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Effects of exercise and creatine on myosin heavy chain isoform composition in patients with Charcot-Marie-Tooth disease.

It is not known whether myosin heavy chain (MHC) content changes in response to exercise training or creatine supplementation in subjects with Charcot-Marie-Tooth disease (CMT). Based on previous data, we hypothesized that resistance exercise and creatine would increase the percentage of type I MHC composition in the vastus lateralis muscle and that myosin isoform changes would correlate with improved chair rise-time in CMT subjects. To test this hypothesis, 18 CMT subjects were randomly assigned to either a placebo or creatine group. All subjects performed a 12-week, home-based, moderate-intensity resistance training program. Chair rise-time was measured before and after the training program. Muscle biopsies were obtained from the vastus lateralis before and after the 12-week program. Gel electrophoresis showed a significant decrease (approximately 30%) in MHC type I in CMT subjects given creatine supplementation when compared with placebo. There was a nonsignificant increase in both MHC type IIa (approximately 23%) and MHC type IIx (approximately 7%) in CMT subjects given creatine. Reduced MHC type I content and increased MHC type IIa content correlated with faster chair rise-times (i.e., improved muscle performance). The training-induced change in MHC IIa content was inversely correlated with chair rise-time in CMT subjects given creatine. When the two subject groups were combined, there was a linear, negative relationship between the change in MHC type IIa content and chair rise-time after training and a positive relationship between the training-induced change in MHC type I content and chair rise-time. These data suggest that improved function (chair rise-time) was associated with a lower level of MHC type I and increased MHC type IIa composition. Furthermore, the data are consistent with the hypothesis that creatine supplementation alters MHC composition in CMT patients undergoing resistance training and that MHC changes associated with creatine supplementation can improve muscle function.

Adult↗

Double trouble (McArdle's disease and myasthenia gravis): how can exercise help?

We report a 29-year-old patient with McArdle's disease and myasthenia gravis. She had been debilitated with McArdle's disease since childhood (with marked rhabdomyolysis) and was obese. Myasthenia gravis was diagnosed at 24 years of age. After 3 months of aerobic exercise training, her exercise capacity increased significantly and she regained the ability to live independently. We conclude that even patients with profound neuromuscular diseases may benefit from carefully prescribed exercise training.

Adult↗

Physical activity and motor decline in older persons.

We tested the hypothesis that physical activity modifies the course of age-related motor decline. More than 850 older participants of the Rush Memory and Aging Project underwent baseline assessment of physical activity and annual motor testing for up to 8 years. Nine strength measures and nine motor performance measures were summarized into composite measures of motor function. In generalized estimating equation models, global motor function declined during follow-up (estimate, -0.072; SE, 0.008; P < 0.001). Each additional hour of physical activity at baseline was associated with about a 5% decrease in the rate of global motor function decline (estimate, 0.004; SE, 0.001; P = 0.007). Secondary analyses suggested that the association of physical activity with motor decline was mostly due to the effect of physical activity on the rate of motor performance decline. Thus, higher levels of physical activity are associated with a slower rate of motor decline in older persons.

Activities of Daily Living↗

Changes in bone mineral density in survivors of childhood acute lymphoblastic leukemia.

BACKGROUND: There is little information about factors modulating bone mineral density (BMD) in survivors of childhood acute lymphoblastic leukemia (ALL). PROCEDURE: We analyzed data from 57 survivors (26 male, 52 Caucasian) who underwent two serial quantitative computed tomography (QCT) studies of BMD. Using multiple linear regression, we evaluated the association of BMD change with demographic variables, treatment history, hormone therapy, exercise, and tobacco and alcohol use. RESULTS: The median age was 3.4 years (range, 0.9-17.4 years) at diagnosis of ALL; the median age at the first QCT (Study I) was 15.0 years (range, 10.6-31.0 years) and at the second QCT (Study II) was 18.2 years (range, 14.2-35.3 years). Mean height increased 4.7 cm and mean weight increased 8.8 kg between Studies I and II. While the mean BMD increased 9.33 mg/cc (P = 0.003), the BMD Z-score increased only slightly (0.21 SD, P = 0.035). Cortical bone density increased significantly (approximately 25.3 mg/cc; P = 0.001), but the ratio of trabecular to cortical BMD decreased significantly (P = 0.045). Factors independently associated with unfavorable BMD changes included older age at diagnosis (P = 0.001), female sex (P = 0.018), and nutritional supplementation (0.032). Alcohol (P = 0.009) was an unfavorable factor in a univariable analysis. CONCLUSIONS: Bone mineral accretion during adolescence is attenuated in childhood ALL survivors by a comparative deficit in trabecular versus cortical bone deposition. BMD is influenced favorably by exercise in early adolescence and unfavorably by the use of nutritional supplements and alcohol. These results provide new information about behavioral factors that affect bone accrual in survivors of childhood ALL and warrant definitive evaluation in a larger cohort.

Adolescent↗

Usefulness of a program of hospital-supervised physical training in patients with cystic fibrosis.

Exercise is an important part of normal childhood, but the ability to exercise may be impaired in chronic lung diseases such as cystic fibrosis (CF). Improving exercise performance by training is very attractive. The aim of the present study was the evaluation of the effects of a physical aerobic training program, performed in the Children's Hospital and Research Institute "Bambino Gesù" (Rome, Italy) in outpatient CF children, supervised by a physician. Twelve patients (mean forced expiratory flow in 1 sec (FEV1), 71%), age range 12-24 years (16.7 +/- 4.4 years), were enrolled. They performed a maximal exercise stress test on the treadmill (modified Bruce protocol) with breath-by-breath determination of oxygen consumption (VO2) to maximum at end-exercise; we measured time of exercise (TE), maximal heart rate (Hrmax) in beats per minute (bpm), and maximal systolic blood pressure (SBPm) in mmHg. The program consisted of 12 weeks of training twice a week. Each training session consisted of walking or running on the treadmill for 30 min at the speed that allowed the child to attain 60% of the maximal heart rate obtained during a baseline stress test for 4 weeks, 70% in the following 4 weeks, and 80% in the last 4 weeks, under strict medical supervision. HR was continously monitored. There was no change in FEV1 and forced vital capacity after the treatment period. Hrmax and SBPm also remained the same (P = 0.37 and P = 0.25, respectively). There was a significant increase in TE (P < 0.002), VO2, VO2/kg, and pulmonary ventilation (VE) (P < 0.0001, P < 0.001, and P < 0.001, respectively). This pilot study showed that a simple training program improves short-term cardiopulmonary fitness in children with CF. Further studies with a larger sample and for a more prolonged time are necessary to assess if sport can have a long-term effect on lung function or survival in CF patients.

Adolescent↗

A multidimensional scaling analysis of the techniques that physiotherapists use.

Following the essentially 'hands-on' origin of the physiotherapy profession a century ago, the range of techniques available to practitioners has expanded considerably. While this expansion of techniques has been of research interest, the main focus has been upon individual techniques, thus neglecting the fundamental question of the use of techniques in combination. In addition, researchers have tended to treat the profession as homogeneous, thus overlooking possible differences in technique usage. The present study addressed these questions. It postulated that ordered combinations of techniques usage would be employed, and that a typology would exist that differentiates the profession according to patterns of use. The study comprised a questionnaire survey of 230 clinical physiotherapists in England. This sought information on the range and frequency of techniques used over the previous six months. Descriptive analysis indicated a high frequency of use of several techniques, notably exercise therapy, passive mobilisation techniques and ultrasound. A feature of the analysis was the use of two multivariate procedures, multidimensional scalogram analysis (MSA) and smallest space analysis (SSA). The SSAs revealed a clear structure concerning combinations of techniques used, and the MSAs provided a coherent typology based upon this usage. One feature of the techniques in combination that emerged was the frequent use of passive mobilising techniques, which was associated with equally frequent use of electrotherapy techniques. In contrast, respondents who employed neurological or respiratory techniques made little use of electrotherapy. Regarding the typology, differentiation of the profession according to use of particular combinations of techniques indicated probable specialisms. The specialist areas identified were neurological physiotherapy, respiratory physiotherapy and musculo-skeletal physiotherapy. The results reinforce the need for both future research and physiotherapy education to address the issue of techniques in combination. Finally, and on the basis of the analyses, elements of a theoretical model are advanced that may provide directions for future research.

England↗

Physiological response to a short period of exercise training in patients with chronic heart failure.

BACKGROUND AND PURPOSE: Whilst cardiac rehabilitation is regularly offered to patients recovering from a myocardial infarction, people with chronic heart failure are often excluded from exercise training programmes. This study was designed to investigate the effects of eight weeks' exercise training in patients with moderate chronic heart failure. METHOD: Eight male patients entered a 16-week randomized, controlled, crossover study of two months' exercise training versus a control period. They performed supervised training three times a week and measures of indices of aerobic function and exercise tolerance were performed monthly throughout the study period. RESULTS: After training, the time walked on a treadmill during an exercise tolerance test increased from 14.8 min (95% CI; range 10.4-18.1 min) to 15.9 min (range 12.1-15.9 min). Significant changes at peak exercise levels (p < 0.05) were seen in oxygen uptake increasing from 3.4 metabolic equivalents (METs) (95% CI; range 2.6-4.2 METs) to 4.0 METs (range 3.2-4.9 METs), and in cardiac index, improving from 5.0 l/min-1/m-2 (95% CI; range 2.9-8.1 l/min-1/m-2) to 5.5 l/min-1/m-2 (range 3.0-9.4 l/min-1/m-2). Resting heart rate decreased significantly (p < 0.05), from 84 beats/min-1 (95% CI; range 73-105 beats/min-1) to 76 beats/min-1 (range 64-90 beats/min-1). Blood flow measurements by venous occlusion plethysmography showed a 21.5% decrease (range 58.5-238.9%) in resting blood flow to the lower leg and a 31.6% increase (range -7.2% to 52.5%) after submaximal exercise, but these changes were not significant (p > 0.05). All benefits were lost within two months of ceasing regular exercise. CONCLUSION: This study demonstrated that patients with moderate chronic heart failure derived beneficial physiological responses from eight weeks' exercise training, but these were soon lost after cessation of regular exercise.

Adult↗

Change in urethral pressure during voluntary pelvic floor muscle contraction and vaginal electrical stimulation.

The purpose of the study was to compare the effect of voluntary pelvic floor muscle (FFM) contraction and vaginal electrical stimulation on urethral pressure. Twelve women with genuine stress incontinence, mean age 49.4 years (range 33-66) participated in the study. The urethral and bladder pressures were recorded simultaneously through a double-lumen 8 Ch catheter. The patients first performed three voluntary PFM contractions. Then two electrical stimulators, Conmax and Medicon MS 105, 50 Hz, were used in random order. A visual analog scale was used to measure pain and discomfort. Pain was reported to mean 6.8, SEM 0.64 (range 0.7-9.9) and mean 6.1, SEM 0.81 (range 0-9.1) with Conmax and Medicon MS 105, respectively. The mean paired difference in favor of voluntary contraction with Conmax was -8.0, SD 6.7, P = 0.0067, and with Medicon MS 105 it was -12.2, SD 5.9, P = 0.0022. The results demonstrated that voluntary PFM contraction increased urethral pressure significantly more than did vaginal electrical stimulation.

Adult↗

[Indications, technique and results of treatment of fractures of the base of the first metacarpal bone with movement stable osteosynthesis].

We report about the indication, technique and results of 27 fractures of the base of the metacarpus I. All those fractures were treated by open reduction and screw-/plate osteosynthesis according to the AOSIF-technique. Using these implants and the technique of open reduction with an additional autologous bone graft it offers the possibility to start active motion therapy immediately after operation. Perioperative complications can be bypassed with the use of external mini-fixateur as well as in those cases of open fractures with various types of damage patterns. Total active mobility (TAM) of the thumb joints showed in our series of 27 treated fractures an active total mobility of 80% or more in 25 patients. There where three patients with wound healing disturbances. No fracture healed delayed or resulted in non union, no patient showed signs of reflex dystrophy, yet in two cases insufficient joint reconstruction had to be noted. Neither our own short followed-up results or those few cases published so far allow us a final critical judgement of the best technique in treating the fractures of the base of metacarpus I.

Adult↗

Initial signs and diagnosis of diabetes--special considerations of Oriental patients.

Hyperglycemia and other metabolic derangements resulting from absolute or functional deficiency of insulin are accompanied by typical signs and symptoms of diabetes. The clinical signs and the findings of hyperglycemia over 200 mg/dl should establish a diagnosis of diabetes mellitus. An oral glucose tolerance test (O-GTT) is rarely necessary for diagnosis of diabetes in a child. A small proportion of children, however, present less severe symptoms, and may require an O-GTT. Approximately 14% of IDDM children were in coma at diagnosis in Tokyo, and 11 onset deaths (0.94%) were observed among the 1172 newly diagnosed IDDM cases in Japan. A significant decline in the onset mortality, however, has been observed in the past 20 years in Japan in association with the improvement of early management of childhood diabetes. The clinical distinction of IDDM from NIDDM is often difficult in diabetic children of Oriental origin without obesity. Japanese IDDM can be divided into two forms, abrupt and slow onset forms, but they may be essentially the same disease. There was no difference in the frequency of being tested positive for circulating ICA between the two groups of the patients. But a difference in the frequency of HLA DR4 and DRW9 was noticed between the two groups. Clinical features of 107 children with NIDDM were studied and about 75% of these cases were obese. All of them can be detected by routine urinalysis for glucose. Diet and exercise therapy in most of the newly diagnosed patients resulted in remission but some of them may require insulin or an oral hypoglycemic agent to get better glycemic control.

Child↗

Obesity, sleep apnea syndrome, and rhythmogenic risk.

Obstructive sleep apnea is a common disorder and affects approximately 4% of middle-aged men and 2% of middle-aged women. Obstructive sleep apnea is clearly associated with obesity, with more than 50% of patients having a body mass index>30 kg/m2. Substantial evidence identified obstructive sleep apnea as risk factor not only for excessive daytime sleepiness and road traffic accidents, but also for increased cardiovascular morbidity and mortality. In addition, all kinds of arrhythmias have been observed in patients with sleep apnea ranging from asymptomatic sinus bradycardia to sudden cardiac death. Approximately 5-10% of patients with obstructive sleep apnea show marked apnea-related bradyarrhythmias due to enhanced vagal tone and pronounced hypoxia. Therapeutic options in obese patients with obstructive sleep apnea include consequent weight loss and nasal continuous positive airway pressure (CPAP) ventilation as the therapy of first choice. Weight reduction and effective nasal CPAP therapy significantly decrease cardiovascular morbidity and mortality and eliminate sleep-related bradyarrhythmias in 80-90% of patients obviating the need for pacemaker implantation in these patients.

Arrhythmias, Cardiac↗

[Influence of the beta-blocker nebivolol on left ventricular function in patients with chronic heart failure].

BACKGROUND: Sympathetic activity is a significant predictor of a poor prognosis in heart failure. beta-blockers such as carvedilol, metoprolol or bisoprolol have been shown to improve the prognosis of patients with heart failure. AIM: This pilot study examined the tolerability and effect of the new beta-blocker nebivolol on left ventricular ejection fraction in patients with chronic heart failure. PATIENTS AND METHODS: Twelve patients with an ejection fraction of 13-39% were included into a double-blind, placebo-controlled, randomized trial with nebivolol on top of a standard therapy. Exercise time, heart rate, left ventricular function, and tolerability were examined at baseline and after 3 months of orally administered nebivolol (2.5 mg and 5 mg, n = 6) or placebo (n = 6). RESULTS: Nebivolol was well tolerated and NYHA stage improved in four patients. Heart rate decreased while the maximal exercise duration and performance remained stable. Left ventricular function improved (ejection fraction: increase from 29.8 +/- 10.66% to 41.2 +/- 10.53%; p = 0.007) after 12 weeks of nebivolol whereas placebo did not improve cardiac function statistically significant. Left ventricular endsystolic diameter decreased from 56.5 +/- 9.40 mm to 50.2 +/- 9.43 mm in the nebivolol group (p < or = 0.02). CONCLUSION: These data indicate that nebivolol might improve cardiac function in patients with chronic heart failure.

Administration, Oral↗

[Rehabilitation after anterior cruciate ligament reconstruction].

The "Frankfurt rehabilitation regimen" following anterior cruciate ligament reconstruction is presented. ACL rehabilitation is discussed in the light of knowledge on knee biomechanics and proprioception as well as clinical results of reconstruction. Special emphasis is given to exercise therapy.

Anterior Cruciate Ligament↗

[Botulinum toxin A in the treatment of infantile cerebral palsy. Taking into account multilevel, integrated treatment].

Botulinum toxin A represents a significant development in the management of children and adolescents with spastic cerebral palsy. Prerequisites for an adequate result are a correct indication, an exact injection technique and an intensive post-treatment programme. Spastic muscle overactivity and the constant tendency of the involved muscles to shorten with growth cannot be treated by only one method. Therefore a multilevel approach and an integrated treatment schedule including plaster of Paris, orthoses and physiotherapy are currently the best ways to modify the disease process. The inclusion of objective clinical documentation techniques combined with 3-D instrumented gait analysis allows the determination of the indications more exactly and for monitoring the post-treatment results. If started early and correctly, this integrated management approach has the potential to modify the natural history of the disorder, and to reduce the frequency of later surgery.

Adult↗