Lack of assent to dental examination in children with intellectual disabilities: Dentists' practices in Europe and ethical issues.

AIM: (1) To determine the repartition of criteria which can be considered as marks of lack of assent by the child with intellectual disabilities from the dentist's point of view and whether that influences the decision to examine the patient or not. (2) To explain the decision of practitioners and determine the ethical implications of these practices. METHODS: An anonymous and structured questionnaire was distributed online using the scenario of a 9-year-old child with moderate cognitive impairment with eight different oppositional behaviours. The practitioners were asked about their perception of the patient's lack of assent and about their decision to perform the dental examination or not. RESULTS: The proportion of them who performed a dental examination despite the patient's refusal represented between 13% and 28.8% of the population of respondents. CONCLUSION: There was an ambivalence among the practitioners who carried out a dental consultation when children were uncooperative. They adopted a teleological point of view. It calls for us to reflect on the ethical principles of autonomy and beneficence.

Oral findings in children with congenital cholestatic disease: A systematic review of case reports and case series.

Multiple causes of congenital neonatal cholestasis have been identified, and are classified as extrahepatic or intrahepatic. Biliary atresia (BA), Alagille syndrome (AGS), and progressive familial intrahepatic cholestasis (PFIC) are the most common of these. Many factors associated with cholestatic diseases are known to degrade the oral health of these children. What are the oral manifestations associated with these diseases in the pediatric population? The aim of this article was to evaluate the impact of congenital cholestasis on oral health in pediatric patients. A systematic review of case reports and case series was carried out in PubMed, the Cochrane Library, and the Web of Science to identify relevant articles in French and English published up to April 2022. The review included 19 studies, 16 case reports, and three case series. Only studies dealing with BA and AGS were found. These studies showed an impact on jaw morphology, dental structure, and periodontal health. The facial dysmorphism observed in AGS was specific. Exposure to high levels of bilirubin during the period of dental calcification led to particular coloration. Regarding periodontal status, gingival inflammation was common in these patients, probably resulting from the use of certain treatment-associated drugs and poor oral hygiene. Cohort studies are needed to confirm the classification of these children as being at high individual risk of caries. Many major oral manifestations are found in children with AGS and BA, confirming the need to include a dentist in the care team of patients with congenital cholestatic disease as early as possible. It appears necessary to carry out individual prospective studies of each phenotype in order to confirm and better describe the oral impact of these cholestatic diseases and provide adequate medical care.

Early childhood caries: Detection, prevention, and referral. A questionnaire study of general medical practitioners and pediatricians in the south of France.

INTRODUCTION: Early childhood caries (ECC) is an illness defined as the presence of one or more decayed, missing after caries, or filled tooth surfaces in any primary tooth in a child under the age of 6 years. It has a negative impact on the physical and the psychological development of children. General medical practitioners (GPs) and pediatricians, the first professionals involved in the follow-up of young children, are on the frontline of detecting and referring patients with caries or individual high-risk of carious lesions. The aims of this study were (1) to assess the current state of knowledge of pediatricians and GPs in the south of France regarding ECC detection and prevention, and (2) to understand whether there are difficulties in referring young patients for the early detection of carious lesions. METHODS: A semi-structured questionnaire was circulated to GPs and pediatricians working in the Provence-Alpes-Côte d'Azur region (France). The questionnaire was divided into three parts related to the characteristics of the participants, to the current skill and knowledge of practitioners in detecting ECC (using clinical vignettes) and advising preventive measures, and to the dental examination and any difficulties in referring patients. RESULTS: There were 97 participants in the study. Many oral hygiene measures were known but only just over half of the dietary risk factors were recognized. Participants seemed to be involved in detecting ECC, the majority very often examined teeth during their consultations. Practitioners recognized a carious lesion in only one of two cases. The lack of knowledge of the recommended age for the first consultation could be a barrier in referring patients to a dentist, for which pain remains the main reason for referral. CONCLUSION: GPs and pediatricians should play a key role in the detection and prevention of ECC. Participants showed great interest in the topic of oral health. For better management, it would be beneficial to provide training resources with quick and efficient access to information.

Amelogenesis imperfecta: Next-generation sequencing sheds light on Witkop's classification.

Amelogenesis imperfecta (AI) is a heterogeneous group of genetic rare diseases disrupting enamel development (Smith et al., Front Physiol, 2017a, 8, 333). The clinical enamel phenotypes can be described as hypoplastic, hypomineralized or hypomature and serve as a basis, together with the mode of inheritance, to Witkop's classification (Witkop, J Oral Pathol, 1988, 17, 547-553). AI can be described in isolation or associated with others symptoms in syndromes. Its occurrence was estimated to range from 1/700 to 1/14,000. More than 70 genes have currently been identified as causative. Objectives: We analyzed using next-generation sequencing (NGS) a heterogeneous cohort of AI patients in order to determine the molecular etiology of AI and to improve diagnosis and disease management. Methods: Individuals presenting with so called "isolated" or syndromic AI were enrolled and examined at the Reference Centre for Rare Oral and Dental Diseases (O-Rares) using D4/phenodent protocol (www.phenodent.org). Families gave written informed consents for both phenotyping and molecular analysis and diagnosis using a dedicated NGS panel named GenoDENT. This panel explores currently simultaneously 567 genes. The study is registered under NCT01746121 and NCT02397824 (https://clinicaltrials.gov/). Results: GenoDENT obtained a 60% diagnostic rate. We reported genetics results for 221 persons divided between 115 AI index cases and their 106 associated relatives from a total of 111 families. From this index cohort, 73% were diagnosed with non-syndromic amelogenesis imperfecta and 27% with syndromic amelogenesis imperfecta. Each individual was classified according to the AI phenotype. Type I hypoplastic AI represented 61 individuals (53%), Type II hypomature AI affected 31 individuals (27%), Type III hypomineralized AI was diagnosed in 18 individuals (16%) and Type IV hypoplastic-hypomature AI with taurodontism concerned 5 individuals (4%). We validated the genetic diagnosis, with class 4 (likely pathogenic) or class 5 (pathogenic) variants, for 81% of the cohort, and identified candidate variants (variant of uncertain significance or VUS) for 19% of index cases. Among the 151 sequenced variants, 47 are newly reported and classified as class 4 or 5. The most frequently discovered genotypes were associated with MMP20 and FAM83H for isolated AI. FAM20A and LTBP3 genes were the most frequent genes identified for syndromic AI. Patients negative to the panel were resolved with exome sequencing elucidating for example the gene involved ie ACP4 or digenic inheritance. Conclusion: NGS GenoDENT panel is a validated and cost-efficient technique offering new perspectives to understand underlying molecular mechanisms of AI. Discovering variants in genes involved in syndromic AI (CNNM4, WDR72, FAM20A … ) transformed patient overall care. Unravelling the genetic basis of AI sheds light on Witkop's AI classification.

Microabrasion in the management of enamel discolorations in paediatric dentistry: a systematic review.

Structural abnormalities of the anterior teeth could be aesthetically compromising in young patients. The dentist must provide solutions while preserving dental tissue. Microabrasion approach can be a solution. We conducted a systematic literature review to evaluate whether microbrasion treatment in paediatric dentistry can improve aesthetic in cases of pre- or post-eruptive discolorations on tooth enamel. 741 articles published up to September 2021 were selected from 3 databases using the key word "microabrasion". 11 prospective studies including 6 randomized were relevant to the inclusion criteria. Microabrasion appears to be an effective and reliable technique for the management of pre and post enamel discoloration in paediatric dentistry, especially in fluorosis. More high-powered, well-conducted randomized studies with complete evaluation criteria are needed for other types of spots. Standardization of criteria for assessing treatment success and of the protocol required should be explored.

Oral Care in Kindler Syndrome: 7-Year Follow-up of 2 Brothers.

BACKGROUND: Kindler poikiloderma is an inherited autosomal genodermatosis characterized by blistering of the epidermis and mucosae. Its prevalence is unknown. CASE REPORT: We monitored two brothers suffering from this pathology. Oral manifestations mainly take the form of periodontal lesions. In our patients we noted gingivitis progressing to periodontitis as follow-up care was not effective. We also diagnosed enamel hypoplasia, described more rarely in this pathology. CONCLUSION: Periodontitis in Kindler Syndrome responds to maintenance therapy, but the absence of surveillance is penalized by a deterioration in periodontal condition and complication of management. All restorative, endodontic, surgical, periodontal and orthodontic treatments should be performed with appropriate precautions.

Inequalities in treatment planning for children with intellectual disabilities: A questionnaire study of dentists in Europe.

BACKGROUND: Children with intellectual disabilities experience major inequality in the field of oral health, including a higher number of extracted teeth. The literature explains this difference in terms of higher levels of dental disease but does not mention the possibility of inequality in the treatment options offered these children. AIM: The aim is to investigate whether the same treatment options are offered by practitioners to children with and without intellectual disability in equivalent clinical circumstances. DESIGN: A scenario involving a clinical dental situation was developed, with one varying parameter: the patient described was a child with or without cerebral palsy. RESULTS: One hundred and thirty-nine dental specialists from France and Europe were recruited. A large number of practitioners (68%) chose the same treatment for both patients, but 32% declared modifying the dental treatment planning in the case of the child with cerebral palsy. The most frequently chosen treatment for the scenario of irreversible pulpitis for the child without disability was conservative endodontic treatment (73%) whereas the most frequently chosen treatment for the child with intellectual disability was tooth extraction (54%). DISCUSSION: These results are discussed in terms of beneficence, fear of restorative failure, lack of guidelines, practitioner experience and the implications for equity in healthcare.

Effectiveness of Resin-Based Sealants with and without Fluoride Placed in a High Caries Risk Population: Multicentric 2-Year Randomized Clinical Trial.

A split-mouth randomized clinical trial was carried out to assess the effectiveness of sealants in preventing carious ICDAS (International Caries Detection and Assessment System) 3-6 lesions within a 2-year follow-up. We evaluated the effectiveness of 2 types of resin-based sealants, with and without fluoride, their retention rates, and the caries risk factors related to their outcomes. The study included 663 tooth pairs in 400 children (aged 5-15 years) considered to be at high individual caries risk (ICR) and presenting permanent molars free of caries or affected by ICDAS 1-2 lesions. In the first randomization, molars were either randomized to the treatment group receiving a dental sealant or the control group (nontreatment), and in the second randomization the sealant material to be used in the intervention group was selected. Uni- and multivariate Cox proportional hazard models were employed. At the 2-year follow-up, 483 tooth pairs were assessed: sealed molars had 83% (adjusted HR = 0.17; 95% CI: 0.15-0.20) less risk of developing ICDAS 3-6 lesions than molars without sealant. The magnitude of the protective effect was lower among teeth with ICDAS 1-2 lesions or with occlusal deep fissures than without. If the total retention rate of sealants was 70% at 2 years, sealant loss was not associated with the risk of caries (HR = 1.29, 95% CI: 0.92-1.79, p = 0.14). Sealants allow the prevention of new ICDAS 3-6 lesions or progression of noncavitated carious lesions in children at high ICR, and the effect of the sealant was similar regardless of whether it contained fluoride or not.

Ethical issues and dentists' practices with children with intellectual disability: A qualitative inquiry into a local French health network.

BACKGROUND: The provision of dental care for children with intellectual disability raises many ethical questions. OBJECTIVE: The aim of this qualitative study was to explore approaches to dental treatment in an anxious child with intellectual disability and the ethical dilemmas that ensue. METHODS: Semi-structured interviews were conducted between February and May 2012. A clinical scenario was used to establish a starting point for a discussion of the clinical approach and lead to an ethical reflection. Four topics were discussed: first contact with the patient, information, attitude towards the patient and outcome from the practitioner's viewpoint. The coding procedure used thematic content analysis. RESULTS: Most practitioners fetched the patient from the waiting room personally, greeted them, gave them special attention, and either began the consultation at once, or used distraction to relax the patient. Verbal language and tell-show-do were most often used to provide information. Anxiety and pain were evaluated using parental assessment and standardized scales. A reassuring attitude was adopted. An ethical dilemma arose if the patient refused care or had to be restrained. Practitioners reported sacrificing ethical values (patient autonomy, beneficence and non-maleficence) when making a clinical decision. CONCLUSIONS: There is a wide range of practices but no consensus. This study shed some light on the different perspectives of dentists, most of whom adopted a utilitarianist viewpoint. In this context, ethical reflection is necessary to avoid a detached attitude or, worse, abuse. Further study would enrich this reflection.

A targeted next-generation sequencing assay for the molecular diagnosis of genetic disorders with orodental involvement.

BACKGROUND: Orodental diseases include several clinically and genetically heterogeneous disorders that can present in isolation or as part of a genetic syndrome. Due to the vast number of genes implicated in these disorders, establishing a molecular diagnosis can be challenging. We aimed to develop a targeted next-generation sequencing (NGS) assay to diagnose mutations and potentially identify novel genes mutated in this group of disorders. METHODS: We designed an NGS gene panel that targets 585 known and candidate genes in orodental disease. We screened a cohort of 101 unrelated patients without a molecular diagnosis referred to the Reference Centre for Oro-Dental Manifestations of Rare Diseases, Strasbourg, France, for a variety of orodental disorders including isolated and syndromic amelogenesis imperfecta (AI), isolated and syndromic selective tooth agenesis (STHAG), isolated and syndromic dentinogenesis imperfecta, isolated dentin dysplasia, otodental dysplasia and primary failure of tooth eruption. RESULTS: We discovered 21 novel pathogenic variants and identified the causative mutation in 39 unrelated patients in known genes (overall diagnostic rate: 39%). Among the largest subcohorts of patients with isolated AI (50 unrelated patients) and isolated STHAG (21 unrelated patients), we had a definitive diagnosis in 14 (27%) and 15 cases (71%), respectively. Surprisingly, COL17A1 mutations accounted for the majority of autosomal-dominant AI cases. CONCLUSIONS: We have developed a novel targeted NGS assay for the efficient molecular diagnosis of a wide variety of orodental diseases. Furthermore, our panel will contribute to better understanding the contribution of these genes to orodental disease. TRIAL REGISTRATION NUMBERS: NCT01746121 and NCT02397824.

Dental occlusion and postural control in adults.

We studied the influence of a dental occlusion perturbation on postural control. The tests were performed in three dental occlusion conditions: (Rest Position: no dental contact, Maximal Intercuspal Occlusion: maximal dental contact, and Thwarted Laterality Occlusion: simulation of a dental malocclusion) and four postural conditions: static (stable platform) and dynamic (unstable platform), with eyes open and eyes closed. A decay of postural control was noted between the Rest Position and Thwarted Laterality Occlusion conditions with regard to average speed and power indexes in dynamic conditions and with eyes closed. However, the head position and stabilization were not different from those in the other experimental conditions, which means that the same functional goal was reached with an increase in the total energetic cost. This work shows that dental occlusion differently affects postural control, depending on the static or dynamic conditions. Indeed, dental occlusion impaired postural control only in dynamic postural conditions and in absence of visual cues. The sensory information linked to the dental occlusion comes into effect only during difficult postural tasks and its importance grows as the other sensory cues become scarce.

Evaluation of safe and effective administration of nitrous oxide after a postgraduate training course.

BACKGROUND: Conscious sedation is used in dentistry to improve access and quality of care in patients who have difficulty coping with treatment. The aim of this prospective study was to describe a postgraduate training course in conscious sedation for dentists, with specific evaluation of the safe and effective administration of a 50% nitrous oxide in oxygen premix. METHODS: 45 practitioners were trained between 2002 and 2004. They carried out 826 sessions of inhalation sedation in 662 patients. The clinical competency of this group was compared with an expert group. RESULTS: There was no difference between trainees and experts in ability to complete the planned dental treatment under sedation (89.6% vs 93.2%). Trainees were less successful than experts for patients with intellectual disability (87.4% vs 94.2%, p < 0.01). For both groups, the degree of cooperation improved between initial induction and each perioperative step (Wilcoxon test, p < 0.01). However, for trainees, Venham behaviour scores varied with the type of patient (Kruskal Wallis test, p < 0.001). No major adverse effects were recorded. Trainees reported more minor adverse effects than experts (13% vs. 5.3% respectively, Fisher exact test, p < 0.001) CONCLUSION: The trainee practitioners provided effective and safe inhalation sedation. This challenges the current French restriction of the 50% nitrous oxide in oxygen premix to the hospital setting. Further emphasis is required on the teaching of behaviour management skills for patients with intellectual disability.

Sedation with 50% nitrous oxide/oxygen for outpatient dental treatment in individuals with intellectual disability.

Persons with intellectual disability have difficulty in cooperating with outpatient care, and many are referred for general anaesthesia. Intellectual disability has traditionally been a contraindication for conscious sedation. We evaluated the behavioural impact, effectiveness, and tolerance of sedation in this population using a fixed 50% nitrous oxide/oxygen mixture as a single agent. We used dental treatment as a model of outpatient care; 349 patients (192 males, 157 females; mean age 22y [SD 14]; range 3-81y) were recruited over a 12-month period at seven centres. Sedation was deemed successful if planned dental treatment was completed. Behaviour was scored with the modified Venham scale. Out of 605 sessions, 91.4% were successful. No serious adverse effects occurred. Minor adverse events (such as nausea) occurred in 10.1% of sessions. We conclude that the use of safe and effective conscious sedation may reduce the indications for general anaesthesia.

Retention of resin-based pit and fissure sealants: A systematic review.

The aim of the present study was to perform a systematic review on the retention of resin-based sealants (RBSs) according to the material used and the clinical procedure. An electronic search in MEDLINE, EMBASE, Cochrane library and SCOPUS was completed by a hand search in conference proceedings. One hundred and twenty-four studies were identified, 31 of which were included. The retention rate of auto-polymerized and light-cured RBSs did not differ significantly. Light-cured RBSs had a significantly higher retention rate than fluoride-containing light-cured RBSs at 48 months (RR = 0.80, 95% CI: 0.72-0.89) and more. Concerning the clinical procedure, the scarcity of well-conducted studies made judgement difficult, except for the isolation stage. If using a rubber dam did not affect retention of auto-polymerized RBSs, it did for fluoride-containing light-cured RBSs (RR = 2.03, 95% CI: 1.51-2.73).