Dataset Information

Oral health-related quality of life in X-linked hypophosphataemia and osteogenesis imperfecta.

ABSTRACT: X-linked hypophosphataemia (XLH) and osteogenesis imperfecta (OI) are rare congenital disorders characterised by skeletal dysplasia. The two disorders may include dental anomalies potentially affecting individual well-being. The aims of study were (a) to assess the oral health-related quality of life (OHRQoL) in Danish adults with XLH or OI, and (b) to compare the results of the groups. A cross-sectional study including 35 adults with XLH, 56 adults with OI type I and 17 adults with OI types III-IV was conducted. The OHRQoL was assessed by the 49-item version of the questionnaire Oral Health Impact Profile (OHIP). Summed domain scores (seven) were compared between XLH and OI groups. Prevalence of severe impact on OHRQoL (scores 3-4) was compared between groups. The median scores in XLH group exceeded the medians in OI (P < .05) in the domains functional limitation (XLH:6.5; OI:4.0), pain (XLH:9.5; OI:5.0), psychological discomfort (XLH:5.5; OI:2.0), psychological disability (XLH:2.0; OI:0.0), handicap (XLH:2.0; OI:0.0) and total OHIP (XLH:35.0; OI:14.0). Differences in domains physical disability (XLH: 4.0; OI: 1.0) and social disability (XLH: 0.0; OI: 0.0) were not significant. Prevalence of severe impact on OHRQoL in the XLH group significantly exceeded the level in OI group in the domains functional limitation (XLH: 59%; OI: 35%), psychological discomfort (XLH: 38%; OI: 20%) and physical disability (XLH: 32%; OI: 13%). In conclusion, adults with XLH experience a higher negative impact on their OHRQoL than adults with OI. Only to a minor degree, individuals with OI types III-IV experience a higher impact on OHRQoL than individuals with OI type I.

SUBMITTER: Gjorup H

PROVIDER: S-EPMC7839549 | biostudies-literature | 2021 Feb

REPOSITORIES: biostudies-literature

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Oral health-related quality of life in X-linked hypophosphataemia and osteogenesis imperfecta.

Gjørup Hans H Beck-Nielsen Signe Sparre SS Hald Jannie Dahl JD Haubek Dorte D

Journal of oral rehabilitation 20201119 2

X-linked hypophosphataemia (XLH) and osteogenesis imperfecta (OI) are rare congenital disorders characterised by skeletal dysplasia. The two disorders may include dental anomalies potentially affecting individual well-being. The aims of study were (a) to assess the oral health-related quality of life (OHRQoL) in Danish adults with XLH or OI, and (b) to compare the results of the groups. A cross-sectional study including 35 adults with XLH, 56 adults with OI type I and 17 adults with OI types III ...[more]

PMID: 33058298

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Project description:Osteogenesis imperfecta (OI) is a rare, connective tissue disorder characterised by bone fragility, resulting in recurrent fractures and skeletal deformities. Extra-skeletal manifestations include dentinogenesis imperfecta, hearing abnormalities and lung disease. These co-morbidities combined with recurrent fractures can exert a significant impact on health-related quality of life (HR-QOL). It is important to assess HR-QOL throughout adulthood because the prevalence of some OI-specific complications increases with age. PubMed, EMBASE and CENTRAL databases were searched on 2nd February 2022 to identify studies reporting quantitative assessments of HR-QOL in adults with OI. The primary endpoint was to determine the impact of an OI diagnosis on adult's HR-QOL. Secondary endpoints were to (i) examine how frequently various HR-QOL assessment tools were used (ii) identify differences in HR-QOL between OI types and (iii) investigate the determinants of HR-QOL in adults with OI. Search results were exported to Endnote where two reviewers independently conducted title/abstract and full-text reviews. Data from accepted studies were extracted into Microsoft Excel. A narrative synthesis was then undertaken. The review identified 17 studies with a total of 1,648 adults. The Short Form-36 (SF-36) was the most frequently reported HR-QOL assessment tool and was used in nine studies. Physical HR-QOL was reduced in adults with OI. Physical component scores (PCS) or individual physical domains of the SF-36 were lower in eight of nine studies. Mental component scores (MCS) were preserved in all six studies, however individual mental health domains of the SF-36 were reduced in some studies. The prevalence of anxiety/depression was relatively low in adults with OI. Those with type III OI had lower physical and respiratory HR-QOL but preserved mental HR-QOL compared with type I. The prevalence of fatigue and pain was higher in adults with OI compared with reference populations. Age and cardio-pulmonary co-morbidities were associated with lower HR-QOL. OI in adulthood has a wide-ranging negative impact on HR-QOL. Physical and respiratory HR-QOL were lower, while the prevalence of pain and fatigue were higher than in reference populations. Mental HR-QOL was relatively preserved, although some deficits were identified. Age and cardio-pulmonary co-morbidities were associated with lower HR-QOL.

Project description:Osteogenesis imperfecta (OI) is characterized by bone fragility and secondary features such as blue sclerae, dentinogenesis imperfecta, hearing loss, ligamentous laxity, and short stature. It was thought that health-related quality of life (QoL) in patients with OI mainly depends on the severity of the skeletal deformities. However, it has become clear that additional factors can affect the QoL in all patients with OI. In this study, we compare dimensions of QoL in adults with OI with a control population. The SF-36 questionnaire was distributed among 330 adult patients with different OI types. Results were compared with two control populations from the Netherlands. Age-matched comparisons were made with one of the two control populations. The results were summarized in eight domains: general and mental health, physical and social function, bodily pain, vitality, and physical and emotional role. General health and physical function in all types of OI are low compared with controls, except patients with OI type 4 aged 55+ years. Bodily pain in patients with OI appeared significantly worse than in the control population. There was no significant difference between OI types regarding pain and vitality. Vitality was only in the OI type 1 group significantly lower compared with controls. Patients with OI type 1 had a significantly reduced mental health. Social functioning appeared most effective in type 3 around 20 years of age. QoL in adult patients with OI should be an important outcome measure in every OI clinic, but the amount of baseline data on this subject is sparse. This baseline measurement study is the largest study to date investigating QoL in adult patients with OI. The mean scores indicate that people with OI generally have a significantly lower QoL than the control population. Further qualitative evaluation of QoL and its influences is important for future management. © 2020 The Authors. JBMR Plus published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research.

Project description:OBJECTIVES:To integrate items from two widely used oral health-related quality of life (OHRQoL) questionnaires, the General Oral Health Assessment Index (GOHAI) and the Oral Impacts on Daily Performances (OIDP), as well as culturally-specific items of the Oral Health Impact Profile (OHIP) into a four-dimensional OHRQoL model consisting of Oral Function, Orofacial Pain, Orofacial Appearance, and Psychosocial Impact. METHODS:Subjects came from an ancillary study of the Dimensions of Oral Health-Related Quality of Life Project (N=267 patients, mean age±SD: 54.0±17.2years, 58% women.) Patients filled in the original 49 items of OHIP and 22 additional OHRQoL items in a cross-sectional study. These additional items consisted of 7 culturally specific OHIP items and 15 GOHAI or OIDP items with unique content not covered in OHIP-49. Before data collection, three experts hypothesized to which of the four OHRQoL dimensions these items belong. Hypotheses were tested in correlation analyses between the 22 items and the four dimension scores that were derived from OHIP-49. RESULTS:Five of the 22 items did not provide sufficient information to which dimension they belong. In 16 of the remaining 17 items, the pattern of correlation coefficients fitted experts' a priori hypotheses. Acceptance of 16 of the 17 hypotheses was interpreted as evidence that additional (not in OHIP-49 contained) OHRQoL items can be assigned to Oral Function, Orofacial Pain, Orofacial Appearance, and Psychosocial Impact. CONCLUSION:Items of three OHRQoL instruments can be integrated into a dimensional OHRQoL model consisting of Oral Function, Orofacial Pain, Orofacial Appearance, and Psychosocial Impact. CLINICAL SIGNIFICANCE:Oral Function, Orofacial Pain, Orofacial Appearance, and Psychosocial Impact can serve as a simple and clinically appealing set of oral health-related quality of life (OHRQoL) dimensions and therefore provide an opportunity for simpler, but psychometrically improved OHRQoL measurement in the future.

Dataset Information

Oral health-related quality of life in X-linked hypophosphataemia and osteogenesis imperfecta.

Publications

Oral health-related quality of life in X-linked hypophosphataemia and osteogenesis imperfecta.

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