Diagnostic and clinical course of pulmonary interstitial glycogenosis: The tip of the iceberg

Pediatr Pulmonol. 2018 Dec;53(12):1659-1661. doi: 10.1002/ppul.24167. Epub 2018 Sep 26.

Abstract

"Pulmonary Interstitial Glycogenosis: Diagnostic Evaluation and Clinical Course," written by Liptzin et al is a timely and insightful phenotypic summary of a rare pediatric interstitial lung disease. Twenty-four infants with biopsy-proven pulmonary interstitial glycogenosis (PIG) were reviewed at their center. Genetic analysis, bronchoscopy results, imaging, biopsy, and cardiology findings were described, and treatment decision and clinical outcomes were discussed.

Keywords: childhood interstitial lung disease; interstitial lung disease (ILD); mesenchymal cells; pulmonary interstitial glycogenosis.

Publication types

  • Comment

MeSH terms

  • Biopsy
  • Bronchoscopy
  • Child
  • Glycogen Storage Disease*
  • Humans
  • Infant
  • Lung Diseases, Interstitial*
  • Pulmonary Alveoli