Citation, DOI, disclosures and article data
Citation:
Weerakkody Y, Luong D, Foster T, et al. Pulmonary interstitial glycogenosis. Reference article, Radiopaedia.org (Accessed on 20 Apr 2024) https://doi.org/10.53347/rID-69106
Pulmonary interstitial glycogenosis (PIG) is a rare pediatric interstitial lung disease associated with alveolar growth abnormalities.
Patients may present in the neonatal period with disproportionate respiratory distress (neonatal respiratory distress).
It is histologically characterized by abundant glycogen-laden mesenchymal cells within the pulmonary interstitium 1.
Associations
Many cases show concurrent extrapulmonary comorbidities with congenital cardiac disease considered the most common of these 3.
CT chest
Features are nonspecific, including ground-glass opacification (considered most common 1), consolidation, linear opacities, and mosaic attenuation without a uniform pattern.
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1. Weinman JP, White CJ, Liptzin DR, Deterding RR, Galambos C, Browne LP. High-resolution CT findings of pulmonary interstitial glycogenosis. (2018) Pediatric radiology. 48 (8): 1066-1072. doi:10.1007/s00247-018-4138-4 - Pubmed
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2. Lanfranchi M, Allbery SM, Wheelock L, Perry D. Pulmonary interstitial glycogenosis. (2010) Pediatric radiology. 40 (3): 361-5. doi:10.1007/s00247-009-1455-7 - Pubmed
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3. Seidl E, Carlens J, Reu S, Wetzke M, Ley-Zaporozhan J, Brasch F, Wesselak T, Schams A, Rauch D, Schuch L, Kappler M, Schelstraete P, Wolf M, Stehling F, Haarmann E, Borensztajn D, van de Loo M, Rubak S, Lex C, Hinrichs B, Reiter K, Schwerk N, Griese M. Pulmonary interstitial glycogenosis - A systematic analysis of new cases. (2018) Respiratory medicine. 140: 11-20. doi:10.1016/j.rmed.2018.05.009 - Pubmed
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4. Cutz E, Chami R, Dell S, Langer J, Manson D. Pulmonary interstitial glycogenosis associated with a spectrum of neonatal pulmonary disorders. (2017) Human pathology. 68: 154-165. doi:10.1016/j.humpath.2017.06.026 - Pubmed
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5. Canakis AM, Cutz E, Manson D, O'Brodovich H. Pulmonary interstitial glycogenosis: a new variant of neonatal interstitial lung disease. (2002) American journal of respiratory and critical care medicine. 165 (11): 1557-65. doi:10.1164/rccm.2105139 - Pubmed
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