Oral Health-related Quality of Life in X-linked Hypophosphatemia and Osteogenesis Imperfecta.

BACKGROUND X-linked hypophosphatemia (XLH) and Osteogenesis Imperfecta (OI) are rare congenital disorders characterized by skeletal dysplasia. The two disorders may include dental anomalies potentially affecting individual well-being. OBJECTIVES i) to assess the oral health-related quality of life (OHRQoL) in Danish adults with XLH or OI, and ii) to compare the results of the groups. METHODS A cross-sectional study including 35 adults with XLH, 56 adults with OI type I, and 17 adults with OI types III-IV. 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. RESULTS The median scores in XLH group exceeded the medians in OI (p<0.05) 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%). CONCLUSIONS 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.