ArticlePediatric pulmonology2018
Pulmonary interstitial glycogenosis: Diagnostic evaluation and clinical course.
Article in Pediatric pulmonology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 1 of them a synthesis that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
13 citing papers in PubMed, 1 synthesis or guideline pooled it, 34 citations in OpenAlex.
- Old and emerging therapies for childhood interstitial lung disease (chILD): a systematic review.European respiratory review : an official journal of the European Respiratory Society · 2026Pooled it
- The evolving pathophysiology of bronchopulmonary dysplasia.Physiological reviews · 2026Review
- Infant Pulmonary Function Testing in Pediatric Diffuse Parenchymal Lung Disease.CHEST pulmonary · 2025Review
- Placental transmogrification of the lung inERJ open research · 2025Article
- Interstitial lung disease in the newborn.Journal of perinatology : official journal of the California Perinatal Association · 2025Review
- New Insights: P.I.G in Preterm Infants With Isolated PDA and Severe Pulmonary Hypertension.Case reports in pediatrics · 2025Article
- Innovations in Childhood Interstitial and Diffuse Lung Disease.Clinics in chest medicine · 2024Review
- Pulmonary interstitial glycogenosis in two neonates: Early recognition and use of corticosteroids.Respiratory medicine case reports · 2024Article
- Interstitial Lung Disease in Children: "Specific Conditions of Undefined Etiology" Becoming Clearer.Children (Basel, Switzerland) · 2022Review
- Persistence of persistent pulmonary hypertension of the newborn: A case of de novo TBX4 variant.Pulmonary circulation · 2022Article
- Neonatal interstitial lung disease in a girl with Jacobsen syndrome: a case report.Journal of medical case reports · 2022Article
- Transition of patients with interstitial lung disease from paediatric to adult care.ERJ open research · 2021Article
- Children's Interstitial and Diffuse Lung Diseases (ChILD) in 2020.Children (Basel, Switzerland) · 2020Review
Corrections and comments
- Commented on by
Authors and funding
8 authors at 4 institutions in 1 country.
Funding
Abstract
objectivesWe sought to describe the phenotype for patients with P.I.G. including presentation, evaluation, cardiac co-morbidities, high resolution computed tomography findings, and outcomes.
methodsWith 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.
resultsSixty-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%).
conclusionsThe 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.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.