Oral health-related quality of life in Loeys-Dietz syndrome, a rare connective tissue disorder: an observational cohort
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(2019) 14:291
RESEARCH
Open Access
Oral health-related quality of life in LoeysDietz syndrome, a rare connective tissue disorder: an observational cohort study Quynh C. Nguyen1 , Olivier Duverger1* , Rashmi Mishra1 , Gabriela Lopez Mitnik2 , Priyam Jani1 , Pamela A. Frischmeyer-Guerrerio3 and Janice S. Lee1*
Abstract Background: Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder whose oral manifestations and dental phenotypes have not been well-characterized. The aim of this study was to explore the influence of oral manifestations on oral health-related quality of life (OHRQoL) in LDS patients. Material and methods: LDS subjects were assessed by the craniofacial team at the National Institutes of Health Clinical Center Dental Clinic between June 2015 and January 2018. Oral Health Impact Profile (OHIP-14) questionnaire, oral health self-care behavior questionnaire and a comprehensive dental examination were completed for each subject. OHRQoL was assessed using the OHIP-14 questionnaire with higher scores corresponding to worse OHRQoL. Regression models were used to determine the relationship between each oral manifestation and the OHIP-14 scores using a level of significance of p ≤ 0.05. Results: A total of 33 LDS subjects (51.5% female) aged 3–57 years (19.6 ± 15.1 years) were included in the study. The OHIP-14 scores (n = 33) were significantly higher in LDS subjects (6.30 [SD 6.37]) when compared to unaffected family member subjects (1.50 [SD 2.28], p < 0.01), and higher than the previously reported scores of the general U.S. population (2.81 [SD 0.12]). Regarding oral health self-care behavior (n = 32), the majority of LDS subjects reported receiving regular dental care (81%) and maintaining good-to-excellent daily oral hygiene (75%). Using a crude regression model, worse OHRQoL was found to be associated with dental hypersensitivity (β = 5.24; p < 0.05), temporomandibular joints (TMJ) abnormalities (β = 5.92; p < 0.01), self-reported poor-to-fair oral health status (β = 6.77; p < 0.01), and cumulation of four or more oral manifestations (β = 7.23; p < 0.001). Finally, using a parsimonious model, self-reported poor-to-fair oral health status (β = 5.87; p < 0.01) and TMJ abnormalities (β = 4.95; p < 0.01) remained significant. Conclusions: The dental hypersensitivity, TMJ abnormalities, self-reported poor-to-fair oral health status and cumulation of four-or-more oral manifestations had significant influence on worse OHRQoL. Specific dental treatment guidelines are necessary to ensure optimal quality of life in patients diagnosed with LDS. Keywords: Rare diseases, Rare connective tissue disorders, Loeys-Dietz syndrome (LDS), Oral health-related quality of life (OHRQoL), Oral health impact profile (OHIP-14)
* Correspondence: [email protected]; [email protected] The research was presented at the 2019 National Oral Health Conference. 1 Craniofacial Anomalies and Regeneration Section, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD, USA Ful
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