Titre
Molecular Diagnostics and In Utero Therapeutics for Orofacial Clefts.
Type
synthèse (review)
Institution
UNIL/CHUV/Unisanté + institutions partenaires
Périodique
Auteur(s)
Oliver, J.D.
Auteure/Auteur
Turner, E.C.
Auteure/Auteur
Halpern, L.R.
Auteure/Auteur
Jia, S.
Auteure/Auteur
Schneider, P.
Auteure/Auteur
D'Souza, R.N.
Auteure/Auteur
Liens vers les personnes
Liens vers les unités
ISSN
1544-0591
Statut éditorial
Publié
Date de publication
2020-10
Volume
99
Numéro
11
Première page
1221
Dernière page/numéro d’article
1227
Peer-reviewed
Oui
Langue
anglais
Notes
Publication types: Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Review
Publication Status: ppublish
Publication Status: ppublish
Résumé
Orofacial clefts and their management impose a substantial burden on patients, on their families, and on the health system. Under the current standard of care, affected patients are subjected to a lifelong journey of corrective surgeries and multidisciplinary management to replace bone and soft tissues, as well as restore esthetics and physiologic functions while restoring self-esteem and psychological health. Hence, a better understanding of the dynamic interplay of molecular signaling pathways at critical phases of palate development is necessary to pioneer novel prenatal interventions. Such pathways include transforming growth factor-β (Tgfβ), sonic hedgehog (Shh), wingless-integrated site (Wnt)/β-catenin, bone morphogenetic protein (Bmp), and fibroblast growth factor (Fgf) and its associated receptors, among others. Here, we summarize commonly used surgical methods used to correct cleft defects postnatally. We also review the advances made in prenatal diagnostics of clefts through imaging and genomics and the various in utero surgical corrections that have been attempted thus far. An overview of how key mediators of signaling that drive palatogenesis are emphasized in the context of the framework and rationale for the development and testing of therapeutics in animal model systems and in humans is provided. The pros and cons of in utero therapies that can potentially restore molecular homeostasis needed for the proper growth and fusion of palatal shelves are presented. The theme advanced throughout this review is the need to develop preclinical molecular therapies that could ultimately be translated into human trials that can correct orofacial clefts at earlier stages of development.
PID Serval
serval:BIB_41FCE1137086
PMID
Date de création
2020-07-03T14:48:27.315Z
Date de création dans IRIS
2025-05-20T15:37:55Z
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Nom
R032. Oliver et al.pdf
Version du manuscrit
postprint
Taille
1.14 MB
Format
Adobe PDF
PID Serval
serval:BIB_41FCE1137086.P003
URN
urn:nbn:ch:serval-BIB_41FCE11370864
Somme de contrôle
(MD5):e0fc2dec39c1240fb45538a110c7efe1