Exploring Bone Health in Cystic Fibrosis: A Study From a Lung Transplantation Center and Strategy for Clinical Care

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 FEV₁ 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.