Trends in bone mineral density in young adults with cystic fibrosis over a 15 year period

Abstract

Background Improvements in clinical care have led to increased life expectancy in patients with cystic fibrosis (CF) over the past several decades. Whether these improvements have had significant effects on bone health in patients with CF is unclear. Methods This is a cross-sectional study comparing clinical characteristics and bone mineral density (BMD) measured by dual energy X-ray absorptiometry (DXA) in adults with CF evaluated in 1995–1999 to age-, race-, and gender matched patients with CF evaluated in 2011–2013 at the same center on calibrated DXA machines. Results The cohorts were similar in terms of age, BMI, pancreatic insufficiency, presence of F508del mutation, and reproductive history. In the most recent cohort, pulmonary function was superior, and fewer patients had vitamin D deficiency or secondary hyperparathyroidism. Areal BMD measures of the PA spine, lateral spine, and distal radius were similarly low in the two cohorts. Conclusions Although pulmonary function and vitamin D status were better in patients in the present-day cohort, areal BMD of the spine was reduced in a significant number of patients and was no different in patients with CF today than in the late 1990s. Further attention to optimizing bone health may be necessary to prevent CF-related bone disease.

Update on Bone Health in Pediatric Chronic Disease

“…Known risk factors for CFBD include poor nutrition, chronic lung infection and inflammation, pancreatic insufficiency, delayed puberty, decreased physical activity, and medications, including glucocorticoids and transplant-related therapies. 15,16 The presence of impaired glucose metabolism in CF-related diabetes and possible direct effect of the CFTR on osteoclast activation 12,13 may also be responsible for the bone disease reported in both children and adults.…”

A retrospective analysis of longitudinal changes in bone mineral content in cystic fibrosis

“…Several cross-sectional reports have reported low bone mass in both post-pubertal children and adults with CF, although adults tend to be more affected [18][19][20][21][22]. Monitoring bone mass and providing preventive care are necessary from childhood through adolescence to minimise CF-related bone disease in adults with CF [8].…”

Maladie osseuse liée à la mucoviscidose : mise au point

“…Des données récentes semblent indiquer que malgré l'amélioration globale de l'état de santé des patients, la fréquence de la déminéralisation osseuse ne diminue pas en proportion. Ainsi, dans une étude comparant 2 cohortes de patients ajustées sur l'âge, le genre et l'indice de masse corporelle, l'une suivie dans les années 1990 et l'autre dans les années 2011-2013, la prévalence du déficit osseux (Z-score < À2) mesuré au niveau de la colonne lombaire et du radius restait globalement identique malgré un meilleur état respiratoire, nutritionnel et vitaminique [9]. Ces résultats méritent toutefois d'être comparés à ceux d'autres centres.…”