WNT10A gene encodes a canonical wingless pathway signaling molecule involved in cell fate specification as well as morphogenetic patterning of the developing ectoderm, nervous system, skeleton, and tooth. In patients, WNT10A mutations are responsible for ectodermal-derived pathologies including isolated hypo-oligodontia, tricho-odonto-onycho-dermal dysplasia and Schöpf-Schulz-Passarge syndrome (SSPS). Here we describe the dental, ectodermal, and extra-ectodermal phenotypic features of a cohort of 41 patients from 32 unrelated families. Correlations with WNT10A molecular status (heterozygous carrier, compound heterozygous, homozygous) and patient's phenotypes were performed. Mild to severe oligodontia was observed in all patients bearing biallelic WNT10A mutations. However, patients with compound heterozygous mutations presented no significant difference in phenotypes compared with homozygous individuals. Anomalies in tooth morphology were frequently observed with heterozygous patients displaying hypodontia. No signs of SSPS, especially eyelids cysts, were detected in our cohort. Interestingly, extra-ectodermal signs consisted of skeletal, neurological and vascular anomalies, the latter suggesting a wider phenotypic spectrum associated with WNT10A mutations. Indeed, the Wnt pathway plays a crucial role in skeletal development, lipid metabolism, and neurogenesis, potentially explaining patient's clinical manifestations.
AimsSevere oligodontia is one of the most important symptoms in children with hypohidrotic ectodermal dysplasia (HED). The growth of the maxilla is a key consideration in restoring their mouth. The aim of this study was to evaluate the transversal maxillary sutural growth, after passive masticatory stimulation, in HED children. We also thought to assess the efficiency and functional outcome of the proposed propriocep-tive passive expansion (PPE) prosthetic device.Materials and methodsWe studied 13 children (age 6-11 years) suffering from HED with severe oligodontia. Their maxilla was restored by a PPE device formed from two parts and joined by a passive slide system. Distance between the two parts was noted at the anterior and posterior regions at each control visit over an average of 23 months. We also conducted and filled a satisfaction questionnaire over the same period.We tested the hypothesis that the posterior expansion is greater than the anterior expansion (one-tailed Student’s t-test with p-value <0.05). Best-fit linear and quadratic models were used to explore the relationship between age, duration of observation, and the rate of growth.ResultsThe average opening of the device was 2.27 mm in the anterior region and 2.96 mm in the posterior region. The questionnaire response was positive for all children. There are no significant linear or quadratic relationships between the data at the 5% significance level. The posterior expansion is greater than the anterior expansion at the 5% significance level (p-value 0.000394).LimitationsFurther studies are mandatory to assess the reliability of our particular intervention and treatment modalities for these cases.ConclusionThe PPE device, we propose, assures function and esthetics in the long- term. It enhances stimulation by a passive way that leads to physiological growth of the palatal suture.Clinical significanceUsing this PPE device to restore the maxilla in children with HED promotes physiological growth. The passive nature of this prosthesis helps by eliminating the need for any changes or replacement over time.How to cite this articleSfeir E, Nahass MG, Mourad A. Evaluation of Masticatory Stimulation Effect on the Maxillary Transversal Growth in Ectodermal Dysplasia Children. Int J Clin Pediatr Dent 2017;10(1):55-61.
AimTwo eruption surgical techniques are commonly described for the treatment of upper impacted central incisors (ICIs): Open and closed. Currently, the closed-eruption surgical technique (CEST) is the most commonly used, as it allows for the best esthetic and periodontal results.The aim of this study was to determine the effect of traction discontinuation on maxillary central incisor sulcal depth and alveolar bone ridge levels compared with contralateral incisors, when CEST is used.Materials and methodsOur study involved 28 unilateral impacted maxillary central incisors treated by CEST. Thirteen teeth were subjected to traction interruption for a month at the time of emergence of the crown, while 15 teeth underwent continuous traction. One year after treatment, periapical digital X-rays, anterosuperior cone beam computerized tomography (CBCT) scanning, and periodontal probing of the ICIs and contralateral central incisors (CCIs) were performed. Student’s t-test was used to study whether a statistically significant difference between continuous and interrupted tractions takes place while using the CCI measurements as reference.ResultsThere was a statistically significant difference between the two techniques only for the following measurements: Mesial probing (p-value 0.039352), labial bone level (p-value 2.58E-08), and palatal bone level (p-value 2.56E-06).LimitationsA larger sample size and longer term follow-up are needed to draw more robust conclusions.ConclusionA temporary discontinuation during traction of the tooth appears to positively impact treatment outcome on ICIs.Clinical significance• The CEST leads to the best periodontal status for ICIs.• The discontinuation of traction at the emergence of the tooth allows the supracrestal fibers to insert into the cement in a proper way.How to cite this article: Sfeir E, Gholmieh M, Skaf Z, Mourad A. Alveolar Bone and Epithelial Attachment Status following Two Different Closed-eruption Surgical Techniques for Impacted Maxillary Central Incisors. Int J Clin Pediatr Dent 2018;11(4):317-322.
AimThis study was carried out to assess the reliability of measurements of the remaining dentin thickness under deep carious lesions as estimated from digital radiographs. The goal is to allow clinicians to correlate the radiographic measurement to the exact value of the remaining dentin thickness. The results obtained will be tested further in a study that will evaluate the histopathologic pulpal state according to the caries’ lesion depth.Materials and methodsThe study was conducted in the Pediatric Dentistry Department at the Lebanese University, in collaboration with the research platform of the same university. Fifty deciduous molars with deep caries on proximal surfaces liable to extraction were collected. Before extraction, a digital in vivo periapical radiograph was taken, followed by manual excavation of the caries. After excavation, another radiograph was taken before the tooth was sectioned through the deepest site of the lesion. Another radiograph was then obtained for each tooth fragment. To evaluate the exact thickness of the remaining dentin, each fragment was measured on a histologic macropho-tograph. The measurements were then compared statistically using a paired-samples t-test, and a correlation was sought.ResultsNo significant difference was observed in the radiographs between the measurement of the remaining dentin thickness before and after the excavation of caries. In contrast, the radiographic measurements of remaining dentin thickness were underestimated by an average of 20% compared with those made with macrophotographs.LimitationsInterpretation of radiographs varies from one practitioner to another and is a function of the operator’s visual acuity.ConclusionMeasuring the residual dentin thickness on a radiograph underestimates the actual thickness by about 20%. Further studies are needed to confirm these results.Clinical significanceOur results indicate that remaining dentin thickness is greater in reality than is shown on a radiograph. This information can help clinicians to refine their diagnoses and treatment plans.How to cite this article: Berbari R, Khairallah A, Kazan HF, Ezzedine M, Bandon D, Sfeir E. Measurement Reliability of the Remaining Dentin Thickness below Deep Carious Lesions in Primary Molars. Int J Clin Pediatr Dent 2018;11(1):23-28.
Background. The timing of eruption of permanent teeth can be a precious help to the pediatric practitioner in identifying an appropriate treatment plan. Usually, it presents a more precise sign of an early or late growth of the child. Aims. It is to determine whether the timing of the permanent teeth eruption in Lebanese pediatric population can be considered as standard Lebanese values, since no other study was previously performed. Materials and Methods. A cross-sectional study is conducted, and the clinical emergence data are collected for a sample of 2317 Lebanese children aged between 5.5 years and 13 years (1129 girls and 1188 boys) from different socioeconomic groups in rural and urban communities spread over different regions in Lebanon. The study investigates dental emergence patterns. Results. Statistical analysis is performed with the SPSS Software. A confidence interval of 95% and significance level of 5% are adopted. The trend is for males to begin their permanent teeth eruption later than girls. Emergence of all permanent mandibular teeth is earlier than maxillary ones. Symmetry is found between the right and left teeth in the maxilla, as well as in the mandibula. Conclusion. In this study, the results indicate that premolars and second permanent molars erupt earlier in the Lebanese children compared to children of other countries reflected in several studies.
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