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CC BY 4.0 · Journal of Child Science 2022; 12(01): e108-e111
DOI: 10.1055/s-0042-1757143
Case Report

An Infant with Persistent Respiratory Failure Associated with Refractory Pulmonary Hypertension: Pulmonary Interstitial Glycogenosis

Gianfranco Tomarelli 
1   Pediatric Intensive Care Unit, Hospital Clínico Dra. Eloísa Díaz I. La Florida, Santiago, Chile
,
Alejandro Donoso
1   Pediatric Intensive Care Unit, Hospital Clínico Dra. Eloísa Díaz I. La Florida, Santiago, Chile
,
Francisca Andrades
2   Department of Pediatrics, Diego Portales University, Santiago, Chile
,
Soledad Montes
3   Department of Pediatric Pulmonology, Hospital Clínico Dra. Eloísa Díaz I, La Florida, Santiago, Chile
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Abstract

Pulmonary interstitial glycogenosis (PIG) is a disease of unknown etiology. It is part of the interstitial lung diseases, corresponding to the compartment of the fetal pulmonary interstitium. It typically presents within the first week of life as refractory respiratory distress with tachypnea and persistent hypoxemia, and it is not associated with glycogen deposition in other organs. Usually, there is a clinical improvement and good prognosis after steroid therapy unless there are associated conditions such as congenital heart disease, pulmonary hypertension, or genetic disorders. We report a case diagnosed by lung biopsy at 4 months of age in a male preterm born, small for gestational age infant, who developed refractory hypoxemia and pulmonary hypertension with fatal outcome. There was no response to steroids and hydroxychloroquine. He was not candidate for extracorporeal membrane oxygenation. PIG should be considered in the differential diagnosis of persistent respiratory distress and hypoxemia despite standard treatment, even after the first month of life.

Keywords

pulmonary interstitium - pulmonary interstitial glycogenosis - interstitial lung disease - pulmonary hypertension - respiratory failure


Publication History

Received: 23 November 2021

Accepted: 28 June 2022

Article published online:
29 September 2022

© 2022. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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