Dental Manifestations Research Articles

SHORT Syndrome: Systematic Appraisal of the Medical and Dental Phenotype.

SHORT syndrome is a rare autosomal dominant condition described by its acronym of short stature, hyperextensibility of joints and/or inguinal hernia, ocular depression, Rieger abnormality, and teething delay. Individuals have a distinct progeroid craniofacial appearance with a triangular face, frontal bossing, hypoplastic or thin alae nasi, large low-set ears, and mandibular retrognathia. To systematically appraise the literature and update the clinical phenotype with emphasis on the dental condition. A systematic literature search was carried out to update the clinical phenotype, identifying reports of individuals with SHORT syndrome published after August 2015. The same search strategy but not limited to publication date was carried out to identify reports of the dental phenotype. Two independent reviewers screened 1937 articles with 55 articles identified for full-text review. Nineteen individuals from 11 families were identified. Facial dysmorphism including ocular depression, triangular shaped face, frontal bossing, large low-set ears, and micrognathia were the most consistent features followed by lipodystrophy, insulin resistance, and intrauterine growth restriction. Teething delay, microdontia, hypodontia, and enamel hypoplasia have all been reported. Features that comprise the SHORT acronym do not accurately or completely describe the clinical phenotype. The craniofacial appearance is one of the most consistent features. Lipodystrophy and insulin resistance may also be considered cardinal features. After teething delay, enamel hypoplasia and microdontia are the most common dental manifestations. We present recommendations for the dental and orthodontic/orthognathic management of individuals with SHORT syndrome.

Oral manifestations of eating disorders in adolescent patients. A review.

Eating disorders (ED) are a group of psychopathological disorders that affect the patient's relationship with food and his own body and that are manifested mainly in adolescence and in young-adult age. ED include anorexia nervosa (AN), bulimia nervosa (BN) and other eating disorders as classified in the DMS-V. ED can result in several oral and dental manifestations that often occur in the early stages of ED and may allow early detection. The aim of the study is to describe the different oral and dental manifestations in patients with ED in order to offer a classification for their identification during an extra/intra-oral examination. A search on PubMed, Medline and Cochrane Library data bases has been performed. Oral manifestations in ED patients include a variety of signs and symptoms, which involve the oral mucosa and perioral tissues (exfoliative cheilitis, labial erythema, atrophic glossitis, glossodynia, yellow-orange colouration of the soft palate, cheek/lip biting, candidiasis), the teeth (dental erosion, tooth hypersensitivity, dental caries), periodontal diseases, and salivary manifestations (sialoadenosis, alterations in salivary flow). The oral signs are caused by a number of factors, including nutritional deficiencies and consequent metabolic changes, poor personal hygiene, altered eating habits and pharmacological therapies. There is a very specific link between oral manifestations and ED in the presence of self-induced vomiting. The paediatric dentist may be the first professional to detect the clinical signs thus improving the interception, early diagnosis, characterisation and prognosis of ED. In addition, the oral manifestations of ED can cause alterations of the oral function, discomfort, oral pain, and worsen aesthetics of the face and the quality of life.

Dental mask of gastroesophageal reflux disease

In clinical practice, there are patients for whom traditional methods of treatment do not allow getting rid of a dental disease. Dental mask of underlying pathology may be a potential cause of this. The aim of the study was to study the features of the clinical course of dental manifestations of gastroesophageal reflux disease. Methods. The study group included 45 patients aged 20 to 50 years with complaints of pathological changes in the oral cavity that did not stop during the last month. The survey included a survey and clinical examination. The GerdQ questionnaire was used to verify gastrointestinal disorders. Results. It was found that 85% of the surveyed complained of a burning sensation behind the breastbone, the ingress of acidic contents into the oral cavity. 100% of the surveyed presented dental complaints. At the same time, the frequency of dental complaints was significantly higher compared to the frequency of complaints of esophageal symptoms. According to the results of the GerdQ questionnaire, the average score among the subjects was 11.0, which corresponds to the presence of gastroesophageal reflux disease. According to the results of the dental examination, 87% of the examined patients had pathological elements on the oral mucosa, and the pathological elements of aphthous and herpetic stomatitis were found significantly more often in patients with an exacerbation of gastroesophageal reflux disease (p<0.05). Desquamative glossitis was detected in 33% of the examined patients, benign migratory glossitis (“geographical” tongue) in 59% of patients, exfoliative cheilitis (dry form) in 70% of patients, angular cheilitis in 23% of patients. The pathology of hard dental tissues was represented by multiple carious lesions in 75% of patients and non-carious lesions in 53% of patients. Conclusions. Diseases of the oral mucosa and non-carious lesions accompany patients with gastrointestinal tract pathology in the form of gastroesophageal reflux disease.

A Report of Incontinentia Pigmenti in an 11-year-old Girl

Introduction: Incontinentia pigmenti (IP) is a rarely diagnosed x-linked dominant disease affecting tissues of ectodermal and mesodermal origin such as cutaneous tissues, teeth, eyes, hair, and the central nervous system. Dermatologic manifestations are often the first signs observed in patients diagnosed with IP and are present in nearly all the subjects, but they are less harmful and do not require treatment. Oral manifestations in patients diagnosed with IP might affect both the deciduous and permanent teeth, with tooth shape anomalies and hypodontia, delayed tooth eruption, cleft palate, and high arched palate. These oral abnormalities influence feeding, quality of life (QoL), and self-esteem of the patient but can be successfully corrected by oral rehabilitation. Case Presentation: Here, we report the case of a female patient, aged 11 years, presenting with dental manifestations such as hypodontia, conical teeth, delayed tooth eruption, narrow and atrophic dental ridge, and also some non-dental findings of IP. Her dental ‎management included oral hygiene instructions, extraction of all unrestorable primary molars, and composite filling of all primary canines. Conclusions: A removable space maintainer was constructed for the patient, which resulted in favorable esthetic outcomes, proper re-establishment of mastication, and improved self-esteem.

Oral and dental manifestation of Allgrove syndrome: A case report

Oral and dental manifestation of Allgrove syndrome: A case report

Key dental challenges during the COVID-19 pandemic: an interdisciplinary platform

Relevance. The article presents an analysis of the key dental problems during the pandemic of the novel coronavirus infection (COVID-19), discussed on the platform of the interdisciplinary all-Russian online conference „Prevention of cross-infection at dental appointments in the context of the COVID-19 pandemic”. The high professional interest of dentists in the problems of high risk of transmission of infection at a dental appointment, the role of dental manifestations in the general symptom complex of COVID-19, the possibilities of testing saliva for SARS-CoV-2, the peculiarities of providing specific types of dental care in different periods of a pandemic, dental (hygienic) accompanying patients in outpatient and inpatient treatment, including in intensive care units. Based on the analysis of existing data on the immunopathogenesis of the disease, the main risk factors and routes of infection, the principles of systematization of COVID-associated diseases (conditions) of the oral cavity, the proposed approaches to their correction and treatment were discussed. Based on the results of our own observations and analysis of foreign publications, clinical variants of the manifestation of the disease (or its medication support) in the oral cavity have been analyzed. The important role of interdisciplinary approaches (dentists, healthcare organizers, epidemiologists, microbiologists, immunologists, etc.) to solving dental problems during a pandemic is emphasized. The role of modern IT-technologies, telemedicine and teledentistry in the implementation of educational and scientific-practical activities for the medical and dental community during a pandemic is highlighted.

Diagnostic Validity of Orthopantomogram Compared to Dual Energy X-ray Absorptiometry Scan in Detecting Osteoporosis

Introduction Osteoporosis is one of the most common and rampant metabolic bone disorders among the geriatric, particularly affecting postmenopausal women. Even though resorption tends to occur more rapidly in bones with a higher proportion of trabecular bone (e.g., vertebrae, pelvis, calcaneus), bones with significant cortical bone content also do undergo resorption, for example, mandible. The dental manifestations that may indicate low-bone density include loose teeth, receding gums, and ill-fitting or loose dentures. Objective To validate the efficacy of orthopantomograms (OPGs) in recognizing bone mineral density (BMD) changes of the mandible using mandibular cortical index (MCI) and substantiate the same with dual energy X-ray absorptiometry (DEXA) scan on femoral neck and spine. Materials and Methods This cross-sectional study comprised 60 geriatric patients of both genders. All the patients were subjected to panoramic radiographs wherever clinically indicated. The visual analysis was done based on the radiographic appearance of the mandibular cortical border and results were compared with DEXA scan reports, followed by an analysis of three grades of MCI and BMD statistically. Results In our study, out of 40 patients in C2 and C3 subgroups, 67% and 20% were normal, respectively. The incidence of osteopenia was 33% in the C2 group and 70% in the C3 group, whereas Osteoporosis was present only among 10% of the population in the C3 group. The difference between the groups are statistically significant (p = 0.01). These findings imply that a progressive link exists between BMD and deteriorating cortical morphology. Conclusion The purpose of this study is that dentists will be able to refer patients to physicians of suspected low BMD, based on incidental findings on panoramic radiographs for further examination. There is a statistically significant correlation present between DEXA and MCI, so the latter can also be used for screening BMD changes.

Research routes on improved sleep bruxism metrics: Toward a standardised approach.

A recent report from the European Sleep Research Society's task force "Beyond AHI" discussed an issue that has been a long-term subject of debate - what are the best metrics for obstructive sleep apnoea (OSA) diagnosis and treatment outcome assessments? In a similar way, sleep bruxism (SB) metrics have also been a recurrent issue for >30years and there is still uncertainty in dentistry regarding their optimisation and clinical relevance. SB can occur alone or with comorbidities such as OSA, gastroesophageal reflux disorder, insomnia, headache, orofacial pain, periodic limb movement, rapid eye movement behaviour disorder, and sleep epilepsy. Classically, the diagnosis of SB is based on the patient's dental and medical history and clinical manifestations; electromyography is used in research and for complex cases. The emergence of new technologies, such as sensors and artificial intelligence, has opened new opportunities. The main objective of the present review is to stimulate the creation of a collaborative taskforce on SB metrics. Several examples are available in sleep medicine. The development of more homogenised metrics could improve the accuracy and refinement of SB assessment, while moving forward toward a personalised approach. It is time to develop SB metrics that are relevant to clinical outcomes and benefit patients who suffer from one or more possible negative consequences of SB.

Mouthguards for a childhood hypophosphatasia patient to protect periodontal tissue of immature permanent teeth – Case report

Hypophosphatasia (HPP) is an inherited skeletal disorder that features early exfoliation of primary teeth as a major dental manifestation. A 6Y4M Japanese girl diagnosed with childhood HPP came to our clinic. Deep probing depth for all teeth and severe mobility of primary incisors were observed. At 7Y1M, the mandibular central incisors received a traumatic injury, with no treatment performed and slight mobility observed thereafter. Such traumatic injuries were repeated, thus mouthguards (MGs) to protect weak periodontal tissue were applied, which helped to maintain stable conditions. Use of a MG may be helpful for HPP patients.

Oral Manifestations of Rett Syndrome-A Systematic Review.

Rett Syndrome is an x linked developmental disorder which becomes apparent in females after 6 to 18 months of age. It leads to severe impairments including loss of speech, loss of hand movements/manual dexterity, characteristic hand movements such as hang wringing and intellectual disability/learning problems. This systematic review was carried out to identify the dental manifestation of Rett syndrome and to shed light on treatment options available for oral health problems associated with Rett syndrome. A systematic literature search was conducted on the PubMed, Scopus, Biomed, Web of Science, Embase, Google Scholars, Cochrane and CINAHL using the following entries: Rett syndrome (n = 3790), Oral health and Rett syndrome (n = 17), dental health of Rett syndrome patients (n = 13), and the MeSH terms listed below: Rett syndrome and Oral Health (n = 17), Rett syndrome and dentistry (n = 29). The final review included 22 search articles. The most common oral findings was bruxism. Masseteric hypertrophy was also reported. Anterior open bite and non-physiological tooth wear was observed. Other oral manifestations of Rett syndrome included mouth breathing, tongue thrusting, digit/thumb sucking, high arch palate. Increased awareness and dental education amongst dentists and assistants regarding the dental manifestations of Rett syndrome and similar neurodevelopmental disorders is required to improve the level of care and empathy they can provide to these differently able patients. Research on dental aspects of Rett is scarce and this remains a neglected topic.

Dental and Skeletal Manifestation of Sickle-Cell Anaemia and Thalassemia in Saudi Arabia; A Systematic Review

Dental and Skeletal Manifestation of Sickle-Cell Anaemia and Thalassemia in Saudi Arabia; A Systematic Review

Response of papillon–Lefevre syndrome to acitretin

Papillon–Lefevre (PLS) is a rare autosomal recessive disorder of keratinization characterized by symmetric, trans-gradient type palmoplantar keratoderma (PPK), rapidly progressive periodontopathy, and precocious loss of dentition. The management of this condition is difficult and needs a combined approach of both dermatologist and periodontologist. Different treatment options tried previously include keratolytic agents such as salicylic acid, urea, topical steroids in combination with keratolytic, propylene glycol, and systemic retinoids such as etretinate, isotretinoin, and acitretin. Both skin and dental manifestations show good therapeutic responses to systemic retinoids. Thereby, we report a case of PLS in a young female child who showed an excellent response to oral acitretin therapy.

Periodontal status evaluation in adolescents with hereditary rickets-like diseases

Hypophosphatemic rickets (HPPR) and hypophosphatasia (HPP) are hereditary pathologies accompanied by phosphorus metabolism problems and rickets deformities of the skeleton. Periodontal diseases, their diagnostic methods and factors affecting the progress of periodontal tissue damage are the least studied among the dental manifestations of HPPR and HPP. The aim of present cohort study was evaluation of periodontal tissue status in adolescents with HPPR and HPP. A dental examination of 20 adolescents aged 12 to 18 years with genetically and biochemically confirmed hereditary rickets-like diseases (E83.3 Disorders of phosphorus metabolism and phosphatases: hypophosphatemic rickets (HPPR) and hypophosphatasia (HPP)) was performed at the Pediatric Dentistry Department of Moscow State University of Medicine and Dentistry. In the present study clinical examination and cone-beam computed tomography (CBCT) data of patients were analyzed. According to the clinical examination data, a strong direct correlation was found between patient's age and the periodontal diseases severity (correlation coefficient r=0.87) in the study group. CBCT revealed the significant alveolar bone hypomineralization in adolescents with HPPR and HPP in comparison to control group (p<0.05). According to the measurement results obtained a strong inverse correlation between alveolar bone mineral density and alveolar bone reduction (correlation coefficient r= -0.74) in adolescents with hereditary rickets-like diseases was revealed. The diagnostic options of CBCT allow to begin comprehensive dental treatment of patients with HPPR and HPP at an early stage of periodontal disease preventing further development of pathology.

Dental Caries in Adults with Atopic Dermatitis: A Nationwide Cross-Sectional Study in Korea.

BackgroundDental caries is the most prevalent chronic infectious oral disease of multifactorial etiology. Increased risk of dental caries development in patients with asthma and allergic rhinitis has been frequently reported. In contrast, only a few studies on dental caries in patients with atopic dermatitis (AD) have been reported.ObjectiveWe investigated the association between AD and dental caries development in an adult population in the Republic of Korea.MethodsA total of 21,606 adults who participated in the Korean National Health and Nutrition Examination Survey, a nationwide, population-based, cross-sectional survey between 2010 and 2015, were included in the study. Multiple logistic regression analyses with confounder adjustment suggested odds ratios (ORs) to identify the possible association between AD and decayed, missing, filled teeth (DMFT) experience compared to non-AD participants. Multiple Poisson regression analyses estimated the mean ratio of the DMFT index according to the presence of AD.ResultsAfter adjusting for various confounding factors, the prevalence of DMFT was significantly associated with AD (OR, 1.58; 95% confidence interval (CI), 1.08~2.29; p=0.017). In addition, the mean value of the DMFT index was significantly different between the AD and non-AD groups (mean ratio, 1.07; 95% CI, 1.00~1.14; p=0.046).ConclusionAD was significantly associated with the development of dental caries. Dermatologists should be aware of the dental manifestations of AD patients and recommend regular dental check-ups for the early detection of caries.

Oral manifestations of celiac disease in French children

Celiac disease (CD) is an immune-mediated systemic disorder caused by ingestion of the gluten found in wheat, rye, and barley. The currently estimated prevalence in children is about 1%. CD is a chronic enteropathy with gastrointestinal manifestations including diarrhea, abdominal distension and weight loss, but extra-intestinal features are increasingly being reported. Dental and oral manifestations such as dental enamel defects (ED), delay in dental eruption, and recurrent aphthous stomatitis (RAS) are well-recognized manifestations of CD. The aim of this study was to compare the frequency of oral manifestations (ED, RAS and delay in dental eruption) on deciduous and permanent teeth between children with CD and a control population. An oral examination was performed on 28 CD children and 59 control children. All children were younger than 12 years old and had deciduous or mixed dentition. CD children had significantly more ED and RAS than the control group (67.9% vs. 33.9% P=0.004 and 50.0% vs. 21.8% P=0.011, respectively). No delay in dental eruption was observed in CD children. ED were mainly grade I and II of Aine's classification (color defects and slight structural defects). ED were more often seen on CD children's deciduous teeth than on permanent teeth (57.1% and 13.6%, respectively; P<0.001). The main teeth affected by ED are the second molar and canines of the deciduous teeth, and the first molar, central incisor, and lateral incisors of the permanent teeth. RAS and ED that were symmetrical in all quadrants and occurred firstly in teeth that mineralize during the first year of life both seem to be signs of CD. Thus, more information for dentists and pediatricians on these oral manifestations should help improve detection of CD.

Dentoalveolar Defects of Hypophosphatasia are Recapitulated in a Sheep Knock-In Model.

Hypophosphatasia (HPP) is the inherited error-of-metabolism caused by mutations in ALPL, reducing the function of tissue-nonspecific alkaline phosphatase (TNAP/TNALP/TNSALP). HPP is characterized by defective skeletal and dental mineralization and is categorized into several clinical subtypes based on age of onset and severity of manifestations, though premature tooth loss from acellular cementum defects is common across most HPP subtypes. Genotype-phenotype associations and mechanisms underlying musculoskeletal, dental, and other defects remain poorly characterized. Murine models that have provided significant insights into HPP pathophysiology also carry limitations including monophyodont dentition, lack of osteonal remodeling of cortical bone, and differing patterns of skeletal growth. To address this, we generated the first gene-edited large-animal model of HPP in sheep via CRISPR/Cas9-mediated knock-in of a missense mutation (c.1077C>G; p.I359M) associated with skeletal and dental manifestations in humans. We hypothesized that this HPP sheep model would recapitulate the human dentoalveolar manifestations of HPP. Compared to wild-type (WT), compound heterozygous (cHet) sheep with one null allele and the other with the targeted mutant allele exhibited the most severe alveolar bone, acellular cementum, and dentin hypomineralization defects. Sheep homozygous for the mutant allele (Hom) showed alveolar bone and hypomineralization effects and trends in dentin and cementum, whereas sheep heterozygous (Het) for the mutation did not exhibit significant effects. Important insights gained include existence of early alveolar bone defects that may contribute to tooth loss in HPP, observation of severe mantle dentin hypomineralization in an HPP animal model, association of cementum hypoplasia with genotype, and correlation of dentoalveolar defects with alkaline phosphatase (ALP) levels. The sheep model of HPP faithfully recapitulated dentoalveolar defects reported in individuals with HPP, providing a new translational model for studies into etiopathology and novel therapies of this disorder, as well as proof-of-principle that genetically engineered large sheep models can replicate human dentoalveolar disorders. © 2022 The Authors. Journal of Bone and Mineral Research published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research (ASBMR).

The Oral Mucosa Status and the Correlation between the Functional Parameters and the Level of Nitric Oxide Metabolites in Saliva among Patients with GERD.

The study involved 91 patients (48 women and 43 men), aged from 18 to 70 years with GERD. All patients underwent the clinical dental examination according to a single scheme including general clinical manifestations (nausea, single vomiting, belching, heartburn, pain in the epigastrium and around the navel, and poor appetite) and dental manifestations of GERD. The objective assessment of the dental status of the examined patients included the measurement of the functional parameters of the mixed saliva, buffer capacity (BC) of saliva, and the detection of the nitric oxide metabolites (NOx) content in saliva from the right parotid salivary gland (“SRPSG”) and in blood serum using the indirect method based on the determination of the stable metabolites: nitrates and nitrites using the Griess reaction. It was established that salivation rate among patients with GERD with the prevailing of ACR and SACR was at the lower limit of normal values (0.32 + 0.19 ml/min), and the salivation rate among patients with the prevailing of SALCR was low (0.10 + 0.04 ml/min). The BC of saliva among patients with the prevailing of ACR and SACR was high (9.07 + 1.23 mmol eq/l and 9.40 + 1.71 mmol eq/l, respectively) and was reduced among patients with the prevailing of SALCR (7.63 + 0.18 mmol eq/l). The NOx level in SRPSG among patients with GERD was increased (especially in Group 3 (20.93 + 11.23 umol/l)). The direct correlation between the indicators of sialometry, the level of the BC of saliva, and the NOx level in SRPSG were established during the study.

Gastro-esophageal reflux disease: Key messages for clinicians.

Gastroesophageal reflux disease (GERD) is a chronic common disorder for which patients often refer to specialists. In the last decades, numerous studies helped to clarify the pathophysiology and the natural history of this disease. Currently, in the clinical setting, GERD is defined by the presence of symptoms that, when endoscopic investigation is required, permit to distinguish between cases with or without associated esophageal mucosal injuries. These conditions are called erosive reflux disease and non-erosive reflux disease (NERD), respectively. The latter is the most common manifestation of GERD. Symptoms are defined typical, as heartburn and regurgitation, and atypical (also called extra-esophageal), as coughing and/or wheezing, hoarseness, sore throat, otitis media, and dental manifestations. In this context, it is crucial for clinicians to investigate the presence of features of suspected malignancy, as unexplained weight loss, anemia, dysphagia, persistent vomiting, familiar history of cancer, long history of GERD, and beginning of GERD symptoms after the age of 50 years. The presence of these risk factors should induce to perform an endoscopic examination. Particular attention should be given to functional conditions that can mimic GERD, such as functional heartburn and hypersensitive esophagus as well as, more rarely, eosinophilic esophagitis. The former ones have different pathophysiology and this explains the frequent non-response to proton pump inhibitor drugs. This narrative review provides to clinicians a useful and practical overview of the state-of-the-art on advancements in the knowledge of GERD.

Cleidocranial dysplasia- case report

Cleidocranial dysplasia (CCD) is a rare syndrome, occurring at a rate of 1:10,000,000 in the form presented in this clinical case. This syndrome occurs due to an autosomal dominant inheritance characterized by changes in skeletal formation and development, as a result of a mutation associated with the RUNX2 gene, the main regulatory gene for osteoblastic differentiation, resulting in manifestations such as hypoplastic or aplastic clavicles, brachycephaly, hypoplasia of the middle third of the face, and delay or non-closure of fontanelles. Specifically, in the intraoral region, atypical dental manifestations occur, such as failure in the exfoliation of the primary dentition, delayed eruption of permanent teeth, and multiple supernumerary teeth. Thus, this study aims to present a case report of a patient with the pathognomonic characteristics of the syndrome in its rarest form, as well as the radiographic, imaging and clinical manifestations that allow its diagnosis and a discussion on the common manifestations in such patients, forms of treatment, and the conduct of treatment according to the specific needs of that case..

Effects of Enzyme Replacement Therapy for Primary Teeth in a Patient with Infantile Hypophosphatasia

Hypophosphatasia (HPP) is a skeletal disorder characterized by hypomineralization of bone, with early exfoliation of primary teeth. Alkaline phosphatase enzyme replacement therapy (ERT) has been shown to improve bone hypomineralization for patients with HPP, although its dental effects are unknown. A 20-month-old Japanese boy diagnosed with infantile HPP was referred to our clinic because of early exfoliation of primary teeth. The patient had been followed by a pediatrician since the age of 3 months, due to slow weight gain. At the age of 12 months, primary incisors showed sudden exfoliation; at the age of 19 months, a diagnosis of HPP was made based on bone and dental manifestations. ERT was initiated at the age of 21 months. The patient demonstrated stable periodontal conditions of primary molars that erupted after initiation of ERT, due to improved alveolar bone and tooth mineralization. Thus, ERT may improve both dental and systemic conditions.