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BMD and body composition in children and young patients affected by cystic fibrosis
Maria Luisa Bianchi1, Giovanna Romano, Silvia Saraifoger
1Centro Malattie Metaboliche Ossee, Istituto Auxologico Italiano IRCCS, Milano, Italy. ml.bianchi@auxologico.it
Insights
Low bone density is common in young cystic fibrosis (CF) patients, affecting 66%. Impaired lung function and steroid use significantly impact bone health and body composition in CF.
Area of Science:
- Pediatric Endocrinology
- Pulmonology
- Bone Metabolism
Background:
- Bone mineral density (BMD) complications are frequent in adult cystic fibrosis (CF) patients.
- Data on BMD in pediatric CF populations are less consistent.
Purpose of the Study:
- To longitudinally evaluate BMD in young CF patients over 12-24 months.
- To identify key determinants of BMD and body composition in this cohort.
Main Methods:
- A longitudinal study of 136 young CF patients (ages 3-24).
- Evaluation of bone mineral density (BMD), bone mineral content (BMC), and body composition.
- Analysis of determinants including pulmonary function (FEV1), puberty, and steroid dose.
Main Results:
- 66% of young CF patients exhibited reduced BMD (Z score) in spine and total body.
- Low BMD prevalence was consistent across children, adolescents, and young adults.
- Key determinants of BMD included pulmonary function (FEV1), puberty, platelet count, and cumulative steroid dose.
- Reduced fat mass and lean mass were observed; lean mass correlated with total body and lower limb BMD, while fat mass correlated with spine BMD.
- FEV1 influenced both BMD and fat-free mass (FFM) changes over time.
Conclusions:
- A significant proportion of young CF patients have low BMD, irrespective of age or sex.
- Pulmonary function, steroid dose, and advanced liver disease are critical factors influencing BMD.
- Pulmonary function and puberty are primary drivers of BMD increase in CF.
- Cystic fibrosis significantly alters body composition, with pulmonary function impacting FFM.
Unlabelled:
Longer survival in cystic fibrosis has led to more bone complications. One hundred thirty-six young patients were studied for 12-24 months. Low BMD was found in 66%. Fat mass and lean mass were also reduced. Impaired pulmonary function and total steroid dose had the greatest negative influence on bone.
Introduction:
Low BMD is reported as a frequent complication in adult patients affected by cystic fibrosis (CF), but the available data are less consistent for younger patients.
Materials And Methods:
This study was designed to evaluate BMD longitudinally over 12-24 months in a sample of 136 young patients (3-24 years of age) and to investigate its major determinants. BMC and body composition were also evaluated.
Results:
BMD (expressed as Z score) of spine and of total body was reduced in 66% of patients. The prevalence of low BMD was the same in children, adolescents, and young adults. The main determinants of BMD were forced expiratory volume in 1 s (FEV1; as an index of pulmonary function), puberty, platelet count (as an index of portal hypertension), and cumulative steroid dose. Changes of FEV1 over time influenced BMD changes. Bone mass, fat mass (FM) and fat-free (lean) mass (FFM) were reduced in CF patients at both total body and subregions (trunk, limbs). Lean mass influenced BMD of total body and lower limbs, whereas fat mass (and BMI) influenced spine BMD. FEV1 also influenced FFM.
Conclusions:
Low BMD was present in a significant proportion of CF patients, independent of sex and age. BMD depended on pulmonary function, steroid dose, and presence of advanced liver disease. Pulmonary function and puberty were the main stimuli for the increase of BMD over time. CF also altered body composition, and FFM was influenced by pulmonary function.
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