Physical Exercise in Patients with Fabry Disease - a Pilot Study.

The aim of this study was to assess the extent of exercise intolerance in Fabry disease (FD) patients and to report individual effects of physical exercise. Exercise capacity and strength of 14 patients (mean age 46 years, 6 females) were determined using cycle ergometry and isokinetic measurements. Patients performed a strength/circuit exercise training protocol for 12 months. The mean relative maximum performance of the group was low at baseline and increased by 12.1% (baseline: 1.9 [0.9-3.4] W·kg-1vs. re-test: 2.1 [1.1-3.8] W·kg-1; p=0.035) during the study. Patients' mean baseline maximum performance blood lactate of 5.4 [1.3-9.9] mmol·L-1 increased to a mean of 7.2 (2.4-10.2) mmol·L-1 (p=0.038). Mean strength of the lower limbs (left/right extensors and flexors, total work of 5 sets) changed from 2269 (1017-2913) kg·m2·s - 2 to 2325 (1359-3107) kg·m2·s-2 (not significant). Patients reported increased well-being, daily activity and reduced fatigue during the study. Our results indicate that exercise intolerance in FD patients often results from physical inactivity. FD patients may perform exercise training to improve exercise capacity and muscle strength. Future studies will address the clinical benefits of exercise in FD.

The laser-pointer assisted angle reproduction test for evaluation of proprioceptive shoulder function in patients with instability.

INTRODUCTION: Over the last decade, proprioceptive function gained increasing attention in joint disorders such as instability of the shoulder. Common tests for evaluation of proprioception are limited by their complexity and high technical demands. Thus, they are hardly applicable during daily routine. Our hypothesis was that the simplified "laser-pointer assisted angle reproduction test" (LP-ART) presented here allows for clinically feasible assessment of proprioceptive shoulder function. METHODS: Active angle reproduction capability as an aspect of sensorimotor function was evaluated with the new method in patients with shoulder instability (n = 24) and healthy controls (n = 24). 15 patients had traumatic, 9 non-traumatic anterior instability (6 bilateral), 17 were treated surgically, 13 non-operatively. Tests were performed in flexion and abduction in different angles (55°, 90°, 125°) in randomized order. RESULTS: Angle reproduction capability was worst below shoulder level (55°) in all groups. Best results were achieved at shoulder level (90°). Healthy controls showed overall better results than patients with instability. Patients after surgical stabilization had better results in 55° and 90° abduction compared to instability patients before surgery. CONCLUSIONS: The new LP-ART presented here is a technically simple, yet effective instrument for evaluation of the proprioceptive function of the shoulder. In contrast to former test setups it is feasible in daily routine. Compared to healthy controls, patients with unstable shoulder joints show significant proprioceptive disorders that can be quantified by the LP-ART.

Participation in sports and physical activity of haemophilia patients.

Modern therapy options offer haemophiliacs more and more possibilities for an active participation in sports. The purpose of the present study was to investigate the attitude of these patients towards sports, their participation in school and leisure time sports activities, and differences between juveniles and adults. We investigated 44 children and adolescents (aged from 4 to 16 years) and 27 adults (aged from 18 to 72 years) with haemophilia by means of a questionnaire. 79.6% of the juvenile patients participated always or almost always in school sports, while this percentage was significantly (P < 0.05) lower in the former school time of the adults (37%). Sports play an important or very important role in leisure time activities for 75% of the adolescent and 55.5% of the adult haemophiliacs (P < 0.05). Bleeding complications occurred in 17.6% of all patients; there was no correlation with any particular type of sports. There were only slight differences between both groups, regarding their motivation to participate in sports activities. The main reasons involved social aspects and having fun. The results show that the modern therapy of haemophilia probably leads to a more positive attitude towards sports and to a wider spectrum of practised sports. This, however, may be associated with an increasing potential of health risks, which require a high level of sports medical care.

Muscle strength in patients with unicompartmental arthroplasty.

OBJECTIVE: The aim of the current study was to evaluate the isokinetic strength of the knee in patients with unicondylar prostheses, to compare these results with healthy control subjects of the same age, and to correlate these results with clinical scores. DESIGN: Seventeen patients were examined an average of 21.5 mos after surgery. Clinical examination was done using the Hospital for Special Surgery, Knee Society, and patellar scores and a visual analog scale for pain. Quality of life was assessed by the Short Form 36 Health Questionnaire. Isokinetic evaluation of knee extensor and flexor muscles was done using a Cybex 6000 dynamometer at angular velocities of 60 and 180 degrees/sec. Eleven healthy subjects of comparable age served as a control group. RESULTS: Clinical results differed significantly in all categories. Quality of life differed only in the items of physical functioning, role limitation because of physical problems, and bodily pain. Isokinetic strength in patients showed a loss of torque of approximately 30% in extension and flexion at 60 and 180 degrees/sec compared with the control group. The flexion and extension ratio and the angles of maximum torque did not differ between the groups. CONCLUSION: In comparison with healthy control subjects, persons with an implanted unilateral sledge prosthesis have strength deficits in extension and flexion. A deficit of the extensor muscles, as it has been described previously for patients with a total knee arthroplasty, could not be found.

Decoupling of intracellular calcium signaling in granulocytes after exhaustive exercise.

There is growing evidence that exhaustive exercise can induce a suppression of the innate immune functions. Most studies so far describe exercise induced changes in cell counts or functional responses while information regarding intracellular signal transduction parameters is lacking. Therefore in the present study we investigated in granulocytes the regulation of intracellular calcium ([Ca2+]i) which is an important intracellular second messenger. Healthy volunteers underwent a treadmill exercise test at 80% of their maximal oxygen uptake until exhaustion. Granulocytes were separated before and 1 hour after the test. [Ca2+]i was analyzed spectrophotometrically using the Ca2+ sensitive fluorescent dye Fura-2, while the oxidative burst and phagocytosis were measured by flow cytometry. While resting [Ca2+]i levels were unchanged, the Ca2+ transient induced N-formyl-methionyl-leucyl phenylalanine (fMLP) and platelet activating factor (PAF) were enhanced 1 hour after the test compared to pre-exercise values although fMLP receptor density did not change. In contrast, oxidative burst and phagocytosis evoked by fMLP and phorbol-myristate-acetate (PMA) were decreased after exercise. Together, our data support the view that exhaustive exercise affects regulation of Ca2+ signaling in granulocytes. The potentiation of Ca2+ signals is not accompanied by an enhancement of cellular functional parameters suggesting a blockade in intracellular signalling pathways.

Creatine loading and resting skeletal muscle phosphocreatine flux: a saturation-transfer NMR study.

31P saturation-transfer nuclear magnetic resonance spectroscopy was used to study skeletal muscle phosphocreatine (PCr) flux in healthy male volunteers. Data analysis included consideration of effects from incomplete saturation and radiofrequency spillover. Spectra were recorded from the resting gastrocnemius muscle before and after 6 days of creatine monohydrate (Cr-H2O) intake (20 g/day). Parallel to an improved muscle performance during maximal intermittent exercise following Cr-H2O supplementation, the concentration of PCr increased (P=0.01) by 23% (34.9+/-2.8 mmol/l vs. 28.6+/-2.7 mmol/l), whereas other metabolites were unaffected (inorganic phosphate: 4.3+/-1.4 mmol/l, free intracellular Mg(2+): 1.1+/-0.7 mmol/l, cytosolic pH: 7.04+/-0.02). Forward and reverse fluxes through the creatine kinase (CK) reaction did not change significantly from their baseline levels (v(for): 11.8+/-5.4 mmol/l per second vs. 15.3+/-6.8 mmol/l per second, (v(rev): 9.5+/-3.4 mmol/l per second vs. 10.9+/-3.7 mmol/l per second). The rate of PCr resynthesis in resting muscle is not limited by the CK reaction, which is near equilibrium. Consequently, the post-load increase in total creatine has no effect on the unidirectional CK reaction rates.

[Effect of external stabilization aids of the upper ankle joint on sports-specific performance with reference to isokinetic strength measurements]. / Einfluss von äusseren Stabilisierungshilfen des oberen Sprunggelenks auf sportspezifische Fähigkeiten unter Berücksichtigung isokinetischer Kraftmessungen.

Two questions were investigated: Do ankle devices have any influence on sports performance? Are there any differences between the devices? 31 participants could be included. The average age was 24.5 (+/- 4.1). The participants exercised 5.8 hours/week. We tested 41 ankles without any previous injury. With the Cybex 6000 four parameters were evaluated. Torque maximum for the plantarflexion, range of motion, work in Joule. The ankles were tested with three different ortheses, ankle taping and without any device. The Friedman Test was used to evaluate differences between the five test conditions. All measurements showed significant worse results for the devices and taping. The protective effect of ankle devices is well known. In this investigation we could show a restriction of the performance. Thus it should be decided individually, if an ankle device is useful.

Alterations in intracellular calcium signaling of lymphocytes after exhaustive exercise.

PURPOSE: Exhaustive exercise is accompanied by pronounced quantitative changes in leukocytes. Whereas most studies on lymphocytes have concentrated on their proliferative responses or cytokine secretion, not much is known about exercise-induced changes in intracellular signal transduction processes. In lymphocytes, the concentration of intracellular free calcium ([Ca(2+)](i)) is an important intracellular second messenger linking extracellular stimuli to cellular responses. The aim of the present study was to examine the effect of exhaustive exercise on the calcium homeostasis of lymphocytes. METHODS: Healthy volunteers underwent treadmill exercise at 80% of their maximal oxygen uptake until exhaustion. Blood samples were taken before, immediately after, 1 h after, and 1 d after the test. Lymphocyte subsets were analyzed by flow cytometry; isolation of lymphocytes was performed by density gradient centrifugation. [Ca(2+)](i) was measured using the calcium-sensitive fluorescent dye Fura-2. RESULTS: Compared with preexercise conditions, basal [Ca(2+)](i) was increased immediately after exercise, whereas there was no change after 1 h or 1 d. The anti-CD3- and phytohemagglutinin-induced Ca(2+) responses demonstrated a bivalent pattern. Immediately after exercise, Ca(2+) transients were impaired, whereas 1 h after and 1 d after the test, the Ca(2+) responses were increased. In contrast, the Ca(2+) responses induced by thapsigargin were not different at any time interval. Lymphocyte subsets increased immediately after exercise, especially natural killer cells and CD8+ T cells, and decreased below preexercise levels after 1 h. One day after exercise, cell counts were not different from preexercise levels. CONCLUSIONS: Taken together, this novel approach demonstrates that exhaustive exercise has a profound influence on intracellular calcium signaling of lymphocytes. These effects may explain changes in lymphocyte function that have previously been reported.

Interdependence of clinical and isokinetic results after bicondylar knee prostheses with special emphasis on quality of life results.

Nineteen patients were examined at an average follow-up of 2 years after total knee joint replacement without patella resurfacing. They were compared with 22 healthy subjects of the same age. Using the Hospital for Special Surgery (HSS) score the operated knee joints scored an average of 77 points, the contralateral side scored 87 points and the control group 97 points. In the SF-36 health questionnaire the patients showed highly significant deviations. Isokinetic measurements revealed a clear loss of isokinetic strength of more than 50% on average in flexion as in extension when compared to the control group and there were considerable asymmetries between the operated and the contralateral legs.

Proprioceptive function in knees with and without total knee arthroplasty.

The goal of this study was to evaluate the differences in angle reproduction capability after nonconstrained posterior cruciate ligament retaining total knee arthroplasty after a follow-up time of 63.9 months compared with the healthy contralateral leg and a control group. In 28 total knee arthroplasty patients (mean age, 65.7 yr) and 25 control subjects (mean age, 55.7 yr), 16 measurements were made between 0 degrees and 90 degrees in 30 degree steps. The leg was positioned by the examiner and then relaxed; afterward, the subject was asked to reproduce the original position. Each measurement was made with the patient or control being blindfolded and not blindfolded to assess the influence of visual control. We found significant differences in total knee arthroplasty patients in contrast to healthy subjects. Without visual control, the mean deviation of the total knee arthroplasty group starting with a 0 degree angle was 7.7+/-5.9 degrees and 4.6+/-4.7 degrees for the healthy subjects. With visual control, the mean deviation in the patient group was 11+/-7.5 degrees, and in the control group, it was 7.2+/-5.0 degrees. Total knee arthroplasty patients did not show significant differences between the operated on and the contralateral knee. Also, in the total knee arthroplasty group, significant differences could not be found comparing reproduction with and without visual control and comparing both starting positions. In the control group, significant differences could be found comparing visual and nonvisual control in the 60 degree angle. The comparison between the 30 and 60 degree repositioning from both 0 and 90 degree starting positions showed a significant difference in the 60 degree angle. In summary, reduced proprioceptive capabilities are present after knee arthroplasty in both the operated on and the contralateral leg in our study group. Postoperative complaints can perhaps be explained by a loss of proprioceptive capabilities, especially at the 60 degree angle. The operative technique should involve special care of ligaments and muscles to preserve stability and receptors as well. Rehabilitation should pay more attention to the remaining receptors.

[Long-term effects of ankle joint orthoses on sports specific capability in handball]. / Langfristige Auswirkungen von Sprunggelenksorthesen auf sportspezifische Fertigkeiten im Handball.

UNLABELLED: We documented the effect on sports specific capabilities of long lasting wear of ankle braces in 14 handball players. For a period of 4 months half of the team performed all athletic activities with an ankle brace. The other players served as control performing the same athletic activities without wearing a brace. For documentation of the coordinative capabilities a special jump-test with a COMET-system was used. This test was performed after a standardised warm-up at the beginning and at the end of the 4 months observation period with and without an ankle brace. The results showed that wearing the used ankle brace for a period of 4 months did not lead to a negative effect on jumping capabilities. The brace group showed even an increased ability in the used test. This could be documented in short as well as long term effects. CONCLUSION: Long wear of ankle braces do not lead to negative side-effects in sports specific capabilities that are represented by the performed jump-test.

[Proprioceptive abilities of patients with post-traumatic instability of the glenohumeral joint]. / Propriozeptive Fähigkeiten von Patienten mit posttraumatischer Instabilität des glenohumeralen Gelenkes.

PURPOSE: The purpose of this study was to evaluate joint position sense (JPS) in patients with posttraumatic glenohumeral instability. MATERIALS AND METHODS: In 28 patients with posttraumatic instability and in a matched control group of 30 subjects proprioception capability was evaluated. For documentation of proprioception an angle reproduction test (ART) was performed with which joint positions sense (JPS) was measured for abduction, flexion, and rotation in three angles each. RESULTS: In both groups there was a significant better JPS with visual control than without. In contrast to the control group the patients were not able to increase angle reproduction capability without visual control when comparing positions below shoulder level with positions at or above shoulder level. When comparing the patients to the controls there were differences in most of the ARTs with worse results in the patient group. These differences were significant in 150 degrees flexion with and without visual control, in 150 degrees abduction without and in 100 degrees abduction with visual control. For rotation there were trends for almost all joint positions, however, the differences were significant only in the -45 position. When comparing the noninjured contralateral shoulder of the patients with the control group, there still were differences. Again these were not in all joint positions significant, but significant worse JPS could be demonstrated in 150 degrees abduction without visual control, 50 degrees flexion without visual control, -45 degrees rotation without and 0 degrees rotation with visual control. CONCLUSIONS: A proprioceptive deficit can be documented in patients with posttraumatic glenohumeral instability. This may be one reason for permanent instability. The contralateral joint also shows reduction in joint position sense. For consecutive treatment as well as for rehabilitation both shoulder joint should be addressed.

[Joint stress in inline skating--a biomechanical study]. / Gelenkbelastung beim Inline-Skating--eine biomechanische Untersuchung.

UNLABELLED: In 8 male subjects we undertook acceleration measurements under standardised conditions at the tibia, the hip and the head. The measurements were performed on a normal flat street as well as on a bicycle route with velocities of 15 km/h, 20 km/h and 25 km/h. Additionally we documented the range of motion at the knee joint. Maximal positive acceleration at the head sensor was 10.75 m/s2 at a velocity of 25 km/h on the bicycle route. At the hip the highest measured value was documented with 15.10 m/s2 at 25 km/h also on the bicycle route. Die maximal tibia acceleration was 55.8 m/s2 at 25 km/h again on the bicycle surface. The amplitude averaged signals showed the highest suppression between tibia and hip. CONCLUSION: Compared to other athletic activities acceleration and joint load in in line-skating is only low.

[Incidence of injuries in parachuting]. / Verletzungsinzidenz beim Fallschirmsport.

During the German championships in parachuting 78 paratroopers were asked about acute injuries and chronic pain using a questionnaire. A total amount of 131 injuries was described. These were evaluated in terms of dimension and localisation. Upper and lower parts of the body were injured with a comparable frequency. Bruises (42%), fractures (19%), sprainings (16%) and dislocations (10%) were most often described. The overall injury rate according to the total number of descents (0.09%) was lower than that reported by previous literature. Therefore it can be concluded that parachuting for experienced jumpers is less dangerous than assumed until today.

[The influence of knee bandages on sport specific capabilities in healthy volunteers and patients with rupture of the anterior cruciate ligament]. / Der Einfluss von Kniebandagen auf sportspezifische Fertigkeiten bei gesunden Probanden und Patienten mit Rupturen des vorderen Kreuzbandes.

In 15 male healthy volunteers and 10 male patients with ACL-deficiency sports specific capabilities were evaluated with a 9-3-6-3-9-run and a single-leg jumping test. Each subject was tested with and without different knee bandages. We evaluated the Patelladyn-, the Kasseler-, the Super-Genuplus-, and the Genuhit-bandage. Additionally a subjective assessment of each bandage was performed by the volunteers. There were no significant difference performing the 9-3-6-3-9-run between all of the bandages in the control-group. Comparing the injured group with the control there were significant differences. All bandages led to improvement without reaching the running time, which was used by the controls. Performing the single leg jumping test there were also no differences with the different bandages in the healthy control group. However, after applying a bandage in the injured subjects, the time improved significantly. In the subjective assessment the Patelladyn-bandage scored best.

Injury pattern and acceptance of passive and active injury prophylaxis for inline skating.

In a field study the injury pattern as well as the active and passive injury prophylaxis of 1036 inline skaters were evaluated. Of them, 60% had already been injured. Every 124 h an injury occurred during inline skating. While 61% of the injuries affected only soft tissue, 31% were joint distorsions, and 8% were fractures. Altogether 37% of the 626 injured skaters required medical treatment. The upper extremities are especially at risk: 78% of the fractures and 48% of the distorsions affected the fingers, wrist, forearm or elbow. It is obvious that the inline skaters' passive prophylaxis measures are still unsatisfactory. Only 17% of the questioned used complete protection, whereas 16% were wearing no protective gear at all. Of the rest, 49% were skating with wrist guards, 66% were using knee pads, and 31% elbow pads. A high percentage of injuries is due to the lack of basic knowledge and techniques. The survey revealed that only 24% can stop immediately. While 51% had only slight problems with braking, 16% admitted having great difficulties, and 7% were not able to brake at all. These numbers reveal that there are still large deficits regarding braking techniques and education on the risks of injury. Therefore, athletes who use this equipment for the first time should learn the basic techniques of skating, braking and falling with the help of a qualified instructor. Most of all those questioned wanted to have more information, and half would be interested in special educational programmes.

[Effects of a proprioceptive training program on sensorimotor capacities of the lower extremity in patients with anterior cruciate ligament instability]. / Auswirkungen eines propriozeptiven Trainingsprogramms auf die sensomotorischen Fähigkeiten der unteren Extremität bei Patienten mit einer vorderen Kreuzbandinstabilität.

Sensomotory function after ACL-reconstruction was tested prior and after a proprioceptive rehabilitation program with the Kinesthetic Ability Trainer (KAT 2000) which documented the static balance index (SBI). A control group consisting of healthy subjects was also tested two times with a time interval of six weeks. The results showed an adaptation effect to the testing device in the control group with an 17.12% increase of the SBI. There were no differences between the dominant and non dominant extremity. The active sensomotory function in the healthy knee of the operated patients showed no difference to the control group. Proprioceptive capabilities of the acl-reconstructed knee joints was significant reduced 6.42 weeks after surgery. A special proprioceptive rehabilitation program for six weeks significantly reduced this deficit. In the patient group sensomotory function of both knee joints could be increased to a level higher than in the control group. In the operated extremity the static balance index increased 27.75% and in the non injured extremity the SBI increased 16.88%.

[Automobile head supports--adjustment possibilities and utilization. Results of a field study]. / PKW-Kopfstützen--Einstellungsmöglichkeiten und deren Ausnutzung. Ergebnisse einer Feldstudie.

An important detail referring to the whiplash syndrome is the relationship between the position of the head restraint and the head, because the head restraint protects the head in case of a rear-end accident. This relationship was evaluated in a representative study in 601 nonselected volunteers. A horizontal distance between the head and head restraint of maximal 9 cm was present in 69.6%. An optimal distance of 0 cm was only found in 7.4%. Just 50% of the adjustment distance was used by the car drivers. Even in completely distracted adjustments in 50% a deficit of more than 8 cm, and in 20.9% a deficit of even more than 12 cm was present. These results show that passive protection, on the one hand, is not guaranteed because of the lack of proper height adjustment. On the other, the volunteers did not use the best adjustment each time.

[Proprioceptive capacities of the knee joint with and without endoprosthesis]. / Propriozeptive Fähigkeiten des Kniegelenkes mit und ohne Endoprothese.

PURPOSE OF THE STUDY: To compare the proprioceptive abilities of total knee arthroplasty patients and a control group. METHOD: In a knee arthroplasty patient group of 28 and in 25 healthy volunteers the proprioceptive function was examined. To measure the proprioceptive function the motion analysis system with reflecting markers was chosen. In each patient 16 measurements from different starting points in different joint positions with and without visual controlling were performed. RESULTS: The results demonstrated significant differences between the patients and volunteers independent of the starting point, the joint position and the visual controlling. Also the comparison with the 30 and 60 degree position showed significant differences. In the patient group were not shown significant values comparing the starting position, the visual controlling and the comparison with the healthy leg. CLINICAL RELEVANCE: Knee arthroplasty conducts to loss of proprioception in the operated and healthy leg. Visual controlling can not solve these problems. The worst results were achieved in 60 degrees position. The starting position can not influence the results.