Decreased mechanical efficiency in clinically stable patients with COPD

BACKGROUND: It has recently been reported that total daily energy expenditure (TDE) is increased in patients with chronic obstructive pulmonary disease (COPD) and it was hypothesised that these patients may have a decreased mechanical efficiency during activities. The purpose of the present study was to measure the mechanical efficiency of submaximal leg exercise, and to characterise patients with a potentially low efficiency in terms of body composition, resting energy expenditure, lung function, and symptom limited exercise performance. METHODS: Metabolic and ventilatory variables were measured breath by breath during submaximal cycle ergometry exercise performed at 50% of symptom limited achieved maximal load in 33 clinically stable patients with COPD (23 men) with forced expiratory volume in one second (FEV1) of 40 (12)% predicted. Net mechanical efficiency was calculated adjusting for resting energy expenditure (REE). RESULTS: Median mechanical efficiency was 15.5% and ranged from 8.5% to 22.7%. Patients with an extremely low mechanical efficiency (< 17%, n = 21) demonstrated an increased VO2/VE compared with those with a normal efficiency (median difference 4.7 ml/l, p = 0.005) during submaximal exercise. There was no difference between the groups differentiated by mechanical efficiency in blood gas tensions at rest, airflow obstruction, respiratory muscle strength, hyperinflation at rest, resting energy expenditure or body composition. There was a significant difference in total airways resistance (92% predicted, p = 0.03) between the groups differentiated by mechanical efficiency. CONCLUSIONS: It is concluded that many patients with severe COPD have decreased mechanical efficiency. Furthermore, based on the results of this study it is hypothesised that an increased oxygen cost of breathing during exercise contributes to the decreased mechanical efficiency.


     

The effect of acute maximal exercise on the regional distribution of ventilation using ventilation MRI in CF

BackgroundThe importance of exercise in the management of people with CF is well recognised, yet the effect of exercise on lung function is not well understood. FEV₁ is insensitive to the detection of small changes in lung function. Ventilation MRI and LCI are both more sensitive to mild lung disease than FEV₁ and may be better suited to assess the effects of exercise. Here we assessed the short-term effects of maximal exercise on the distribution of ventilation using ventilation MRI and LCI.MethodsPatients with CF and a range of lung disease were assessed. Baseline LCI and ventilation MRI was followed by a maximal cardio-pulmonary exercise test (CPET). Repeated ventilation MRI was performed within 30 minutes of exercise termination, followed by LCI and finally by FEV₁.Results13 patients were recruited and completed all assessments. Mean (SD) age was 25 (10) years and mean (SD) FEV₁ z-score was -1.8 (1.7). Mean LCI at baseline was 8.2, mean ventilation defect percentage on MRI (VDP) was 7.3%. All patients performed maximal CPET. Post-exercise, there was a visible change in lung ventilation in 85% of patients, including two patients with increased ventilation heterogeneity post-CPET who had normal FEV₁. VDP and LCI were significantly reduced post-exercise (p < 0.05) and 45% of patients had a significant change in VDP.ConclusionsAcute maximal exercise directly affects the distribution of ventilation on ventilation MRI in patients with CF. This suggests that exercise is beneficial in CF and that ventilation MRI is suitable to assess airway clearance efficacy.     

Resting energy expenditure in cystic fibrosis as an indicator of disease severity.

BACKGROUND: An imbalance of energy expenditure has been reported in Cystic Fibrosis (CF). High-energy diets, in combination with pancreatic enzymes, do not always compensate for energy requirements. The aim of this study was to investigate the relation between Resting Energy Expenditure (REE) and disease severity in CF. METHODS: REE was measured with indirect calorimetry in a group of 38 clinically stable CF patients (32 with pancreatic insufficiency and 6 with pancreatic sufficiency). Correlations between REE and pulmonary function as well as clinical status were studied. RESULTS: Increased REE expressed as a percentage of predicted (REE%) was demonstrated in pancreatic insufficient CF patients (PI) (113.3+/-2.5%), as compared to pancreatic sufficient (98.9+/-2.5%) and healthy subjects. The elevated REE% in the PI group was not affected by gender and exhibited a strong correlation with the clinical status of the patients (r=-0.641). CONCLUSIONS: Our data analysis supports the argument that the percentage of predicted REE is an objective indicator of disease severity and progression as well as energy requirements in the assessment of CF patients.     

Resting energy expenditure and lung disease in cystic fibrosis.

Optimal nutritional support is considered to be an integral part in the management of cystic fibrosis (CF). Several factors contribute to increased resting energy expenditure (REE), which itself can lead to energy imbalance and thus contribute to deterioration of the nutritional status. We aimed to assess the impact of lung parenchyma damage on REE and correlated these findings with forced expiratory volume in 1 s (FEV(1)). Twenty patients performed respiratory function testing (FEV(1)), pulmonary high-resolution computed tomography (HRCT) and assessment of REE with open circuit indirect calorimetry. HRCT was scored by using a modified Bhalla method. Mean HRCT score was 8.4 and mean REE value was 108.4% predicted vs. 96.5% predicted of 16 healthy subjects (P<0.01). There was a significant correlation between HRCT score and REE (P<0.01), HRCT score and FEV(1) (P<0.001) and REE and FEV(1) (P<0.05). The correlations demonstrate a close correlation between lung damage and elevated REE in people with CF. Prevention of negative energy balance is an important part in follow-up of patients with CF. Any increase in REE should raise suspicion of progress in lung impairment.     

Metabolism in patients with small cell lung carcinoma compared with patients with non-small cell lung carcinoma and healthy controls

BACKGROUND: Weight loss is a frequently occurring problem in patients with lung cancer due to an increased resting energy expenditure (REE) and a decreased energy intake. The aim of the present study was to compare the metabolic and inflammatory characteristics of patients with small cell lung carcinoma (SCLC) and non-small cell lung carcinoma (NSCLC). The metabolic parameters of the lung cancer population were compared with those of a healthy control group. METHODS: REE was measured in 66 patients with lung cancer, subdivided according to their histology, and in 33 healthy controls matched for sex, age, and fat free mass (FFM). Inflammatory mediators were measured in the plasma of the patients with lung cancer. RESULTS: An increased REE adjusted for FFM was found in the patients with lung cancer. Those with small cell lung carcinoma (SCLC) had an increased REE adjusted for FFM (mean 1925 kcal/day) compared with those with non-small cell lung carcinoma (NSCLC) (mean 1789 kcal/day, 95% CI for difference 36 to 236). FFM accounted for 69% and 48% of the inter-individual variation in REE in controls and those with NSCLC, respectively, while FFM accounted for only 25% of the variation in REE in patients with SCLC in whom the fat mass (FM) also contributed significantly (28%) to the variation in REE. Increased concentrations of soluble TNF-receptor 75 (sTNF-R75) and cortisol were found in patients with SCLC compared with those with NSCLC. Lipopolyasccharide binding protein (LBP) and sTNF-R55 were related to plasma levels of cortisol. CONCLUSION: An enhanced REE adjusted for FFM occurred in patients with SCLC compared with those with NSCLC.


     

Ghrelin and leptin levels in young adults with cystic fibrosis: relationship with body fat.

BACKGROUND: Ghrelin and leptin are hormones implicated in energy balance coordination and body weight regulation. There are conflicting data regarding the levels and role of leptin while ghrelin has not been studied in CF. The aim of this study was to investigate fasting serum ghrelin and leptin levels in CF adolescents as compared to healthy controls and analyze their association with body fat. METHODS: Fourteen CF adolescents having pancreatic insufficiency and twenty healthy adolescents were enrolled in the study. Diabetic patients were excluded. In all participants' height, weight, body mass index (BMI) and body fat % (BF %) were estimated. Ghrelin and leptin levels were determined after an overnight fast. RESULTS: Weight, BMI and BF% were significantly lower in CF adolescents than those of controls. Fasting leptin levels in CF were significantly higher in CF patients (p=0.030), compared to controls and significantly lower in CF males as compared to CF females (p=0.01). Fasting ghrelin levels were significantly lower in CF males as compared to male controls (p<0.001) and comparable in females. CONCLUSIONS: As the overall clinical outcome of CF patients is related to the nutritional status and body weight, the role of ghrelin and leptin in these patients needs to be elucidated.     

Shwachman-Kulczycki score and resting energy expenditure in cystic fibrosis.

BACKGROUND: Disease severity assessed by clinical scores in cystic fibrosis (CF) has been a topic of investigation for many years, although a correlation of clinical scores with resting energy expenditure (REE) has not been described yet. We aimed to assess disease severity as evaluated by the Shwachman-Kulczycki (SK) score and to correlate these findings with REE and forced expiratory volume in 1 s (FEV1). METHODS: Twenty-eight patients performed respiratory function testing (FEV1), and assessment of REE with open circuit indirect calorimetry. The SK score was evaluated according to general activity, physical examination, nutrition and conventional chest X-ray findings. RESULTS: Mean SK score was 75.3 +/- 15.7. Mean REE was 109.1% of predicted vs. 96.5% predicted in 16 healthy subjects (P = 0.002). There was a significant correlation between the SK score and REE (P = 0.001), the SK score and FEV1 (P < 0.001) and REE and FEV1 (P = 0.034). CONCLUSIONS: The correlations between the SK score, REE and FEV1 demonstrate a close connection between disease severity, caloric requirement and lung damage. They confirm the clinical value of the SK score, which is easy to assess in a clinical setting.     

Components of energy expenditure in tumor-bearing animals

Single snapshot measurements of resting energy expenditure (REE) suggest that hypermetabolism contributes to cancer cachexia, but tumor impact on total 24-hr energy expenditure (TEE) is unknown. Automated multicage indirect calorimetry was employed to measure daily energy expenditure in adult Buffalo rats (n = 16) randomized to tumor inoculation or controls. Measurements included baseline REE, activity EE (AEE), thermic response to food (TEF), and TEE. Rats (n = 16) were randomized. Metabolic measurements, tumor size, and body weight were recorded weekly. Animals were sacrificed at Week 5 for analysis of host and tumor composition. Significant depletion of total lean body mass occurred in TB rats (greater than 15% wt loss, ANOVA P less than 0.001) which inversely correlated with tumor growth (r = -0.81, P less than 0.001). REE, TEF, AEE, and TEE did not change in controls (ANOVA P = n.s.). In TB rats, a 19.5% increase in REE occurred (119.4 +/- 3.3 to 138.7 +/- 1.8 kcal/kg LBM/day, P less than 0.01). TEE remained unchanged (157.3 +/- 5.6 vs 152.9 +/- 3.6 kcal/kg LBM/day, P = n.s.) due to a 66% decrease in AEE (32.9 +/- 3.1 to 10.5 +/- 1.7 kcal/kg LBM/day, P = 0.01). TEF did not change (4.7 +/- 0.8 vs 5.0 +/- 0.3 kcal/kg LBM/day, P = n.s.). Both TB and controls demonstrated a decreased REE in response to a 24-hr fast (7.9% vs 4.8%, P = n.s.). Respiratory quotient decreased in both groups when comparing fed to fasted values: TB (0.86 to 0.76) and controls (0.86 to 0.71), but the decline was greater in controls (P = 0.04).(ABSTRACT TRUNCATED AT 250 WORDS)     

Evaluation of the exercise intensity generated by active video gaming in patients with cystic fibrosis and healthy individuals

BackgroundAdherence of patients with cystic fibrosis (CF) to exercise is challenging. Here we compared the physiological responses during the use of interactive video games (VG) with the cardiopulmonary exercise test (CPET) in healthy and CF subjects.MethodsCross-sectional study including CF and healthy (CON) subjects older than 6 years. Individuals were evaluated in two visits. At visit one, anthropometric measures, spirometry and CPET were performed. In the second visit, a physical activity questionnaire was applied and gas analyses performed during the use (10 min) of both Nintendo Wii (Wii Fit Plus: (1) Obstacle Course, (2) Rhythm Boxing and (3) Free Run) and Xbox One (Just Dance 2015: (1) Love Me Again, (2) Summer and (3) Happy).ResultsTwenty-five CON and 30 CF patients were included. The mean FEV₁ (%) was significantly lower in the CF group compared to CON. There were no differences between groups at peak exercise (CPET) for heart rate (HR), oxygen consumption (VO₂) and minute ventilation (V_E). In the CON group, games 2 and 3 (Xbox) and game 3 (Nintendo) increased HR to values similar to the anaerobic threshold (AT), while for the CF group this occurred for games 2 (Xbox) and 3 (Nintendo). As for VO₂ and V_E, both groups obtained similar responses as compared to AT values in games 2 (Xbox) and 3 (Nintendo).ConclusionThe use of VG generated a cardiorespiratory response similar to AT levels found during CPET, indicating that it may be an alternative for exercise training of CF individuals.     

Raised CRP levels mark metabolic and functional impairment in advanced COPD

BACKGROUND: C-reactive protein (CRP) is often used as a clinical marker of acute systemic inflammation. Since low grade inflammation is evident in chronic diseases such as chronic obstructive pulmonary disease (COPD), new methods have been developed to enhance the sensitivity of CRP assays in the lower range. A study was undertaken to investigate the discriminative value of high sensitivity CRP in COPD with respect to markers of local and systemic impairment, disability, and handicap. METHODS: Plasma CRP levels, interleukin 6 (IL-6) levels, body composition, resting energy expenditure (REE), exercise capacity, health status, and lung function were determined in 102 patients with clinically stable COPD (GOLD stage II-IV). The cut off point for normal versus raised CRP levels was 4.21 mg/l. RESULTS: CRP levels were raised in 48 of 102 patients. In these patients, IL-6 (p<0.001) and REE (adjusted for fat-free mass, p = 0.002) were higher while maximal (p = 0.040) and submaximal exercise capacity (p = 0.017) and 6 minute walking distance (p = 0.014) were lower. The SGRQ symptom score (p = 0.003) was lower in patients with raised CRP levels, as were post-bronchodilator FEV1 (p = 0.031) and reversibility (p = 0.001). Regression analysis also showed that, when adjusted for FEV1, age and sex, CRP was a significant predictor for body mass index (p = 0.044) and fat mass index (p = 0.016). CONCLUSIONS: High sensitivity CRP is a marker for impaired energy metabolism, functional capacity, and distress due to respiratory symptoms in COPD.     

Caloric Expenditure in the Morbidly Obese Using Dual Energy X-ray Absorptiometry

Total caloric expenditure is the sum of resting energy expenditure (REE) and caloric expenditure during physical activity. In this study, we examined total caloric expenditure in 25 morbidly obese patients (body mass index ≥ 35 kg/m²) using dual energy X-ray absorptiometry (DXA) scanning and cardiorespiratory exercise testing. Our results show average REE for all individuals was 2027 ± 276 kcal/d and mean net caloric expenditure during 30 min of exercise was 115 ± 16 kcals. Assuming the mean of all input values, a strict 1500 kcal/d diet combined with 150 min per wk of structured physical activity, the projected weight change was − 7% (8.8 ± 6.2 kg) for 6 mo. We conclude that morbidly obese individuals should be able to achieve only a modest weight loss by following minimal national guidelines. These data suggest that more aggressive energy expenditure and caloric restriction targets for long periods of time are needed to result in significant weight loss in this population.     

Visceral adipose tissue is associated with poor diet quality and higher fasting glucose in adults with cystic fibrosis

BackgroundBody fat distribution and diet quality influence clinical outcomes in general populations but are understudied in individuals with cystic fibrosis (CF). The aim of this pilot study was to assess body fat distribution and diet quality in relation to fasting glucose and lung function in adults with CF.MethodsSubjects were 24 adults (ages 18–50) with CF and 25 age-matched controls. The Healthy Eating Index 2015 (HEI-2015) was calculated from 3-day food records and data were adjusted per 1000 kcal. Whole and regional body composition, including visceral adipose tissue (VAT), was assessed by dual energy X-ray absorptiometry.ResultsSubjects with CF reported more added sugar intake [26.1 (IQR 18.1) vs. 12.9 (12.5) g/1000 kcal, p < 0.001] and had lower HEI-2015 scores [48.3 (IQR 9.9) vs. 63.9 (27.3), p < 0.001] compared to controls. There were no differences in BMI, total body fat, or lean body mass (LBM) between subjects with CF and controls (p > 0.05 for all), although subjects with CF had higher VAT than control subjects [0.3 (IQR 0.3) vs 0.1 (0.3) kg, p = 0.02]. Among subjects with CF, VAT was positively associated with added sugar intake (p < 0.001) and fasting blood glucose (p = 0.04). Lung function was positively associated with BMI (p = 0.005) and LBM (p = 0.03) but not with adiposity indicators.ConclusionsThese novel data link body fat distribution with diet quality and fasting glucose levels in adults with CF, whereas LBM was associated with lung function. This study highlights the importance of increasing diet quality and assessing body composition and fat distribution in the CF population.     

Energy expenditure and nutritional adequacy of rehabilitation paraplegics with asymptomatic bacteriuria and pressure sores

OBJECTIVE: To measure resting energy expenditure (REE) in a group of people with postacute paraplegia, quantify the impact of asymptomatic bacteriuria and pressure sore(s) on patients' metabolic rate, and estimate the adequacy of patients' nutritional intakes to preserve patients' protein levels. MATERIAL AND METHODS: Ten males with post-acute paraplegia aged 42.1+/-18.7 years. We evaluated: height, body mass index (BMI), resting energy expenditure (REE), total daily calorie requirement (E), 24-h urine creatinine excretion (Cru), creatinine index (CI), and nitrogen balance (NB). RESULTS: Subjects with paraplegia showed high erythrocyte sedimentation rates. As a group, they had normal resting calorie consumption when REE was normalized for unit of urine creatinine (REE/Cru), it was higher in patients than in controls. Six of the 10 patients had a low calorie intake: of these only three had a negative nitrogen balance. CONCLUSION: In conclusion, the resting energy expenditure of the subjects with significant bacteriuria and pressure sore(s) of 23.7 kcal/kg/day suggests that a large portion of patients may have an inadequate calorie protein intake to preserve their nutritional status. The clinical significance of this study is that 28.5 kcal/kg/day may be the lower calorie threshold to meet the metabolic demands of people with apyretic paraplegia with bacteriuria and pressure sore(s).     

Do patients with nonmetastatic non-small cell lung cancer demonstrate altered resting energy expenditure?

BACKGROUND: The cancer cachexia syndrome occurs in patients with non-small cell lung cancer (NSCLC) and includes elevated resting energy expenditure (REE). This increase in REE leads to weight loss, which in turn confers a poor prognosis. This study was undertaken to determine whether the cancer cachexia syndrome occurs in patients with nonmetastatic NSCLC. METHODS: In this case-control study, 18 patients with nonmetastatic NSCLC (stages IA to IIIB) were matched to healthy controls on age (+/- 5 years), gender, and body mass index (+/- 3 kg/m2). Only 4 cancer patients had experienced > 5% weight loss. Cancer patients and controls were compared on the basis of: (1) unadjusted REE, as measured by indirect calorimetry; (2) REE adjusted for lean body mass, as measured by dual x-ray absorptiometry; (3) REE adjusted for body cell mass, as measured by potassium-40 measurement; and (4) REE adjusted for total body water, as measured by tritiated water dilution. RESULTS: We observed no significant difference in unadjusted REE or in REE adjusted for total body water. However, with separate adjustments for lean body mass and body cell mass, cancer patients manifested an increase in REE: mean difference +/- standard error of the mean: 140+/-35 kcal/day (p = 0.001) and 173+/-65 kcal/day (p = 0.032), respectively. Further adjustment for weight loss yielded similarly significant results. CONCLUSIONS: These results suggest that the cancer cachexia syndrome occurs in patients with nonmetastatic NSCLC and raise the question of whether clinical trials that target cancer cachexia should be initiated before weight loss.     

The 1-min sit-to-stand test in cystic fibrosis — Insights into cardiorespiratory responses

Background

We aimed to characterize the cardiopulmonary response during a 1-min sit-to-stand (STS) test and compare peak exercise cardiorespiratory variables to a maximal cardiopulmonary exercise test (CPET) in cystic fibrosis (CF). We further aimed to assess the validity of the STS power index (Power_STS) as a measure of exercise capacity.

Thermic effect of food and resting energy expenditure after sleeve gastrectomy for weight loss in adolescent females

BackgroundFew studies have addressed the effect of bariatric surgery on factors related to energy balance, including resting energy expenditure (REE) and thermic effect of food (TEF). To our knowledge, very few studies have examined changes in REE and none have investigated modifications in TEF after sleeve gastrectomy (SG) performed in adolescents.ObjectiveTo assess energy expenditure in females who underwent SG as adolescents and matched-control participants as preliminary data about the potential of SG to confer differences in postprandial energy expenditure.SettingChildren’s Hospital Medical Center, Cincinnati, Ohio, United States.MethodsIn this observational study, REE and respiratory quotient (RQ) were measured via indirect calorimetry, followed by a standardized meal and assessment of TEF and postprandial RQ. Plasma drawn before and every 15 minutes after the meal was assayed for insulin, glucose, and C-peptide. Usual dietary intake was estimated using 24-hour recall interviews.ResultsFasting REE and RQ were similar between surgical and control groups. Postmeal TEF also did not differ between groups. The surgical group had higher RQ early in the postprandial period, whereas the control group RQ was higher after 125 minutes post meal. Compared with the control group, the surgical group had lower postprandial glucose, higher insulin and C-peptide, and consumed less daily energy during usual intake.ConclusionsPostprandial RQ was consistent with the rapid gastric emptying typical of SG, yet we observed no group differences in REE or TEF. These findings may have been due to limited statistical power. More comprehensive studies of EE after SG are warranted.     

Analysis of energy utilization and body composition in kidney,bladder, and adrenal cancer patients

ObjectiveTo investigate resting energy expenditure (REE) and body composition and the relationship between substrate utilization and energy expenditure in urologic cancer patients.Patients and methodsMeasured resting energy expenditure (mREE) was detected by indirect calorimetry in 122 urologic cancer patients and 131 control subjects. Extracellular fluid (ECF), intracellular fluid (ICF), and total water (TW) were measured by bioelectrical impedance appliance. Fat oxidation rate (F-O), carbohydrate oxidation rate, fat mass (FM), and fat free mass (FFM) were further determined.ResultsCompared with the controls, cancer patients showed significantly elevated mREE and mREE/FFM (P = 0.049; P < 0.001). Of all the cancer patients, 50% (n = 61) were hypermetabolic, 43.4% (n = 53) normometabolic, and 6.6% (n = 8) hypometabolic, whereas 35.1% (n = 46) of the controls were hypermetabolic, 56.5% (n = 74) normometabolic, and 8.4% (n = 11) hypometabolic. REE was correlated to substrate oxidation rate (R² = 0.710). Cancer patients exhibited no significant difference in FM, FM/body weight (BW) and FFM, compared with controls. Cancer patients presented no significant difference in TW compared with controls (P = 0.791), but they had increased ECF (P < 0.001) and decreased ICF (P < 0.001).ConclusionAberrations in substrate utilization may contribute to the elevated energy expenditure in urologic cancer patients. Cancer type and pathologic stage are influential factors of REE.     

Early enteral nutrition does not decrease hypermetabolism associated with burn injury

BACKGROUND: A prospective, randomized study was performed to compare the effects of early versus late enteral feeding on postburn metabolism. METHODS: Burn patients were randomized to receive enteral feedings either within 24 hours (early) or 7 days (late) of injury. Basal energy expenditure (BEE) was calculated from Harris-Benedict equations and resting energy expenditure (REE) was obtained from indirect calorimetry. The average daily energy expenditure (DEE) was expressed as REE/BEE. RESULTS: Average age, burn size, infections, and length of stay were similar between groups. Mortality between groups was similar (early, 28%; late, 38%) and not significantly influenced by inhalation injury. When controlled for percentage of total body surface area burn, inhalation injury, and age, the early group had an increased rather than decreased DEE, with a mean DEE calorie 0.17 more than the late group (p = 0.07). CONCLUSION: Early enteral feeding does not decrease the average energy expenditure associated with burn injury.     

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.     

Test-retest reliability,minimal detectable change and minimal clinically important differences in modified shuttle walk test in children and adolescents with cystic fibrosis

BackgroundIn youths with cystic fibrosis (CF) the modified shuttle walk test (MSWT) has been shown to be useful for assessing exercise tolerance; however, no studies to date have evaluated the reliability of MSWT, the minimal detectable change (MDC) and the minimal clinically important differences (MCID) for the MSWT distance for children and adolescents with CF.MethodsTest-retest reliability: 35 CF patients and 34 healthy controls performed two MSWTs (separated by 2–4 days). MCID: 33 patients were invited to participate in a 6-week home-based exercise program consisting of 30–60-min sessions, 5 days a week.ResultsThe test-retest reliability of the MSWT distance for children and adolescents with CF was excellent, obtaining a MDC₉₀ and MDC₉₅ of 97.08 m and 115.32 m, respectively. The test-retest reliability also was excellent in healthy controls, founding a MDC₉₀ and MDC₉₅ of 69.12 m and 82.11 m, respectively. The ROC curve analysis established (with a sensitivity of 82% and specificity of 76%) that a change of 60 m represented MCID.ConclusionsThe MSWT is a reliable tool to measure of exercise tolerance in children/adolescents with CF and those without CF. We propose a change in the score of at least 97.08 m (MDC₉₀) as the most appropriate value for assessing the exercise response of children and adolescents with CF, given that this value exceeds the MCID based on the children's perception.