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 (XLH6.5; OI4.0), pain (XLH9.5; OI5.0), psychological discomfort (XLH5.5; OI2.0), psychological disability (XLH2.0; OI0.0), handicap (XLH2.0; OI0.0) and total OHIP (XLH35.0; OI14.0). 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.