C66 PEDIATRIC CASE REPORTS: DIFFUSE LUNG DISEASES: Persistent Pulmonary Hypertension As A Result Of Pulmonary Interstitial Glycogenosis

Due to lack of improvement on conventional therapy for pulmonary hypertension, which included nitric oxide and vasopressors, she was placed on ECMO secondary to severe hypoxic respiratory failure. Discussion of novelty Pulmonary interstitial glycogenosis (PIG) is a rare idiopathic form of childhood...

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Bibliographic Details
Published inAmerican journal of respiratory and critical care medicine Vol. 195
Main Authors Still, G G, Sammut, P H
Format Journal Article
LanguageEnglish
Published New York American Thoracic Society 01.01.2017
Subjects
Biopsy
Lung diseases
Nitric oxide
Pediatrics
Pulmonary arteries
Pulmonary hypertension
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Summary:Due to lack of improvement on conventional therapy for pulmonary hypertension, which included nitric oxide and vasopressors, she was placed on ECMO secondary to severe hypoxic respiratory failure. Discussion of novelty Pulmonary interstitial glycogenosis (PIG) is a rare idiopathic form of childhood interstitial lung disease characterized by accumulation of glycogen in the lung interstitial cells.
ISSN:1073-449X
1535-4970