BONE DENSITOMETRY IN PATIENTS WITH NEUROMUSCULAR DISORDERS

该子项目是利用NIH/NCRR资助的中心赠款提供的资源的许多研究子项目之一。子项目和研究者（PI）可能从另一个NIH来源获得主要资金，因此可以在其他CRISP条目中表示。所列机构为中心，不一定是研究者所在机构。严重的骨质疏松症是脊髓损伤的一个众所周知的后果，它会导致高钙血症、高钙尿症、肾结石、软组织钙化和病理性骨折的风险增加。病理性骨折的总发生率为1.4-6%，最常见于轮椅福尔斯跌倒。已经证明，长时间的骨骼卸载，如严格的卧床休息，由于骨折和空间飞行导致的固定，导致负钙平衡和骨质疏松症。一名俄罗斯宇航员最近返回地球，发现他在微重力环境中仅呆了140天，跟骨骨密度就下降了19%。 脊髓损伤、神经肌肉疾病和微重力对骨代谢有类似的影响。随着负重的减轻，骨吸收显著增加，将骨钙释放到循环中。在脊髓损伤或神经肌肉功能显著降低后的七个月内，受影响骨骼区域的骨量减少高达30%。这增加了骨折、高钙血症、高钙尿症、肾结石和异位骨化的发生率。我们将招募100名患有脊髓损伤或其他原因导致的神经肌肉功能障碍的成年患者，如多发性硬化症、脑瘫、肌营养不良等。符合以下标准的受试者将被考虑入组：a）21岁以上的神经肌肉疾病患者，在BMC接受治疗; B）由于脊髓损伤或神经肌肉疾病而不能活动或不能活动明显减少的受试者; c）尿妊娠试验阴性; d）能够提供口头或书面同意。我们将观察骨密度随时间的变化。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Severe osteoporosis is a well known consequence of spinal cord injury, which causes hypercalcemia, hypercalciuria, kidney stones, soft tissue calcification and increased risk of pathologic fractures. The overall incidence of pathologic fractures is 1.4-6% and occurs most commonly from wheelchair falls. It has been demonstrated that prolonged periods of skeletal unloading such as strict bed rest, immobilization due to fractures and space flight leads to a negative calcium balance and osteoporosis. A Russian cosmonaut recently returned and was found to have a 19% decrease in his calcaneous bone density after only 140 days in microgravity. Spinal cord injury, neuromuscular disorders and microgravity have similar effects on bone metabolism. With the loss of weight bearing, there is a marked increase in bone resorption releasing bone calcium into the circulation. Within seven months after a spinal cord injury, or significant reduction in neuromuscular function, there is up to a 30% reduction in bone mass of affected skeletal areas. This increases the incidence of fractures, hypercalcemia, hypercalciuria, kidney stones and heterotopic ossification. We will enroll 100 adult patients with either spinal cord injury, or neuromuscular dysfunction due to other causes, such as, multiple sclerosis, cerebral palsy, muscular dystrophy, etc. Subjects who meet the following criteria will be considered for enrollment: a) >21 y/o with neuromuscular disease who are cared for at BMC; b) subjects who are immobilized or have marked decrease immobility due to either spinal cord injury or a neuromuscular disorder; c) negative urine pregnancy test; d) ability to provide verbal or written consent. We will look at changes in bone density over time.