Pulmonary growth abnormalities as etiologies for pediatric pulmonary hypertension

Pulmonary growth abnormality (PGA) is a common type of diffuse lung disease in infants. Although the histologic and radiographic features of PGA have been described in the literature in varying detail, the clinical spectrum of disease has not. The array of case series and case reports has led to a c...

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Bibliographic Details
Published in:Pediatric pulmonology 2021-03, Vol.56 (3), p.678-685
Main Authors: Mallory, George B., Spielberg, David R., Silva‐Carmona, Manuel
Format: Article
Language:English
Subjects:
filamin A mutation
Lung diseases
Pediatrics
pulmonary growth abnormality
Pulmonary hypertension
pulmonary hypoplasia
pulmonary interstitial glycogenosis
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Summary:Pulmonary growth abnormality (PGA) is a common type of diffuse lung disease in infants. Although the histologic and radiographic features of PGA have been described in the literature in varying detail, the clinical spectrum of disease has not. The array of case series and case reports has led to a clinical picture that could be confusing to clinicians. We describe three subsets of PGA, including its association with the histologic marker of pulmonary interstitial glycogenosis, and its common association with pulmonary hypertension. We propose a new approach to what we consider an increasingly broad array of different disease entities.
ISSN:8755-6863
1099-0496
DOI:10.1002/ppul.24998