ArticleAmerican journal of respiratory and critical care medicine2018
Novel Molecular and Phenotypic Insights into Congenital Lung Malformations.
Article in American journal of respiratory and critical care medicine, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
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
26 citing papers in PubMed.
- Congenital cystic adenomatoid malformations of the lung: an epithelial transcriptomic approach.Respiratory research · 2020Trial
- Composition and Function of Decellularized Human Lung Extracellular Matrix from Congenital Pulmonary Airway Malformation.Journal of clinical medicine · 2026Article
- Congenital Pulmonary Hybrid Lesions: Clinical Presentation, Surgical Management, and Outcomes.Indian journal of pediatrics · 2025Article
- Unilateral Congenital Pulmonary Hypoplasia: A Rare Cause of Severe Respiratory Distress in a Three-Weeks-Old Neonate.Clinical case reports · 2025Article
- Atypical mesenchyme in congenital pulmonary airways malformation: a promising new focus.Respiratory research · 2025Article
- KLF5 Shapes Developing Respiratory Tubules by Inhibiting Actin Asymmetry in Epithelial Cells.American journal of respiratory cell and molecular biology · 2025Article
- Novel compound heterozygous OBSCN variants in Chinese children with congenital pulmonary airway malformation.Italian journal of pediatrics · 2025Article
- The dynamics of tubulogenesis in development and disease.Development (Cambridge, England) · 2025Review
- Integrative analysis of bulk and single-cell RNA sequencing reveals the gene expression profile and the critical signaling pathways of type II CPAM.Cell & bioscience · 2024Article
- Congenital Lung Malformations: A Pictorial Review of Imaging Findings and a Practical Guide for Diagnosis.Children (Basel, Switzerland) · 2024Review
- Congenital lung malformations.Nature reviews. Disease primers · 2023Review
- Integrated bulk and single-cell RNA-sequencing reveals SPOCK2 as a novel biomarker gene in the development of congenital pulmonary airway malformation.Respiratory research · 2023Article
- Cancer or Not Cancer: That Is the Question.American journal of respiratory and critical care medicine · 2023Article
- Defining the spatial landscape of KRAS mutated congenital pulmonary airway malformations: a distinct entity with a spectrum of histopathologic features.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2022Article
- Transcriptome analysis of lncRNA expression patterns in human congenital lung malformations.BMC genomics · 2021Article
- Review
- mTORC1 is a mechanosensor that regulates surfactant function and lung compliance during ventilator-induced lung injury.JCI insight · 2021Article
- In vitro models of fetal lung development to enhance research into congenital lung diseases.Pediatric surgery international · 2021Article
- Genomic, epigenomic, and biophysical cues controlling the emergence of the lung alveolus.Science (New York, N.Y.) · 2021Article
- Clinicopathological features of resected pediatric congenital lung and mediastinal lesions: A single institution experience.Saudi medical journal · 2021Article
Corrections and comments
- Commented on by
Authors and funding
9 authors.
Funding
Abstract
rationaleDisruption of normal pulmonary development is a leading cause of morbidity and mortality in infants. Congenital lung malformations are a unique model to study the molecular pathogenesis of isolated structural birth defects, as they are often surgically resected.
objectivesTo provide insight into the molecular pathogenesis of congenital lung malformations through analysis of cell-type and gene expression changes in these lesions.
methodsClinical data, and lung tissue for DNA, RNA, and histology, were obtained from 58 infants undergoing surgical resection of a congenital lung lesion. Transcriptome-wide gene expression analysis was performed on paired affected and unaffected samples from a subset of infants (n = 14). A three-dimensional organoid culture model was used to assess isolated congenital lung malformation epithelium (n = 3). MEASUREMENTS AND MAIN
resultsCongenital lung lesions express higher levels of airway epithelial related genes, and dysregulated expression of genes related to the Ras and PI3K-AKT-mTOR (phosphatidylinositol 3-kinase-AKT-mammalian target of rapamycin) signaling pathways. Immunofluorescence confirmed differentiated airway epithelial cell types throughout all major subtypes of congenital lung lesions, and three-dimensional cell culture demonstrated a cell-autonomous defect in the epithelium of these lesions.
conclusionsThis study provides the first comprehensive analysis of the congenital lung malformation transcriptome and suggests that disruptions in Ras or PI3K-AKT-mTOR signaling may contribute to the pathology through an epithelial cell-autonomous defect.
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.