INTRODUCTION
X-linked hypophosphatemic rickets (XLH) is a rare disease and its natural history affects the quality of life of patients, as it leads to bone deformities and architectural and qualitative changes in the bone.
MATERIALS AND METHODS
Sixteen XLH patients were selected from referral hospitals in Rio de Janeiro (12 women and four men) and compared to 16 healthy age- and sex-matched control individuals. All XLH were receiving calcitriol and oral phosphate supplements. Bone assessment was performed by high-resolution peripheral quantitative computed tomography (HR-pQCT) and dual-energy X-ray absorptiometry (DXA).
RESULTS
XLH patients showed higher lumbar spine BMD Z-score, but lower 33% distal radius BMD and Z-score values as compared to control group. Low BMD for age (Z-score ≤ -2.0 SD) was only detected in the XLH group: specifically in 3 of the 5 postmenopausal women, 3 of the 7 premenopausal women, and 2 of the 4 men. HR-pQCT evaluation showed lower total vBMD at both peripheral sites and impaired trabecular parameters, particularly at the distal tibia, after correction for multiple comparisons.
CONCLUSION
Our study sheds light on the involvement of both trabecular and cortical compartments in the XLH group, despite conventional treatment. We also emphasize the limitation of DXA in assessing central sites in the XLH group. The 33% radius may represent a useful complementary skeletal site for DXA assessment, particularly when HR-pQCT is not available.
Iara Marcelino Sant Ana, Maria Caroline Alves Coelho, Joyce Cantoni et al.· Journal of Bone and Mineral...· 0 citations
Objective To evaluate bone density and microstructure, fragility fractures, and hormones in men 50 years and older receiving antiseizure medications (ASMs). Subjects and methods Study population included 39 men with difficult-to-control epilepsy and 42 healthy men as control group. Spine and hip areal bone mineral density (aBMD), body composition and vertebral fracture assessment (VFA) were evaluated using Dual-Energy X-Ray Absorptiometry (DXA). Volumetric bone mineral density (vBMD) and microarchitecture of trabecular and cortical compartments were evaluated using High-Resolution Peripheral Quantitative Computed Tomography (HR-pQCT). Physical activity was tested by the International Physical Activity Questionnaire (IPAQ). The fracture risk was estimated by fracture risk assessment tool (FRAX). Results ASMs patients showed lower cortical bone thickness (Ct.Th) in distal tibia (1.196 ± 0.295 vs. 1.341 ± 0.241 mm, p = 0.03), more fractures (12 vs. 2, p = 0.003) mostly morphometric vertebral fractures, and a higher risk for major and hip fractures based on FRAX. Hormonal changes included higher median parathyroid hormone (PTH) (50.4 vs. 24 pg/mL, p < 0.001) possibly associated with vitamin D insufficiency, (25(OH)D levels below 30 ng/mL in 43.6% of ASMs patients), lower median Estradiol that was an independent factor for fractures (p = 0.04) and bioavailable Testosterone in the lowest quartile in 52 % of them. Conclusion Men on ASMs exhibit lower Ct.Th and hormone changes that may contribute to bone fragility. While clinical fractures are infrequent, it is crucial to actively search for morphometric fractures, either during DXA or through the more available method of spine X-rays.
Cristian Pieper, L. Vieira, Eduardo Ferreira da Gama et al.· Archives of Endocrinology an...· 0 citations
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