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Divergent Clinical Courses in Biopsy‐Proven Pulmonary Interstitial Glycogenosis: A Case Series

Year: 2026

Type: article

Abstract: Courses in Biopsy-Proven Pulmonary Interstitial Glycogenosis: A Case Series" for consideration in Pediatric Pulmonology .This report describes two infants with biopsy-confirmed pulmonary interstitial glycogenosis (PIG) who demonstrated markedly different clinical trajectories-one requiring emergent extracorporeal membrane oxygenation on the first day of life and another diagnosed months later after initial discharge, ultimately requiring tracheostomy and pulmonary hypertension-targeted therapy.This correspondence highlights the heterogeneity of PIG presentation and outcomes, emphasizing the importance of early recognition and multidisciplinary management in infants with suspected interstitial lung disease and pulmonary vascular involvement.The contrasting courses of these cases underscore the evolving understanding of PIG as a potentially dynamic and treatable form of developmental lung disease.

Source: Pediatric Pulmonology

DOI: 10.1002/ppul.71465