Characterization of 45 cases of congenital lung malformation and association of inflammation with symptomatology
Nuutinen, Susanna; Lappi-Blanco, Elisa; Ronkainen, Eveliina; Dunder, Teija; Harju, Terttu; Serlo, Willy; Sinikumpu, Jaakko (2025-04-23)
Springer
23.04.2025
Nuutinen, S., Lappi-Blanco, E., Ronkainen, E. et al. Characterization of 45 cases of congenital lung malformation and association of inflammation with symptomatology. Sci Rep 15, 14207 (2025). https://doi.org/10.1038/s41598-025-98743-0.
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© The Author(s) 2025. This article is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License, which permits any non-commercial use, sharing, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if you modified the licensed material. You do not have permission under this licence to share adapted material derived from this article or parts of it. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by-nc-nd/4.0/.
https://creativecommons.org/licenses/by-nc-nd/4.0/
© The Author(s) 2025. This article is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License, which permits any non-commercial use, sharing, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if you modified the licensed material. You do not have permission under this licence to share adapted material derived from this article or parts of it. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by-nc-nd/4.0/.
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Julkaisun pysyvä osoite on
https://urn.fi/URN:NBN:fi:oulu-202504252907
https://urn.fi/URN:NBN:fi:oulu-202504252907
Tiivistelmä
Abstract
Lung development begins during week 4 of pregnancy and continues through fetal development until early infancy. Various malformations can develop during the fetal period. There is histology overlap in several of them and partly joint etiology has been suggested as the cause of malformation. Early surgery has been supported after the diagnosis of a local pulmonary malformation due to potential malignancy risk and an increased lung infection risk. However, the evidence supporting surgery for asymptomatic patients is scarce. This comprehensive single-center study in a tertiary children’s hospital included all 45 surgically treated patients at any age between 1986 and 2015. The study population consisted of 45 patients. Histology, preoperative symptoms, and radiological and operative findings were systematically reevaluated. Small cyst type congenital pulmonary airway malformation (CPAM) was the most common type of detected congenital lung malformation. There were no malignant or premalignant findings. Of all the patients, 23 (48.9%) were asymptomatic at the time of surgery. However, histology showed that four out of them (18%) had ongoing inflammation. Overall, one third of patients (n = 15, 34%) presented to have inflammation histologically. While chronic inflammation results in alveolar fibrosis, a high reported inflammation rate (34%) supports early malformation excision, regardless of the symptoms.
Lung development begins during week 4 of pregnancy and continues through fetal development until early infancy. Various malformations can develop during the fetal period. There is histology overlap in several of them and partly joint etiology has been suggested as the cause of malformation. Early surgery has been supported after the diagnosis of a local pulmonary malformation due to potential malignancy risk and an increased lung infection risk. However, the evidence supporting surgery for asymptomatic patients is scarce. This comprehensive single-center study in a tertiary children’s hospital included all 45 surgically treated patients at any age between 1986 and 2015. The study population consisted of 45 patients. Histology, preoperative symptoms, and radiological and operative findings were systematically reevaluated. Small cyst type congenital pulmonary airway malformation (CPAM) was the most common type of detected congenital lung malformation. There were no malignant or premalignant findings. Of all the patients, 23 (48.9%) were asymptomatic at the time of surgery. However, histology showed that four out of them (18%) had ongoing inflammation. Overall, one third of patients (n = 15, 34%) presented to have inflammation histologically. While chronic inflammation results in alveolar fibrosis, a high reported inflammation rate (34%) supports early malformation excision, regardless of the symptoms.
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- Avoin saatavuus [37957]
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