Malignant Transformation Rate of Oral Submucous Fibrosis: A Systematic Review and Meta-Analysis.

Oral submucous fibrosis (OSF) is a chronic progressive condition affecting the oral cavity, oropharynx and upper third of the oesophagus. It is a potentially malignant disorder. The authors collated and analysed the existing literature to establish the overall malignant transformation rate (MTR). A retrospective analysis of medical and dental scientific literature using online indexed databases was conducted for the period 1956 to 2021. The quality of the enrolled studies was assessed by the Newcastle-Ottawa Scale (NOS). A meta-analysis using a random effects model of a single proportion was performed along with statistical tests for heterogeneity. The overall proportion of malignancy across all studies was 0.06 (95% CI, 0.02-0.10), indicating an overall 6% risk of malignant transformation across all studies and cohorts. Sub-group analyses revealed strong differences in proportion of malignancy according to ethnicity/cohort; Chinese = 0.02 (95% CI 0.01-0.02), Taiwanese = 0.06 (95% CI, 0.03-0.10), Indian = 0.08 (95% CI, 0.03-0.14) and Pakistani = 0.27 (95% CI 0.25-0.29). Overall, the MTR was 6%; however, wide heterogeneity of the included studies was noted. Geographic variations in MTR were noted but were not statistically significant. Further studies are required to analyse the difference between cohort groups.

Development of a new tool for predicting the behavior of individuals with intellectual disability in the dental office: A pilot study.

BACKGROUND: The dental treatment of individuals with intellectual disability can represent a considerable professional challenge. OBJECTIVE: To develop a model for predicting the behavior of patients with intellectual disability in the dental office. METHODS: The study group comprised 250 patients with Down syndrome (DS), autism spectrum disorder (ASD), cerebral palsy (CP), idiopathic cognitive impairment or rare disorders. We collected their demographic, medical, social and behavioral information and identified potential predictors (chi-squared test). We developed stratified models (Akaike information criterion) to anticipate the patients'behavior during intraoral examinations and to discern whether the dental treatment should be performed under general anesthesia. These models were validated in a new study group consisting of 80 patients. Goodness of fit was quantified with sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and area under the receiver operating characteristic curve (AUC). We developed a mathematical algorithm for executing the models and developed software for its practical implementation (PREdictors of BEhavior in Dentistry, "PREBED"). RESULTS: For patients with DS, ASD and CP, the model predicting the need for physical restraint during examination achieved a PPV of 0.90, 0.85 and 1.00, respectively, and an NPV of 0.66, 0.76 and 1.00, respectively. The model predicting the need for performing treatment under general anesthesia achieved a PPV of 0.63, 1.00 and 1.00, respectively, and an NPV of 1.00, 1.00 and 0.73, respectively. However, when validating the stratified models, the percentage of poorly classified individuals (false negatives + false positives) ranged from 24% to 46.6%. CONCLUSIONS: The results of the PREBED tool open the door to establishing new models implementing other potentially predictive variables.

Genetic Susceptibility to Periodontal Disease in Down Syndrome: A Case-Control Study.

Severe periodontitis is prevalent in Down syndrome (DS). This study aimed to identify genetic variations associated with periodontitis in individuals with DS. The study group was distributed into DS patients with periodontitis (n = 50) and DS patients with healthy periodontium (n = 36). All samples were genotyped with the "Axiom Spanish Biobank" array, which contains 757,836 markers. An association analysis at the individual marker level using logistic regression, as well as at the gene level applying the sequence kernel association test (SKAT) was performed. The most significant genes were included in a pathway analysis using the free DAVID software. C12orf74 (rs4315121, p = 9.85 × 10-5, OR = 8.84), LOC101930064 (rs4814890, p = 9.61 × 10-5, OR = 0.13), KBTBD12 (rs1549874, p = 8.27 × 10-5, OR = 0.08), PIWIL1 (rs11060842, p = 7.82 × 10-5, OR = 9.05) and C16orf82 (rs62030877, p = 8.92 × 10-5, OR = 0.14) showed a higher probability in the individual analysis. The analysis at the gene level highlighted PIWIL, MIR9-2, LHCGR, TPR and BCR. At the signaling pathway level, PI3K-Akt, long-term depression and FoxO achieved nominal significance (p = 1.3 × 10-2, p = 5.1 × 10-3, p = 1.2 × 10-2, respectively). In summary, various metabolic pathways are involved in the pathogenesis of periodontitis in DS, including PI3K-Akt, which regulates cell proliferation and inflammatory response.

In Vitro Anti-Biofilm and Antibacterial Properties of Streptococcus downii sp. nov.

The aim of this study was to evaluate the potential anti-biofilm and antibacterial activities of Streptococcus downii sp. nov. To test anti-biofilm properties, Streptococcus mutans, Actinomyces naeslundii, Veillonella parvula, Fusobacterium nucleatum, Porphyromonas gingivalis, and Aggregatibacter actinomycetemcomitans were grown in a biofilm model in the presence or not of S. downii sp. nov. for up to 120 h. For the potential antibacterial activity, 24 h-biofilms were exposed to S. downii sp. nov for 24 and 48 h. Biofilms structures and bacterial viability were studied by microscopy, and the effect in bacterial load by quantitative polymerase chain reaction. A generalized linear model was constructed, and results were considered as statistically significant at p < 0.05. The presence of S. downii sp. nov. during biofilm development did not affect the structure of the community, but an anti-biofilm effect against S. mutans was observed (p < 0.001, after 96 and 120 h). For antibacterial activity, after 24 h of exposure to S. downii sp. nov., counts of S. mutans (p = 0.019) and A. actinomycetemcomitans (p = 0.020) were significantly reduced in well-structured biofilms. Although moderate, anti-biofilm and antibacterial activities of S. downii sp. nov. against oral bacteria, including some periodontal pathogens, were demonstrated in an in vitro biofilm model.

Oral health needs of athletes with intellectual disability in Southern Europe: Greece, Italy and Spain.

AIMS: The population with intellectual disabilities (ID) has been reported to be a vulnerable population in terms of oral health. The aims of this study were to evaluate the oral condition and treatment needs of Special Olympics (SO) athletes from Greece, Italy and Spain. METHODS AND RESULTS: A cross-sectional study was performed with data collected in annual SO events held in Greece, Italy and Spain, between 2010 and 2012. The recorded parameters were the presence/absence of edentulism, untreated decay, filled or missing teeth, sealants, tooth injury and signs of gingival disease. Among the main findings, the prevalence of untreated decay was 57.0% in Greece, 48.8% in Italy and 41.7% of the Spanish athletes. The prevalence of signs of gingival disease was 61.1% in Greece, 60.6% in Italy and 66.1% in Spain. While the majority of the athletes were in need of dental treatment. CONCLUSIONS: Oral disease is an unresolved problem among athletes with ID in these Mediterranean countries. Therefore efforts should be directed to meet their treatment needs and to prevent oral disease.

Oral Manifestations of Wolf-Hirschhorn Syndrome: Genotype-Phenotype Correlation Analysis.

BACKGROUND: Wolf-Hirschhorn syndrome (WHS) is a rare disease caused by deletion in the distal moiety of the short arm of chromosome 4. The objectives of this study were to report the most representative oral findings of WHS, relate them with other clinical characteristics of the disease, and establish possible phenotype-genotype correlation. METHODS: The study was conducted at 6 reference centers distributed throughout Spain during 2018-2019. The study group consisted of 31 patients with WHS who underwent a standardized oral examination. Due to behavioral reasons, imaging studies were performed on only 11 of the children 6 years of age or older. All participants had previously undergone a specific medical examination for WHS, during which anatomical, functional, epilepsy-related, and genetic variables were recorded. RESULTS: The most prevalent oral manifestations were delayed tooth eruption (74.1%), bruxism (64.5%), dental agenesis (63.6%), micrognathia (60.0%), oligodontia (45.5%), and downturned corners of the mouth (32.3%). We detected strong correlation between psychomotor delay and oligodontia (p = 0.008; Cramér's V coefficient, 0.75). The size of the deletion was correlated in a statistically significant manner with the presence of oligodontia (p = 0.009; point-biserial correlation coefficient, 0.75). CONCLUSION: Certain oral manifestations prevalent in WHS can form part of the syndrome's phenotypic variability. A number of the characteristics of WHS, such as psychomotor delay and epilepsy, are correlated with oral findings such as oligodontia and bruxism. Although most genotype-phenotype correlations are currently unknown, most of them seem to be associated with larger deletions, suggesting that some oral-facial candidate genes might be outside the critical WHS region, indicating that WHS is a contiguous gene syndrome.

The Subgingival Microbiome in Patients with Down Syndrome and Periodontitis.

OBJECTIVE: To describe the subgingival microbiome of individuals with Down syndrome (DS). METHODS: We conducted a cross-sectional observational study that obtained bacterial DNA samples from 50 patients with DS, 25 with periodontitis (PDS) and 25 with a healthy periodontal condition (HDS). The samples were analyzed by sequencing the 16S rRNA gene V3-V4 hypervariable region using the MiSeq System. Taxonomic affiliations were assigned using the naïve Bayesian classifier integrated in QIIME2 plugins. We evaluated the difference in bacteria abundance between the sample groups using Wilcoxon and Kruskal-Wallis tests. We evaluated the alpha diversity of the identified species using the Observed, Chao1metric, ACE and Shannon indices and evaluated beta diversity with principal coordinate analysis (registration code: 2018/510). RESULTS: Twenty-one genera and 39 bacterial species showed a significantly different abundance between the study groups. Among the genera, Porphyromonas, Treponema, Tannerella and Aggregatibacter were more abundant in the PDS group than in the HDS group, as were the less commonly studied Filifactor, Fretibacterium and Desulfobulbus genera. Among the species, Porphyromonas spp. and Tannerella spp. were the most abundant in the PDS group; the most abundant species in the HDS group were Pseudomonas spp., Granulicatella spp. and Gemella spp. CONCLUSION: Well-recognized periodontal pathogens and newly proposed pathogenic taxa were associated with periodontitis in patients with DS.

International meeting on Wolf-Hirschhorn syndrome: Update on the nosology and new insights on the pathogenic mechanisms for seizures and growth delay.

"An International Meeting on Wolf-Hirschhorn Syndrome (WHS)" was held at The University Hospital La Paz in Madrid, Spain (October 13-14, 2017). One hundred and twenty-five people, including physicians, scientists and affected families, attended the meeting. Parent and patient advocates from the Spanish Association of WHS opened the meeting with a panel discussion to set the stage regarding their hopes and expectations for therapeutic advances. In keeping with the theme on therapeutic development, the sessions followed a progression from description of the phenotype and definition of therapeutic endpoints, to definition of genomic changes. These proceedings will review the major points of discussion.

Expanding the clinical and molecular spectrum of the CWC27-related spliceosomopathy.

Cyclophilins are a type of peptidyl-prolyl cis-trans isomerases. CWC27, one of the known human cyclophilins, is recruited by the spliceosome for the pre-mRNA splicing process. Biallelic deleterious variants in CWC27 lead to a spectrum of overlapping phenotypes including retinal degeneration, skeletal anomalies, short stature, and neurological defects. The present work reports a woman showing these clinical features, in addition to hypergonadotropic hypogonadism, hypoplastic/agenesic teeth, and cataracts, not previously associated with such phenotypic spectrum. Whole exome sequencing on this patient identified a novel CWC27 homozygous variant predicted to originate a severely truncated protein and the consequent loss of functionality. The clinical and genetic characterization of such patient could provide further insight into the underlying causes of the spliceosomopathies.

The effect of dentist experience on the treatment of individuals with disability under general anesthesia.

AIMS: The aim of the present work was to assess in patients with severe disability operated under general anesthesia whether the progressive acquisition of experience by the dental team affects the type of procedure performed and the duration of operations. METHODS AND RESULTS: A study group of 911 patients who underwent dental treatment under general anesthesia between 1997 and 2014 was conformed. Information was collected from every patient including: dental diagnosis, dental procedures and duration of the operating times. To analyze the impact of the operators' experience, the study period was divided into three 6-year periods. The most prevalent systemic diagnosis was "mental disease and behavior disorders" (42.9%). The most common dental procedures were extractions and fillings. Fewer extractions were performed during the third 6-year period (p = .000). Compared with the other two periods, during the first period fewer composite fillings (p = .000 and p = .000, respectively) and more fissure sealants (p = .001 and p = .023, respectively) were performed. The number of amalgam fillings decreased progressively (p = .000). There was a statistically significant reduction in the mean duration of the operations between the first and third period (p = .002). CONCLUSION: The dental team's experience entailed a significant reduction in the duration of operations.

Brief Report: Estimating the Dental Age of Children with Autism Spectrum Disorders.

Determining a patient's dental age is essential from the dental standpoint but can also have connotations of a forensic, anthropological and medicolegal nature. In this study, we assessed the correspondence between dental age and chronological age in a group of 50 children with autism spectrum disorders, with a chronological age range of 3-17 years. The dental age was calculated using panoramic radiography images, applying linear regression models derived from the classical indices by Nolla and Demirjian. In 2 of every 3 boys, the dental age was ahead of the chronological age, and in almost 1 of every 3 cases, the difference was ≥ 12 months. In the girls, conversely, we found no significant differences between dental age and chronological age.

Relationship between functional masticatory units and cognitive impairment in elderly persons.

BACKGROUND: Studies on the elderly have reported that the risk of cognitive impairment is affected by chewing difficulty. OBJECTIVE: To determine whether there is a relationship between the number of pairs of antagonist teeth that come into contact when the mouth is closed (functional masticatory units [FMUs]) and the level of cognitive impairment. METHODS: We conducted a cross-sectional observational study with 502 institutionalised White individuals older than 65 years, living in the northwest of Spain and Portugal. Through a direct visual inspection, we recorded the number of FMUs. Cognitive impairment was assessed by applying the Mini-Cognitive Examination (MCE), a test derived from the Mini-Mental State Examination. To describe the statistical relationship between the FMUs and the MCE values, a generalised linear model (GLM) was applied. We assessed the GLM predictive capacity for detecting cognitive impairment (MCE ≤23) in a new study group consisting of 156 elderly individuals. RESULTS: A large number of FMUs was significantly associated with a lower probability of cognitive impairment, regardless of the nature of the contact and its location (explained deviance, 30.1%). The model's discriminatory capacity for cognitive impairment based on the FMUs was "good" (0.820). The model's predictive capacity for cognitive impairment was "acceptable" (sensitivity, 0.786; positive predictive value, 0.900; accuracy, 0.729). CONCLUSION: In White, elderly institutionalised individuals, the absolute number of FMUs is significantly related to their MCE scores.

Benefits of Maxillary Expansion for a Patient With Spinal Muscular Atrophy Type 2.

This case report involves a 10-yr-old boy diagnosed with spinal muscular atrophy type 2 who underwent nighttime mechanical ventilation with bilevel positive airway pressure. The oral examination revealed restricted mouth opening, lip interposition, dental crowding, and maxillary compression. After maxillary expansion, the upper airway volume increased 18.6%; 13 episodes of airway infections (20 days of hospitalization) were recorded in the 2 yrs before the maxillary expansion and only 4 episodes (no hospital admissions) in the 2 subsequent years. In conclusion, maxillary expansion in children with systemic disease that involves respiratory impairment may, in some cases, provide functional and clinical improvements, increase upper airway airflows, and possibly decrease the number of respiratory infections.

Effect of chewing an apple on dental plaque removal and on salivary bacterial viability.

OBJECTIVES: Studies on dental plaque removal by chewing an apple are scarce and dated, with conflicting findings. This study aimed to determine whether chewing an apple produced mechanical removal of dental plaque or had any effect on salivary bacterial viability. METHODS: The study group consisted of 20 healthy adults with good oral health status who were randomly assigned to brush their teeth or eat an apple. After 2 weeks, the experiment was repeated with the order reversed. Plaque index (PI) and the bacterial viability (BV) in a sample of whole saliva (spit) were determined before brushing or apple eating (baseline, B), immediately afterward (A) and 24 hours afterward (24). RESULTS: After chewing an apple, PI-A was significantly higher than both PI-B (P < .001) and PI-24 (P < .001). BV-A was significantly lower than BV-B (P < .001), with a return to baseline values at the BV-24 measurement. CONCLUSIONS: Chewing an apple does not remove dental plaque, and may favor plaque regrowth during the first 24 hours, but it does produce an immediate reduction in salivary bacterial viability similar to that after tooth brushing.

Denosumab-related osteonecrosis of the jaw following non-surgical periodontal therapy: A case report.

INTRODUCTION: Osteonecrosis of the jaw associated with bisphosphonates is currently called medication-related osteonecrosis of the jaw (MRONJ), given that in addition to bisphosphonates, jaw osteonecrosis has been related to the administration of other antiresorptive and antiangiogenic drugs, such as denosumab, sunitinib, bevacizumab and ipilimumab. CASE PRESENTATION: A 77-year-old patient with osteoporosis treated with subcutaneous injections of denosumab at an interval of 6 months is presented. The patient developed MRONJ after receiving a non-surgical periodontal therapy. Although the MRONJ was initially classified as a stage I lesion in this patient, cone beam computed tomography images confirmed the presence of a significant osteolytic lesion. Treatment consisted of the administration of chlorhexidine mouthwash and systemic doxycycline, exodontia of the involved teeth, sequestrectomy and complete surgical debridement of the necrotic bone. CONCLUSION: To our knowledge, this is the first case reported in the literature of MRONJ following non-surgical periodontal therapy in a patient with osteoporosis treated with denosumab. The risk of MRONJ development after a periodontal procedure and how to prevent this complication are still unknown.

Prevention of medication-related osteonecrosis of the jaws secondary to tooth extractions. A systematic review

Special Patients Unit. Medical-Surgical Dental Research Group (OMEQUI).. (Spain) BACKGROUND: A study was made to identify the most effective protocol for reducing the risk of osteonecrosis of the jaws (ONJ) following tooth extraction in patients subjected to treatment with antiresorptive or antiangiogenic drugs. MATERIAL AND METHODS: A MEDLINE and SCOPUS search (January 2003 - March 2015) was made with the purpose of conducting a systematic literature review based on the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. All articles contributing information on tooth extractions in patients treated with oral or intravenous antiresorptive or antiangiogenic drugs were included. RESULTS: Only 13 of the 380 selected articles were finally included in the review: 11 and 5 of them offered data on patients treated with intravenous and oral bisphosphonates, respectively. No randomized controlled trials were found - all publications corresponding to case series or cohort studies. The prevalence of ONJ in the patients treated with intravenous and oral bisphosphonates was 6,9% (range 0-34.7%) and 0.47% (range 0-2.5%), respectively. The main preventive measures comprised local and systemic infection control. CONCLUSIONS: No conclusive scientific evidence is available to date on the efficacy of ONJ prevention protocols in patients treated with antiresorptive or antiangiogenic drugs subjected to tooth extraction

Prevention of medication-related osteonecrosis of the jaws secondary to tooth extractions. A systematic review.

BACKGROUND: A study was made to identify the most effective protocol for reducing the risk of osteonecrosis of the jaws (ONJ) following tooth extraction in patients subjected to treatment with antiresorptive or antiangiogenic drugs. MATERIAL AND METHODS: A MEDLINE and SCOPUS search (January 2003 - March 2015) was made with the purpose of conducting a systematic literature review based on the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. All articles contributing information on tooth extractions in patients treated with oral or intravenous antiresorptive or antiangiogenic drugs were included. RESULTS: Only 13 of the 380 selected articles were finally included in the review: 11 and 5 of them offered data on patients treated with intravenous and oral bisphosphonates, respectively. No randomized controlled trials were found - all publications corresponding to case series or cohort studies. The prevalence of ONJ in the patients treated with intravenous and oral bisphosphonates was 6,9% (range 0-34.7%) and 0.47% (range 0-2.5%), respectively. The main preventive measures comprised local and systemic infection control. CONCLUSIONS: No conclusive scientific evidence is available to date on the efficacy of ONJ prevention protocols in patients treated with antiresorptive or antiangiogenic drugs subjected to tooth extraction.

Fixed multibracket dental therapy has challenges but can be successfully performed in young persons with Down syndrome.

Purpose The objective of this study was to analyze the feasibility of orthodontic treatment with fixed multibracket dental appliances (FMAs) in patients with Down syndrome (DS). Methods The study sample was formed of 25 patients with DS who underwent orthodontic treatment with FMAs. Dental and skeletal characteristics, aspects of FMA treatment, retainer use and recurrences were analyzed. Results were compared with a control group of healthy, age-, sex- and PAR index-matched individuals. Results All the controls underwent a single desensitization session, whereas 2-3 sessions were necessary in 11 patients with DS (p < 0.001). All the controls underwent bimaxillary treatment, compared with 11 patients with DS (p < 0.001). Only 8 patients with DS wore intermaxillary elastics, compared with 19 controls (p = 0.004). Complications were more common in patients with DS than in controls (p = 0.003), due particularly to traumatic ulcers development. There were more failures during the maintenance phase in patients with DS than in controls (p = 0.001). The mean duration of treatment was 37 months in patients with DS and 23 months in controls. More cemented retainers were used in the controls than in patients with DS (p = 0.050). Conclusions It is possible to perform orthodontic treatment with FMAs in patients with DS, although treatment may take longer than usual and the frequency of complications is higher than in controls. Implications for Rehabilitation Certain dental characteristics of Down syndrome (DS) make these children firm candidates for orthodontic intervention. There is little literature available on orthodontic treatment in patients with DS, particularly with regard to the use of fixed appliances. This case-control study shows that it is possible to perform orthodontic treatment with fixed appliances in adequately selected patients with DS. However, in patients with DS orthodontic treatment may take longer than usual and the frequency of complications is higher than in the general population.

Dimensions of central incisors, canines, and first molars in subjects with Down syndrome measured on cone-beam computed tomographs.

INTRODUCTION: The literature on tooth dimensions in subjects with Down syndrome (DS) is scarce. To our knowledge, no studies have yet been published in which the morphometry of the teeth in DS subjects has been determined using computed tomography. METHODS: The study group consisted of 40 subjects with DS, aged 10 to 40 years. An age- and sex-matched control group was selected. Cone-beam computed tomography images were retrieved from the archive of the Santiago de Compostela University in Spain. The maxillary central incisors, canines, and first molars were evaluated. The following variables were analyzed: overall tooth length, crown height, root length, mesiodistal diameter, vestibular-palatine diameter, crown-to-root ratio, and cervical circumference. RESULTS: The teeth of subjects with DS were smaller than those of the controls, although the crown-to-root ratio was maintained. No clear sexual dimorphism was detected, but the root lengths of the incisors were greater in male subjects. Crown height, mesiodistal diameter, and crown-to-root ratio showed progressive reductions with age. There appeared to be a degree of "fluctuating dental asymmetry" with respect to vestibular-palatine diameter and cervical circumference. CONCLUSIONS: Our findings confirm microdontia of the permanent teeth and progressive reductions in tooth sizes with age in persons with DS. These variations might be relevant to orthodontic treatment planning.