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Pulmonary growth abnormalities as etiologies for pediatric pulmonary hypertension

George B Mallory1, David R Spielberg1, Manuel Silva-Carmona1

  • 1Division of Pediatric Pulmonology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas.

Pediatric Pulmonology
|August 1, 2020
PubMed

Insights

Pulmonary growth abnormality (PGA) in infants is a common lung disease. This study clarifies its clinical spectrum, identifying three subsets and associations with pulmonary interstitial glycogenosis and hypertension.

Area of Science:

  • Pediatric Pulmonology
  • Neonatal Medicine
  • Histopathology

Background:

  • Pulmonary growth abnormality (PGA) is a frequent diffuse lung disease in infants.
  • Existing literature details PGA's histologic and radiographic features but lacks a clear clinical spectrum.
  • This ambiguity can complicate clinical diagnosis and management.

Observation:

  • Case series and reports present a varied clinical picture of PGA.
  • Three distinct subsets of PGA were identified.
  • Associations with pulmonary interstitial glycogenosis and pulmonary hypertension were noted.

Findings:

  • PGA presents with a spectrum of clinical manifestations, not a single entity.
  • Pulmonary interstitial glycogenosis is a significant histologic marker associated with certain PGA subsets.
  • Pulmonary hypertension is a common comorbidity in infants with PGA.

Implications:

  • A refined classification of PGA is needed to improve clinical understanding.
  • Recognizing distinct PGA subsets can guide targeted diagnostic and therapeutic strategies.
  • Further research into the pathogenesis and long-term outcomes of PGA subsets is warranted.

Keywords:
filamin A mutationpulmonary growth abnormalitypulmonary hypertensionpulmonary hypoplasiapulmonary interstitial glycogenosis

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