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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
04:44

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Interstitial lung disease in children

Salvatore Cazzato1, Emanuela di Palmo, Vincenzo Ragazzo

  • 1Department of Pediatrics, University of Bologna, S. Orsola-Malpighi Hospital Bologna, Italy.

Early Human Development
|August 27, 2013
PubMed

Insights

Children's interstitial lung disease (ILD) comprises rare respiratory disorders in children. Early diagnosis and tailored treatment strategies are crucial for managing these complex conditions.

Area of Science:

  • Pediatric Pulmonology
  • Rare Respiratory Diseases
  • Interstitial Lung Disease

Background:

  • Children's interstitial lung disease (ILD) encompasses diverse rare respiratory disorders.
  • These conditions present significant morbidity and mortality in pediatric populations.
  • Pathogenesis involves genetic factors, systemic diseases, and inflammatory/fibrotic repair patterns.

Purpose of the Study:

  • To review entities unique to pediatric interstitial lung disease.
  • Focus on clinical characteristics, histologic definitions, and radiologic-pathologic correlations.
  • To discuss therapeutic strategies for pediatric ILD.

Main Methods:

  • Comprehensive review of literature on pediatric ILD.
  • Emphasis on entities specific to children.
  • Integration of clinical, imaging, genetic, and histopathologic data.

Main Results:

  • Key pediatric ILDs include diffuse developmental disorders, alveolar growth abnormalities, genetic surfactant disorders, pulmonary interstitial glycogenosis, and neuroendocrine cell hyperplasia of infancy.
  • Diagnostic challenges can lead to delayed treatment.
  • Multidisciplinary approach including imaging, PFTs, genetic testing, BAL, and biopsy is essential.

Conclusions:

  • Pediatric ILD requires specialized diagnostic and management approaches.
  • Understanding unique pediatric entities is vital for timely and effective treatment.
  • Radiologic-pathologic correlation and genetic insights guide therapeutic strategies.

Keywords:
Bronchiolitis obliteransComputed tomographyDiffuse lung diseaseInterstitial lung diseaseSurfactant

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