Oliveri M B, Cassinelli H, Bergadá C, Mautalen C A
Laboratorio de Osteopatías Médicas, Hospital de Clinicas, Centro de Investigaciones Endocrinológicas, Buenos Aires, Argentina.
Bone Miner. 1991 Feb;12(2):91-100. doi: 10.1016/0169-6009(91)90038-2.
X-linked hypophosphatemic rickets (XLH) is characterized by inadequate skeletal mineralization. The bone mineral density (BMD) of the radius shaft and the lumbar spine was determined in 13 children with XLH. Ten patients were on treatment, whereas three patients had discontinued treatment 20-32 months prior to this study. Two of them had radiological evidence of rickets. The radius shaft BMD was significantly diminished: Z score was -1.33 +/- 0.89 (P less than 0.001), while the BMD of lumbar spine was significantly augmented (Z score +1.95 +/- 1.17, P less than 0.001). A positive correlation was found between the Z scores for the BMD of the radius shaft and spine. The two patients with overt rickets had lower radius shaft BMD values and a lesser increment of BMD of the spine. The BMD deficit of cortical bone may be related to the lack of efficacy of the treatment and/or to an intrinsic defect of the bone on this disease. On the other hand, the augmented BMD of the lumbar spine might reflect the overabundance of partially mineralized osteoid. The determination of the BMD of the radius shaft by SPA was a sensitive method for detecting abnormalities of the bone mass in XLH patients under treatment without radiological signs of rickets.
X连锁低磷性佝偻病（XLH）的特征是骨骼矿化不足。对13例XLH患儿测定了桡骨干和腰椎的骨密度（BMD）。10例患者正在接受治疗，而3例患者在本研究前20 - 32个月已停止治疗。其中2例有佝偻病的放射学证据。桡骨干BMD显著降低：Z值为-1.33±0.89（P<0.001），而腰椎BMD显著升高（Z值+1.95±1.17，P<0.001）。桡骨干和脊柱BMD的Z值之间存在正相关。2例有明显佝偻病的患者桡骨干BMD值较低，脊柱BMD的增量较小。皮质骨的BMD不足可能与治疗效果不佳和/或该疾病中骨骼的内在缺陷有关。另一方面，腰椎BMD升高可能反映了部分矿化类骨质过多。通过单光子吸收法（SPA）测定桡骨干BMD是一种检测正在接受治疗且无佝偻病放射学征象的XLH患者骨量异常的敏感方法。
