ArticleThe Journal of pediatrics2015
Fontan-associated protein-losing enteropathy and plastic bronchitis.
Article in The Journal of pediatrics, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers, 1 of them a synthesis that pooled it.
What it found
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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.
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Who cites it
33 citing papers in PubMed, 1 synthesis or guideline pooled it, 94 citations in OpenAlex.
- Opportunities and pitfalls of social media research in rare genetic diseases: a systematic review.Genetics in medicine : official journal of the American College of Medical Genetics · 2021Pooled it
- A Multicenter, Open-Label Study to Assess the Safety of Nebulized Tissue Plasminogen Activator for the Acute Treatment of Pediatric Plastic Bronchitis: The PLATyPuS Trial.Pharmacotherapy · 2025Trial
- Lymphatic Imaging and Interventions in Children: Current Practice.Cardiovascular and interventional radiology · 2026Review
- Fontan-Related Lymphatic Complications: Incidence, Risk Factors, and Impact on Extracardiac Outcomes and Morbidity.Journal of the American Heart Association · 2026Article
- Long-Term Care in Fontan Circulation: Surveillance and Management of Fontan-Associated Liver Disease and Protein-Losing Enteropathy.Current cardiology reports · 2026Review
- Article
- Sustained remission of post-Fontan protein-losing enteropathy achieved by combination therapy with high-dose spironolactone and the somatostatin analogue octreotide: a case report.BMC pediatrics · 2026Article
- Dielectric elastomer actuator-based valveless pump as Fontan failure assist device: introduction and preliminary study.Interdisciplinary cardiovascular and thoracic surgery · 2024Article
- The Impact of Dominant Ventricular Morphology on the Early Postoperative Course After the Glenn Procedure.Pediatric cardiology · 2023Article
- Liver stiffness in Fontan patients: the effect of respiration and food intake.Frontiers in medicine · 2023Article
- Transient liver elastography in the follow-up of Fontan patients: results of a nation wide survey in Germany.Frontiers in pediatrics · 2023Article
- Lymphatic anomalies in congenital heart disease.Pediatric radiology · 2022Review
- Stakeholders' views on drug development: the congenital disorders of glycosylation community perspective.Orphanet journal of rare diseases · 2022Article
- Protein-losing enteropathy in Fontan circulation: Pathophysiology, outcome and treatment options of a complex condition.International journal of cardiology. Congenital heart disease · 2022Review
- Protein losing enteropathy after the Fontan operation.International journal of cardiology. Congenital heart disease · 2022Review
- Dynamic contrast-enhanced magnetic resonance lymphangiography.Pediatric radiology · 2022Review
- Current Treatment Options for the Failing Fontan Circulation.Current cardiology reviews · 2022Review
- Transcatheter interventions in patients with a Fontan circulation: Current practice and future developments.Frontiers in pediatrics · 2022Review
- Plastic Bronchitis-A Serious Rare Complication Affecting Children Only after Fontan Procedure?Journal of clinical medicine · 2021Article
- Risk Factors and Long-Term Prognosis for Chylothorax After Total Cavopulmonary Connection in Children: A Retrospective Study From a Single Center.Frontiers in pediatrics · 2021Article
Corrections and comments
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Authors and funding
10 authors at 1 institution in 1 country.
Funding
Abstract
objectiveTo characterize the medical history, disease progression, and treatment of current-era patients with the rare diseases Fontan-associated protein-losing enteropathy (PLE) and plastic bronchitis. STUDY
designA novel survey that queried demographics, medical details, and treatment information was piloted and placed online via a Facebook portal, allowing social media to power the study. Participation regardless of PLE or plastic bronchitis diagnosis was allowed. Case control analyses compared patients with PLE and plastic bronchitis with uncomplicated control patients receiving the Fontan procedure.
resultsThe survey was completed by 671 subjects, including 76 with PLE, 46 with plastic bronchitis, and 7 with both. Median PLE diagnosis was 2.5 years post-Fontan. Hospitalization for PLE occurred in 71% with 41% hospitalized ≥ 3 times. Therapy varied significantly. Patients with PLE more commonly had hypoplastic left ventricle (62% vs 44% control; OR 2.81, 95% CI 1.43-5.53), chylothorax (66% vs 41%; OR 2.96, CI 1.65-5.31), and cardiothoracic surgery in addition to staged palliation (17% vs 5%; OR 4.27, CI 1.63-11.20). Median plastic bronchitis diagnosis was 2 years post-Fontan. Hospitalization for plastic bronchitis occurred in 91% with 61% hospitalized ≥ 3 times. Therapy was very diverse. Patients with plastic bronchitis more commonly had chylothorax at any surgery (72% vs 51%; OR 2.47, CI 1.20-5.08) and seasonal allergies (52% vs 36%; OR 1.98, CI 1.01-3.89).
conclusionsPatient-specific factors are associated with diagnoses of PLE or plastic bronchitis. Treatment strategies are diverse without clear patterns. These results provide a foundation upon which to design future therapeutic studies and identify a clear need for forming consensus approaches to treatment.
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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.