The impact of osteogenesis imperfecta severity on oral health-related quality of life in Spain: a cross-sectional study.

BACKGROUND: Osteogenesis imperfecta (OI) affects dental and craniofacial development; therefore, it can influence oral health-related quality of life (OHRQoL). The objective of this study was to explore the influence of the severity of OI on OHRQoL in adults older than eighteen years residing in Spain. METHOD: Adults with OI were recruited from the Spanish Association of Crystal Bone (AHUCE) foundation. OHRQoL was evaluated using the Spanish version of the Oral Health Impact Profile questionnaire (OHIP-14sp), oral hygiene habits, and a dental care survey. Clinical and radiological dental examinations were performed to evaluate the patients' oral conditions. RESULTS: A total of 65 adults (n = 46 females) aged between nineteen and sixty-two years who were diagnosed with OI and classified as type I, III, and IV (n = 20, 14, and 31, respectively) participated in this research. The total OHIP-14sp scores were significantly greater (worse) for type III (23 [SD = 10]) and type IV (21.4 [SD = 12]) than for type I (13.8 [SD = 6]) (P < 0.05). The negative impact of OHRQoL was due to the association of type III OI with all domains except for the handicap domain, while type IV OI was associated with the physical disability, social disability, and handicap domains (P < 0.05 for all). CONCLUSION: The severity of OI negatively impacted OHRQoL in adults. This association was statistically significant.

Malocclusion traits and oral health-related quality of life in children with osteogenesis imperfecta: A cross-sectional study.

BACKGROUND: The incidence of malocclusion is higher among people with osteogenesis imperfecta (OI) than the general population, and treatment options are limited due to the weak structure of bones and teeth. Focusing on those malocclusion traits that might have a high impact on a patient's oral health-related quality of life (OHRQoL) is warranted. METHODS: A total of 138 children and adolescents with OI were examined for malocclusion traits. OHRQoL was measured using age-specific versions (8 through 10 years and 11 through 14 years) of the Child Perceptions Questionnaire (CPQ), considering the following domains: oral symptoms, functional limitation, emotional well-being, and social well-being. Higher scores implied worse OHRQoL. Multivariable ordinal logistic regression was used to estimate the association between malocclusion traits and OHRQoL. RESULTS: Among children aged 8 through 10 years (n = 56), the CPQ and its constituent domain scores were relatively similar between those with malocclusion (higher scores) and those without. In the adolescent (n = 82) group aged 11 through 14 years; however, those with posterior crossbite (odds ratio, 5.01; 95% confidence interval, 1.40 to 12.41) or open bite (odds ratio, 3.21; 95% confidence interval, 1.21 to 10.23) experienced statistically significantly higher degrees of functional limitations (a higher functional limitation score) than those without. CONCLUSIONS: Adolescents with OI and posterior open bites or crossbites have substantial self-reported functional limitations and worse oral symptoms, which warrants additional investigation and therapeutic trials in an attempt to improve the malocclusion. In addition, the authors found that the CPQ can be a useful tool in a clinical trial of orthodontic interventions in OI.

Caries prevalence and experience in individuals with osteogenesis imperfecta: A cross-sectional multicenter study.

OBJECTIVE: Dentinogenesis Imperfecta (DI) forms a group of dental abnormalities frequently found associated with Osteogenesis Imperfecta (OI), a hereditary disease characterized by bone fragility. The objectives of this study were to quantify the dental caries prevalence and experience among different OI-types in the sample population and quantify how much these values change for the subset with DI. METHODS: To determine which clinical characteristics were associated with increased Caries Prevalence and Experience (CPE) in patients with OI, the adjusted DFT scores were used to account for frequent hypodontia, impacted teeth and retained teeth in OI population. For each variable measured, frequency distributions, means, proportions and standard deviations were generated. Groups means were analyzed by the unpaired t-test or ANOVA as appropriate. For multivariate analysis, subjects with caries experience of zero were compared with those with caries experience greater than zero using logistic regression. RESULTS: The stepwise regression analysis while controlling for all other variables demonstrated the presence of DI (OR 2.43; CI 1.37-4.32; P = 0.002) as the significant independent predictor of CPE in the final model. CONCLUSION: This study found no evidence that CPE of OI subjects differs between the types of OI. The presence of DI when controlled for other factors was found to be the significant predictor of CPE.

Oral health-related quality of life in children and adolescents with osteogenesis imperfecta: cross-sectional study.

BACKGROUND: Osteogenesis imperfecta (OI) affects dental and craniofacial development and may therefore impair Oral Health-Related Quality of Life (OHRQoL). However, little is known about OHRQoL in children and adolescents with OI. The aim of this study was to explore the influence of OI severity on oral health-related quality of life in children and adolescents. METHODS: Children and adolescents aged 8-14 years were recruited in the context of a multicenter longitudinal study (Brittle Bone Disease Consortium) that enrolls individuals with OI in 10 centers across North America. OHRQoL was assessed using the Child Perceptions Questionnaire (CPQ) versions for 8 to 10-year-olds (CPQ8-10) and for 11 to 14-year-olds (CPQ11-14). RESULTS: A total of 138 children and adolescents (62% girls) diagnosed with OI types I, III, IV, V and VI (n = 65, 30, 37, 4 and 2, respectively) participated in the study. CPQ8-10 scores were similar between OI types in children aged 8 to 10 years. In the 11 to 14-year-old group, CPQ11-14-scores were significantly higher (i.e. worse) for OI types III (24.7 [SD 12.5]) and IV (23.1 [SD 14.8]) than for OI type I (16.5 [SD 12.8]) (P < 0.05). The difference between OI types was due to the association between OI types and the functional limitations domain, as OI types III and IV were associated with significantly higher grade of functional limitations compared to OI type I. CONCLUSION: The severity of OI impacts OHRQoL in adolescents aged 11 to 14 years, but not in children age 8 to 10 years.