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Molecular mechanisms underlying fibrosis and elastin destruction in childhood interstitial lung diseases

Enas A Hamed1, Mostafa M El-Saied1, Khaled Saad1

  • 1Department of Pediatrics, Faculty of Medicine, Assiut University, Assiut, Egypt.

Pathophysiology : the Official Journal of the International Society for Pathophysiology
|October 1, 2016
PubMed

Insights

Childhood interstitial lung disease (chILD) shows increased fibrosis and elastin destruction markers. Targeting these pathways, including transforming growth factor-beta 1 (TGF-β1), offers potential new treatments for chILD.

Area of Science:

  • Pediatric Pulmonology
  • Biomarkers
  • Interstitial Lung Diseases

Background:

  • Childhood interstitial lung disease (chILD) is a group of rare, heterogeneous lung disorders.
  • Accurate assessment of fibrosis and elastin destruction in chILD is crucial for understanding disease progression and developing treatments.

Purpose of the Study:

  • To evaluate markers of fibrosis and elastin destruction in pediatric patients diagnosed with interstitial lung disease.
  • To correlate these markers with disease severity and clinical outcomes in chILD.

Main Methods:

  • Sixty chILD patients and twenty healthy children were enrolled.
  • Plasma levels of transforming growth factor-beta 1 (TGF-β1), connective tissue growth factor (CCN2), soluble factor related apoptosis (sFas), and long non-coding RNAs were measured.
  • Urinary desmosine/urinary creatinine (UDes/UCr) levels were assessed as a marker of elastin degradation.

Main Results:

  • chILD patients exhibited significantly higher plasma levels of TGF-β1, CCN2, sFas, and lncrRNA-2700086A05Rik, along with elevated UDes/UCr, compared to healthy controls (P < 0.002).
  • Positive correlations were observed between fibrosis markers (TGF-β1, CCN2, sFas) and elastin destruction marker (UDes/UCr) in chILD patients.
  • Morbidity and mortality rates in the studied chILD cohort were 46.70% and 10.00%, respectively.

Conclusions:

  • Elevated levels of fibrosis (TGF-β1, sFas, CCN2) and elastin destruction (UDes/UCr) markers are characteristic of chILD.
  • These markers, particularly in patients with prolonged disease duration, suggest potential therapeutic targets.
  • Inhibition of these identified pathways may represent a novel strategy for managing chILD.
Abstract

Keywords:
Connective tissue growth factor (CCN2)Interstitial lung disease of childrenLong non-coding RNAsTransforming growth factor (TGF)β-(1)Urinary desmosine (UDes)

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