Specialist and transitional care provision for amelogenesis imperfecta: a UK-wide survey.

Background Amelogenesis imperfecta (AI) can be challenging to manage due to the complexity and variation of presentation. Clear care pathways between general practice, specialist paediatric dentistry and adult services are required.Aim To assess the provision of specialist care and transitional care arrangements for paediatric patients with AI in the UK.Method An online survey was disseminated to members of the British Society of Paediatric Dentistry in January 2020. Descriptive analysis was used to interpret the quantitative and qualitative results.Results In total, 115 clinicians across all four nations participated. Most respondents (54%; n = 66), were based in the hospital dental service. Overall, 29% (n = 33) were consultants and 24% (n = 28) were specialists in paediatric dentistry. The most common patient age group seen was 6-12 years old. No clear AI referral pathway into specialist care was reported by 49% (n = 47). A clear transitional care pathway was deemed not to exist by 77% (n = 72), with 85.9% (n = 73) indicating a need. Qualitative analysis themes included 'unclear care pathways' and 'specialist care access problems'.Conclusion Access to specialist paediatric dental care and transition to adult services is not readily available throughout the UK for AI patients. There is a clear need to establish and improve existing pathways.

The voice of the child in dental care plans: a service evaluation.

Introduction The voice of the child (VOC) is vitally important in the dental setting as 'patient participation' is a key clinical governance pillar. A recent Care Quality Commission review of Bradford District Care NHS Foundation Trust identified 'the voice of the patient was not always clear in the care plans stored on the electronic system'.Aims To determine if Bradford District Care NHS Foundation Trust's Community Dental Service was recording and acting upon the voice of children and young people (CYP) regarding their dental care.Methods Retrospective case note analysis of CYP who had completed a course of treatment between January and March 2019. Patient notes were reviewed to determine if key indicators of the VOC were included. Verbal communication, communication barriers and evidence treatment options were discussed with CYP wherever feasible and whether comments by CYP were used to tailor their care.Results Review of 200 patients aged 4-16 years old found that no CYP notes recorded all key VOC indicators. In total, 36% of dentists recorded if the CYP had verbal communication or otherwise; 26% of notes evidenced treatment options had been discussed; and 11% of notes evidenced that CYP comments were used to tailor their care.Discussion Presentation of results to clinicians locally highlighted the importance of the VOC and the need to improve documentation. Subsequently, implementation of a modified clinical note pro forma has taken place which includes VOC indicators, thus providing evidence that CYP are being listened to and, where feasible, their opinions used to tailor care.Conclusion The VOC was not routinely being documented in clinical notes, thus it was difficult to prove/disprove if the VOC was being listened to.

Spectrum of pathogenic variants and founder effects in amelogenesis imperfecta associated with MMP20.

Amelogenesis imperfecta (AI) describes a heterogeneous group of developmental enamel defects that typically have Mendelian inheritance. Exome sequencing of 10 families with recessive hypomaturation AI revealed four novel and one known variants in the matrix metallopeptidase 20 (MMP20) gene that were predicted to be pathogenic. MMP20 encodes a protease that cleaves the developing extracellular enamel matrix and is necessary for normal enamel crystal growth during amelogenesis. New homozygous missense changes were shared between four families of Pakistani heritage (c.625G>C; p.(Glu209Gln)) and two of Omani origin (c.710C>A; p.(Ser237Tyr)). In two families of UK origin and one from Costa Rica, affected individuals were homozygous for the previously reported c.954-2A>T; p.(Ile319Phefs*19) variant. For each of these variants, microsatellite haplotypes appeared to exclude a recent founder effect, but elements of haplotype were conserved, suggesting more distant founding ancestors. New compound heterozygous changes were identified in one family of the European heritage: c.809_811+12delinsCCAG; p.(?) and c.1122A>C; p.(Gln374His). This report further elucidates the mutation spectrum of MMP20 and the probable impact on protein function, confirms a consistent hypomaturation phenotype and shows that mutations in MMP20 are a common cause of autosomal recessive AI in some communities.

One-to-one oral hygiene advice provided in a dental setting for oral health.

BACKGROUND: Effective oral hygiene measures carried out on a regular basis are vital to maintain good oral health. One-to-one oral hygiene advice (OHA) within the dental setting is often provided as a means to motivate individuals and to help achieve improved levels of oral health. However, it is unclear if one-to-one OHA in a dental setting is effective in improving oral health and what method(s) might be most effective and efficient. OBJECTIVES: To assess the effects of one-to-one OHA, provided by a member of the dental team within the dental setting, on patients' oral health, hygiene, behaviour, and attitudes compared to no advice or advice in a different format. SEARCH METHODS: Cochrane Oral Health's Information Specialist searched the following databases: Cochrane Oral Health's Trials Register (to 10 November 2017); the Cochrane Central Register of Controlled Trials (CENTRAL; 2017, Issue 10) in the Cochrane Library (searched 10 November 2017); MEDLINE Ovid (1946 to 10 November 2017); and Embase Ovid (1980 to 10 November 2017). The US National Institutes of Health Ongoing Trials Register (ClinicalTrials.gov) and the World Health Organization International Clinical Trials Registry Platform were also searched for ongoing trials (10 November 2017). No restrictions were placed on the language or date of publication when searching the electronic databases. Reference lists of relevant articles and previously published systematic reviews were handsearched. The authors of eligible trials were contacted, where feasible, to identify any unpublished work. SELECTION CRITERIA: We included randomised controlled trials assessing the effects of one-to-one OHA delivered by a dental care professional in a dental care setting with a minimum of 8 weeks follow-up. We included healthy participants or participants who had a well-defined medical condition. DATA COLLECTION AND ANALYSIS: At least two review authors carried out selection of studies, data extraction and risk of bias independently and in duplicate. Consensus was achieved by discussion, or involvement of a third review author if required. MAIN RESULTS: Nineteen studies met the criteria for inclusion in the review with data available for a total of 4232 participants. The included studies reported a wide variety of interventions, study populations, clinical outcomes and outcome measures. There was substantial clinical heterogeneity amongst the studies and it was not deemed appropriate to pool data in a meta-analysis. We summarised data by categorising similar interventions into comparison groups.Comparison 1: Any form of one-to-one OHA versus no OHAFour studies compared any form of one-to-one OHA versus no OHA.Two studies reported the outcome of gingivitis. Although one small study had contradictory results at 3 months and 6 months, the other study showed very low-quality evidence of a benefit for OHA at all time points (very low-quality evidence).The same two studies reported the outcome of plaque. There was low-quality evidence that these interventions showed a benefit for OHA in plaque reduction at all time points.Two studies reported the outcome of dental caries at 6 months and 12 months respectively. There was very low-quality evidence of a benefit for OHA at 12 months.Comparison 2: Personalised one-to-one OHA versus routine one-to-one OHAFour studies compared personalised OHA versus routine OHA.There was little evidence available that any of these interventions demonstrated a difference on the outcomes of gingivitis, plaque or dental caries (very low quality).Comparison 3: Self-management versus professional OHAFive trials compared some form of self-management with some form of professional OHA.There was little evidence available that any of these interventions demonstrated a difference on the outcomes of gingivitis or plaque (very low quality). None of the studies measured dental caries.Comparison 4: Enhanced one-to-one OHA versus one-to-one OHASeven trials compared some form of enhanced OHA with some form of routine OHA.There was little evidence available that any of these interventions demonstrated a difference on the outcomes of gingivitis, plaque or dental caries (very low quality). AUTHORS' CONCLUSIONS: There was insufficient high-quality evidence to recommend any specific one-to-one OHA method as being effective in improving oral health or being more effective than any other method. Further high-quality randomised controlled trials are required to determine the most effective, efficient method of one-to-one OHA for oral health maintenance and improvement. The design of such trials should be cognisant of the limitations of the available evidence presented in this Cochrane Review.

School based oral health education.

Data sourcesThe methodology followed the Cochrane Handbook for systematic reviews of interventions with MEDLINE/PubMed, CENTRAL, Embase and LILACS databases searched. Publication date was limited to 1995-2015 with no restriction on language.Study selectionTwo independent reviewers selected randomised controlled clinical trials involving oral health education provided by a dental care professional to children aged between five and 18 years old within a school setting. Eligible studies were those which had outcomes including caries, plaque accumulation, gingivitis, toothache or tooth loss. Randomisation was at group (school and/or classroom) or individual level. The control groups were not provided with an educational programme on oral health, however they could have been given an action that belonged to the school's curricular framework.Data extraction and synthesisThe title and abstract of each study was reviewed and critically assessed by two independent reviewers. Risk of bias was assessed using the Cochrane Handbook. Studies where the data of interest were presented in charts or were of dichotomous data were not included in meta-analysis.ResultsTwelve studies were included in this systematic review. Five studies showed plaque level reduction in the intervention groups and two studies found no effect of the interventions on gingivitis. There was insufficient evidence on effectiveness of the interventions in reducing dental caries.ConclusionsTraditional oral health educational actions were effective in reducing plaque in the short-term, but not gingivitis. There was no long-term evidence regarding the effectiveness of traditional oral health educational actions in the school environment on preventing plaque accumulation, gingivitis and dental caries in schoolchildren.Acknowledgements This study was funded in part by the Coordination for the Improvement of Higher Education Personnel (CAPES).

A Fourth KLK4 Mutation Is Associated with Enamel Hypomineralisation and Structural Abnormalities.

"Amelogenesis imperfecta" (AI) describes a group of genetic conditions that result in defects in tooth enamel formation. Mutations in many genes are known to cause AI, including the gene encoding the serine protease, kallikrein related peptidase 4 (KLK4), expressed during the maturation stage of amelogenesis. In this study we report the fourth KLK4 mutation to be identified in autosomal recessively-inherited hypomaturation type AI, c.632delT, p.(L211Rfs*37) (NM_004917.4, NP_004908.4). This homozygous variant was identified in five Pakistani AI families and is predicted to result in a transcript with a premature stop codon that escapes nonsense mediated decay. However, the protein may misfold, as three of six disulphide bonds would be disrupted, and may be degraded or non-functional as a result. Primary teeth were obtained from one affected individual. The enamel phenotype was characterized using high-resolution computerized X-ray tomography (CT), scanning electron microscopy (SEM), energy dispersive X-ray spectroscopy (EDX), and microhardness testing (MH). Enamel from the affected individual (referred to as KLK4 enamel) was hypomineralised in comparison with matched control enamel. Furthermore, KLK4 inner enamel was hypomineralised compared with KLK4 outer enamel. SEM showed a clear structural demarcation between KLK4 inner and outer enamel, although enamel structure was similar to control tissue overall. EDX showed that KLK4 inner enamel contained less calcium and phosphorus and more nitrogen than control inner enamel and KLK4 outer enamel. MH testing showed that KLK4 inner enamel was significantly softer than KLK4 outer enamel (p < 0.001). However, the hardness of control inner enamel was not significantly different to that of control outer enamel. Overall, these findings suggest that the KLK4 c.632delT mutation may be a common cause of autosomal recessive AI in the Pakistani population. The phenotype data obtained mirror findings in the Klk4-/- mouse and suggest that KLK4 is required for the hardening and mineralization of the inner enamel layer but is less essential for hardening and mineralization of the outer enamel layer.

Defects in the acid phosphatase ACPT cause recessive hypoplastic amelogenesis imperfecta.

We identified two homozygous missense variants (c.428C>T, p.(T143M) and c.746C>T, p.(P249L)) in ACPT, the gene encoding acid phosphatase, testicular, which segregates with hypoplastic amelogenesis imperfecta in two unrelated families. ACPT is reported to play a role in odontoblast differentiation and mineralisation by supplying phosphate during dentine formation. Analysis by computerised tomography and scanning electron microscopy of a primary molar tooth from an individual homozygous for the c.746C>T variant revealed an enamel layer that was hypoplastic, but mineralised with prismatic architecture. These findings implicate variants in ACPT as a cause of early failure of amelogenesis during the secretory phase.

A comparison of inhalation sedation agents in the management of children receiving dental treatment: a randomized, controlled, cross-over pilot trial.

AIMS: First, to compare the relative effectiveness of inhalation sedation using (A) nitrous oxide and oxygen with (B) nitrous oxide, sevoflurane, and oxygen in the management of children receiving dental extractions. Secondly, to determine patient and guardian preference between the two sedation techniques. MATERIALS AND METHODS: A randomized, controlled, double-blinded, cross-over, pilot clinical trial was undertaken. Thirty patients aged 6-15 years, ASA category I or II, who required two identical dental extractions with inhalation sedation were recruited. At the first session, patients were randomly allocated to receiving treatment with sedation Method A or B. At the second session, the alternative sedation protocol was employed. RESULTS: Overall, 80% of patients successfully completed treatment at both appointments. There was no statistically significant difference between either the success rate of the two methods or in guardian preference between the two modes of sedation. There was a statistically significant difference in patient preference in favour of Method B. CONCLUSIONS: The results from this pilot study would suggest no increased benefit, in terms of treatment completion, from the additional use of sevoflurane in combination with nitrous oxide and oxygen. There was, however, a small but significant patient preference in favour of nitrous oxide with sevoflurane and oxygen.

The use of theatre time for paediatric dentistry under general anaesthesia.

OBJECTIVE: The aim of this paper was to determine the use of theatre time for all procedures performed under general anaesthetic on a paediatric dental list. METHODS: A prospective study of paediatric dental general anaesthetic procedures was undertaken at Ninewells Hospital and Medical School, NHS Tayside, Dundee, UK. Data were collected prospectively for 71 operating lists over a 3-year period from April 2003 to March 2006. Both operator status and the procedure being undertaken were recorded. In addition, pre-anaesthetic, anaesthetic, operating and disconnection times were recorded. RESULTS: Of the 71 lists examined, 61 either finished early or on time, with a median unused time of 32.50 min (interquartile range = 19.50, 50.00 min), whilst 10 lists finished late with a median overrun time of 30.50 min (interquartile range = 9.25, 45.50 min). Comparing lists which finished late with those which were completed within time, the median pre-anaesthetic time was significantly longer (Mann-Whitney U-test, W = 20.05, P = 0.048). Overall, the theatre was in use for 78.22% of time combining pre-anaesthetic, anaesthetic, operating and disconnection times; hence, there was poor time utilization of theatre for 21.78% of the total theatre time. CONCLUSIONS: Overall, 85.9% of theatre sessions for dental procedures under general anaesthetic in children finished early or on time. Where lists finished late, the duration of the pre-anaesthetic time appeared to be the significant factor.

An assessment of rubber dam usage amongst specialists in paediatric dentistry practising within the UK.

OBJECTIVE: Rubber dam is recommended by the British Society of Paediatric Dentistry (BSPD) for various restorative and endodontic procedures. To date, there has been no report of actual usage of rubber dam within the speciality of paediatric dentistry. The aim of this study was to assess the usage of rubber dam amongst paediatric dentistry specialists within the UK. METHODS: A postal questionnaire was distributed to all practitioners registered on the UK General Dental Council's 2004 specialist list in paediatric dentistry. RESULTS: Data were available for 162 questionnaires (a 75% response rate), and of these, 85% of respondents worked in the National Health Service (NHS), 4% were private practitioners and the remainder had a mixed NHS/private practice. Regarding the benefits of rubber dam, 65% and 52% of respondents quoted patient safety and moisture control, respectively. Perceived difficulties of dam usage were lack of patient cooperation and the non-necessity for a particular treatment, as quoted in 64% and 36% of the completed questionnaires, respectively. The most common modes of isolation for anterior and posterior teeth were Dry Dam(R) (58%), and clamp and dam (80%), respectively. CONCLUSION: Current BSPD guidelines recommend rubber dam usage for many restorative procedures; however, it would appear that there is wide variability in the application, as well as under-use, of rubber dam.