Persistent Pulmonary Hypertension Without Underlying Cardiac Disease as a Presentation of Pulmonary Interstitial Glycogenosis

Authors

Gordon Gray Still, Department of Pediatrics, University of Nebraska Medical Center , Omaha, NE , United States.
Shuo Li, Department of Radiology, Yale New Haven Health Bridgeport Hospital , Bridgeport , CT , United States.
Mark Wilson, Department of Radiology, Yale New Haven Health Bridgeport Hospital , Bridgeport , CT , United States.
Paul Sammut, Department of Radiology, Yale New Haven Health Bridgeport Hospital , Bridgeport , CT , United States.

Document Type

Article

Publication Title

Fetal and pediatric pathology

Abstract

INTRODUCTION: Pulmonary interstitial glycogenosis (PIG) is an idiopathic lung condition that remains clinically underrecognized despite a growing body of literature. CASE REPORT: We present a case of PIG with pulmonary hypertension without underlying cardiac disease. This patient presented with respiratory distress and spontaneous pneumothorax at 6 months of age. Laboratory and imaging investigations demonstrated nonspecific features, but refractory pulmonary hypertension was confirmed on cardiac catheterization. Lung tissue histopathology showed glycogen positive staining of the interstitial cells, consistent with PIG. CONCLUSION: This unique case demonstrates that pulmonary hypertension can be seen in the setting of PIG without associated cardiac anomalies, and persists despite treatment in an otherwise self-limited condition.

First Page

22

Last Page

26

DOI

10.1080/15513815.2017.1397071

Publication Date

2-1-2018

Recommended Citation

Still, Gordon Gray; Li, Shuo; Wilson, Mark; and Sammut, Paul, "Persistent Pulmonary Hypertension Without Underlying Cardiac Disease as a Presentation of Pulmonary Interstitial Glycogenosis" (2018). All Research. 226.
https://scholar.bridgeporthospital.org/all_research/226

Identifier

29336713 (pubmed); 10.1080/15513815.2017.1397071 (doi)