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Epidemiology of childhood interstitial lung disease in France: the RespiRare cohort

Camille Fletcher1,2, Alice Hadchouel3,4, Caroline Thumerelle5

  • 1Paediatric Pulmonology Department and Reference Center for Rare Lung Diseases, RespiRare, Sorbonne University, AP-HP, Armand Trousseau Hospital, Paris, France.

Thorax
|July 4, 2024
PubMed

Insights

This study reveals a higher incidence of childhood interstitial lung disease (chILD) in France than previously known. Findings highlight the need for improved data collection and standardized care for these rare pediatric lung conditions.

Area of Science:

  • Pediatric Pulmonology
  • Epidemiology
  • Rare Diseases

Background:

  • Childhood interstitial lung disease (chILD) encompasses rare and severe pediatric lung conditions.
  • Existing epidemiological data on chILD are scarce, often limited to small patient cohorts.
  • Accurate prevalence and incidence data are crucial for understanding the burden of chILD.

Purpose of the Study:

  • To determine the prevalence and incidence of chILD in France.
  • To analyze the characteristics, management, and outcomes of pediatric patients with chILD.
  • To establish a baseline for future national and international chILD research.

Main Methods:

  • A multicenter retrospective observational study was conducted within the RespiRare network (2000-2022).
  • A prospective evaluation of chILD incidence was performed between February 2022 and 2023.
  • Data on patient demographics, diagnoses, investigations, management, and outcomes were collected.

Main Results:

  • The study identified 790 chILD cases across 42 centers.
  • Estimated 2022 prevalence was 44/million children; incidence was 4.4/million children.
  • Common etiologies included surfactant metabolism disorders, neuroendocrine cell hyperplasia of infancy, diffuse alveolar hemorrhage, and connective tissue diseases. Survival rates varied significantly by age at diagnosis.

Conclusions:

  • This comprehensive epidemiological study demonstrates a higher prevalence and incidence of chILD than previously reported.
  • The findings underscore the need for optimized case collection and harmonized diagnostic and management practices.
  • Further collaborative efforts are essential for advancing international chILD research and improving patient care.
Abstract

Keywords:
ChildClinical EpidemiologyPaediatric Lung DisaesePaediatric interstitial lung diseaseRare lung diseases

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