Royal Children's Hospital Melbourne, Parkville, Victoria, Australia.
Murdoch Children's Research Institute, Royal Children's Hospital, Parkville, Victoria, Australia.
Pediatr Pulmonol. 2021 Jul;56(7):2014-2022. doi: 10.1002/ppul.25375. Epub 2021 Apr 1.
For Australians living with cystic fibrosis (CF), increased longevity means greater consideration needs to be given to long-term endocrine sequelae such as CF-related bone disease. Deficits in bone mass accrual are most likely to occur during childhood and adolescence. Current guidelines in Australia suggest repeat dual-energy X-ray absorptiometry (DXA) scans every 2 years. This study aims to stratify clinical factors that determine future bone health in the Australian CF population and use this to guide a more streamlined approach to bone health screening.
This study was a retrospective audit of all patients diagnosed with CF who were treated at the Royal Children's Hospital Melbourne, Australia from 2000 to 2016 (n = 453). Two hundred and two patients had a DXA scan in the study period (191 with height-adjusted data) and 111 patients had more than one scan (108 with height-adjusted data). An investigation into the associations between bone mineral density (BMD) Z score and potential risk factors was conducted using DXA and historical data.
The main predictor of future BMD was the previous BMD Z score (p < .001). Other factors found to be determinants of BMD included nutritional status, lung function (FEV ), age, history of previous fracture, oral corticosteroid use, and the number of hospital admissions. However, after adjusting for previous BMD, evidence of an association remained only with nutritional status, FEV , and number of hospital admissions.
Second yearly scans may be unnecessary in children with an adequate DXA score on the initial scan who remain clinically stable. However, clinical deterioration in those whose BMD was previously normal, may require closer monitoring of bone health. We propose a guideline for the frequency of DXA monitoring in relation to clinical risk factors.
对于患有囊性纤维化 (CF) 的澳大利亚人来说，寿命的延长意味着需要更多地考虑长期的内分泌后遗症，如 CF 相关的骨骼疾病。骨量积累的不足很可能发生在儿童和青少年时期。目前澳大利亚的指南建议每 2 年重复进行双能 X 射线吸收法 (DXA) 扫描。本研究旨在对决定澳大利亚 CF 人群未来骨骼健康的临床因素进行分层，并利用这些因素指导更简化的骨骼健康筛查方法。
这是对 2000 年至 2016 年期间在澳大利亚墨尔本皇家儿童医院接受治疗的所有 CF 患者进行的回顾性审计(n=453)。在研究期间，有 202 名患者进行了 DXA 扫描（191 名患者有身高调整数据），111 名患者进行了多次扫描（108 名患者有身高调整数据）。使用 DXA 和历史数据对骨矿物质密度 (BMD) Z 评分与潜在危险因素之间的关联进行了调查。
未来 BMD 的主要预测因素是之前的 BMD Z 评分（p<0.001）。还发现其他决定 BMD 的因素包括营养状况、肺功能 (FEV1)、年龄、既往骨折史、口服皮质类固醇的使用以及住院次数。然而，在调整了之前的 BMD 后，仅与营养状况、FEV1 和住院次数有关。
对于初始扫描时 BMD 评分足够且临床稳定的儿童，第二年的扫描可能没有必要。然而，那些之前 BMD 正常但临床状况恶化的人可能需要更密切地监测骨骼健康。我们提出了一个与临床危险因素有关的 DXA 监测频率的指南。
