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Bone acquisition and loss in children and adults with cystic fibrosis: a longitudinal study
G S Bhudhikanok1, M C Wang, R Marcus
1Department of Pediatrics, Stanford University of Medicine, California 94305-5208, USA.
Insights
Children and young adults with cystic fibrosis (CF) show reduced bone mineral density (BMD) and inadequate bone acquisition. Glucocorticoid use and disease severity significantly impact BMD changes in CF patients.
Area of Science:
- Pediatrics
- Bone Metabolism
- Cystic Fibrosis Research
Background:
- Cystic Fibrosis (CF) is associated with significant skeletal complications, including osteopenia.
- Understanding bone mineral acquisition patterns is crucial for managing CF-related bone disease.
Purpose of the Study:
- To investigate bone mineral acquisition in pediatric and young adult CF patients.
- To identify clinical and laboratory factors associated with changes in bone mineral density (BMD).
Main Methods:
- Dual-energy x-ray absorptiometry (DXA) assessed bone mineral content in 41 CF patients over 1.5 years.
- Evaluated associations between BMD/BMAD changes and clinical factors like weight, glucocorticoid use, and disease severity.
Main Results:
- Reduced mean BMD Z-scores were observed at baseline and decreased further in youths at follow-up, indicating impaired bone accrual.
- Bone mineral apparent density (BMAD) was also reduced, ruling out small bone size as the sole explanation for osteopenia.
- Glucocorticoid use, body mass changes, physical activity, and disease severity were significant correlates of BMD changes.
Conclusions:
- Osteopenia in CF primarily results from insufficient bone mineral gains, with potential for bone loss, especially with glucocorticoid therapy.
- While weight gain and puberty may lead to some bone mineral recovery, it remains incomplete in CF patients.
Objectives:
To determine patterns of bone mineral acquisition in children and young adults with cystic fibrosis (CF) and to identify clinical and laboratory correlates of change in bone mineral density (BMD).
Study Design:
Bone mineral and clinical status were assessed in 41 patients with CF (26 female, aged 9 to 50 years) at baseline and 1.5 years later. Bone mineral content of the lumber spine, femoral neck, and whole body was determined by dual-energy x-ray absorptiometry and expressed as BMD and bone mineral apparent density (BMAD). Changes in weight, height, pubertal status, glucocorticoid use, physical activity, disease severity, and biochemical markers of bone turnover were examined for associations with changes BMD and BMAD.
Results:
Mean BMD Z-scores (adjusted for age and sex) were reduced at the spine, hip, and whole body at baseline in both adults and youths, and decreased further at all sites among youths at follow-up (-0.4 at spine, p < 0.05; -0.3 at hip, p < 0.10; -0.5 for whole body, p < 0.0005). These data indicate failure to gain bone mineral at the expected rate. BMAD was also reduced at follow-up, suggesting that the observed osteopenia could not be explained by small bone size. Bone loss at multiple sites was observed in four youths and two adults. In general glucocorticoid use, change in body mass, physical activity, and disease severity were the most significant correlates for change in BMD and in BMD Z-score.
Conclusions:
Osteopenia in CF generally reflects inadequate gains in bone mineral, although bone loss may occur, particularly in patients requiring glucoc therapy. Late gains in bone mineral may accompany weight gain and pubertal development, but the catch-up appears to be incomplete.