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

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Authors: Corey A. Chartan, Claire Champion, PharmD Lisa Musembi, Annemarie Wolfe, Lisa Barber, Sandhya R. Ramlogan, Charles D. Fraser
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References: 4

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.

Fields of Study & Subjects

Glycogen Storage Diseases and Myoclonus Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis Cystic Fibrosis Research Advances

Publication details

Journal / source
Pediatric Pulmonology
Volume
61
Issue
1
Pages
e71465–e71465
Publication date
1 January 2026
Language
EN