Sakai Tomoko, Honzawa Shiho, Kaga Makiko, Iwasaki Yuji, Masuyama Tatsuo
Department of Rehabilitation Medicine, Tokyo Medical and Dental University, Tokyo, Japan.
Department of Pediatrics, Tokyo Metropolitan Tobu Medical Center for Persons with Developmental/Multiple Disabilities, Tokyo, Japan.
Brain Dev. 2020 Mar;42(3):256-263. doi: 10.1016/j.braindev.2019.12.010. Epub 2020 Jan 22.
We assessed the severity and pathology of osteoporosis in children and adults with severe motor and intellectual disabilities (SMID) by evaluating bone enzymes, by which we aimed to determine adequate treatment approaches for preventing fractures.
Ninety patients (44 men, 46 women; mean age, 34.5 years) underwent bone quality assessment. Quantitative ultrasonography (QUS) was used to measure the T-score and Z-score of the calcaneus, and blood tests were used to measure bone-specific alkaline phosphatase and tartrate-resistant acid phosphatase 5b levels as bone formation and resorption markers, as well as calcium, phosphorous, and parathyroid hormone levels as routine examination.
Bone formation and resorption marker levels were within normal ranges in adults, although they were high during the growth period in children and adolescents and in elderly women. Patients receiving tube feeding showed a significantly lower Z-score than those without tube feeding. Tube feeding was a significant factor for the Z-score, whereas age, vitamin supplements, and anti-epileptic drugs were not.
The severity of osteoporosis in SMID started during the growth period and seems to be caused by a lack of an effective increase in bone mineral density. Any treatment should be started during the growth period. More study about tube feeding is needed.
我们通过评估骨酶来评估重度运动和智力残疾（SMID）儿童及成人骨质疏松症的严重程度和病理情况，旨在确定预防骨折的适当治疗方法。
90名患者（44名男性，46名女性；平均年龄34.5岁）接受了骨质量评估。采用定量超声（QUS）测量跟骨的T值和Z值，并通过血液检测测量骨特异性碱性磷酸酶和抗酒石酸酸性磷酸酶5b水平作为骨形成和骨吸收标志物，以及钙、磷和甲状旁腺激素水平作为常规检查项目。
成人的骨形成和骨吸收标志物水平在正常范围内，尽管在儿童、青少年生长发育期以及老年女性中这些指标较高。接受管饲的患者Z值显著低于未接受管饲的患者。管饲是影响Z值的一个重要因素，而年龄、维生素补充剂和抗癫痫药物则不是。
SMID患者骨质疏松症的严重程度始于生长发育期，似乎是由于骨矿物质密度未能有效增加所致。任何治疗都应在生长发育期开始。需要对管饲进行更多研究。
