Search

Introduction/Aims: Persons with spinal muscular atrophy (pwSMA) report progressive muscle weakness but also reduced endurance when performing repetitive tasks in daily life, referred to as "performance fatigability" (PF). Data regarding the effects of the new disease‐modifying drugs on PF are scarce. Thus, our main objective was to examine PF in adult ambulatory pwSMA treated long‐term with nusinersen. Methods: Six‐minute walk test (6MWT) data from 14 adult pwSMA treated with nusinersen for up to 70 months were retrospectively analyzed to determine PF. Performance fatigability was defined as the percentage change in the distance covered between the last and first minute of the 6MWT. In addition, relationships between PF and other clinical features were assessed. Results: Performance fatigability was found in 12/14 pwSMA (85.7%) prior to treatment. The mean distance walked in the sixth minute (71.1 m) was shorter than the distance covered in the first minute (81.8 m), corresponding to a mean PF of 13.1% (95% confidence interval (CI): 6.5–19.6, p =.0007). During treatment with nusinersen, there was a mean reduction in PF of 5.6% (95% CI: −10.0 to −1.3, p =.0148). We found no relationship between PF and fatigue as measured by the Fatigue Severity Scale. Discussion: This study demonstrates the presence of PF as an independent component of motor impairment and as a potential therapeutic target in our cohort of adult ambulatory pwSMA. Furthermore, the observations in our cohort suggest that nusinersen may have a beneficial effect on PF. [ABSTRACT FROM AUTHOR]

Aims: Frailty is an obstacle to performing cardiopulmonary exercise test (CPET) in patients with chronic heart failure (CHF). We evaluated the usefulness of oxygen uptake efficiency slope (OUES) using a 6 min walk test (6MWT) with portable gas analysis compared with CPET‐derived parameters in patients with CHF. Methods and results: Patients with CHF who underwent both the 6MWT with portable gas analysis and CPET between December 2016 and May 2020 were retrospectively investigated. The 6MWT‐derived and echocardiographic parameters were compared with the OUES and peak oxygen consumption (VO2) from the CPET. Forty patients were analysed; 50% were male with a mean age of 55.45 ± 14.70 years. Twenty‐six patients (65%) had New York Heart Association Functional Classification II or III dyspnoea. Twenty‐five patients (62.5%) had heart failure (HF) with preserved ejection fraction (EF) (left ventricular EF > 50%), and nine patients (22.5%) had HF with reduced EF (EF < 40%). During the 6MWT, the peak VO2 was 14.97 ± 3.80 mL/kg/min, which was only 74% of the peak VO2 in the CPET, 20.18 ± 5.64 mL/kg/min. The OUES in the 6MWT was lower than that in the CPET (1528.87 ± 579.01 in the 6MWT vs. 1638.69 ± 601.31 in the CPET). The 6 min walk distance (6MWD) and OUES in the 6MWT were positively correlated with the OUES in the CPET (6MWD, r = 0.434, P = 0.005; OUES, r = 0.729, P < 0.001). The OUES in the 6MWT showed the strongest correlation with the OUES in the CPET. When we divided patients into two groups according to peak VO2 in the CPET, the correlation between OUES values of the 6MWT and that of the CPET was consistently confirmed (peak VO2 ≥ 20 mL/kg/min group, r = 0.661, P = 0.001; peak VO2 < 20 mL/kg/min group, r = 0.526, P = 0.021). In addition, the 6MWD, OUES, and peak VO2 in the 6MWT were associated with peak VO2 in the CPET (6MWD, r = 0.627, P < 0.001; OUES, r = 0.452, P = 0.003; and peak VO2, r = 0.492, P = 0.001). Conclusions: In frail patients with CHF who have difficulty performing maximal exercises, the OUES through the 6MWT may be applied instead of the OUES and peak VO2 from the CPET. [ABSTRACT FROM AUTHOR]

Background: Major abdominal surgery is associated with a high rate of post‐operative complications with increased risk of adverse surgical outcomes due to the presence of frailty. This study aims to evaluate the effectiveness of the multimodal Recovery of Surgery in the Elderly (ROSE) prehabilitation program with supervised exercise in mitigating postoperative functional decline when compared to standard care. Method: The ROSE program enrolled ambulant patients who were 65 years and above, had a Clinical Frailty Scale score of 4 or more and were planned for major abdominal surgery. Participation in supervised exercise sessions before surgery were compared with standard physiotherapy advice. The primary outcome was 6‐min walk test (6MWT) distance assessed at baseline, after prehabilitation and 30 days follow‐up after surgery. Secondary outcomes included physical performance, length of hospital stay and postoperative morbidity. Results: Data from 74 eligible patients, 37 in each group, were included. Median age was 78 years old. Forty‐two patients (22 in Prehab group and 20 in control group) with complete 6MWT follow‐up data at 30 days follow‐up were analysed for outcomes. Most patients underwent laparoscopic surgery (63.5%) and almost all of the surgeries were for abdominal malignancies (97.3%). The Prehab group had an increase in 6MWT distance at the 30‐day follow up, from a baseline mean (SD) of 277.4 (125) m to 287.6 (143.5) m (p = 0.415). The 6MWT distance in the control group decreased from a baseline mean (SD) of 281.7 (100.5) m to 260.1 (78.6) m at the 30‐day follow up (p = 0.086). After adjusting for baseline 6MWT distance and frailty score, the Prehab group had significantly higher 6MWT distance at 30‐day follow‐up than control (difference in adjusted means 41.7 m, 95% confidence interval 8.7–74.8 m, p = 0.015). There were no significant between‐group differences in the secondary outcomes. Conclusion: A multimodal prehabilitation program with supervised exercise within a short time frame can improve preoperative functional capacity and maintain baseline functional capacity in frail older adults undergoing major abdominal surgery. [ABSTRACT FROM AUTHOR]

The possible benefits of inspiratory muscle training (IMT) on mechanical and clinical outcomes in patients with Coronavirus disease‐2019 (COVID‐19) remain controversial. We conducted a meta‐analysis to evaluate the effect of IMT in the rehabilitation strategy of patients with COVID‐19. The Pubmed, Embase, Web of Science (WOS), and Cochrane Central Register of Controlled Trials (CENTRAL) were searched to identify trials evaluating the efficacy of IMT in the treatment of patients with COVID‐19. The primary outcome included change from baseline of VO2max, maximal inspiratory pressure (PImax), 6‐min walk test(6MWT), forced expiratory volume in the first second predicted (FEV1%pred), and quality of life (QOL). Six studies with 349 participants were analyzed. Significant improvements were found in change from baseline of VO2max (MD: 4.54, 95% confidence interval [CI]: 1.79–7.30, Z = 3. 32, I2 = 0, p = 0.001), PImax (MD: 21.43, 95% CI: 1.33–41.52, Z = 2.09, I2 = 90%, p = 0.04), 6MWD (MD: 40.13, 95% CI: 24.92–55.35, Z = 5.17, I2 = 0, p < 0.00001) and FEV1%pred (MD: 8.73, 95% CI 3.07–14.39, Z = 3.02, p = 0.002) while no statistical improvements were found in QOL (SMD: 0.70, 95% CI: 0.37–1.03, Z = 4.15, I2 = 89% p = 0.32) between IMT group and control group. The application of IMT might elicit mechanical and clinical improvement in patients with COVID‐19. IMT could be recommended as an effective strategy of pulmonary rehabilitation for COVID‐19. However, the proper timing, optimal duration, as well as appropriate frequency and intensity of IMT remain uncertain and further studies are needed. [ABSTRACT FROM AUTHOR]

Objectives: The six‐minute walk test (6MWT) is a commonly used measure of functional capacity. This study is the first to investigate the test‐retest reliability, minimal detectable difference (MDD) and the minimal clinically important difference (MCID) for people attending a persistent pain service. Relationships between change in 6MWT performance and change in self‐reported physical, functional and psychological outcome measures were also explored. Methods: A cross‐sectional repeated measures design was used with people having >9 months of pain attending an 8‐week outpatient persistent pain programme. For reliability and MDD, 27 people were recruited, for MCID calculations, 32 people were recruited. The MCID was examined by dichotomising people into "improvers", or "non‐improvers" based upon the Global Rating of Change (GRC) in physical abilities score. Results: The mean (SD) 6MWT distance was 389.4 (93.6) m at programme start, and 427.8 (83.0) m at week eight completion. The test‐retest reliability was good (intraclass correlation coefficient = 0.89) and the MDD = 86.1 m. As there was no relationship between change in 6MWT distance and GRC physical abilities at week eight (r = 0.132, p = 0.472) the MCID could not be calculated. Furthermore, no relationships were found between change in 6MWT distance and other self‐reported measures. Changes in GRC physical abilities and 6MWT were frequently discordant, with increased 6MWT for 7/11 "GRC non‐improvers" and decreased 6MWT for 7/21 "GRC improvers". Conclusions: Amongst this cohort, change in physical ability may or may not be reflected by self‐reported change. Objective tests of physical ability are recommended for people attending pain services, and validated tests should align with intervention aims. [ABSTRACT FROM AUTHOR]

Aims: Exercise games (exergames) have been recently proposed as a mode of facilitating physical activity in patients with chronic diseases. Although patients supported with left ventricular assist devices (LVADs) benefit from physical activity, specific LVAD‐related issues hinder their ability to exercise properly. The objective of this study was to assess the feasibility and safety of exergaming in LVAD‐supported patients. Methods and results: Eleven LVAD‐supported patients were enrolled in a 4 week exergaming programme using Nintendo Wii console with five sport games. Patients were instructed to play for 30 min a day, 5 days a week. Data on exercise capacity and exergaming were collected by using the 6 min walk test (6MWT) and a daily self‐report diary, respectively. Feasibility of using the console and its safety was assessed by a semi‐structured patient interview. Quality of life was assessed by the Minnesota Living with Heart failure Questionnaire (MLHFQ) and the Cantril's Ladder of Life. Safety was assessed by patient's report in interview and diary. The study group consisted of 10 male patients and 1 female patient, mean age of 67 ± 7 years, of whom 10 were supported with the HeartMate 3 LVAD for a median of 10 (interquartile range 3, 21) months. Baseline exercise capacity assessed by the 6MWT ranged from 240 to 570 m (mean 448 ± 112). After 4 weeks of exergaming, 6MWT distance increased from a mean of 448 ± 112 (evaluated in six patients) to 472 ± 113 m (P = 0.023). Patients' Cantril's Ladder of Life score improved numerically from an average of 6.13 to 7.67, as did their MLHFQ score from 45.9 ± 27 to 38.7 ± 18, with higher and lower scores, respectively, reflecting higher quality of life. No specific LVAD‐related safety issues regarding exergaming were reported. Conclusions: Exergaming was found to be a safe and feasible mode for encouraging physical activity in LVAD‐supported patients and carries a potential for improving exercise capacity and quality of life in these patients. Larger scale studies are warranted to further investigate the effect of exergaming in this patient population. [ABSTRACT FROM AUTHOR]

Introduction: Exercise capacity has been established as a protective factor against joint impairment in people with haemophilia (PWH). However, little is known about how exercise capacity is affected in PWH. Aim: To analyse exercise capacity, as assessed by standardised laboratory or field tests in PWH. Methods: A systematic review was conducted to identify manuscripts investigating physical capacity in PWH. An electronic search of PubMed/MEDLINE, Embase, Web of Science, CENTRAL and CINAHL was conducted from inception to 13 April, 2022. Two independent reviewers performed data extraction and assessed study quality using the critical appraisal tools of the Joanna Briggs Institute. Results: Nineteen studies with 825 patients were included. Most studies used the six‐min walk test (6MWT) or peak/maximal oxygen consumption (VO2max). In children, the distance walked ranged from 274 ± 36.02 to 680 ± 100 m. In adults, the distance walked ranged from 457.5 ± 96.9 to 650.9 ± 180.3 m. VO2max ranged from 37 ± 8 to 47.42 ± 8.29 ml kg–1 min–1. Most studies reported lower values of exercise capacity compared to standardised values. Overall, the quality of the studies was moderate. Conclusion: Most of the studies showed that PWH have lower exercise capacity compared to reference values of 6MWT or VO2max. Based on these results, it is necessary to emphasise in both the promotion and the prescription of physical exercise in PWH. [ABSTRACT FROM AUTHOR]

Aims: ACTIVITY‐HF was a randomized, double‐blind, active‐controlled study, which assessed the short‐term effect of sacubitril/valsartan compared with the active comparator enalapril on improving maximal exercise capacity in patients with heart failure with reduced ejection fraction (HFrEF). Methods and results: A total of 201 ambulatory patients with HFrEF (left ventricular ejection fraction ≤ 40%, New York Heart Association class III) across 34 centres in Germany were randomized (1:1) to receive sacubitril/valsartan 97/103 mg bid (n = 103) or enalapril 10 mg bid (n = 98). The primary endpoint of the study was the change from baseline in peak oxygen consumption (VO2; adjusted to body weight) after 12 weeks, and the key secondary endpoint was change from baseline in peak VO2 after 6 weeks. The study population was predominantly male (81.1%) with a mean age of 66.9 years and a body mass index of 29.4 kg/m2. Change in peak VO2 from baseline to Week 12 was similar between sacubitril/valsartan and enalapril groups [least squares mean difference: 0.32 mL/min/kg; 95% confidence interval (CI) −0.21, 0.85; P = 0.2327]. Similarly, no significant differences were observed between the two treatment groups in minute ventilation to carbon dioxide production slope, exercise capacity at first ventilatory threshold or Borg scale at either Week 6 or Week 12. Change in heart rate at first ventilatory threshold was lower in the sacubitril/valsartan group compared with the enalapril group at Week 12 (mean −3.75 bpm; 95% CI −7.03, −0.48; P = 0.0248). The safety of sacubitril/valsartan was comparable to enalapril. Conclusion: In patients with HFrEF, short‐term treatment with sacubitril/valsartan for 12 weeks did not result in significant benefits on peak VO2 when compared with enalapril. [ABSTRACT FROM AUTHOR]

This cross‐sectional Physical Fitness Study compared cardiorespiratory fitness (VO2 peak), six‐minute walk test (6MWT), muscle strength (30sSTS), balance (BESS), and self‐reported physical activity level (IPAQ) in Norwegian adults with achondroplasia (ACH) to reference values of average‐statured individuals. The feasibility of the physical fitness tests and IPAQ was explored. Forty‐three adults (22 women) participated. Mean age was 38 years (range 16–69 years). Mean differences (95% CI) for men and women with ACH compared to reference values were: VO2 peak. −7.0 m/min/kg (−13.6 to −0.5, p = .037), and − 7.9 ml/kg/min (−11.6 to −4.3, p < .001); 6MWT −270.8 m (−340.4 to −201.2, p < .001), and − 196.7 m (−244.3 to −149.0, p = .001); 30sSTS ‐4.6 repetitions (−7.8 to −1.5, p = .006), and − 1.1 repetitions (−3.4 to 1.1, p = .335). There were no differences within ACH participants, except for VO2 peak, where men performed better. Sufficient physical activity (> 600 metabolic equivalent of task weekly) was achieved by 79% of the participants. The feasibility of the 6MWT and 30sSTS was good. There was a strong correlation between the VO2 peak and 6MWT (men: r = 0.63, p = .007; women: r = 0.71, p < .001). The findings indicate that the 6MWT and 30sSTS test are useful in assessing functional exercise capacity and muscle strength in adults with ACH. [ABSTRACT FROM AUTHOR]

Aims: OUTSTEP‐HF compared the effect of sacubitril/valsartan vs. enalapril on 6‐min walk test (6MWT) distance, non‐sedentary daytime physical activity and heart failure (HF) symptoms in patients with HF with reduced ejection fraction (HFrEF). Methods and results: Ambulatory patients (n = 621) with stable symptomatic HFrEF were randomised 1:1 to sacubitril/valsartan (n = 310) or enalapril (n = 311). Changes in physical activity and mean daily non‐sedentary daytime activity from baseline to Week 12 were measured using 6MWT and a wrist‐worn accelerometer device, respectively. After 12 weeks, 6MWT improved by 35.09 m with sacubitril/valsartan [97.5% confidence interval (CI) 27.85, 42.32] and by 26.11 m with enalapril (97.5% CI 18.78, 33.43); however, there was no significant difference between groups [least squares means treatment difference: 8.98 m (97.5% CI −1.31, 19.27); P = 0.0503]. Mean daily non‐sedentary daytime activity decreased by 27 min with sacubitril/valsartan and by 21 min with enalapril [least squares means treatment difference: −6 min (97.5% CI −25.7, 13.4), P = 0.4769] after 12 weeks. 6MWT improved by ≥30 m in 51% of patients in the sacubitril/valsartan group vs. 44% of patients treated with enalapril (odds ratio 1.251, 95% CI 0.895, 1.748). At Week 4, non‐sedentary daytime activity increased by ≥10% in 58% of patients treated with sacubitril/valsartan vs. 64% with enalapril; 58% of patients treated with sacubitril/valsartan reported improved HF symptoms as assessed by patient global assessment vs. 43% with enalapril. However, these differences did not persist at Week 12. Conclusion: After 12 weeks of treatment, there was no significant benefit of sacubitril/valsartan on either 6MWT or daytime physical activity measured by actigraphy compared with enalapril. [ABSTRACT FROM AUTHOR]

PURPOSE: Identify the prevalence of the nursing diagnosis (ND) Fatigue in patients with heart failure (HF) and to analyze the accuracy of its defining characteristics (DCs). METHODS: 50 patients were assessed for Fatigue through a distance ambulated <300m during the Six‐Minute Walking Test (6MWT) and the report of insufficient energy. Sensitivity, specificity, positive and negative predictive values were calculated for the DCs associated with Fatigue. FINDINGS: The prevalence of Fatigue was 48%. No DCs were sensitive and specific for determining Fatigue. CONCLUSIONS: There were no DCs accurate enough to infer Fatigue. IMPLICATIONS FOR PRACTICE: A distance ambulated <300m during the 6MWT should be included as a DC of Fatigue. [ABSTRACT FROM AUTHOR]

Introduction: With the advent of disease-altering therapies for spinal muscular atrophy (SMA), there is a requirement to better characterize outcome measures, particularly in milder forms of disease.Methods: Maximal voluntary isometric contraction testing and 6-minute walk test (6MWT) performed in ambulatory SMA adults as part of the SMA-VALIANT trial were analyzed. Test-retest reliability and correlation with other candidate biomarkers and outcomes were investigated.Results: Maximal voluntary isometric contraction testing and 6MWT showed good test-retest reliability (intraclass correlation coefficient = 0.98 and 0.85, respectively). Maximal voluntary isometric contraction testing and 6MWT demonstrated very strong correlation (r = 0.83, P <. 0001), and each correlated with the SMA Functional Rating Scale (r = 0.7, P < .0001 and r = 0.65, P = .0001, respectively), lean muscle mass (r = 0.68, P < .0001 and r = 0.56, P = .001, respectively), and ulnar compound muscle action potential (r = 0.57, P = .0008 and r = 0.47, P = .008, respectively).Discussion: Maximal voluntary isometric contraction testing and 6MWT are suitable outcomes for use in ambulatory adults with SMA. Maximal voluntary isometric contraction testing may be preferable because of superior test-retest reliability and closer associations with other outcomes and biomarkers of neuromuscular function. [ABSTRACT FROM AUTHOR]

Aims: Patients with heart failure and preserved ejection fraction (HFpEF) are characterized by functional impairment and an abnormal haemodynamic response to exercise. The six-minute walk test (6MWT) serves as a standardized test for functional capacity quantification in heart failure patients, and is associated with cardiovascular outcomes. However, as the association between 6MWT and haemodynamic parameters during rest and exercise in HFpEF patients is unknown, we sought to elucidate this relationship.Methods and Results: Overall, 64 patients enrolled in the REDUCE LAP-HF trial completed a 6MWT at baseline. Univariate and multivariable linear regression models were used to assess the associations between 6MWT and measured or derived haemodynamic variables at baseline, during light/moderate exercise (20 W), and at peak supine exercise. The average 6MWT distance was 318 ± 106 m. At rest, in a multivariable model, only pulmonary capillary wedge pressure (PCWP) was significantly associated with 6MWT [coefficient: -5.4, 95% confidence interval (CI) -10.4, -0.5, P = 0.033]. During light/moderate exercise, mean pulmonary artery pressure was associated with 6MWT in a multivariable model (coefficient: -3.5, 95% CI -6.8, -0.3, P = 0.033). During peak exercise, central venous pressure, cardiac index (CI), and PCWP/CI correlated with 6MWT; however, workload corrected PCWP was the only variable independently associated with 6MWT (coefficient: -0.8, 95% CI -1.3, -0.4, P < 0.001). The variance in 6MWT was modestly explained by measured or derived haemodynamic variables at rest or at any stage of exercise (r2  = 7-17%).Conclusion: Workload corrected PCWP correlated best with 6MWT performance in HFpEF patients. Baseline haemodynamic variables were modestly correlated with 6MWT, suggesting that 6MWT performance in HFpEF patients may be significantly influenced by extra-cardiac factors. [ABSTRACT FROM AUTHOR]

Aim This study investigated the associated factors of 6-min walk test (6MWT) and its predictive value of outcome in patients undergoing peritoneal dialysis (PD). Methods This is a single centre prospective observational cohort study. Stable ambulatory PD patients in our centre between 1 May 2010 and 30 April 2011 were enrolled in this study. All included subjects performed 6MWT, and 6-min walk distances (6MWDs) were recorded. Patients were divided into two groups according to 6MWD and prospectively followed up until death, cessation of PD or to the end of the study (30 September 2012). Results A total of 145 patients were enrolled, including 63 (43%) males. Multiple stepwise regression showed that age (β = -0.295, P = 0.001), diastolic blood pressure (DBP) (β = 0.292, P = 0.001), left ventricular ejection fraction (LVEF) (β = 0.198, P = 0.019) were independently associated with lower 6MWD. By the end of the study, six (8%) patients died in long 6MWD group while 15 (20%) died in the short 6MWD group, a significantly lower patient survival was observed in short 6MWD group (Log-rank = 4.983, P = 0.026). Patients with short 6MWD also showed inferior technique survival (Log-rank = 4.838, P = 0.028). There was no significant difference in peritonitis-free survival between the two groups (Log-rank = 0.801, P = 0.371). However, more patients in short 6MWD group had been transferred to hemodialysis due to peritonitis (25% vs 4.2%, P = 0.013). Conclusion Age, diastolic blood pressure, LVEF are independent associated factors of 6MWD in patients undergoing PD. Having the advantages of easy applicability and safety, 6MWT may be proposed as an important predictor of outcome in ambulatory PD patients. [ABSTRACT FROM AUTHOR]

Introduction: The Six-Minute Walk Test (6MWT) was adopted as a clinical outcome measure for ambulatory spinal muscular atrophy (SMA). However, a systematic review of measurement properties reported significant variation among chronic pediatric conditions. Our purpose was to assess the reliability/validity of the 6MWT in SMA.Methods: Thirty participants performed assessments, including the 6MWT, strength, and function. Reproducibility was evaluated by intraclass correlation coefficients. Criterion/convergent validity were determined using Pearson correlation coefficients.Results: Test-retest reliability was excellent. The 6MWT was associated positively with peak oxygen uptake, Hammersmith Functional Motor Scale Expanded (HFMSE), lower extremity manual muscle testing, knee flexion hand-held dynamometry, and inversely with 10-m walk/run. The 6MWT discriminates between disease severity, unlike the HFMSE.Conclusions: This study documents measurement properties of reproducibility, positive criterion validity, and convergent validity with established clinical assessments and reaffirms the value of the 6MWT as a pivotal outcome measure in SMA clinical trials. Muscle Nerve 54: 836-842, 2016. [ABSTRACT FROM AUTHOR]

ABSTRACT Introduction: Spinal muscular atrophy (SMA) causes muscle weakness and fatigue. Better understanding of the relationship between weakness and fatigue may help identify potential targets for rehabilitation. Methods: Gait and surface electromyography (EMG) from 4 muscle groups were measured during the Six-Minute Walk Test (6MWT) in 10 ambulatory participants, aged 9-49 years. Average root mean square amplitude (RMS) of muscle activity was calculated. Strength was assessed using manual and quantitative methods. Results: RMS, stride length, and velocity decreased during the 6MWT. Knee flexor and hip abductor strength was associated with fatigue-related changes; overall strength correlated with disease duration; and leg strength was associated with 6MWT distance. Conclusions: Clinical measures are valid in assessing fatigue and function in SMA, and these assessments can be enhanced by use of gait analysis and EMG. Disease duration and strength measures may represent further stratification refinements when enrolling patients in clinical trials. Muscle Nerve 50: 34-39, 2014 [ABSTRACT FROM AUTHOR]

The purpose of the pretransplant assessment in lung transplantation is to determine a patient's need for transplant as well as their potential survival post-procedure. In 2005, the UNOS introduced the LAS, a calculation based on multiple physiologic measures to determine need and likelihood for survival. Measures include NYHA class and the 6- MWT. Some adult studies indicate a positive correlation with 6- MWT and waiting list survival. In pediatric/adolescent patients, there are minimal data regarding the predictive value of physiologic markers in either wait list survival or post-transplant outcome. A retrospective cohort study of 60 consecutive lung transplantations from 1990 to 2008 was performed at a pediatric tertiary care facility. Functional pretransplant assessments were abstracted from the medical record and compared with outcomes after transplantation. Results: a 6- MWT of >1000 ft (305 m) prior to transplantation correlated with a shorter ICU stay (7 vs. 11 days, p = 0.046) and fewer days of mechanical ventilation (2 vs. 4, p = 0.04). A pretransplant 6- MWT greater than 750 ft (229 m) correlated with shorter overall hospitalization (37 vs. 20 days, p = 0.03). Measuring pretransplant 6- MWT tests for pediatric patients is valuable in predicting peri-operative outcomes after lung transplantation. [ABSTRACT FROM AUTHOR]

Tomás MT, Santa-Clara MH, Monteiro E, Baynard T, Carnero EÁ, Bruno PM, Barroso E, Sardinha LB, Fernhall B. Body composition, muscle strength, functional capacity, and physical disability risk in liver transplanted familial amyloidotic polyneuropathy patients. Clin Transplant 2011: 25: E406-E414. © 2011 John Wiley & Sons A/S. Abstract: Background: Familial amyloidotic polyneuropathy (FAP) is a neurodegenerative disease leading to sensory and motor polyneuropathies, and functional limitations. Liver transplantation is the only treatment for FAP, requiring medication that negatively affects bone and muscle metabolism. The aim of this study was to compare body composition, levels of specific strength, level of physical disability risk, and functional capacity of transplanted FAP patients (FAPTx) with a group of healthy individuals (CON). Methods: A group of patients with 48 FAPTx (28 men, 20 women) was compared with 24 CON individuals (14 men, 10 women). Body composition was assessed by dual-energy X-ray absorptiometry, and total skeletal muscle mass (TBSMM) and skeletal muscle index (SMI) were calculated. Handgrip strength was measured for both hands as was isometric strength of quadriceps. Muscle quality (MQ) was ascertained by the ratio of strength to muscle mass. Functional capacity was assessed by the six-minute walk test. Results: Patients with FAPTx had significantly lower functional capacity, weight, body mass index, total fat mass, TBSMM, SMI, lean mass, muscle strength, MQ, and bone mineral density. Conclusion: Patients with FAPTx appear to be at particularly high risk of functional disability, suggesting an important role for an early and appropriately designed rehabilitation program. [ABSTRACT FROM AUTHOR]

Aim: To investigate the correlation between 6-min walk test (SMWT) and incremental treadmill exercise stress test (EST) as indicators for the functional capacity in children. Methods: Healthy children aged 9–12 years were included. The anthropometric data, SMWT and EST were prospectively measured using the standard protocols. Various parameters were analysed to define the correlation between SMWT and EST. Results: A total of 100 subjects (53 boys) aged 10.3 ± 1.0 years participated in the study. The SMWT distance was 586.1 ± 44.0 m. Height (r = 0.59, R2 = 35%), length of the leg (r = 0.64, R2 = 41%), heart rate at the end of SMWT (r = 0.59, R2 = 35%) and heart rate difference at the end of SMWT (r = 0.71, R2 = 50%) were found to have significant correlation with SMWT distance. The estimated maximal oxygen consumption (eVO2) obtained during the EST tended to be greater in boys than in girls. Among the parameters obtained during EST, maximal heart rate (r = 0.33, R2 = 11%) and the eVO2 (r = 0.54, R2 = 53%) were found to have significant correlation with SMWT. Conclusions: SMWT distance is significantly correlated with the eVO2 obtained during the EST. This indicates that SMWT is also one of the predictive markers for EST performance. [ABSTRACT FROM AUTHOR]

This cross-sectional Physical Fitness Study compared cardiorespiratory fitness (VO 2 peak), six-minute walk test (6MWT), muscle strength (30sSTS), balance (BESS), and self-reported physical activity level (IPAQ) in Norwegian adults with achondroplasia (ACH) to reference values of average-statured individuals. The feasibility of the physical fitness tests and IPAQ was explored. Forty-three adults (22 women) participated. Mean age was 38 years (range 16-69 years). Mean differences (95% CI) for men and women with ACH compared to reference values were: VO 2 peak. -7.0 m/min/kg (-13.6 to -0.5, p = .037), and - 7.9 ml/kg/min (-11.6 to -4.3, p < .001); 6MWT -270.8 m (-340.4 to -201.2, p < .001), and - 196.7 m (-244.3 to -149.0, p = .001); 30sSTS -4.6 repetitions (-7.8 to -1.5, p = .006), and - 1.1 repetitions (-3.4 to 1.1, p = .335). There were no differences within ACH participants, except for VO 2 peak, where men performed better. Sufficient physical activity (> 600 metabolic equivalent of task weekly) was achieved by 79% of the participants. The feasibility of the 6MWT and 30sSTS was good. There was a strong correlation between the VO 2 peak and 6MWT (men: r = 0.63, p = .007; women: r = 0.71, p < .001). The findings indicate that the 6MWT and 30sSTS test are useful in assessing functional exercise capacity and muscle strength in adults with ACH., (© 2020 The Authors. American Journal of Medical Genetics Part A published by Wiley Periodicals LLC.)