Pulmonary interstitial glycogenosis: Diagnostic evaluation and clinical course
Pediatric Pulmonology2018Vol. 53(12), pp. 1651–1658
Citations Over TimeTop 11% of 2018 papers
Deborah R. Liptzin, Christopher D. Baker, Jeffrey R. Darst, Jason P. Weinman, Megan K. Dishop, Csaba Galambos, John T. Brinton, Robin R. Deterding
Abstract
The P.I.G. phenotype has not been comprehensively described, and poor recognition and misconceptions about P.I.G. persist. P.I.G. is a disease that presents in early infancy, requires significant medical intervention, and frequently is seen in association with alveolar simplification and/or cardiovascular disease. CT findings include ground glass opacities and cysts. Patients should be monitored for pulmonary hypertension. Without life-threatening comorbidities, many patients do well over time, although respiratory symptoms may persist into adolescence.
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