PATHOGENESIS OF OSTEOPOROSIS IN YOUNG CHILDREN WITH CYSTIC FIBROSIS

这个子项目是利用由NIH/NCRR资助的中心拨款提供的资源的许多研究子项目之一。子项目和调查员(PI)可能从另一个NIH来源获得了主要资金，因此可能会出现在其他CRISE条目中。列出的机构是针对中心的，而不一定是针对调查员的机构。骨质疏松症、骨骼疼痛和骨折是囊性纤维化的常见后遗症。CF患者的骨病发病机制是复杂的，既有减少骨形成的过程，也有增加骨吸收的过程。成人CF患者的骨转换异常，骨吸收增加，但骨形成正常。目前尚无全面评估幼年CF儿童维生素D代谢的研究。这项研究将比较患有CF的儿童和他们未受影响的兄弟姐妹。选择兄弟姐妹作为对照，以提供骨转换测量和骨密度测量的对照组，并探讨杂合性CF跨膜受体突变是否对骨矿物质积累有影响。对患有骨质疏松症的青春期前儿童的骨量和骨代谢的评估将有助于确定这些因素在这些个体发生骨质疏松症中的作用。这将使制定合理的管理和预防性干预和治疗成为可能。

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. Osteoporosis, skeletal pain, and fractures are frequent sequelae of cystic fibrosis (CF). The pathogenesis of bone disease in individuals with CF is complex, resulting both from processes that decrease bone formation and that increase bone resorption. Bone turnover in adults with CF has been shown to be abnormal with increased bone resorption yet normal bone formation. NO study as comprehensively assessed vitamin D metabolism in young children with CF. This study will compare children with CF to their unaffected siblings. Siblings were chosen as the controls in order to provide a comparison group for bone turnover measures and densitometric measurements and also to explore if there are effects of heterozygous CF transmembrane receptor mutations on bone mineral accrual. Assessment of bone mass and bone metabolism in prepubertal children with CF will allow determination of the role of these factors in the development of osteoporosis in these individuals. This will enable development of rational management and prophylactic interventions and therapies.