The possibilites of prevention of oral diseases in children with epidermolysis bullosa dystrophica

Abstract

Epidermolysis bullosa (EB) is a genetic multisystem, genetically and clinically heterogeneous group of diseases characterized by extreme sensitivity of the skin and mucous membranes, where blisters and erosions occur after minor mechanical trauma. Patients suffering from epidermolysis bullosa dystrophica (DEB) are highly-risk group of patients for caries and periodontal diseases due to poor oral hygiene, specific diet and changed function of the oral cavity (blisters and erosions on mucousa, microstomia, ankyloglossia, loss of the vestibular space, absence of lingual papillae and palatal folds and enamel hypoplasia). The aim of this study was to determine the level of knowledge and opinions of parents about the implementation of preventive measures and quality of dental care of affected children, then to examine whether there are statistically significant differences in the parameters of oral health status among children with DEB and healthy control group and then to examine whether the oral health status of children with DEB may significantly improve with the continuous and regular use of dental preventive and prophylactic measures. Materials and Methods: This study was conducted at the Clinic for Pediatric and Preventive Dentistry at School of Dental Medicine at the University of Belgrade. Participants of this study included a group of 24 patients (8 (33.0%) female and 16 (67.0%) male sex) suffering from epidermolysis bullosa dystrophica (4 (16.7%) had dominant, and 20 (83.3%) recessive type of epidermolysis bullosa dystrophica) who live on the territory of Serbia and who are in parental custody. Healthy controls were correspondingly matched individually by sex and age (±6 months). The level of knowledge and opinion of parents about the quality of dental care of affected children was obtained by filling a specifically designed questionnaires for this study. In the clinical examination there were investigated and recorded parameters of oral health status: dental status, oral hygiene status, condition of the gingiva and condition of oral mucosa...

Epidermolysis bullosa (EB) je nasledna multisistemska, genetički i klinički heterogena grupa bolesti koju karakteriše izuzetna osetljivost kože i sluzokoža, na kojima se bule i erozije javljaju nakon slabe mehaničke traume. Pacijenti oboleli od epidermolysis bullosa dystrophica (DEB) predstavljaju visko rizičnu grupu pacijenata za nastanak karijesa i parodontalnih oboljenja zbog loše oralne higijene, posebnog režima ishrane i izmenjene funkcije usne duplje (bule i erozije po sluzokoži, mikrostomija, ankiloglosija, smanjen vestibulum, odsustvo jezičnih papila i palatinalnih plika i hipoplazija gleđi). Cilj ove studije je bio da se utvrdi nivo znanja i mišljenja roditelja o primeni preventivnih mera u stomatologiji i kvalitetu stomatološke zaštite obolele dece, zatim da se ispita da li postoje statistički značajne razlike u vrednostima parametara stanja oralnog zdravlja izmedju dece obolele od DEB i zdrave kontrolne grupe i zatim ispitati da li se stanje oralnog zdravlja dece obolele od DEB može statistički značajno poboljšati kontinuiranom i redovnom primenom stomatoloških preventivnih i profilaktičkih mera. Materijal i metode: Istraživanje je sprovedeno na Klinici za dečju i preventivnu stomatologiju, Stomatološkog fakulteta, Univerziteta u Beogradu. U okviru studije pregledana su 24 pacijenta (8 (33.0%) ženskog i 16 (67.0%) muškog pola) obolela od epidermolysis bullosa dystrophica (4 (16.7%) je imalo dominantan, a 20 (83.3%) recesivan oblik distrofične bulozne epidermolize) koji žive na teritoriji Srbije i koji su na roditeljskom staranju. Kontrolnu grupu predstavljao je isti broj zdrave dece odgovarajudeg pola i uzrasta (±6meseci), koji su odabirani po principu individualnog mečovanja. Nivo znanja i mišljenje roditelja o stomatološkoj zaštiti obolele dece je dobijen popunjavanjem specijalno dizajniranih anketnih upitnika. Na kliničkom pregledu ispitivani su i zabeleženi parametri stanja oralnog zdravlja: stanje zuba, stanje oralne higijene, stanje gingive i stanje oralne sluzokože. Preventivne i profilaktičke mere bile su sprovedene kroz 3 kontrolna pregleda, na 6 meseci, u periodu od godinu i po dana i zabeležene u stomatološki karton pacijenta...