Bone Mineral Density in Children with Non-Cystic Fibrosis Bronchiectasis (original) (raw)
Related papers
Pediatric pulmonology, 2017
Improvements in the medical management of cystic fibrosis (CF) in recent years have resulted in increased prevalence of long-term sequelae of the condition, such as low bone mineral density (BMD) and hence an increased risk of fractures in later life. Aim To explore the interaction between BMD and lung function, nutrition, and genotype. This study was a retrospective audit of 202 children with CF from August 2000 to January 2016 to investigate associations between BMD Z-scores with clinical status, nutrition, and genetics using dual-energy absorptiometry X-ray data from the Royal Children's Hospital Melbourne, Australia. Severity of both lung disease (P < 0.0001) and nutritional status (P < 0.05) was found to be strongly associated with BMD Z-scores. This is the biggest study to date to provide further evidence that the severity of pulmonary disease is related to BMD in CF patients and therefore screening guidelines for bone health in children with CF should target individ...
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.
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.
Jornal de Pediatria, 2013
Objective: To assess bone mineral density in patients with cystic fibrosis (CF), and to correlate it with possible intervening variables. Methods: Children and adolescents diagnosed with CF, aged 6 to 18 years, followed at the outpatient clinic were included in the study. First, demographic data were collected and, subsequently, patients underwent a spirometric test. All patients answered the Cystic Fibrosis Quality of Life Questionnaire (CFQ) and underwent the six-minute walk test (6MWT) and bone densitometry (DXA). Results: A total of 25 CF patients were included, of which 56% were males. The mean age was 12.3±3.4 years; mean height was 149.2±14.4 cm; and mean weight was 44.4±13.9 kg. Most results on pulmonary function and bone mineral density (BMD) were within normal limits. The mean forced expiratory volume in one second (FEV 1 ) was 92.5±23.6 (% of predicted), mean forced vital capacity (FVC) was 104.4±21.3 (% of predicted), and mean BMD z-score was 0.1±1.0. BMD was moderately correlated with FEV 1 (r = 0.43, p = 0.03) and FVC (r = 0.57, p = 0.003). Regarding chronological age and age at diagnosis, 0,003, respectivamente). Entretanto, não foram encontradas correlações significativas com os dados do QFC, TC6 e índice de massa corporal. Conclusão: A maioria dos pacientes avaliados apresenta DMO dentro dos limites de normalidade e possui correlação positiva com a função pulmonar e negativa com a idade cronológica e a idade de diagnóstico.
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 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.
Update on Cystic Fibrosis-Related Bone Disease: A Special Focus on Children
Paediatric Respiratory Reviews, 2009
Cystic fibrosis (CF) is the most frequent lethal autosomal recessive disease in the white population. It is linked to mutations in the CF transmembrane regulator (CFTR) gene, which in turn leads to dysfunction of the multifunctional CFTR protein located at the apical membrane of epithelia and is mainly involved in modulation of transepithelial ion transport, hydration of epithelial lining fluids, pH regulation, and inflammation. 1 Classically, the clinical phenotype causes chronic obstructive airways disease with Pseudomonas aeruginosa sputum colonization, pancreatic insufficiency, malnutrition, and an elevated sweat chloride concentration. The increased life span of patients with CF has lead to the appearance of new complications that are still being better defined. First reported in 1979, 2 low bone mineral density (BMD) is now recognized as a common problem, characteristic of this disease in adults with CF, and its complications have become a difficult management problem. Recent investigations however suggest that these problems actually begin during childhood, especially at puberty. 3 Although nutritional deficits, chronic infection, Vitamin D deficiency, hypogonadism, delayed puberty, and reduced physical activity may all jeopardize bone health in this population (Fig. 1), the pathogenesis of low BMD in individuals with CF still remains uncertain. This paper will review the main studies of CF-related bone disease in CF patients, the most important risk factors for low BMD in CF, and strategies for optimizing bone health, which includes screening and monitoring of BMD, prevention, and pharmacologic interventions. EPIDEMIOLOGY OF CF-RELATED BONE DISEASE Adult studies More than 50 reports have observed low bone mass and fractures in individuals with CF (reviewed in reference 4). Low bone mineral density (BMD) is commonplace in both post pubertal adolescents and adults with CF, although adults tend to be more affected. Cross-sectional surveys of adults have found that 20%-34% of adults with CF have standard Z-scores (age and genderadjusted standard deviation scores) <À2 while 10% have T-scores (standard deviations compared with young healthy adults) <À2.5. The prevalence of osteopenia (T-score <À1) has been reported to be as high as 85% in advanced-stage (adult) CF 5,6. The prevalence of bone disease appears to increase with severity of lung disease and malnutrition. Younger and healthier individuals may have normal BMD (reviewed in reference 4) but bone disease almost invariably develops as patients get older because of malabsorption of vitamin D, poor nutritional status, physical inactivity, glucocorticoid therapy, delayed pubertal maturation, intermittent hypogonadism (mainly due to chronic disease), and systemic inflammation. Several studies have demonstrated a positive correlation between BMD T-or Z-scores and both FEV 1 % and body mass index (BMI). 4,7-9 Haworth et al. (1999) reported mean annualized losses in BMD of 0.5%, 2.1% and 1.8% at the lumbar spine (LS), femoral neck (FN), and total hip, respectively, in a cohort of 114 patients ages 15-49 (mean age 25 yrs). 8 Aris et al. (2004) found annualized losses of similar magnitude (1.8% at the spine and 0.7% at the hip) in a slightly older cohort (mean age 27 yrs), which acted as a control arm for an alendronate trial. 10 More recently, other
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.
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.
Scientific Reports
The prevalence of osteopenia/osteoporosis has not been sufficiently studied in people with bronchiectasis not due to cystic fibrosis (BC), nor has its relationship with other variables (clinical, body composition and bone turnover and inflammation markers) been sufficiently studied. Our aim was to determine the prevalence of osteopenia and osteoporosis and related factors in patients with BC. We did a cross-sectional study in people with BC in a clinically stable state. Spirometric parameters, annual exacerbations and analysis with bone turnover markers (BTM) and inflammation markers were evaluated. Densitometry (DXA) was performed for body composition, bone mineral density (BMD) and handgrip strength. 123 patients were studied (65% women, mean age 49.6 ± 18.8, Body Mass Index (BMI) 24.8 ± 4.7 kg/m2). 62.8% and 62.5% of men and women, respectively, had normal bone mineral density, 30.2% and 22.2% osteopenia and 7% and 15% osteoporosis. 52 patients (56.2%) had low fat-free mass: 68.9...