Cellular origins and translational approaches to congenital diaphragmatic hernia
Congenital Diaphragmatic Hernia (CDH) is a complex developmental abnormality characterized by abnormal lung development, a diaphragmatic defect and cardiac dysfunction. Despite significant advances in management of CDH, mortality and morbidity continue to be driven by pulmonary hypoplasia, pulmonary...
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| Main Authors: | , , , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
August 2024
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| In: |
Seminars in pediatric surgery
Year: 2024, Volume: 33, Issue: 4, Pages: 1-11 |
| ISSN: | 1532-9453 |
| DOI: | 10.1016/j.sempedsurg.2024.151444 |
| Online Access: | Verlag, kostenfrei, Volltext: https://doi.org/10.1016/j.sempedsurg.2024.151444 Verlag, kostenfrei, Volltext: https://www.sciencedirect.com/science/article/pii/S1055858624000659 
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| Author Notes: | Marietta Jank, Fabian Doktor, Augusto Zani, Richard Keijzer |
| Summary: | Congenital Diaphragmatic Hernia (CDH) is a complex developmental abnormality characterized by abnormal lung development, a diaphragmatic defect and cardiac dysfunction. Despite significant advances in management of CDH, mortality and morbidity continue to be driven by pulmonary hypoplasia, pulmonary hypertension, and cardiac dysfunction. The etiology of CDH remains unknown, but CDH is presumed to be caused by a combination of genetic susceptibility and external/environmental factors. Current research employs multi-omics technologies to investigate the molecular profile and pathways inherent to CDH. The aim is to discover the underlying pathogenesis, new biomarkers and ultimately novel therapeutic targets. Stem cells and their cargo, non-coding RNAs and agents targeting inflammation and vascular remodeling have produced promising results in preclinical studies using animal models of CDH. Shortcomings in current therapies combined with an improved understanding of the pathogenesis in CDH have given rise to novel promising experimental treatments that are currently being evaluated in clinical trials. This review provides insight into current developments in translational research, ranging from the cellular origins of abnormal cardiopulmonary development in CDH and the identification of novel treatment targets in preclinical CDH models at the bench and their translation to clinical trials at the bedside. |
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| Item Description: | Online verfügbar: 2. Juli 2024, Artikelversion: 11. Juli 2024 Gesehen am 27.01.2025 |
| Physical Description: | Online Resource |
| ISSN: | 1532-9453 |
| DOI: | 10.1016/j.sempedsurg.2024.151444 |