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Rare interstitial lung disease in a preterm neonate with congenital lobar emphysema
  1. Swati Jayaram1,
  2. Caroline Abramovich2,
  3. Aparna Roy1 and
  4. Gurinder Kumar1
  1. 1Paediatrics, Case Western Reserve University Hospital, Cleveland, Ohio, USA
  2. 2Pathology, Case Western Reserve University Hospital, Cleveland, Ohio, USA
  1. Correspondence to Dr Gurinder Kumar; kumargurinder{at}


Congenital lobar emphysema (CLE)/congenital alveolar overdistension/congenital lobar overinflation or infantile lobar emphysema is a rare developmental anomaly of the lower respiratory tract which is characterised by hyperinflation of one or more of the pulmonary lobes. Histopathology may be variable, which may show abnormality in the cartilage, granulation tissue, mucosal folds, etc. We report a rare underlying histopathology in a preterm neonate with CLE. This entity referred to as pulmonary interstitial glycogenosis (PIG) is a group of heterogeneous lung disease affecting the lung parenchyma and is characterised by the presence of glycogen laden cells in the lung interstitium. This impairs the gas exchange and typically manifest as tachypnoea, retractions, hypoxia and increased respiratory support. Our case report highlights the association of CLE and PIG and a review of literature. One should always have a detailed histopathology in children presenting with CLE to rule out PIG.

  • Neonatal health
  • Pathology
  • Interstitial lung disease

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  • Contributors SJ and GK conceptualised the manuscript, drafted the initial manuscript and reviewed and revised the manuscript. CA reviewed the histopathological slides, drafted the pathological aspect of case report and reviewed and revised the manuscript. AR conceptualised and designed the study, coordinated and supervised the data collection, and critically reviewed the manuscript for important intellectual content. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.