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      Oral health-related quality of life in Loeys-Dietz syndrome, a rare connective tissue disorder: an observational cohort study

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          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.

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          Most cited references20

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          Derivation and validation of a short-form oral health impact profile.

          Growing recognition that quality of life is an important outcome of dental care has created a need for a range of instruments to measure oral health-related quality of life. This study aimed to derive a subset of items from the Oral Health Impact Profile (OHIP-49)-a 49-item questionnaire that measures people's perceptions of the impact of oral conditions on their well-being. Secondary analysis was conducted using data from an epidemiologic study of 1217 people aged 60+ years in South Australia. Internal reliability analysis, factor analysis and regression analysis were undertaken to derive a subset (OHIP-14) questionnaire and its validity was evaluated by assessing associations with sociodemographic and clinical oral status variables. Internal reliability of the OHIP-14 was evaluated using Cronbach's coefficient alpha. Regression analysis yielded an optimal set of 14 questions. The OHIP-14 accounted for 94% of variance in the OHIP-49; had high reliability (alpha = 0.88); contained questions from each of the seven conceptual dimensions of the OHIP-49; and had a good distribution of prevalence for individual questions. OHIP-14 scores and OHIP-49 scores displayed the same pattern of variation among sociodemographic groups of older adults. In a multivariate analysis of dentate people, eight oral status and sociodemographic variables were associated (P < 0.05) with both the OHIP-49 and the OHIP-14. While it will be important to replicate these findings in other populations, the findings suggest that the OHIP-14 has good reliability, validity and precision.
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            Temporomandibular disorders and oral health-related quality of life. A systematic review.

            Oral health-related quality of life (OHRQoL) is considered an important aspect of different oral conditions. It has also gained increased attention in temporomandibular disorders (TMDs) in recent years. The purpose of this study was to systematically review the literature on OHRQoL and TMDs. A systematic search of the dental literature was performed using the Medline and Cochrane Library databases, supplemented by a hand search. Various combinations of search terms related to OHRQoL and TMDs were used. Among numerous titles found in Medline, abstracts and eventually full papers of potential interest were reviewed. Twelve papers fulfilled the inclusion criteria and were included in the review. Most studies used the Oral Health Impact Profile, an instrument with good psychometric properties, for evaluation. All articles described a substantial impact on OHRQoL in TMD patients. Only a small proportion of all patients, a few percent, reported no impact at all. The difference between men and women was small and not significant. The impact appeared to be more pronounced in patients with more signs and symptoms. The perceived impact of pain on OHRQoL seems to be substantial. Two studies found that the impact increased with age among TMD patients. The reviewed studies convincingly demonstrated that OHRQoL was negatively affected among TMD patients.
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              Impact of oral disease on quality of life in the US and Australian populations.

              The US National Health and Nutrition Examination Survey (NHANES 2003-2004) evaluated oral health quality of life for the first time using a previously untested subset of seven Oral Health Impact Profile (OHIP) questions, i.e. the NHANES-OHIP. (i) To describe the impact of dental conditions on quality of life in the US adult population; (ii) to evaluate construct validity and adequacy of the NHANES-OHIP in NHANES 2003-2004 and a comparable Australian survey. In the cross-sectional NHANES 2003-2004 survey of a nationally representative sample of US adults (n=4907), prevalence was quantified as the proportion of adults who reported experiencing one or more impacts fairly often or very often within the past year. Construct validity was tested by comparing prevalence estimates across categories of sociodemographic, dental health and utilization characteristics known to vary in oral health. In 2002, Australian cross-sectional survey of a nationally representative sample of adults (n=2644), adequacy of the NHANES-OHIP questions were tested with reference to a slightly modified version of the OHIP-14 questions. NHANES-OHIP prevalence estimates were markedly similar in the United States (15.3%) and Australia (15.7%). In the US construct, validity was evidenced by higher NHANES-OHIP scores among groups with greater levels of tooth loss, perceived treatment need and problem-oriented visiting and with lack of private dental insurance and low income. In Australia, prevalence for the NHANES-OHIP closely resembled prevalence estimates of the modified OHIP-14. Both varied to a similar degree across levels of tooth loss, perceived treatment need, problem-oriented visiting, and private dental insurance and income, demonstrating adequacy of the NHANES-OHIP as a brief independent instrument. There was acceptable construct validity and adequacy of the NHANES-OHIP questionnaire. In the United States, the impact of oral disease disproportionately affected disadvantaged groups, a finding that supports application of the US Healthy People 2010 major goals of improved quality of life and reduced health disparities.
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                Author and article information

                Contributors
                olivier.duverger@nih.gov
                janice.lee@nih.gov
                Journal
                Orphanet J Rare Dis
                Orphanet J Rare Dis
                Orphanet Journal of Rare Diseases
                BioMed Central (London )
                1750-1172
                16 December 2019
                16 December 2019
                2019
                : 14
                : 291
                Affiliations
                [1 ]ISNI 0000 0001 2205 0568, GRID grid.419633.a, Craniofacial Anomalies and Regeneration Section, National Institute of Dental and Craniofacial Research, National Institutes of Health, ; Bethesda, MD USA
                [2 ]ISNI 0000 0001 2205 0568, GRID grid.419633.a, Program Analysis and Reporting Branch, National Institute of Dental and Craniofacial Research, National Institutes of Health, ; Bethesda, MD USA
                [3 ]ISNI 0000 0001 2164 9667, GRID grid.419681.3, Food Allergy Research Unit, National Institute of Allergy and Infectious Diseases, National Institutes of Health, ; Bethesda, MD USA
                Author information
                https://orcid.org/0000-0003-2084-4473
                http://orcid.org/0000-0003-3200-1019
                https://orcid.org/0000-0002-3696-750X
                https://orcid.org/0000-0003-3985-4890
                https://orcid.org/0000-0001-7768-7865
                https://orcid.org/0000-0003-2259-5546
                https://orcid.org/0000-0001-5164-5958
                Article
                1250
                10.1186/s13023-019-1250-y
                6915860
                31842932
                4c558435-74cc-45d3-9cb7-354aace27704
                © The Author(s). 2019

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 3 July 2019
                : 1 November 2019
                Funding
                Funded by: FundRef http://dx.doi.org/10.13039/100000072, National Institute of Dental and Craniofacial Research;
                Award ID: ZIA DE000746 04 and ZID DE000728 10
                Award Recipient :
                Funded by: FundRef http://dx.doi.org/10.13039/100000060, National Institute of Allergy and Infectious Diseases;
                Award ID: ZIA AI001203 04
                Award Recipient :
                Categories
                Research
                Custom metadata
                © The Author(s) 2019

                Infectious disease & Microbiology
                rare diseases,rare connective tissue disorders,loeys-dietz syndrome (lds),oral health-related quality of life (ohrqol),oral health impact profile (ohip-14)

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