scholarly journals Dysregulation of Rho GTPases in orofacial cleft patients-derived primary cells leads to impaired cell migration, a potential cause of cleft/lip palate development

2021 ◽  
Vol 165 ◽  
pp. 203656
Author(s):  
Mirvat El-Sibai ◽  
Joelle El Hajj ◽  
Maria Al Haddad ◽  
Nada El Baba ◽  
Mounir Al Saneh ◽  
...  
Keyword(s):  
Cell Migration ◽  
Rho Gtpases ◽  
Cleft Lip ◽  
Orofacial Cleft ◽  
Primary Cells ◽  
Palate Development ◽  
Cleft Lip Palate

scholarly journals Pregnancy Termination in the Case of an Orofacial Cleft: An Investigation of the Concept of Reproductive Autonomy

2020 ◽  
Vol 57 (9) ◽  
pp. 1134-1139
Author(s):  
Kristien Hens ◽  
Greet Hens
Keyword(s):  
Pregnant Woman ◽  
Cleft Lip ◽  
Pregnancy Termination ◽  
Clinical Information ◽  
Orofacial Cleft ◽  
Reproductive Autonomy ◽  
Ethical Theories ◽  
Cleft Lip Palate ◽  
Ethical Approaches ◽  
The Right

Objective: To describe ethical approaches to the issue of pregnancy termination after prenatal detection of cleft lip ± palate. Results: Gynecologists and cleft surgeons are sometimes confronted with the demand for a pregnancy termination after ultrasound detection of an isolated cleft lip/cleft palate. In this article, we discuss different ethical theories and principles that can be applied to the dilemma at hand. We formulate recommendations that will respect the right to autonomy of the pregnant woman and at the same time acknowledge that a termination of pregnancy for a cleft lip may in most cases not be the best option. Conclusion: The recognition of each person’s right to reproductive autonomy also entails that clinicians should make sure that prospective parents are provided with up-to-date and relevant clinical information.

Maternal Drug Use and Infant Cleft Lip/Palate with Special Reference to Corticoids

2003 ◽  
Vol 40 (6) ◽  
pp. 624-628 ◽  
Author(s):  
Bengt Källén
Keyword(s):  
Drug Use ◽  
Cleft Palate ◽  
Early Pregnancy ◽  
Cleft Lip ◽  
First Trimester ◽  
Orofacial Cleft ◽  
Orofacial Clefts ◽  
Maternal Drug Use ◽  
Median Cleft ◽  
Cleft Lip Palate

Objective To study the association between maternal drug use in early pregnancy and orofacial cleft in the infant. Design Register analysis based on prospectively collected information. Patients All delivered women in Sweden July 1, 1995, through December 31, 2001. Main outcome measure Presence of orofacial cleft in infant. Results Prospective information on maternal drug use during the first trimester, as reported in early pregnancy, was studied in 1142 infants with orofacial clefts, isolated or with other malformations, excluding chromosome anomalies. Any drug use was not associated with clefts (odds ratio [OR] = 0.98, 95% confidence interval [95% CI] = 0.85 to 1.13), with isolated clefts (OR = 0.92) with isolated median cleft palate (OR = 1.03, 95% CI = 0.79 to 1.36) or with isolated cleft lip with or without cleft palate (OR = 0.86, 95% CI = 0.71 to 1.05). Reported use of multivitamins, folic acid, or B12 was not associated with a decrease in orofacial cleft risk (OR = 1.00, 95% CI = 0.63 to 1.52). ORs above 2 were seen for some drugs: sulfasalazine, naproxen, and anticonvulsants, but only a few exposed cases occurred. An association between glucocorticoid use and infant cleft was indicated and seemed to be strongest for median cleft palate. Conclusion Maternal drug use seems to play only a small role for the origin of orofacial clefts, at least in Sweden.

scholarly journals Effects of anesthesia and oral cleft types on academic achievement

2020 ◽  
Author(s):  
Saskia Gladys Nadal
Keyword(s):  
Academic Achievement ◽  
Cleft Lip ◽  
Outcome Measure ◽  
Orofacial Cleft ◽  
Oral Cleft ◽  
Percentage Point Increase ◽  
School Year ◽  
Point Increase ◽  
Different Types ◽  
Cleft Lip Palate

ObjectiveResearch looking simultaneously at the effects of anesthesia and oral cleft types on academic achievement is scarce. Available results are contradictory: some studies suggest that anesthesia exposure is responsible for underachievement, and others that responsibility falls instead on the type of orofacial cleft. This study investigates the potential compound effects of exposure to anesthesia and orofacial cleft types on the risk of academic underachievement.DesignCentre Labio Palatin Albert Coninck, Cliniques universitaires Saint-Luc, Brussels, Belgium, nationwide register-based study.SettingBirth cohort 1995-2007.PatientsTwenty-nine children with isolated orofacial clefts exposed to anesthesia.InterventionsAverage duration of exposure to anesthesia before the Certificat d Etudes de Base (CEB) exam was 382 minutes.Main Outcome Measure(s)Scores obtained by patients at the CEB exam were compared with the scores of the 6th grade Belgian general population who passed the same exam controlling for gender, school year, year they passed the exam, medical illnesses, duration of exposure to anesthesia in minutes and socioeconomic confounders.ResultsDoubling the time of anesthesia exposure produces a 17 percentage point increase in the probability that patients will underachieve. Cleft lip reduces while cleft right-left increases the duration of anesthesia exposure relative to cleft lip palate. Results do not change when anesthesia exposure only up to 4 years and socioeconomic factors are considered.ConclusionsBoth exposure to anesthesia and different types of orofacial cleft may result in underachievement at the CEB exam.

Massively Increased Caries Susceptibility in an Irf6 Cleft Lip/Palate Model

2016 ◽  
Vol 96 (3) ◽  
pp. 315-322 ◽  
Author(s):  
B. Tamasas ◽  
T.C. Cox
Keyword(s):  
Salivary Gland ◽  
Rho Gtpases ◽  
Cleft Lip ◽  
Alveolar Bone ◽  
Bacterial Colonization ◽  
Fold Increase ◽  
Oral Epithelium ◽  
Periodontal Pathogens ◽  
Direct Role ◽  
Cleft Lip Palate

Patients with cleft lip/palate (CLP) have been reported, in some studies, to exhibit an increased prevalence of caries, although the underlying cause for this increase is unknown. In genetically defined mouse models, studies of postnatal sequelae associated with CLP have been hampered by neonatal lethality. Using a conditional targeting approach, we ablated the major CLP gene Irf6 only in the late embryonic oral epithelium ( Irf6 cKO), bypassing the role of the gene in lip and palate morphogenesis and thus ensuring survival to adulthood. We report that Irf6 cKO mice present with 1) dysplastic salivary glands due to disruptions of epithelial junctional complexes, likely secondary to elevated activation of RHO GTPases, and 2) increased salivary cell proliferation. These changes result in significantly reduced saliva flow rate and buffering capacity and increased mucus acidity. A marked decrease in expression of CCL27, one of the major mucosal and skin cytokines, was found that correlated with increased bacterial colonization of the oral cavity with the cariogenic pathogen Streptococcus mutans and other bacteria. When placed on a high-sugar diet, Irf6 cKO mice show a 35-fold increase in presentation and severity of dental caries as compared with wild-type control mice. Strikingly, within the 8-wk test period, many molars extensively dissolved, and there was progressive loss of the alveolar bone, likely as a result of increased colonization of periodontal pathogens. These data provide the first mechanistic insight into the heightened caries susceptibility associated with CLP and indicate a direct role for the major CLP gene Irf6 in salivary gland development and a significant role in regulating oral immunity. Our data suggest that careful evaluation of salivary gland function and the implementation of early oral health preventive strategies are warranted to reduce the burden of dental care in this at-risk population.

scholarly journals Regulation of Rho GTPases by RhoGDIs in Human Cancers

Cells ◽  
2019 ◽  
Vol 8 (9) ◽  
pp. 1037 ◽  
Author(s):  
Cho ◽  
Kim ◽  
Baek ◽  
Kim ◽  
Lee
Keyword(s):  
Cell Migration ◽  
Cancer Progression ◽  
Anticancer Agents ◽  
Rho Gtpases ◽  
Rho Gtpase ◽  
Regulatory Mechanisms ◽  
Malignant Phenotype ◽  
Cellular Processes ◽  
Human Cancers

Rho GDP dissociation inhibitors (RhoGDIs) play important roles in various cellular processes, including cell migration, adhesion, and proliferation, by regulating the functions of the Rho GTPase family. Dissociation of Rho GTPases from RhoGDIs is necessary for their spatiotemporal activation and is dynamically regulated by several mechanisms, such as phosphorylation, sumoylation, and protein interaction. The expression of RhoGDIs has changed in many human cancers and become associated with the malignant phenotype, including migration, invasion, metastasis, and resistance to anticancer agents. Here, we review how RhoGDIs control the function of Rho GTPases by regulating their spatiotemporal activity and describe the regulatory mechanisms of the dissociation of Rho GTPases from RhoGDIs. We also discuss the role of RhoGDIs in cancer progression and their potential uses for therapeutic intervention.

scholarly journals Advantages of using toothpaste containing propolis and plant oils for gingivitis prevention and oral cavity hygiene in cleft lip/palate patients

2021 ◽  
Vol 142 ◽  
pp. 111992
Author(s):  
Agnieszka Machorowska-Pieniążek ◽  
Tadeusz Morawiec ◽  
Marcin Olek ◽  
Anna Mertas ◽  
David Aebisher ◽  
...  
Keyword(s):  
Oral Cavity ◽  
Cleft Lip ◽  
Plant Oils ◽  
Cleft Lip Palate

scholarly journals Isoform-Specific Roles of Mutant p63 in Human Diseases

Cancers ◽  
2021 ◽  
Vol 13 (3) ◽  
pp. 536
Author(s):  
Christian Osterburg ◽  
Susanne Osterburg ◽  
Huiqing Zhou ◽  
Caterina Missero ◽  
Volker Dötsch
Keyword(s):  
Cleft Lip ◽  
Ectodermal Dysplasia ◽  
Skin Development ◽  
Disease Mechanisms ◽  
Skin Fragility ◽  
Cleft Lip Palate ◽  
Life Threatening ◽  
Functional Consequences ◽  
Development And Differentiation

The p63 gene encodes a master regulator of epidermal commitment, development, and differentiation. Heterozygous mutations in the DNA binding domain cause Ectrodactyly, Ectodermal Dysplasia, characterized by limb deformation, cleft lip/palate, and ectodermal dysplasia while mutations in in the C-terminal domain of the α-isoform cause Ankyloblepharon-Ectodermal defects-Cleft lip/palate (AEC) syndrome, a life-threatening disorder characterized by skin fragility, severe, long-lasting skin erosions, and cleft lip/palate. The molecular disease mechanisms of these syndromes have recently become elucidated and have enhanced our understanding of the role of p63 in epidermal development. Here we review the molecular cause and functional consequences of these p63-mutations for skin development and discuss the consequences of p63 mutations for female fertility.

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