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  • Title: Accrual of Bone Mass in Children and Adolescents With Cystic Fibrosis.
    Author: Sharma S, Jaksic M, Fenwick S, Byrnes C, Cundy T.
    Journal: J Clin Endocrinol Metab; 2017 May 01; 102(5):1734-1739. PubMed ID: 28323913.
    Abstract:
    CONTEXT: Low bone density is a complication of cystic fibrosis (CF). HYPOTHESIS: Accrual of bone mass is most impaired in the sickest children, as judged by nutritional status and pulmonary function. DESIGN: Retrospective analysis of correlation between lumbar spine bone mineral density (BMD), body mass index (BMI), and forced expiratory volume in 1 second (FEV1) z scores in children and adolescents with CF. SETTING: Pediatric hospital specialist CF service. PATIENTS: Sixty participants aged 5.9 to 18.8 years (24 female) with confirmed CF. INTERVENTIONS: Lumbar spine BMD, BMI, and FEV1 z scores measured at first BMD scan; 40 participants had sequential scans. Change in L1-L4 z scores over time was used as a measure of bone accrual, and BMI as a measure of nutritional status. OUTCOME MEASURES: Correlations between lumbar spine BMD, BMI, and FEV1 z scores. RESULTS: Mean BMI and BMD z scores were strongly correlated at the initial scan (P < 0.0001), suggesting that nutritional status is a major determinant of BMD. In the sequential scan at a mean age of 16.1 years, height centile was maintained, indicating normal linear growth. Changes in BMI and BMD z scores were positively correlated (P = 0.001), indicating that patients failing to gain weight appropriately with growth were also failing to acquire bone normally. Change in FEV1 z score was correlated with change in both BMD (P < 0.0001) and BMI z scores (P = 0.02). CONCLUSION: Although young people with CF may be maintaining normal growth in height, bone accrual is impaired in those with the poorest nutritional status and lung function.
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