[Anesthesia for dental care management in children with dystrophic epidermolysis bullosa]. / Anesteziologicheskoe obespechenie stomatologicheskikh vmeshatel'stv u detei s distroficheskim bulleznym epidermolizom.

THE AIM OF THE STUDY: Was to summarize our experience in dental treatment of children with dystrophic epidermolysis bullosa (DEB) under deep intravenous sedation. MATERIAL AND METHODS: The study comprised 11 DEB patients aged 2-17 years who received full oral rehabilitation under deep sedation in Hospital Pediatric Dentistry department of the Central Research Institute of Dentistry and Maxillofacial Surgery in 2021-2022. Intravenous induction was used if a child already had venous access installed or it was feasible before sedation. If not the case inhalation induction with sevoflurane was performed for vein catheterization. Sedation was sustained by propofol infusion up to aimed concentration. Both dental treatment and extractions were carried out. RESULTS: In 11 children 37 teeth were treated and 14 extracted. Mean duration of the procedure under deep sedation was 53 min (30-190 min). In two children multiple dental extractions resulted in extensive oral mucosa injury with bullae and erosions formation in vermillion, cheeks, and hard palate areas. No skin injuries, airway obstruction or long-term complications of both anesthesiologic an dental procedure were observed. The paper describes main preventive measures minimizing risks of skin and mucosa adverse events. CONCLUSION: Intravenous sedation with spontaneous respiration allows full oral rehabilitation in DEB children with minimal risks of skin and mucosal injury associated with intubation techniques when performing described preventive measures. Use of cofferdam is crucial for success and safety of the procedure as it prevents water, dust and dental fragments contamination of oropharynx which is especially important in case of sedation with spontaneous respiration.

[Dental caries prevention program in children and adolescents living in residential institutions]. / Model' profilaktiki kariesa zubov sredi detei i podrostkov, prozhivayushchikh v organizovannom detskom kollektive.

THE AIM OF THE STUDY: Was to assess the effectiveness of a new dental caries prevention program in children of various ages living in residential institutions. MATERIALS AND METHODS: The program of oral health promotion and caries prevention was introduced in the autonomous non-profit organization «Social rehabilitation center Solba¼. The study comprised 98 children aged 12-17 years divided into two groups: the main group of 52 children aged 12-16 years enrolled in the program in 2013 in pre-school (32 children) and primary school age (20 children) and the control group of 46 adolescents aged 12-17 years not included in the program. DMFT and OHI-S index were compared in both groups as well as in children enrolled in the program at various ages. RESULTS: The overall caries prevalence was 84.7%. Statistically significant differences were documented between children enrolled in the program in pre-school and primary school ages regarding both caries prevalence (78% and 89%, respectively) and intensity (3.5 and 5.4 tooth, respectively (p=0.01)). Non-satisfactory, poor and very poor OHI-S was observed in 15.4% of children in the main group and 54.3% of controls. Good oral hygiene was seen in 67.3 and 37%, satisfactory - in 17.3 and 8.7% of children, respectively (p<0.05). CONCLUSION: Programs of dental caries prevention with oral health promotion component are effective for oral hygiene improvement in children living in residential institutions. The key for long-term effectiveness and dental caries prevalence and intensity reduction is the start of the program in pre-school age and education of caregivers and teachers to provide control of dental care in pre-school and primary school children.

[Morphological and functional assessment of the oral mucosa in children with dystrophic epidermolysis bullosa]. / Otsenka morfologicheskogo i funktsional'nogo sostoyaniya slizistoi obolochki rta u detei s distroficheskim bulleznym epidermolizom.

THE AIM OF THE STUDY: To validate modified oral index (MOI) for the assessment of the oral mucosa in children with dystrophic epidermolysis bullosa (DYB). MATERIALS AND METHOS: The study comprised 27 DYB children aged 4 to 18 years. Morphological component of MOI was documented by an intraoral camera with the registration of pathological elements in various of the oral cavity and differentiated scoring. Functional component included registration of ankyloglossia and microstomia measured by Bristol assessment system and orthodontic caliper, correspondingly, and then referred to normal age-matched values. RESULTS: Oral mucosa condition deteriorates in DYB children with age both in morphological and functional aspects. MOI values more than 40 should be seen as prognostically unfavorable as they are always associated with severe functional restrictions. These restrictions are always present in children older than 6. If present in younger age they may indicate poor functional status in future. CONCLUSION: The MOI may be a useful tool for the assessment of the efficacy of the pharmacological agents' impact on the oral mucosa and disease prognosis. Correlation of MOI and general condition of DYB children needs further investigation.

[Oral microbiome in children with dystrophic recessive epidermolysis bullosa]. / Mikrobiota slizistoi obolochki rta u detei s retsessivnym distroficheskim bulleznym epidermolizom.

THE AIM OF THE STUDY: Was to assess bacterial load in oral wounds in children with recessive dystrophic epidermolysis bullosa (RDEB). MATERIALS AND METHODS: The study comprised 77 RDEB children aged 3-18 years (mean age 9.5±3.6 years) and 27 healthy children aged 4-18 years (mean age 9.8±4.1 years) who served as controls. Swabs for bacteriological study were taken from the oral wounds in RDEB patients and non-affected corresponding oral mucosa areas in controls. The microorganism growth was assessed after 24, 48 and 72 hours of incubation (37 °C and 30 °C) with subsequent identification in automatic bacteriological analyzer MicroScan Walk Away (Simens, USA). Results. The study revealed high prevalence and concentrations of Candida albicans (in 40.3% children), Staphilococcus aureus (23.4%), Enterobacter cloacae (9.1%), and Enterobacteria (10.4%) in RDEB children. From these species, only Candida albicans was present in controls (26%). The prevalence and concentration of commensal and pathogenic species correlated positively with age and significant difference was revealed between children at the age of 3-6 and 7-10 years (p=0.001). Thus, bacterial load in oral wounds correlates with the RDEB natural history and fibrosis progression. Delayed oral wound healing was associated with microbiome shift towards biofilm-producing bacteria Staphilococcus aureus and Enterobacter cloacae. CONCLUSION: Oral wounds microbiome may be an indicator of RDEB severity and tendency to oral fibrosis. The decrease of bacterial load in the oral wounds may remove one of the healing obstacles and serve as fibrosis prevention measure.

[Dental age in children with epidermolysis bullosa]. / Zubnoi vozrast u detei s bulleznym epidermolizom.

THE AIM OF THE STUDY: Was to assess dental age and the frequency of dental extractions in children with epidermolysis bullosa (EB) compared to controls. MATERIALS AND METHODS: The study comprised 22 EB children (8 boys and 14 girls aged 4-16 years, mean age 11.1±3.5 years) and 25 healthy children (9 boys and 16 girls aged 4-14 years, mean age 10.1±2.4 years). Dental age was measured on panoramic X-ray by Demirjian method and then compared to child's physical age to estimate retardation or advance in dental maturity. RESULTS: Only in 5 from 22 EB children dental age corresponded to physical age, there was dental age retardation in 7 patients (13 to 68 months, 27±21.5 months in average), but in 10 dental age exceeded physical (4 to 21 months, 12.9±7.8 months in average). In controls where was only one boy with dental age retardation by 18 months, in 7 children dental age corresponded to physical, in 17 dental age advance by 5-45 months (16.0±11.0 months in average) was revealed. Conclusion. Possible dental age retardation in EB patients should be considered when undertaking dental extractions for orthodontic reasons. For more precise orthodontic treatment timing further studies are needed to correlate dental age, skeletal age and BMI in children with EB.

[Age-related dynamics of mouth opening and tongue mobility in children with various forms of epidermolysis bullosa]. / Vozrastnaya dinamika stepeni otkryvaniya rta i podvizhnosti yazyka u detei s razlichnymi formami bulleznogo epidermoliza.

THE AIM OF THE STUDY: Was to assess age-related changes in mouth opening and tongue mobility in children with various forms of epidermolysis bullosa (EB). Materials and methods. The study comprised 50 EB children (mean age 8.8±3.9 years). Oral slit width (the distance between mouth commissures at rest), mouth opening amplitude (the distance between incisive edges of the lower and upper incisors) were measured by orthodontic caliper. Tongue mobility was assessed using Bristol Tongue Assessment Tools (BTAT). The results were compared with 55 healthy age-matched controls (mean age 9.3±3.7 years). RESULTS: Mean moth opening in EB group was 22.6±11.1 mm which is twice lower than in controls (44.3±7.2 mm). The amplitude was progressively increasing in EB patients in the mixed dentition period but then dropped drastically in the permanent dentition because of oral fibrosis. Tongue mobility was lower in EB group when compared to controls even in cases with anatomically appropriate frenulum fixation sites. In permanent dentition maximal tongue raising was 8 times lower than in controls. Microstomia and ankyloglossia were very specific for dystrophic EB patients while in EB simplex, junctional EB and Kindler syndrome the values were similar to healthy controls. CONCLUSION: Microstomia, limited mouth opening and tongue mobility are typical oral features of dystrophic EB patients. Age-related dynamics shows progressive growth of the values in mixed dentition with significant lowering after so preventive measures for oral fibrosis are more feasible before permanent dentition phase.

[Dental care access for dystrophic epidermolysis bullosae children in Russian Federation]. / Dostupnost' stomatologicheskoi pomoshchi dlia detei s distroficheskim bulleznym épidermolizom v regionakh RF.

AIM: To assess the quality and access to dental care for children with dystrophic epidermolysis bullosae (DEB) living in RF regions. MATERIAL AND METHODS: 181 (55.9%) out of 324 DEB children registered in RF were enrolled in the study. The parents filled the survey assessing primary examination time, annual number of visits, quality of dental care and dental facilities, as well as access to urgent dental care, dental care under general anesthesia and dental caries prevention. RESULTS: 37.6% of children do not have regular dental examinations. Among these only in 13 cases the parents do not understand the importance of regular dental care and caries prevention, while in 55 cases the reasons are the absence of pediatric dentist and the refusal of dentist to perform examination and treatment. Only parents of 72 (40%) from 181 children think then can get proper dental care at the place of residence. Parents of 103 children stated the presence of facilities for dental treatment under general anesthesia at the place of residence but 40 of them could not have the access to it in case of urgent dental care need. In 61 cases (33.7%) the family must move to federal centers to receive urgent dental care and almost one third of children (51 from 181) receive any kind of dental treatment in federal institutions in Moscow. CONCLUSION: A large part of children with DEB living in distant RF regions have no access even to urgent dental care with cases of dentist's refusal to perform dental examination and treatment because of 'distressing' visual aspect of some DEB patients. This leads to the necessity for the family to move to state federal centers to receive dental treatment which for the financial reasons makes impossible proper frequency of dental examinations and preventive measures resulting in many cases of advanced oral pathology where multiply teeth extractions are needed.

[Oral manifestations of KID syndrome: rare clinical case]. / Stomatologicheskie proiavleniia KID-sindroma: redkii klinicheskii sluchai.

The paper presents a rare clinical case of an infant with KID (Keratitis, Ichthyosis, Deafness) syndrome (about 100 patients reported so far) admitted for histological verification of oral mucosa lesions. Disease pathogenesis defines inadequate reparation and skin and mucosa innate immunity defect leading to higher incidence of bacterial and fungal infections, so the 4-years old girl received treatment for vegetating candidiasis of the oral mucosa for several weeks with no clinical improvement. Initial examination showed that the oral lesions resulted from sharp edges of severely affected carious teeth. Histological study of multifocal biopsy revealed pyogenic granulomas and no signs of SCC. Teeth extraction and symptomatic treatment leaded to significant clinical improvement and some remained mucosal changes may be attributed to syndrome manifestations.