Calcium acquisition rates do not support age-appropriate gains in total body bone mineral content in prepuberty and late puberty in girls with cystic fibrosis (original) (raw)
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Controlled longitudinal study of bone mass accrual in children and adolescents with cystic fibrosis
Thorax, 2006
Background: A study was undertaken to observe the gains in bone mass in children and adolescents with cystic fibrosis (CF) over 24 months and to examine the relationship between areal bone mineral density (aBMD) and associated clinical parameters including physical activity, nutrition, and 25-hydroxyvitamin D (25OHD). Methods: Areal BMD of the total body (TB), lumbar spine (LS), and total femoral neck (FNt) were repeatedly measured in 85 subjects aged 5-18 years with CF and 100 age and sex matched controls over 2 years. At each visit anthropometric variables, nutritional parameters, pubertal status, disease severity, physical activity, dietary calcium, caloric intake, and serum 25OHD were assessed and related to aBMD. Results: After adjusting for age, sex, and height Z-score, gains in LS aBMD in children (5-10 years) and TB and FNt aBMD in adolescents (11-18 years) with CF were significantly less than in controls. Lean tissue mass was significantly associated with TB and LS aBMD gains in children and adolescents and explained a significant proportion of the aBMD deficit observed. Lung function parameters were significantly associated with aBMD gains in adolescents with CF. Conclusions: Inadequate bone mass accrual during childhood and adolescence contributes to the low bone mass observed in adults with CF. Accounting for the height discrepancy which is frequently observed in those with CF, in addition to age and sex, is important when assessing low bone mass in children and adolescents with CF. To optimise an individual's potential to acquire maximal bone mass, it is necessary to maximise nutritional status and limit the progression of chronic suppurative lung disease.
Journal of Cystic Fibrosis, 2008
Background: Low bone mineral density is common in adults with cystic fibrosis. Children with good lung function compared to controls matched for body size have normal bone mineralisation. There are few data in large unselected populations of children. Methods: All children between five and 16 years were invited to take part. Disease severity was assessed. Bone mineral measurements using a GE-Lunar Prodigy densitometer were expressed as age and gender matched Z-scores. Bone mineral apparent density for L2-L4 was estimated and data from UK Caucasian children used to create age and gender specific reference ranges for predicted values. Z-scores were calculated. Total body analysis utilised the Molgaard method. Blood was sampled for measurement of 25-hydroxyvitamin D, and parathyroid hormone levels. Results: 107 children entered the study. 18 and 10 children had low areal and apparent bone mineral density respectively. Short, narrow bones were common. Fifteen children reported 22 fractures, 20 with associated trauma. The best predictors of bone status were ZBMI and percent predicted FEV 1. Conclusions: Bone mineral density corrected for body size was normal in over 90% of children. These results are similar to previously reported results in small studies of children with well preserved respiratory function.
Bone mineral and body composition alterations in paediatric cystic fibrosis patients
Pediatric Radiology, 2010
Background With the increased life span of cystic fibrosis (CF) patients, CF-related bone diseases could have an increased prevalence and morbidity in this group. In children, previous retrospective and prospective studies have yielded conflicting results on bone mineralization. Objective To monitor body composition and bone mineral status of children with CF. Materials and methods We reviewed the dual-energy X-ray absorptiometry (DXA) data of 161 children with CF (age 10±4.8 years). Total body bone mineral content (BMCt), total lean tissue mass (LTMt) and total fat mass (FMt) were measured and compared to expected data calculated from ideal weight for height (Wi; e.g. BMCti, LTMti, FMti). The bt (BMCt/BMCti), lt (LTMt/LTMti) and ft (FMt/FMti) ratios were used as quantitative variables. Results Low bt ratio was found at all ages (mean bt ratio 0.94±0.10; P<0.001), even in children <6 years of age. However, the children's BMCt was satisfactorily adapted to their weight. lt and ft ratios were not constant across age groups. Children <10 years had 8% reduction of their lt ratio, maintaining normal levels thereafter. The opposite trend was found for ft ratio. Poor clinical, nutritional status and vitamin A levels were correlated with bt and lt ratios. Conclusion Our results indicate that children with CF could have early alterations in their bone status and that lt and ft ratios did not have constant values across ages. Interpreting DXA data using this approach is suitable in children with CF.
Accrual of Bone Mass in Children and Adolescents With Cystic Fibrosis
The Journal of Clinical Endocrinology & Metabolism, 2017
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.
Prevalence of low bone mineral density in adolescents and adults with cystic fibrosis
Revista da Associação Médica Brasileira, 2014
Objective The aim of this cross-sectional study was to evaluate the prevalence of low bone mass density in cystic fibrosis patients as well as to evaluate the factors associated with bone mass in such patients. Methods Bone mass density was measured by dual-photon X-ray absorptiometry of lumbar spine (L1-L4), in patients ≤19 years old, or lumbar spine and femur (total and neck) in patients ≥20 years old. Evaluations of nutritional status, biochemical parameters, and lung function were performed. Medication data were obtained from medical records. Results Fifty-eight patients were included in the study (25 males/ 33 females), mean age 23.9 years (16-53years). The prevalence of bone mass below the expected range for age at any site was 20.7%. None of the subjects had history of fracture. Lumbar spine Z-score in cystic fibrosis patients correlated positively with body mass index (r= 0.3, p=0.001), and forced expiratory volume in the first second (% predicted) (r=0.415, p=0.022). Mean l...
Bone mass density and associated factors in cystic fibrosis patients of young age
Journal of Paediatrics and Child Health, 2008
To investigate bone mineral status in young cystic fibrosis (CF) patients and determine risk factors related with the development of low bone mineral density (BMD). We determined, in 81 patients with CF, 4 to 23-years-old, BMD as well as factors, which are thought to play a role in the development of reduced BMD. BMD Z-score was between -1 and -2.5 in 27 (33%) and lower than -2.5 in 9 (11%). Means of BMD Z-score were lower than the expected value of 0 in the three groups of children, adolescents and young adults (P = 0.004; P &amp;amp;amp;amp;amp;lt; 0.001; P = 0.048, respectively), but they did not differ among them (P = 0.114). Analysis showed that Shwachman-Kulczycki (SK) score, gender and levels of 25-hydroxy-vitamin D were significant predictors of BMD Z-score. Significant also was the interaction between gender and SK score. Our study supports that BMD may be reduced from a young age in CF patients though this needs to be confirmed using true volumetric measures of BMD. This defect is related to disease severity with males being more vulnerable. Inefficient levels of vitamin D are very common and contribute significantly to impaired bone health. The latter finding underlines the need for higher supplementation doses.
Calcium kinetics are altered in clinically stable girls with cystic fibrosis
The Journal of clinical endocrinology and metabolism, 2004
Reduced bone mass in individuals with cystic fibrosis (CF) may result from alterations in calcium metabolism. Bone calcium deposition and resorption rates, calcium balance, and markers of bone turnover were assessed using stable isotopes of calcium in 22 prepubertal and pubertal girls with CF. Bone calcium deposition was associated with the availability of dietary calcium, total serum osteocalcin, and leptin concentrations. Reduced bone mass in individuals with CF may result from inadequate bone calcium (Ca) deposition, and excessive resorption, although these parameters have not been directly assessed in children with CF. We used stable Ca isotopes to measure rates of bone Ca deposition (Vo+), resorption, and retention in 22 clinically stable girls with CF (aged 7-18 yr). Rates of bone Ca deposition were determined by mathematically modeling the disappearance of iv Ca stable isotope ((42)Ca) for 6 d post dosing. Indirect markers of bone turnover and hormones associated with puberta...
Bone mineral status in 134 patients with cystic fibrosis
Archives of Disease in Childhood, 1999
Patients and methods-Whole body bone mineral content (BMC), projected bone area, and bone mineral density (BMD) were determined by dual energy x ray absorptiometry in 134 patients with CF and compared with 396 healthy controls. Results-In patients < 19 years of age, BMD for age was normal in boys and marginally reduced in girls, whereas BMC for age was significantly reduced in both sexes. Height for age and bone area for height were significantly reduced, indicating "short" and "narrow" bones, whereas BMC for bone area was increased, indicating increased size corrected BMC. In patients > 19 years of age, BMD and BMC for age were significantly reduced. Conclusion-Short and narrow bones were the main reasons for reduced BMC for age in patients < 19 years of age, indicating that treatment to prevent osteoporosis in younger patients should be directed at increasing bone size, whereas conventional treatment with calcium and vitamin D supplementation alone might not be as eVective. Because of the significant decrease in BMD and BMC in adult patients, we fear that these patients may develop osteoporotic fractures prematurely. (Arch Dis Child 1999;81:235-240) Keywords: bone mineral content; bone mineral density; cystic fibrosis Dis Child 1999;81:235-240 235 Department of Growth and Reproduction GR,
Journal of Cystic Fibrosis, 2015
Background: The investigation of skeletal health data beyond dual X-ray absorptiometry (DXA) is limited in young individuals with CF. We assessed volumetric bone mineral densities (BMD), and bone and muscle parameters using peripheral quantitative computed tomography (pQCT) in individuals with CF and controls, 7.00-17.99 years. Methods: Peripheral QCT (XCT 3000, Stratec) measurements were made in 53 individuals with CF and 53 controls. Bone mineral content (BMC), total volumetric BMD (vBMD) and cross sectional area (CSA) of the bone were measured at the 4% and 66% sites of the non-dominant tibia and radius. Additionally, trabecular vBMD and bone strength index (BSIc) were measured at the 4% sites, and cortical vBMD, muscle CSA (mCSA) and strength strain index (SSI) were measured at the 66% sites. Results: Pre-pubertal males with CF had greater trabecular vBMD (p = 0.01) and total vBMD (p = 0.00) at 4% tibia, and greater total vBMD (p = 0.02) at 4% radius. Pre-pubertal females with CF had greater total vBMD at 66% tibia (p = 0.02) and radius (p = 0.04), and cortical vBMD (p = 0.04) at the radius. At puberty, the CF cohort had less BMC at 4% tibia (males, p = 0.02; females, p = 0.01), and smaller mCSA at 66% tibia (males, p = 0.02; females, p = 0.01). Pubertal CF females had a smaller bone CSA (p = 0.01) at 4% tibia, and lower bone strength (SSI) at the tibia (p = 0.00) and radius (p = 0.05) sites. Conclusions: Bone strength parameters were not compromised prior to puberty in this CF cohort. At puberty, the bone phenotype changed for this CF cohort, showing several deficits compared to the controls. However, bone strength was adapting to the mechanical demands of the muscle. Altered bone parameters and their implications for lowered bone strength with increased age may be greatly influenced by: the CF cohort remaining smaller for age and/or a reduced bone strain, secondary to reduced muscle force.