ArticleOrphanet journal of rare diseases2019
New insights on congenital pulmonary airways malformations revealed by proteomic analyses.
Article in Orphanet journal of rare diseases, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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10 citing papers in PubMed, 56 citations in OpenAlex.
- Composition and Function of Decellularized Human Lung Extracellular Matrix from Congenital Pulmonary Airway Malformation.Journal of clinical medicine · 2026Article
- Congenital Pulmonary Airway Malformations in Children: Beyond the Pulmonary Cystic Lesion Is There Really an Associated Laryngo-Tracheal Abnormality?Pediatric pulmonology · 2026Review
- Activating KRAS mutations mark premalignant cystic structures in congenital pulmonary airway malformations.Respiratory research · 2026Article
- Atypical mesenchyme in congenital pulmonary airways malformation: a promising new focus.Respiratory research · 2025Article
- 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
- Selection of potential targets for stratifying congenital pulmonary airway malformation patients with molecular imaging: is MUC1 the one?European respiratory review : an official journal of the European Respiratory Society · 2023Review
- Early resection of a rare congenital pulmonary airway malformation causing severe progressive respiratory distress in a preterm neonate: a case report and review of the literature.BMC pediatrics · 2023Review
- Laser microdissection, proteomics, and multiplex immunohistochemistry: a bumpy ride into the study of paraffin-embedded fetal and pediatric lung tissues.Frontiers in medicine · 2023Article
- Application of second-generation sequencing in congenital pulmonary airway malformations.Scientific reports · 2022Article
- In vitro models of fetal lung development to enhance research into congenital lung diseases.Pediatric surgery international · 2021Article
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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
backgroundCongenital Pulmonary Airway Malformation (CPAM) has an estimated prevalence between 0.87 and 1.02/10,000 live births and little is know about their pathogenesis. To improve our knowledge on these rare malformations, we analyzed the cellular origin of the two most frequent CPAM, CPAM types 1 and 2, and compared these malformations with adjacent healthy lung and human fetal lungs.
methodsWe prospectively enrolled 21 infants undergoing surgical resection for CPAM. Human fetal lung samples were collected after termination of pregnancy. Immunohistochemistry and proteomic analysis were performed on laser microdissected samples.
resultsCPAM 1 and 2 express mostly bronchial markers, such as cytokeratin 17 (Krt17) or α-smooth muscle actin (ACTA 2). CPAM 1 also expresses alveolar type II epithelial cell markers (SPC). Proteomic analysis on microlaser dissected epithelium confirmed these results and showed distinct protein profiles, CPAM 1 being more heterogeneous and displaying some similarities with fetal bronchi.
conclusionThis study provides new insights in CPAM etiology, showing clear distinction between CPAM types 1 and 2, by immunohistochemistry and proteomics. This suggests that CPAM 1 and CPAM 2 might occur at different stages of lung branching. Finally, the comparison between fetal lung structures and CPAMs shows clearly different protein profiles, thereby arguing against a developmental arrest in a localized part of the lung.
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