Osteometabolic Disorders Unit, Endocrinology Division, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Enéas de Carvalho Aguiar, 155-PAMB, 8° Andar, Bloco 3, São Paulo, SP, CEP: 05403-900, Brazil.
Hormone and Molecular Genetics Laboratory (LIM/42), Endocrinology Division, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Enéas de Carvalho Aguiar, 155-PAMB, 2° Andar, Bloco 6, São Paulo, SP, CEP: 05403-900, Brazil.
J Bone Miner Metab. 2023 Sep;41(5):666-672. doi: 10.1007/s00774-023-01442-x. Epub 2023 Jul 7.
The aim of this study is to evaluate and compare the trabecular bone scores (TBSs) of 11 children and 24 adults with X-linked hypophosphatemic rickets (XLH) and non-XLH subjects from a tertiary center.
The areal bone mineral density at the lumbar spine (LS-aBMD) and LS-aBMD Z score were analyzed by dual-energy X-ray absorptiometry. The bone mineral apparent density (BMAD) and LS-aBMD Z score adjusted for height Z score (LS-aBMD-HAZ) were calculated. The TBS was determined using TBS iNsight software based on DXA images from the Hologic QDR 4500 device.
The XLH patients exhibited a higher mean LS-aBMD Z score, BMAD, and TBS than the non-XLH subjects (p < 0.01). LS-aBMD-HAZ and BMAD were greater in the XLH children than those in their corresponding non-XLH subjects (p < 0.01 and p = 0.02), and the XLH children trended toward a greater TBS (p = 0.06). The XLH adults had a higher LS-aBMD Z score, BMAD, and TBS than the non-XLH subjects (p < 0.01). When stratified by metabolic status according to the serum values of bone formation markers, compensated adult patients had a higher LS-aBMD Z score, BMAD, and TBS than non-XLH subjects (p < 0.01). Noncompensated patients had higher LS-aBMD Z scores and BMAD results than non-XLH subjects. However, TBS values did not differ statistically significantly between those groups (p = 0.45).
The higher LS-aBMD Z score, BMAD, and TBS result in the XLH patients compared to non-XLH subjects indicates an increased amount of trabecular bone within the lumbar spine, regardless of extraskeletal calcifications.
本研究旨在评估和比较来自三级中心的 11 名儿童和 24 名成 年 X 连锁低磷性佝偻病（XLH）患者与非 XLH 患者的骨小梁骨密度（TBS）。
通过双能 X 线吸收法分析腰椎（LS）的骨矿密度（aBMD）和 LS-aBMDZ 评分。计算骨矿密度表观密度（BMAD）和校正身高 Z 评分的 LS-aBMD（LS-aBMD-HAZ）。使用 Hologic QDR 4500 设备的 DXA 图像，通过 TBS iNsight 软件测定 TBS。
XLH 患者的 LS-aBMDZ 评分、BMAD 和 TBS 均高于非 XLH 患者（p<0.01）。XLH 患儿的 LS-aBMD-HAZ 和 BMAD 均大于相应的非 XLH 患儿（p<0.01 和 p=0.02），而 XLH 患儿的 TBS 则呈上升趋势（p=0.06）。XLH 成年患者的 LS-aBMDZ 评分、BMAD 和 TBS 均高于非 XLH 患者（p<0.01）。根据骨形成标志物的血清值，按代谢状态分层，代偿成人患者的 LS-aBMDZ 评分、BMAD 和 TBS 均高于非 XLH 患者（p<0.01）。非代偿患者的 LS-aBMDZ 评分和 BMAD 结果高于非 XLH 患者。然而，两组间 TBS 值差异无统计学意义（p=0.45）。
与非 XLH 患者相比，XLH 患者的 LS-aBMDZ 评分、BMAD 和 TBS 更高，表明腰椎内的骨小梁数量增加，而与骨骼外钙化无关。
