Abstract
BackgroundTo report on dental characteristics and treatment load in Danish adult patients with osteogenesis imperfecta (OI).MethodsOral examination of 73 patients with OI was performed and OI type I, III, and IV were represented by 75.3%, 8.2%, and 16.4%, respectively. Patients were diagnosed as having dentinogenesis imperfecta (DI) if they had clinical and radiological signs of DI. In the data analysis, mild OI (type I) was compared to moderate-severe OI (type III and IV).ResultsDiscoloration of teeth was prevalent in patients with moderate-severe compared to mild OI (83.3% vs. 5.5%, p < 0.001). Cervical constriction and pulpal obliteration were frequent findings in patients with moderate-severe OI (61.1% and 88.9%, respectively), whereas pulp stones and taurodontism were diagnosed in patients with mild OI only (29.1% and 9.1%, respectively). DI was found in 24.7% of OI patients and considerably more frequent in patients with moderate-severe (94.4%) compared to mild OI (1.8%) (p < 0.001). The number of teeth with artificial crowns was significantly higher in patients with moderate-severe OI than in patients with mild OI (median 1.5, range 0–23 vs. median 0, range 0–14) (p < 0.001). The number of teeth with fillings in patients with mild OI was significantly higher than in patients with moderate-severe OI (mean 9.7, SD 5.1, median 9.0, range 1–21 vs. mean 5.0, SD 4.4, median 4.0, range 0–16) (p < 0.001).ConclusionsOne fourth of patients with OI had DI, and the vast majority of them had moderate-severe OI. Whereas discoloration of teeth, cervical constriction and pulp obliteration were frequent findings in patients with moderate-severe OI, pulp stones and taurodontism were found in patients with mild OI only. In patients with moderate-severe OI, the dental treatment load was dominated by prosthetic treatment, whereas restorative treatment with fillings was more prevalent in patients with mild OI.
Highlights
To report on dental characteristics and treatment load in Danish adult patients with osteogenesis imperfecta (OI)
The quantitative collagen defect was associated with dentinogenesis imperfecta (DI) in only one out of 38 patients, all having mild OI, and the qualitative collagen defect was associated with DI in 17 out of 18 patients, all having moderate-severe OI (p < 0.001) (Table 1)
Pulp stones and taurodontism were found in patients with mild OI only (16 out of 55 (29.1%) (p = 0.008) and 5 out of 55 (9.1%) (p = 0.322), respectively) (Table 2, Fig. 2, d and f)
Summary
To report on dental characteristics and treatment load in Danish adult patients with osteogenesis imperfecta (OI). Osteogenesis imperfecta (OI) is a rare hereditary connective tissue disorder with different degrees of severity. In addition to OI, some patients have dentinogenesis imperfecta (DI) which is a hereditary dentine disorder characterized by greyish-blue to brown discoloration of Thuesen et al BMC Oral Health (2018) 18:175 teeth and pulp obliteration [7]. DI type I is associated with OI and is caused by mutations in the genes, COL1A1 and COL1A2 encoding collagen type I. DI type II and III are caused by mutations in the gene encoding dentine sialophosphoprotein (DSPP) [7], and not found in OI patients. Dentine is composed of hydroxyapatite, an organic phase composed primarily of type I collagen and water. Due to mutations in the genes encoding collagen type I, defects in the dentine occur [8]. In patients with DI type I, both dentitions are typically affected, but the primary dentition is usually more severely affected than the permanent dentition [9]
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