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Refined Murine Model of Idiopathic Pulmonary Fibrosis
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Pulmonary Fibrosis in Children.

Nadia Nathan1,2, Chiara Sileo3, Guillaume Thouvenin4

  • 1Pediatric Pulmonology Department, Reference Center for Rare Lung Diseases (RespiRare), Armand Trousseau Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), 75012 Paris, France. nadia.nathan@aphp.fr.

Journal of Clinical Medicine
|August 29, 2019
PubMed
Summary

Pediatric pulmonary fibrosis (PF) differs significantly from adult forms, necessitating distinct diagnostic approaches. This review clarifies childhood PF pathophysiology and proposes age-appropriate diagnostic criteria for interstitial lung disease.

Keywords:
childreninterstitial lung diseasenonspecific interstitial pneumoniapulmonary fibrosisusual interstitial pneumonia

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Area of Science:

  • Pediatric Pulmonology
  • Interstitial Lung Disease
  • Pathophysiology

Background:

  • Pulmonary fibrosis (PF) is exceptionally rare in children, presenting differently than in adults.
  • Current diagnostic criteria for adult PF, particularly idiopathic PF, are unsuitable for pediatric cases.
  • Age-related differences in PF expression suggest distinct underlying entities in children.

Purpose of the Study:

  • To review the current understanding of pediatric pulmonary fibrosis pathophysiology.
  • To identify and propose suitable diagnostic criteria for childhood PF.
  • To differentiate pediatric PF from adult forms of the disease.

Main Methods:

  • Literature review of pediatric interstitial lung diseases.
  • Analysis of age-specific clinical, radiological, and histological findings in PF.
  • Synthesis of current research on childhood PF pathophysiology.

Main Results:

  • Pediatric PF exhibits unique characteristics distinct from adult presentations.
  • Existing adult diagnostic standards for PF are not applicable to children.
  • PF pathophysiology likely involves age-dependent mechanisms.

Conclusions:

  • Pulmonary fibrosis in children represents a distinct clinical and pathological entity.
  • Development of specific diagnostic criteria for pediatric PF is crucial.
  • Further research is needed to fully elucidate childhood PF mechanisms and improve diagnosis.