ArticlePulmonary circulation
Alveolar capillary dysplasia with misalignment of the pulmonary veins: clinical, histological, and genetic aspects.
Article in Pulmonary circulation. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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Who cites it
29 citing papers in PubMed, 68 citations in OpenAlex.
- Phenotyping persistent pulmonary hypertension of the newborn: recognition of its persistence across world symposium on pulmonary hypertension classifications.Journal of perinatology : official journal of the California Perinatal Association · 2026Review
- Alveolar capillary dysplasia: an example of health system data to understand rare conditions.Pediatric research · 2026Article
- Alveolar capillary dysplasia with misalignment of pulmonary veins in neonates: a multicenter cohort study.Pediatric research · 2026Article
- KLF6 activation marks an angiogenic and apoptosis resistant endothelial phenotype in pulmonary arterial hypertension.Communications biology · 2026Article
- Late presentation of alveolar capillary dysplasia with misalignment of pulmonary veins presenting as refractory pulmonary hypertension in infancy.Respiratory medicine case reports · 2026Article
- Review
- Interstitial lung disease in the newborn.Journal of perinatology : official journal of the California Perinatal Association · 2025Review
- The molecular consequences of FOXF1 missense mutations associated with alveolar capillary dysplasia with misalignment of pulmonary veins.Journal of biomedical science · 2024Article
- Beyond Bronchopulmonary Dysplasia: A Comprehensive Review of Chronic Lung Diseases in Neonates.Cureus · 2024Review
- Rapid genome diagnosis of alveolar capillary dysplasia leading to treatment in a child with respiratory and cardiac failure.Cold Spring Harbor molecular case studies · 2023Article
- Prenatal Detection of aGenes · 2023Article
- Alveolar capillary dysplasia with misalignment of pulmonary veins in a premature newborn: the role of lung ultrasound.The ultrasound journal · 2023Article
- Unusual presentation of alveolar capillary dysplasia with misalignment of the pulmonary veins in a child with respiratory syncytial virus pneumonia: A case report.Respirology case reports · 2023Article
- Case report: High-dose epoprostenol therapy in pediatric patients with pulmonary hypertension and developmental lung disease.Frontiers in pediatrics · 2023Article
- The Vasculature in Pulmonary Fibrosis.Current tissue microenvironment reports · 2022Article
- Haploinsufficiencies of FOXF1, FOXC2 and FOXL1 genes originated from deleted 16q24.1q24.2 fragment related with alveolar capillary dysplasia with misalignment of pulmonary veins and lymphedema-distichiasis syndrome: relationship to phenotype.Molecular cytogenetics · 2022Article
- Weighted gene co-expression network analysis identifies dysregulated B-cell receptor signaling pathway and novel genes in pulmonary arterial hypertension.Frontiers in cardiovascular medicine · 2022Article
- Genetics dictating therapeutic decisions in pediatric pulmonary hypertension? A case report suggesting we are getting closer.Pulmonary circulation · 2022Article
- Successful Management of an Infant with Atypical Presentation of Alveolar Capillary Dysplasia with Misalignment of the Pulmonary Veins.Journal of pediatric intensive care · 2021Article
- Nanoparticle-Facilitated Gene Delivery in Congenital Pulmonary Vascular Disease: Roadmap for Other Forms of Pulmonary Hypertension.Circulation · 2021Article
Corrections and comments
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Authors and funding
10 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV) is a rare and lethal disorder mainly involving the vascular development of the lungs. Since its first description, significant achievements in research have led to a better understanding of the underlying molecular mechanism of ACD/MPV and genetic studies have identified associations with genomic alterations in the locus of the transcription factor FOXF1. This in turn has increased the awareness among clinicians resulting in over 200 cases reported so far, including genotyping of patients in most recent reports. Collectively, this promoted a better stratification of the patient group, leading to new perspectives in research on the pathogenesis. Here, we provide an overview of the clinical aspects of ACD/MPV, including guidance for clinicians, and review the ongoing research into the complex molecular mechanism causing this severe lung disorder.
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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.