Dental and extra-oral clinical features in 41 patients with WNT10A gene mutations: A multicentric genotype-phenotype study.

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.

Evaluation of a modified Pulpotec endodontic approach on necrotic primary molars: a one-year follow-up.

AIM: The aim of this study was to evaluate the effectiveness of a Pulpotec modified endodontic approach on primary molars presenting necrotic pulp and furcation bone loss in a cohort of healthy children. MATERIALS AND METHODS: Forty primary necrotic molars in healthy children, aged between 4 and 6 years underwent clinical and radiological assessment. A chemomechanical removal of pulpal necrotic debris was performed with 1% sodium hypochlorite irrigation. The canals were dried and Pulpotec was inserted in the pulp chamber, and the teeth were then restored. Clinical evaluation, vertical and horizontal measurements of the bone radiolucency were performed for up to one year after the Pulpotec procedure. STATISTICAL ANALYSIS: Wilcoxon signed-rank test was applied for comparison of groups. RESULTS: In this study 67.7% of patients showed healing of bone loss, and a significant difference in height and width of the lesion was observed (respectively 80.6%, 71%; p<0.05; p<0.025). CONCLUSION: This technique can be used as an alternative to conventional endodontic treatment for primary necrotic teeth. This procedure may allow paedodontists the ability to postpone extraction of necrotic teeth in particular situations or until eruption of the first permanent molar. Necrotic primary molars presenting furcation bone lesion due to infection may be treated with this modified Pulpotec procedure. With certain caveats, this procedure will preserve the molar on the dental arch for a certain period of time. In our study this technique yielded significant clinical improvements, but the radiological improvement is considered moderate. Future investigations are warranted in order to determine the possible effects of Pulpotec on the succedaneous teeth as well as their path of eruption.

Use of mini dental implants in ectodermal dysplasia children: follow-up of three cases.

BACKGROUND: Ectodermal dysplasia is a hereditary genodermatosis characterised by a congenital defect of ectodermal structures, causing tooth malformations and anomalies. Implantology has become accepted in these subjects. However cases are often complicated by a reduction in the size of the alveolar process, making the insertion of conventional implants difficult without bone grafting. The reduced diameter of mini-implants and their ease of insertion provide an interesting solution in supporting removable or fixed prosthesis. The purpose of this paper is to report the follow-up of three cases of children (11-12 year- old) with ectodermal dysplasia in which mini-implants were used to support the prostheses. CASE REPORTS: In the first case, two mini-implants were inserted into the anterior part of the mandible for stabilising a removable denture (2 years follow-up). In the other two cases, mini- implants were inserted in the maxilla and mandible to replace missing front teeth with fixed prostheses. Patients were called for follow- up every 6 months: in the sencod case follow-up lasted 4 years in the mandible and 2 years in the maxilla; in the third case, 2 years in the maxilla and 1 year in the mandible. CONCLUSION: The use of mini-implants in children with ectodermal dysplasia can enhance aesthetics, and functional and psychosocial development.