Total body water in children with cystic fibrosis using bioelectrical impedance.

BACKGROUND: The aim of this study was to assess the ability of commercially available single frequency bioelectrical impedance analysis (BIA) to predict total body water and hence body composition in a cohort of children with cystic fibrosis (CF). METHODS: In 56 (31 girls, 25 boys) children aged 4 to 18 years, total body water was predicted using BIA and measured using a deuterium oxide dilution technique. Each child provided a urine sample before consuming 0.5 g/kg body weight 10% deuterium oxide. A further urine sample was collected between 4 and 6 h later. RESULTS: There was a mean bias between predicted and measured total body water of 1.1 L. The bias was consistent across the range of total body water measured in this study. The relationship between height2/impedance and measured total body water was TBW=0.35+0.74*height2/impedance. This equation is similar to other equations found in healthy children. CONCLUSION: We have shown that a commercially available bioelectrical device underestimates measured total body water. We have generated a prediction equation for the population studied, which we believe might enhance the accuracy of the method in children with cystic fibrosis.

Series of laryngomalacia, tracheomalacia, and bronchomalacia disorders and their associations with other conditions in children.

Laryngomalacia, bronchomalacia, and tracheomalacia are commonly seen in pediatric respiratory medicine, yet their patterns and associations with other conditions are not well-understood. We prospectively video-recorded bronchoscopic data and clinical information from referred patients over a 10-year period and defined aspects of interrelationships and associations. Two hundred and ninety-nine cases of malacia disorders (34%) were observed in 885 bronchoscopic procedures. Cough, wheeze, stridor, and radiological changes were the most common symptoms and signs. The lesions were most often found in males (2:1) and on the left side (1.6:1). Concomitant malacia lesions ranged from 24% for laryngotracheobronchomalacia to 47% for tracheobronchomalacia. The lesions were found in association with other disorders such as congenital heart disorders (13.7%), tracheo-esophageal fistula (9.6%), and various syndromes (8%). Even though the understanding of these disorders is in its infancy, pediatricians should maintain a level of awareness for malacia lesions and consider the possibility of multiple lesions being present, even when one symptom predominates or occurs alone.

The use of air displacement plethysmography in children and adolescents with cystic fibrosis.

OBJECTIVES: The purpose of this paper was to evaluate the use of air displacement plethysmography (ADP) in children and adolescents with cystic fibrosis (CF). Specifically, the primary aim of this study was to compare estimates of fat-free mass (FFM) measured from ADP and dual energy X-ray absorptiometry (DEXA) in children and adolescents with CF. The secondary aim was to compare the effect of using predicted thoracic gas volume (VTG) and measured VTG for the calculation of FFM by ADP in this population. METHODS: Cross-sectional FFM measurements were taken using ADP and DEXA in 52 children and adolescents with CF, ranging in age from 6.3 to 16.6 y. RESULTS: Bland-Altman analysis showed that ADP values of FFM were on average 0.59 kg higher than DEXA values (95% limits of agreement = 3.61 to -2.43 kg); however this difference was not significant. There was no significant correlation (r = -0.26, P = 0.07) between the mean FFM and difference in FFM between ADP and DEXA. Using either predicted or measured VTG did not significantly affect FFM estimates in individuals with CF who had normal lung function (bias = -0.39 +/- 0.86 kg; r = -0.02, P = 0.93). CONCLUSIONS: ADP is an appropriate technique for use in children and adolescents with CF.

Controlled longitudinal study of bone mass accrual in children and adolescents with cystic fibrosis.

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.

Bone mineral density in Australian children, adolescents and adults with cystic fibrosis: a controlled cross sectional study.

BACKGROUND: Low bone mineral density (BMD) is recognised in individuals with cystic fibrosis (CF) although the pathogenesis remains unclear. The aims of this study were to compare BMD over a broad continuum of Australian individuals with CF with healthy controls and to examine the relationship between BMD and clinical parameters including physical activity, nutrition, and vitamin D levels. METHODS: BMD of the lumbar spine (LS), total body (TB), femoral neck (FN), cortical wrist (R33%), and distal wrist (RUD) was examined in 153 individuals with CF aged 5.3-55.8 years (84 males) and in 149 local controls aged 5.6-48.3 years (66 males) using dual energy x ray absorptiometry. Anthropometric variables, body cell mass, markers of disease severity, corticosteroid usage, measures of physical activity, dietary calcium and caloric intake and serum vitamin D were assessed and related to BMD. RESULTS: Compared with controls, mean BMD was not significantly different in children aged 5-10 years with CF. Adolescents (females 11-18 years, males 11-20 years) had reduced TB and R33% BMD when adjusted for age, sex, and height (difference in BMD (g/cm2) adjusted means between control and CF: TB=0.04 (95% CI 0.01 to 0.07); R33%=0.03 (95% CI 0.01 to 0.06)). BMD was reduced at all sites except R33% in adults (difference in BMD (g/cm2) adjusted means between control and CF: TB=0.05 (95% CI 0.02 to 0.09); LS=0.08 (95% CI 0.03 to 0.14); FN=0.09 (95% CI 0.03 to 0.15); RUD=0.03 (95% CI 0.01 to 0.05)). In children/adolescents BMD was weakly associated with nutritional status and disease severity. CONCLUSIONS: BMD was normal in a well nourished group of prepubertal children with CF. A BMD deficit appears to evolve during adolescence and becomes more marked in adults. Individuals with CF should optimise nutrition, partake in physical activity, and maximise lung health in order to optimise BMD. Further longitudinal studies are required to understand the evolution of reduced BMD in young people and adults with CF.