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Pulmonary interstitial glycogenosis: Diagnostic evaluation and clinical course.

Abstract：

OBJECTIVES: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. METHODS: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. RESULTS: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, one underwent lung transplantation, and one died. CT findings commonly included ground glass opacities (86%) and cystic change (50%). CONCLUSIONS:The P.I.G. phenotype has not been comprehensively described, and poor recognition and misconceptions about P.I.G. persist. P.I.G. is a disease that presents in early infancy, requires significant medical intervention, and frequently is seen in association with alveolar simplification and/or cardiovascular disease. CT findings include ground glass opacities and cysts. Patients should be monitored for pulmonary hypertension. Without life-threatening comorbidities, many patients do well over time, although respiratory symptoms may persist into adolescence.

journal_name

Pediatr Pulmonol

journal_title

Pediatric pulmonology

authors

Liptzin DR,Baker CD,Darst JR,Weinman JP,Dishop MK,Galambos C,Brinton JT,Deterding RR

doi

10.1002/ppul.24123

subject

Has Abstract

pub_date

2018-12-01 00:00:00

pages

1651-1658

issue

eissn

8755-6863

issn

1099-0496

journal_volume

pub_type

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Pulmonary interstitial glycogenosis: Diagnostic evaluation and clinical course.