Oral manifestations of nine individuals with Williams syndrome. A case series.

AIMS: Williams syndrome (WS) is a congenital developmental disorder characterized, mainly, by distinctive facial features, cardiovascular anomalies, growth delay and a typical neurobehavioral profile. The oral manifestations have not been sufficiently described and, therefore, the aim of the current study was to present the clinical, radiographic and microbiological findings of individuals with WS. METHODS AND RESULTS: A series of nine WS individuals (seven females) with mean age 21 years-old were evaluated. A complete intraoral clinical examination, a radiographic analysis using panoramic and cephalometric x-ray and a supra- and sub-gingival microbiological profiling were performed. We observed abnormal tooth morphology, excessive interdental spacing, congenitally missing permanent teeth and malocclusion. High levels of DMFT were observed and gingivitis was present in all subjects. Bacteria related to periodontal disease were detected in dental plaque. Three patients were classified with a gingival phenotype type I according to the Maynard and Wilson classification. The sella turcica bridging was a novel finding for this group of patients. CONCLUSION: Due to the elevated prevalence of gingivitis, caries and malocclusion, a multidisciplinary approach including dental follow-ups should be the standard of care in WS patients.

Oral manifestations of eleven individuals with Noonan syndrome. A case series.

AIMS: Noonan syndrome (NS) is a clinically and genetically heterogeneous condition characterized by distinctive facial features, short stature, and congenital heart defects. The oral manifestations have not been sufficiently described. In an attempt to enrich our understanding regarding the oral manifestations, the aim of the current study was to present the clinical, radiographic, and microbiological findings of eleven subjects with NS syndrome. METHODS AND RESULTS: A complete intraoral clinical evaluation, a radiographic analysis using panoramic and cephalometric x-rays, and a supra- and sub-gingival microbiological profiling of 20 periodontal and cariogenic microbiota using the checkerboard DNA-DNA hybridization technique were performed in a series of 11 NS individuals. Compared to previous reports very few dental findings were present. Gingivitis and carious lesions were present in all subjects and could be related to poor oral hygiene. High-arched palate and malocclusions such as crossbite, open-bite, and deep-bite were very common findings but without a specific pattern. Two findings that have not been reported before included the different shapes of the condyles and the irregular shape of the sella turcica. CONCLUSION: Due to the elevated prevalence of gingivitis, caries, and malocclusion, a multidisciplinary approach including dental follow-ups should be the standard care in NS patients.

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.

ACTN2 (rs6656267) and MPPED2 (rs11031093 and rs536007) polymorphisms in primary dentition caries: A case-control study.

BACKGROUND: Dental caries represents one of the most common human diseases which can lead to pulpitis, pain, and tooth loss and can negatively affect growth and well-being. Although dietary and environmental factors have been extensively studied towards their contribution of the disease, genetic factors that contribute one's susceptibility over caries development remain rather clouded. AIM: To investigate the possible contribution of ACTN2 (rs6656267) and MPPED2 (rs11031093 and rs536007) polymorphisms in primary dentition caries. DESIGN: Samples from children (5-12 years old) were collected and genotyped for ACTN2 (rs6656267) and MPPED2 (rs11031093 and rs536007) polymorphisms. With regard to dmfs index and socio-economic status, an association between these polymorphisms and primary dentition caries was investigated. RESULTS: ACTN2 (rs6655267) and MPPED2 (rs536007) are not associated with primary dentition caries. MPPED2 (rs11031093, G Allele) is marginally associated. CONCLUSIONS: MPPED2 (rs11031093, G Allele) is marginally associated with caries susceptibility on primary dentition.

Peripheral Tumor with Osteodentin and Cementum-like Material in an Infant: Odontogenic Hamartoma or Odontoma?

The purpose of this report is to describe a peripheral tumor on the mandibular alveolar ridge of a seven-month-old Caucasian boy, consisting of ectomesencymal odontogenic tissues, in particular osteodentin and cementum-like material, in a cellular or loose vascular connective tissue stroma. This case may be considered either a peripheral odontogenic hamartoma or a peripheral odontoma.

Beyond the drugs: nonpharmacologic strategies to optimize procedural care in children.

PURPOSE OF REVIEW: Painful and/or stressful medical procedures mean a substantial burden for sick children. There is good evidence that procedural comfort can be optimized by a comprehensive comfort-directed policy containing the triad of nonpharmacological strategies (NPS) in all cases, timely or preventive procedural analgesia if pain is an issue, and procedural sedation. RECENT FINDINGS: Based both on well-established theoretical frameworks as well as an increasing body of scientific evidence NPS need to be regarded an inextricable part of procedural comfort care. SUMMARY: Procedural comfort care must always start with a child-friendly, nonthreatening environment in which well-being, confidence, and self-efficacy are optimized and maintained. This requires a reconsideration of the medical spaces where we provide care, reduction of sensory stimulation, normalized professional behavior, optimal logistics, and coordination and comfort-directed and age-appropriate verbal and nonverbal expression by professionals. Next, age-appropriate distraction techniques and/or hypnosis should be readily available. NPS are useful for all types of medical and dental procedures and should always precede and accompany procedural sedation. NPS should be embedded into a family-centered, care-directed policy as it has been shown that family-centered care can lead to safer, more personalized, and effective care, improved healthcare experiences and patient outcomes, and more responsive organizations.

Perioperative considerations in children with autism spectrum disorder.

PURPOSE OF REVIEW: Children with autism often present a challenge to the anesthesiologist. This review summarizes the current experiences and recommendations for the perioperative management of this unique group of patients. RECENT FINDINGS: Autism is the fastest growing neurodevelopmental disorder in the world. Increased recognition and public awareness of the disease is driven largely by the advances in research. A large body of evidence exists that identifies the role of genetic, environmental, biological, and developmental factors in the origin of autism. The anesthesia literature consists mostly of case reports. Recent publications are reporting management strategies and evaluation of this patient population's perioperative experiences. SUMMARY: Patients with autism spectrum disorder are a heterogeneous group and often need general anesthesia for different procedures and studies. Familiarity with each patient's behavioral specifics and efforts to alleviate stress is of paramount importance for a smooth perioperative course with minimal adverse events.

Hand-Schüler-Christian disease presenting with recurrent, bilateral, symmetrical mandibular lesions in an 8-year-old boy: report of a case.

INTRODUCTION: Hand-Schüller-Christian disease (HSC) is the unisystem multifocal form of Langerhans Cell Histiocytosis (LCH) and is primarily seen in infants and children. CASE REPORT: An 8-year-old boy was referred for acute mandibular pain. His medical history included otic LCH and diabetes insipidus at age of 11 months. Intraorally, a pressure sensitive swelling, and radiographically, extensive bone loss were revealed on the area of lower second primary molars. The primary molars were extracted and histological examination confirmed the final diagnosis of HSC. The patient was treated with chemotherapy and the lesions decreased considerably. Two years later, a new swelling was recorded in the same area bilaterally. Biopsy confirmed recurrent HSC disease and the patient entered an alternative chemotherapy protocol. Six months later, improvement of the lesions was revealed. CONCLUSIONS: Dentists can contribute to a timely and valid identification of HSC disease by correctly differentially diagnosing lesions of head and neck.

Congenital adrenal hyperplasia: a case report with premature teeth exfoliation and bone resorption.

Congenital adrenal hyperplasia (CAH) is an inherited autosomal recessive disorder characterized by insufficient production of cortisol. The aim of this case report was to present a child with CAH, premature exfoliation of primary teeth and accelerated eruption of his permanent teeth related to bone resorption. A 4.5-year-old Caucasian boy with CAH and long-term administration of glucocorticoids was referred for dental restoration. Clinical examination revealed primary molars with worn stainless steel crowns, severe attrition of the upper canines, and absence of the upper incisors. Before the completion of treatment, abnormal mobility of the first upper primary molars and the lower incisors was detected, and a few days later the teeth exfoliated prematurely. Histologic examination revealed normal tooth structure. Alkaline phosphatase and blood cells values were normal. Eruption of the permanent dentition was also accelerated. Tooth mobility was noticed in the permanent teeth as soon as they erupted, along with bone destruction. Examination revealed an elevated level of receptor activator of nuclear factor-κB ligand and lower-than-normal osteoprotegerin and vitamin D levels. The patient was treated with vitamin D supplements, and his teeth have been stable ever since. CAH is a serious chronic disorder appearing in children with accelerated dental development and possibly premature loss of primary teeth.

Psychometric and biohormonal indices of dental anxiety in children. A prospective cohort study.

The stress of dental treatment often elicits negative emotions in children, expressed as dental fear or anxiety. Highly anxious children obstruct treatment and avoid therapy, further amplifying oral health problems. The aim of this study was to examine the neuroendocrine and autonomic nervous system responses to dental treatment and their possible interactions and associations with psychometric indices of anxiety, caries, previous dental experience, anesthesia, age and gender in school children. Upon informed consent, saliva was obtained from 97 children (59% males, mean age ± SD: 89.73 ± 15 months) in the Clinic of pediatric dentistry before treatment, immediately post-treatment and at the recall visit to determine cortisol and salivary alpha-amylase (sAA) levels. Dental and general anxiety was assessed through specific questionnaires completed by the children. Compared to pre-treatment, cortisol levels were increased following treatment, while sAA levels were higher at the recall. Pre- and post-treatment cortisol and sAA responses were positively correlated. Dental and general anxiety questionnaire scores were also significantly correlated with each other. The integrated autonomic and neuroendocrine responses prior to treatment were correlated with state anxiety and those following treatment with dental anxiety. However, univariable and multivariable linear regression analysis associated post-treatment cortisol, but not sAA, levels with dental anxiety. No associations of cortisol or sAA responses with caries, age, gender, previous dental experience or anesthesia were detected. These data provide some evidence that both sAA and cortisol levels are altered in children in anticipation or during dental treatment, but only cortisol levels are associated to dental anxiety.

Raisins and oral health.

Traditionally, raisins have been thought to promote dental caries due to their suspected "stickiness" and sugar content. Current research identifies some evidence contrary to traditional thought, suggesting that raisins may not contribute to dental caries. This article reviews new findings with regards to raisins and the 3 conditions that are thought to contribute to the formation of dental caries; low oral pH, adherence of food to teeth, and biofilm (bacterial) behavior. The studies reviewed concluded that raisin: consumption alone does not drop oral pH below the threshold that contributes to enamel dissolution, do not remain on the teeth longer than other foods, and contain a variety of antioxidants that inhibit Streptococcus Mutans, bacteria that is a primary cause of dental caries. Further research in this area should be considered.

Giant cell fibroma in children: report of two cases and literature review.

BACKGROUND: Giant cell fibroma is a type of fibrous tumour of the oral mucosa which rarely affects children under the age of 10. The purpose of this paper was to contribute two clinically and histologically documented cases of giant cell fibroma in the free gingiva of a 7 and 6 year old boys. METHODS: Both nodules were presented in the mandibular anterior region. In the differential diagnosis several fibrous hyperplastic lesions were considered such as traumatic fibroma, papilloma, peripheral ossifying fibroma, peripheral odontogenic fibroma, giant cell fibroma and odontogenic hamartoma. RESULTS: The lesions were removed and the histological examination revealed fibrocollagenous connective tissue with the presence of stellate giant cells which confirmed the diagnosis of giant cell fibroma. CONCLUSIONS: Dentists should be aware of the existence of giant cell fibroma in children, which must be included in the differential diagnosis of nodular lesions of the gingiva and adequately diagnosed and treated by removal and histopathological examination.

Psychometric properties of Greek versions of the Modified Corah Dental Anxiety Scale (MDAS) and the Dental Fear Survey (DFS).

BACKGROUND: A growing body of literature describes the performance of dental fear questionnaires in various countries. We describe the psychometric properties of Greek versions of the Modified Dental Anxiety Scale (MDAS) and the Dental Fear Survey (DFS) in adult Greek patients. METHODS: Greek versions of the MDAS and DFS were administered to two samples of adult dental patients. In the first sample, 195 patients attending one of three private practice dental offices in a large city in Greece completed the questionnaires in the waiting room before dental treatment. After treatment, their dentists (who did not know how the patients had answered the questionnaire) rated their anxiety during dental treatment. In the second sample, 41 patients attending a Greek university dental school clinic completed the questionnaire twice at two separate visits, in order to provide test-retest data. Cronbach's alpha was used to compute the internal consistencies, while Spearman's rho was used to compute the test-retest reliabilities. Construct validity was assessed by correlating the responses to the MDAS and DFS by Spearman's rho. Spearman's rho was also used to examine the criterion validities, by comparing the questionnaire responses with the dentists' ratings of anxiety. RESULTS: The internal consistencies for the MDAS were 0.90 and 0.92 in the two samples; for the DFS, the internal consistencies were 0.96 in both samples. The test-retest reliabilities were 0.94 for the MDAS and 0.95 for the DFS. The correlation between the two questionnaires was 0.89. The patients' responses to both questionnaires were significantly related to the dentists' ratings of their anxiety during dental treatment (both p values <0.001). CONCLUSION: The results indicate that the Greek versions of the MDAS and DFS have good internal consistencies and test-retest reliabilities, as well as good construct and criterion validities. The psychometric properties of the Greek versions of these questionnaires appear to be similar to those previously reported in other countries.

Reliability and validity of the Greek version of the Children's Fear Survey Schedule-Dental Subscale.

BACKGROUND: The Children's Fear Survey Schedule-Dental Subscale (CFSS-DS) is a commonly used questionnaire which measures children's dental fear. OBJECTIVE: The aim of this study was to gather data to evaluate the psychometric properties of a Greek version of the CFSS-DS. METHODS: A sample of 260 children aged 4-12 completed the Greek version of the CFSS-DS while in the waiting room of a paediatric dentist. The dentist, who was unaware of the children's scores, rated the children's behaviour during the dental appointment using the Frankl scale. Children who returned for a second dental appointment during the study period completed the CFSS-DS a second time. RESULTS: The mean CFSS-DS score was 24.80 (standard deviation = 9.17). Age and gender were not related to mean scores. Invasiveness of dental treatment was not related to mean scores. Children who were most uncooperative/fearful on the Frankl had the highest mean scores (Kruskal-Wallis chi2 = 9.48; d.f. = 2; P = 0.009). The internal consistency (Cronbach's alpha) was 0.85, and the test-retest reliability (intraclass correlation) was 0.74. CONCLUSIONS: The Greek version of the CFSS-DS appears to be reliable and valid. Further samples should include school samples, to include children who may not go to the dentist.

Nitrous oxide-antinociception is mediated by opioid receptors and nitric oxide in the periaqueductal gray region of the midbrain.

Previous studies have shown that nitrous oxide (N(2)O)-induced antinociception is sensitive to antagonism by blockade of opioid receptors and also by inhibition of nitric oxide (NO) production. The present study was conducted to determine whether these occur within the same brain site. Mice were stereotaxically implanted with microinjection cannulae in the periaqueductal gray (PAG) area of the midbrain. In saline-pretreated mice, exposure to 70% N(2)O resulted in a concentration-dependent antinociceptive effect in the mouse abdominal constriction test. Pretreatment with an opioid antagonist in the PAG significantly antagonized the antinociceptive effect. Pretreatment with an inhibitor of NO production in the PAG also significantly antagonized the antinociceptive effect. These findings suggest that N(2)O acts in the PAG via an NO-dependent, opioid receptor-mediated mechanism to induce antinociception.

Advances in understanding the actions of nitrous oxide.

Nitrous oxide (N(2)O) has been used for well over 150 years in clinical dentistry for its analgesic and anxiolytic properties. This small and simple inorganic chemical molecule has indisputable effects of analgesia, anxiolysis, and anesthesia that are of great clinical interest. Recent studies have helped to clarify the analgesic mechanisms of N(2)O, but the mechanisms involved in its anxiolytic and anesthetic actions remain less clear. Findings to date indicate that the analgesic effect of N(2)O is opioid in nature, and, like morphine, may involve a myriad of neuromodulators in the spinal cord. The anxiolytic effect of N(2)O, on the other hand, resembles that of benzodiazepines and may be initiated at selected subunits of the gamma-aminobutyric acid type A (GABA(A)) receptor. Similarly, the anesthetic effect of N(2)O may involve actions at GABA(A) receptors and possibly at N-methyl-D-aspartate receptors as well. This article reviews the latest information on the proposed modes of action for these clinical effects of N(2)O.

A study of the role of serotonin in the anxiolytic effect of nitrous oxide in rodents.

RATIONALE: In earlier studies, we have shown that nitrous oxide (N2O)-induced behavioral effects in rats and mice are mediated by benzodiazepine receptors. OBJECTIVES: This two-part study was conducted in order to investigate the possible role of serotonin (5-HT) in the behavioral effects of N2O by clarifying its effects on regional brain concentrations of 5-HT and assessing the influence of 5-HT antagonist and reuptake inhibiting drugs on the anxiolytic-like behavioral effect of N2O. METHODS: In experiment A, male, 150-200 g Sprague-Dawley rats were killed following a 15-min exposure to room air or 70% N2O. The frontal cortex, hippocampus, corpus striatum and hypothalamus were dissected out and analyzed by HPLC with electrochemical detection for content of 5-HT and 5-hydroxyindoleacetic acid (5-HIAA); dopamine and 3,4-dihydroxyphenylacetic acid (DOPAC) were also measured. In experiment B, male 18-22 g NIH Swiss mice were pretreated with the 5-HT2 antagonist cinanserin, the 5-HT3 antagonist LY-278,584, the 5-HT reuptake inhibitor fluoxetine or saline and tested in the light/dark exploration test under 70% N2O 30 min after pretreatment. RESULTS: In experiment A, N2O produced differential effects on 5-HT neurons in distinct brain areas. There was increased 5-HT turnover in the hypothalamus, decreased turnover in the frontal cortex but no changes in either hippocampus or corpus striatum. By comparison, dopamine turnover in these brain regions was unaltered by N2O exposure. In experiment B, pretreatment with neither cinanserin, LY-278,584 nor fluoxetine had any appreciable effect on the N2O-induced increase in time spent in the light compartment. Only cinanserin significantly reduced the N2O-induced increase in transitions. CONCLUSIONS: While neurochemical results suggest an effect of N2O on brain 5-HT function, there was no effect of 5-HT2 or 5-HT3 antagonists or 5-HT reuptake inhibitor on N2O-induced anxiolytic-like behavior.