Pulmonary interstitial glycogenosis: Diagnostic evaluation and clinical course

Pediatric Pulmonology
Deborah R LiptzinRobin R Deterding

Abstract

We sought to describe the phenotype for patients with P.I.G. including presentation, evaluation, cardiac co-morbidities, high resolution computed tomography findings, and outcomes. With institutional review board approval, we performed a retrospective review of patients with biopsy-proven P.I.G. Biopsies, high resolution chest computed tomography, and cardiac evaluations were reviewed and characterized by experts in each field. Sixty-two percent of the patients were male. The median gestational age was 37 weeks (range 27-40). The median age at biopsy was 1.6 months (range 0.3-6 months). Structural heart disease was present in 63% of patients. Pulmonary hypertension (diagnosed by echocardiogram and/or cardiac catheterization) was noted in 38% of patients. Alveolar simplification was present in 79% of patients. Fifty percent of available biopsies revealed patchy disease. An increase in age at biopsy was associated with patchy (vs diffuse) disease. Ninety-two percent of patients were treated with systemic corticosteroids. Median age at last follow-up was 1234 days with a range of 37 days to 15 years. At the time of last follow-up, 12 patients were off all support, eight were on supplemental oxygen, two were mechanically ventilated...Continue Reading

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Citations

Mar 7, 2019·American Journal of Physiology. Lung Cellular and Molecular Physiology·Stephen E McGowan
Mar 4, 2020·NeoReviews·Stephanie Satchell, Sebastian Welsh
May 21, 2020·The European Respiratory Journal·Csaba GalambosSteven H Abman
Aug 1, 2020·Pediatric Pulmonology·George B MalloryManuel Silva-Carmona
Dec 16, 2020·Children·Valentina Agnese FerraroSilvia Carraro
Jun 11, 2021·ERJ Open Research·Václav KouckýAndrew Bush
Jul 16, 2021·Pediatric Pulmonology·Deborah R LiptzinRobin R Deterding

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