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Background: Amelogenesis imperfecta (AI) and dentinogenesis imperfecta (DI) are two groups of genetically inherited conditions resulting in abnormal enamel and dentin formation, respectively. Children and young people may be adversely affected by these conditions, with significant reduction in oral health related quality of life. Dental management of children with AI and DI is often complex, which is exacerbated by the absence of clear referral pathways and scarce evidence-based guidelines. Method: The need for increased knowledge and peer support led to the development of a group of UK paediatric dentists with a special clinical interest in the management of children with AI and DI. Purpose: The aims of this paper are to describe the establishment of an AI/DI Clinical Excellence Network (AI/DI CEN) in paediatric dentistry including outputs and future plans, and to share our collective learning to help support others anywhere in the world advance the care of people with AI or DI. [ABSTRACT FROM AUTHOR]

Purpose: Dental bleaching in paediatric patients can be used to address discolouration of teeth due to trauma, endodontic treatment, or enamel and dentine defects. Despite being a minimally invasive and successful treatment, the use of bleaching products in children and young people remains controversial. This evaluation was designed to provide insight into the child's perspective on dental bleaching and the influence that this treatment has upon their life. Method: A dental bleaching patient reported outcome measure (PROM) was developed and piloted in 2019. Data were collected from 3 UK units (January–March 2020). Children attending these units for bleaching reviews were invited to complete the PROM. Results: Twenty seven PROM questionnaires were completed including 19 courses of external bleaching and 8 courses of internal/external bleaching. The average age was 14 years old (9–17 years). The common indications for bleaching were Amelogenesis Imperfecta, dental trauma and Molar Incisor Hypomineralisation. Patients reported improvements in their appearance (89%) and self-confidence (81%). Sensitivity was the most common side effect, reported in 63% of cases. Conclusion: This PROM supports the use of dental bleaching in children and young people when treating dental disease that causes discolouration. Bleaching not only improved the appearance of teeth, but also patients' self-confidence. Sensitivity is a common side effect and clinicians should discuss this common risk and its management with patients and their families. [ABSTRACT FROM AUTHOR]

Purpose: The burden of dental care in Amelogenesis Imperfecta (AI) has not been well described. This condition results in weak, discoloured and often sensitive teeth. Specialist paediatric care is available for AI patients in the UK, but treatment protocols and care provided are inconsistent. The aim of this study was therefore to analyse the provision of treatment and burden of care for children and families with AI across four Paediatric Dentistry centres in the UK. Methods: A retrospective evaluation of AI patient clinical records across four UK consultant-led Paediatric Dentistry centres was completed. Frequency and duration of care were recorded along with treatment and experience of inhalation sedation, local and general anaesthetic. Results: In total, 138 records were available for analysis. The average patient age at first referral was 7.7 years (range 1–16 years) and families travelled an average 21.8 miles per appointment (range 0.2–286 miles). Patients attended on average 4.5 appointments per year for 5.8 years. In total, 65.2% had experience of local anaesthetic, 27.5% inhalation sedation and 31.9% general anaesthetic. Dental treatment including restorations and extractions were commonly required on multiple teeth per patient. Conclusion: AI carries a high burden of specialist dental care to patients and families. Specialist centres are required to provide longitudinal, comprehensive care. [ABSTRACT FROM AUTHOR]

Amelogenesis imperfecta (AI) is a heterogeneous group of genetic diseases characterised by dental enamel malformation. Pathogenic variants in at least 33 genes cause syndromic or non‐syndromic AI. Recently variants in RELT, encoding an orphan receptor in the tumour necrosis factor (TNF) superfamily, were found to cause recessive AI, as part of a syndrome encompassing small stature and severe childhood infections. Here we describe four additional families with autosomal recessive hypomineralised AI due to previously unreported homozygous mutations in RELT. Three families carried a homozygous missense variant in the fourth exon (c.164C>T, p.(T55I)) and a fourth family carried a homozygous missense variant in the 11th exon (c.1264C>T, p.(R422W)). We found no evidence of additional syndromic symptoms in affected individuals. Analyses of tooth microstructure with computerised tomography and scanning electron microscopy suggest a role for RELT in ameloblasts' coordination and interaction with the enamel matrix. Microsatellite genotyping in families segregating the T55I variant reveals a shared founder haplotype. These findings extend the RELT pathogenic variant spectrum, reveal a founder mutation in the UK Pakistani population and provide detailed analysis of human teeth affected by this hypomineralised phenotype, but do not support a possible syndromic presentation in all those with RELT‐variant associated AI. [ABSTRACT FROM AUTHOR]

This paper presents a differential diagnosis of pathological changes observed in the deciduous dentition of a child aged 3-4 years, recovered from a Late Roman (c. ad 270-410) cemetery at Ancaster, England. The entire maxillary dentition exhibited areas of missing enamel and erosion of the underlying dentine, which was most pronounced at the lingual surfaces of the anterior teeth, and lingual and occlusal surfaces of the molars. Additionally, the mandibular second molars and mandibular right first molar displayed superficial enamel erosion of the buccal surfaces. Radiography indicated normal enamel density, and no further dental pathology was identified. Conditions considered in a differential diagnosis included amelogenesis imperfecta, fluorosis, perimolysis, xerostomia-related caries and early childhood caries (ECC). The nature and distribution of lesions implicates ECC - also known as 'nursing caries' or 'baby-bottle caries' - as the most probable diagnosis. The potential significance of the findings for studies of infant health and feeding practices in past populations is discussed. Copyright © 2015 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Background Amelogenesis imperfecta ( AI) is an inherited dental condition affecting enamel, which can result in significant tooth discolouration and enamel breakdown, requiring lifelong dental care. The possible impact of this condition on children and adolescents from their perspectives is not fully understood. Aims The aim of the study was to explore the impact of AI on children and adolescents through in-depth interviewing. The information derived from this was then used to construct a questionnaire to distribute to a larger cohort of AI patients. Design This research involved semistructured in-depth interviews with seven AI patients, and common themes and concepts were then identified using framework analysis. A questionnaire was developed based on the themes and subthemes identified, and completed by 40 AI patients at various stages of treatment. Results Children and adolescents with AI exhibited concerns regarding aesthetics and function. Patients also expressed a high level of concern regarding comments by other people and self-consciousness associated with this. A small number of AI patients highlighted the effect of their dental treatment and health on their personal life. Conclusion The results indicate that there are marked impacts on children and adolescents as a result of AI, including aesthetics, function, and psychosocial. [ABSTRACT FROM AUTHOR]