Article
Biotechnology & Applied Microbiology
Shiho Yoshida, Alexander M. Kreger, George K. Gittes
Summary: We propose that intra-amniotic sildenafil administration is an effective prenatal therapy for CDH-induced pulmonary hypertension. Intra-amniotic sildenafil treatment attenuated peripheral vascular muscularization, enhanced pulmonary blood flow, and improved lung architecture in CDH fetuses. Early intervention with intra-amniotic sildenafil treatment may be preferable to accelerate lung development and improve prognosis in CDH cases.
FRONTIERS IN BIOENGINEERING AND BIOTECHNOLOGY
(2023)
Review
Biochemistry & Molecular Biology
Flaminia Pugnaloni, Irma Capolupo, Neil Patel, Paola Giliberti, Andrea Dotta, Pietro Bagolan, Florian Kipfmueller
Summary: This review highlights the importance of miRNAs in CDH-related PH and summarizes the findings from animal and human CDH studies. The focus on epigenetic modulators of CDH-PH offers potential for developing innovative diagnostic tools and treatment approaches, and enhances researchers' understanding of CDH pathophysiology.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Surgery
Francesca Maria Russo, Marina Gabriela Monteiro Carvalho Mori Da Cunha, Julio Jimenez, Flore Lesage, Mary Patrice Eastwood, Jaan Toelen, Jan Deprest
Summary: The study aimed to evaluate the effect of combining antenatal sildenafil with fetal tracheal occlusion (TO) in fetal rabbits with surgically induced congenital diaphragmatic hernia (CDH) and found that the combination had a complementary effect on lung development.
Article
Acoustics
F. M. Russo, A-G Cordier, D. Basurto, L. Salazar, E. Litwinska, O. Gomez, A. Debeer, J. Nevoux, S. Patel, L. Lewi, A. Pertierra, M. Aertsen, E. Gratacos, K. H. Nicolaides, A. Benachi, J. Deprest
Summary: The antenatal measurement of lung size can predict the survival of fetuses with isolated right-sided congenital diaphragmatic hernia (iRCDH). In cases with severe lung hypoplasia, fetal endoscopic tracheal occlusion (FETO) is associated with a significant increase in survival without an associated increase in neonatal morbidity.
ULTRASOUND IN OBSTETRICS & GYNECOLOGY
(2021)
Review
Acoustics
Y. Chen, R. Xu, X. Xie, T. Wang, Z. Yang, J. Chen
Summary: This study conducted a systematic review and meta-analysis to evaluate the overall effects of fetal endoscopic tracheal occlusion (FETO) on clinical outcomes of fetuses with congenital diaphragmatic hernia (CDH). The results showed that FETO can reduce mortality, rates of pulmonary hypertension, and the usage of extracorporeal membrane oxygenation (ECMO) in severe CDH, while it only reduces the risk of mortality in moderate CDH. Furthermore, FETO increases the risk of preterm birth but not extreme prematurity.
ULTRASOUND IN OBSTETRICS & GYNECOLOGY
(2023)
Article
Pharmacology & Pharmacy
Alecsander F. Bressan, Victoria Oliveira Maia, Beatriz de Souza Rodrigues, Giuliana Bertozi, Sabrina S. Batah, Alexandre T. Fabro, Gloria Pelizzo, Ugo Maria Pierucci, Rita C. Tostes, Lourenco Sbragia, Fernando S. Carneiro
Summary: Congenital diaphragmatic hernia (CDH) can cause abnormalities in the pulmonary arteries of both the contralateral and ipsilateral sides of the diaphragm. Nitric oxide (NO) is commonly used to treat CDH but may not always be effective. This study investigated the different responses of the left and right pulmonary arteries to a NO donor in a rabbit model of left CDH. The results showed that newborns with CDH had increased vasorelaxant responses to the NO donor compared to the control group, possibly due to increased cGMP mobilization.
PHARMACOLOGICAL RESEARCH
(2023)
Review
Pediatrics
Oluyinka O. Olutoye, Walker D. Short, Jamie Gilley, J. D. Hammond, Michael A. Belfort, Timothy C. Lee, Alice King, Jimmy Espinoza, Luc Joyeux, Krithika Lingappan, Jason P. Gleghorn, Sundeep G. Keswani
Summary: Congenital diaphragmatic hernia (CDH) is a complex disease associated with pulmonary hypoplasia and pulmonary hypertension. Fetoscopic endoluminal tracheal occlusion (FETO) has shown potential in improving lung volume and morphology in CDH patients, but its cellular and molecular effects are still an emerging field of research.
FRONTIERS IN PEDIATRICS
(2022)
Article
Medicine, General & Internal
Felix Rafael De Bie, Christopher Gates Halline, Travis Kotzur, Kevin Hayes, Christopher Copeland Rouse, Jonathan Chang, Abby Christine Larson, Sameer Ahmad Khan, Ashley Spina, Samantha Tilden, Francesca Maria Russo, Holly Lee Hedrick, Jan Deprest, Emily Anne Partridge
Summary: In a nitrofen rat model, prenatal administration of treprostinil significantly reduces pulmonary arteriole muscularization. These results suggest that prophylactic treatment may be a potential approach for managing CDH-related PH.
Article
Medicine, General & Internal
Florian Kipfmueller, Suemeyra Akkas, Flaminia Pugnaloni, Bartolomeo Bo, Lotte Lemloh, Lukas Schroeder, Ulrich Gembruch, Annegret Geipel, Christoph Berg, Andreas Heydweiller, Andreas Mueller
Summary: The study suggests that PAAT:ET has potential as a prognostic indicator in neonates with congenital diaphragmatic hernia. This parameter can be used for early assessment of ECMO requirement and mortality risk.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Physiology
Krithika Lingappan, Oluyinka O. Olutoye, Abiud Cantu, Manuel Eliezer Cantu Gutierrez, Nahir Cortes-Santiago, J. D. Hammond, Jamie Gilley, Joselyn Rojas Quintero, Hui Li, Francesca Polverino, Jason P. Gleghorn, Sundeep G. Keswani
Summary: This is the first application of spatial transcriptomics in patients with CDH, revealing the contribution of different lung cellular subpopulations in CDH pathophysiology and highlighting sex-specific differences.
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY
(2023)
Article
Obstetrics & Gynecology
Ben Van Calster, Alexandra Benachi, Kypros H. Nicolaides, Eduard Gratacos, Christoph Berg, Nicola Persico, Glenn J. Gardener, Michael Belfort, Yves Ville, Greg Ryan, Anthony Johnson, Haruhiko Sago, Przemyslaw Kosinski, Pietro Bagolan, Tim Van Mieghem, Philip L. J. DeKoninck, Francesca M. Russo, Stuart B. Hooper, Jan A. Deprest
Summary: This analysis suggests that fetoscopic endoluminal tracheal occlusion improves survival in cases of moderate and severe lung hypoplasia. The timing of balloon insertion may impact the treatment effect, but further research is needed to determine its precise influence due to the limited sample size and confounding factors. In particular, early insertion of the balloon greatly increases the risk of preterm delivery.
AMERICAN JOURNAL OF OBSTETRICS AND GYNECOLOGY
(2022)
Article
Pediatrics
R. Gregorio-Hernandez, C. Ramos-Navarro, S. Vigil-Vazquez, E. Rodriguez-Corrales, A. Perez-Perez, M. Arriaga-Redondo, M. Sanchez-Luna
Summary: Lung ultrasound is a valuable tool for assessing pulmonary aeration in neonates, including those with congenital diaphragmatic hernia (CDH). In this study, lung ultrasound examinations were performed on 8 CDH patients before and after surgical correction, and compared between two groups based on mechanical ventilation duration. Lung ultrasound showed potential for diagnosing postoperative complications without radiation exposure and offered quick and serial assessments. These findings highlight the importance of lung ultrasound as an effective alternative to conventional imaging methods in managing CDH.
EUROPEAN JOURNAL OF PEDIATRICS
(2023)
Article
Pediatrics
Vincent Serapiglia, Chad A. Stephens, Rashika Joshi, Emrah Aydin, Marc Oria, Mario Marotta, Jose L. Peiro, Brian M. Varisco
Summary: Fetal endoscopic tracheal occlusion (FETO) is an emerging surgical therapy for congenital diaphragmatic hernia (CDH). This study found that tracheal occlusion (TO) leads to an increase in basal cells and a decrease in ciliated cells in the lung epithelial cell populations. The increased nuclear Yap, a known regulator of basal cell fate, contributes to the expansion of basal cells.
FRONTIERS IN PEDIATRICS
(2022)
Article
Medicine, General & Internal
Daphne S. Mous, Marjon J. Buscop-van Kempen, Rene M. H. Wijnen, Dick Tibboel, Rory E. Morty, Robbert J. Rottier
Summary: Studies on the activation of TGF beta and BMP signaling pathways in CDH are limited and inconsistent. However, research on a nitrofen-CDH rat model suggests increased activation of TGF beta pathway and decreased activation of BMP pathway in pulmonary vasculature, which may contribute to the development of pulmonary hypertension in CDH.
FRONTIERS IN MEDICINE
(2021)
Article
Pediatrics
Judith Leyens, Lukas Schroeder, Annegret Geipel, Christoph Berg, Bartolomeo Bo, Lotte Lemloh, Neil Patel, Andreas Mueller, Florian Kipfmueller
Summary: This study aims to describe the early course of pulmonary hypertension (PH) in infants with congenital diaphragmatic hernia (CDH) and its relation to prognostic markers and outcome measures. The severity of PH varied significantly in the first 48 hours of life. Patients with mild/no PH had a better prognosis, while patients with severe PH had a higher risk of mortality and ECMO. Assessing PH within 2-6 hours is crucial in the care for CDH neonates.
FRONTIERS IN PEDIATRICS
(2023)