Bone mineral content in cystic fibrosis patients: correlation with fat-free mass.

Salamoni, F; Roulet, M.; Gudinchet, F; Pilet, Marianne; Thiébaud, D.; Burckhardt, P.

doi:10.1136/adc.74.4.314

Cited by 71 publications

(38 citation statements)

References 26 publications

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“…However, deficits in total body bone mineral, lean body mass, or body fat were not found in a similar body composition study of 14 CF patients aged 6-20 years who were specifically selected as being wellnourished based on a normal weight for height ratio. 16 The findings in that select group of children and young adults compared to those of the current study further demonstrate the importance of nutrition in maintaining normal growth in children with CF. Significant deficits in bone mineral, lean, and fat body mass may develop early in life in patients with CF.…”

Section: Discussionsupporting

confidence: 61%

Bone mineral content and body composition in children and young adults with cystic fibrosis

Henderson

Madsen

1999

Pediatr. Pulmonol.

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With dual energy X‐ray absorptiometry (DEXA), it is possible to quantitate important aspects of growth in children with cystic fibrosis (CF), supplementing the usual measures of height and weight. Of particular concern during growth is the accumulation of bone mineral, since osteoporosis and fractures are well‐recognized problems in end‐stage disease. Various measures of growth and body composition were examined in 40 children and young adults (ages 5.7–20.3 years, mean 11.9 years) and compared to age‐, gender‐, and race‐matched normal controls. The mean (± SE) weight Z‐score of the 40 CF patients was −0.70 ± 0.11, and the mean height Z‐score was −0.66 ± 0.15. Relative to their matched normal controls, the CF patients had a deficit in total body bone mineral averaging 19.1% ± 3.0%. The deficits in total body bone mineral correlated with pulmonary and nutritional measures of disease severity. Serum vitamin D levels, calcium intake, age, gender, use of steroids, and CF genotype were not found to be significant factors. In this group of children and young adults with CF, height and weight measures of growth were not dramatically reduced (mean Z‐scores = −0.7), yet large deficits in total body bone mineral averaging nearly 20% were identified. Pediatr Pulmonol. 1999; 27:80–84. © 1999 Wiley‐Liss, Inc.

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Section: Discussionsupporting

confidence: 61%

Bone mineral content and body composition in children and young adults with cystic fibrosis

Henderson

Madsen

1999

Pediatr. Pulmonol.

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“…Recent studies have reported normal overall bone mineral status in well nourished children with mild disease when controls were matched for body size as well as age and gender [23,24,39,40]. These studies were limited, however, by small numbers and exclusion of children with long term corticosteroid use.…”

Section: Discussionmentioning

confidence: 96%

A cross-sectional study of bone mineral density in children and adolescents attending a Cystic Fibrosis Centre

Conway

Oldroyd

Brownlee

et al. 2008

Journal of Cystic Fibrosis

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“…There is strong evidence that disease severity has the greatest impact on BMD in CF (3,4,6,11). Moreover, one report suggested that patients with CF and a normal nutritional status had a normal BMD (24). Apparently, the pathogenesis of CFassociated low bone mass is a secondary result of disease severity and hence physical activity, but no specific effect of cystic fibrosis transmembrane conductance regulator (CFTR) on bone mineral metabolism.…”

Section: Discussionmentioning

confidence: 99%

Bone mineral density and quantitative ultrasound in adults with cystic fibrosis

Flohr

Lutz²,

App³

et al. 2002

European Journal of Endocrinology

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Objective: With increasing life span osteoporosis becomes a more recognized problem in patients with cystic fibrosis (CF). The aim of this cross-sectional study in 75 adult patients with CF (mean age 25.3 years) was to assess the prevalence of low bone mineral density (BMD) by dual-energy x-ray absorptiometry (DEXA) and, for the first time, by quantitative ultrasound (QUS), and to identify predicting factors. Design and Methods: Bone status was assessed at the lumbar spine (L2-L4) and the femoral neck by DEXA, and at the calcaneus by QUS (stiffness index). These data were correlated with a variety of clinical and anthropomorphic variables. Biochemical markers of bone turnover such as osteocalcin, bone-specific alkaline phosphatase, crosslinks in urine, 25-hydroxy vitamin D (25-OH vitamin D), parathyroid hormone, calcium and free testosterone were determined by standard assays. Results: The mean BMD T score (^S.E.M.) was 21:4^0:17 at the lumbar spine, and 20:54^0:16 at the femoral neck. The mean T score of the calcaneal stiffness index was 20:83^0:19: Based on a lumbar spine T score ,22.5 by DEXA, 27% of the patients had osteoporosis. Multiple regression analysis showed that the forced expiratory volume in one second (FEV 1 ) and the use of oral glucocorticoids were independent predictors of low lumbar spine BMD, whereas body mass index (BMI) and the use of oral glucocorticoids were independent predictors of low femoral neck BMD. The stiffness index correlated moderately with BMD (0.49-0.62, P , 0:0001). QUS had a sensitivity and specificity of only 57% and 89% respectively for diagnosing 'osteoporosis' (based on a femoral neck T score ,22.5 by DEXA). Positive and negative predictive values were 36% and 95% respectively. Conclusions: Low BMD is frequent in adults with CF and is most strongly correlated with disease severity (BMI, FEV 1 ) and the use of glucocorticoids. Calcaneal QUS might help to screen out patients with a normal BMD, but sensitivity and specificity were not sufficiently high to replace DEXA in these patients.

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Bone mineral content in cystic fibrosis patients: correlation with fat-free mass.

Cited by 71 publications

References 26 publications

Bone mineral content and body composition in children and young adults with cystic fibrosis

Bone mineral content and body composition in children and young adults with cystic fibrosis

A cross-sectional study of bone mineral density in children and adolescents attending a Cystic Fibrosis Centre

Bone mineral density and quantitative ultrasound in adults with cystic fibrosis

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