on diagnosis and management. It highlights how high-resolution CT imaging aids in differentiating these diverse pulmonary disorders.

Keywords:
Birt-Hogg-Dubé syndromefollicular bronchiolitislymphangioleiomyomatosislymphoid interstitial pneumoniapulmonary Langerhans cell histiocytosis

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Area of Science:

  • Pulmonology
  • Radiology
  • Medical Genetics

Background:

  • Diffuse cystic lung diseases (DCLDs) are a heterogeneous group of lung disorders.
  • These conditions are characterized by thin-walled, air-filled spaces in the lung parenchyma.
  • Understanding their varied pathophysiology is crucial for effective management.

Purpose of the Study:

  • To provide a clinical overview of common DCLDs.
  • To emphasize the role of high-resolution computed tomography (HRCT) in DCLD evaluation.
  • To offer practical guidance for optimal diagnosis and patient management.

Main Methods:

  • Review of clinical literature on DCLDs.
  • Emphasis on HRCT findings including cyst morphology and distribution.
  • Classification of DCLDs based on underlying pathophysiology.

Main Results:

  • HRCT is essential for distinguishing between different DCLDs.
  • Common DCLDs discussed include lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis, Birt-Hogg-Dubé syndrome, and lymphoid interstitial pneumonia/follicular bronchiolitis.
  • Pathophysiological classification aids in understanding DCLDs (neoplastic, genetic, lymphoproliferative, infectious, interstitial lung disease-associated, smoking-related).

Conclusions:

  • Accurate diagnosis and management of DCLDs rely on integrating clinical, imaging, and etiological information.
  • HRCT characteristics are key discriminators among various DCLDs.
  • This review offers practical insights for clinicians managing patients with DCLDs.