Evaluation of bone mineral density and 3D-Shaper parameters in congenital hypophosphatasia of the adult

Abstract

Objetive: To evaluate bone mineral density (BMD) and 3D‐Shaper parameters at the proximal femur (FP) level in adults with genetically confirmed hypophosphatasia (HPP) and to compare them in those subjects with and without fractures. Material and methods: Cross‐sectional analysis of densitometric data and bone architecture from the baseline visit of a longitudinal study in which patients with HPP were included. A densitometric study (Lunar Prodigy, GE iDXA) was carried out in FP using 3D‐Shaper software (version 2.7. Galgo Medical). Results: 33 adults with HPP with heterozygous mutations were included. 63.6% (21/33) were women (42.9% postmenopausal), and 8 of the men (66.6%) were older than 50 years. The mean age was 50.56±15.08 years, 30.3% (10/33) had previous traumatic fractures and 15.2% (5/33) presented stress fractures. The prevalence of osteoporosis in CF was 11.8% (2/17) and of osteopenia, 82.4% (14/17). In premenopausal women and young men, low bone mass was detected for age in 12.5% (2/16). When comparing subjects with and without stress fractures, as well as traumatic ones, there were no differences in BMD. The 3D‐Shaper showed a decrease in cortical thickness (mm) in patients with stress fractures [1.8 (1.77‐1.89)] compared to subjects without them [1.94 (1.87‐2.03, p=0.03)] and compared to those with traumatic fractures [1.97 (1.88‐2.04), p=0.03]. Conclusions: These data reflect a discrete densitometric impact in milder forms of the adult. Bone architecture studies could be of interest in determining patients susceptible to stress fractures.