Kartal Öztürk Gökçen, Halis Ece, Ocak Ece, Eşki Aykut, Gökşen Damla, Özen Samim, Çoksüer Fevziye, Demir Esen, Gülen Figen
Department of Pediatric Pulmonology, Ege University Faculty of Medicine, İzmir, Turkey.
Department of Pediatric Pulmonology, Tepecik Education and Research Hospital, University of Health Sciences, İzmir, Türkiye.
Pediatr Pulmonol. 2025 Jul;60(7):e71210. doi: 10.1002/ppul.71210.
Cystic Fibrosis Bone Disease (CFBD) is a known complication in children with CF and may cause serious problems in adulthood or transplantation processes. This study aimed to identify potential predictable risk factors for the development of low BMD by evaluating pediatric patients screened with DXA as a "Heart-Lung Transplantation Center" and created new strategic plans to improve our CFBD screening program by evaluating our results in literature and guidelines recommendations.
This retrospective cohort study includes 86 children ages 6-18 years with CF who underwent at least one DXA scan between August 2016 and October 2024. Participants were compared according to BMD z scores and the relationship between BMD and disease-related parameters was evaluated.
The rate of DXA screening in our center was 81.1% over 8 years of age and 72.8% over 6 years of age. 41.8% of our population had abnormal BMD (z scores < -1), and the rate of very low BMD (z scores < -2) was 17.4%. The frequency of abnormal BMD was higher in children with BMI< 50th percentile, SKS ≤ 70, low FEV z score, respiratory microorganism colonization, ≥ 2 annual pulmonary exacerbations, required respiratory support, low albumin, and high CRP levels. Systemic inflammation marker CRP increase was the most predictable parameter for low BMD.
This study informs clinical practice by highlighting the need for multidisciplinary interventions, such as earlier evaluation of DXA scans due to the risk factors and poor clinical conditions, a consistent follow-up protocol, individualized nutrition programs with the dietitian, and enhanced physical therapy.
囊性纤维化骨病（CFBD）是囊性纤维化患儿已知的一种并发症，可能在成年期或移植过程中引发严重问题。本研究旨在通过评估作为“心肺移植中心”接受双能X线吸收法（DXA）筛查的儿科患者，确定低骨密度（BMD）发生的潜在可预测风险因素，并通过评估我们在文献和指南建议中的结果，制定新的战略计划以改进我们的CFBD筛查项目。
这项回顾性队列研究纳入了86名6至18岁的囊性纤维化患儿，他们在2016年8月至2024年10月期间至少接受了一次DXA扫描。根据BMD z评分对参与者进行比较，并评估BMD与疾病相关参数之间的关系。
我们中心8岁以上儿童的DXA筛查率为81.1%，6岁以上儿童为72.8%。我们研究人群中41.8%的人骨密度异常（z评分< -1），极低骨密度（z评分< -2）的发生率为17.4%。体重指数（BMI）低于第50百分位数、斯基恩评分（SKS）≤70、第一秒用力呼气容积（FEV）z评分低、呼吸道微生物定植、每年≥2次肺部加重、需要呼吸支持、白蛋白水平低和C反应蛋白（CRP）水平高的儿童中，骨密度异常的频率更高。全身炎症标志物CRP升高是低骨密度最可预测的参数。
本研究通过强调多学科干预的必要性为临床实践提供了参考，比如由于存在风险因素和临床状况不佳而尽早进行DXA扫描评估、一致的随访方案、与营养师共同制定个性化营养计划以及加强物理治疗。
