[Family caregivers and hereditary muscular disorders: association between burden, quality of life and mental health]. / Les aidants familiaux des personnes myopathes: liaisons entre charge de soins, qualité de vie et santé mentale.

OBJECTIVE: The analysis of caregiver difficulties seems important to define the life of the patient. The aim of the study was to evaluate the burden of perceived care, health-related quality of life, and mental health of family caregivers who care for patients with hereditary neuromuscular disease. MATERIALS AND METHOD: Fifty-nine family caregivers were investigated in the course of multidisciplinary consultations in Reims, France, between April 2002 and February 2005. Burden of perceived care, mental functioning and mental health were measured by answers to an burden interview instrument (Zarit Burden Inventory, ZBI), a health-related quality of life questionnaire (the SF-36 and General Health Questionnaire-12 items [GHQ-12]), and an instrument quantifying anxiety and depression (Hospital Anxiety and Depression scale [HAD]). The domain scores of the SF-36 were compared with those of the general population. Non-parametric correlations between scores were calculated. RESULTS: Thirty-five parents, 20 spouses, a sister, a grandmother and two friends cared for patients with hereditary myopathy. The average age of caregivers was 50+/-11 years; 81% were women. Ten percent of the caregivers had above-normal scores on the HAD scale, One-third perceived a significant reduction in health-related quality of life, and more half expressed a moderate or important burden according to their answers on the ZBI. Depression occurred in a great proportion of the caregivers, and they showed reduced health-related quality of life as compared with the general population. CONCLUSION: Taking into account these results, it appears useful to set up a specific support for family caregivers of patients with hereditary myopathy.

[Outcome of early fitting by silicone liner with lock system for dysvascular below-knee amputation: assessment of walking abilities by a specific questionnaire]. / Appareillage précoce par manchon silicone avec attache terminale après amputation vasculaire sous le genou: évaluation par score de marche du devenir fonctionnel.

PURPOSE: To assess by a specific questionnaire the functional outcome of patients with below-the-knee amputation after early prosthetic fitting by the ICEROSS silicone liner, which had demonstrated improvement of stump healing and length of hospital stay. PATIENTS AND METHODS: In this retrospective study, walking ability was assessed by a specific score resulting from answers on a questionnaire. The outcome variables were walking inside and outside, transfer from sitting, climbing stairs, and use of walking aids. Following amputation, the ICEROSS system was used for compression therapy, then for temporary prosthesis. The questionnaire was administered at the fitting stabilized state. RESULTS: Twenty-nine of 51 patients who underwent trans-tibial amputation were included: 5 women (mean age 72.8+/-4.1 years) and 24 men (mean age: 69+/-7.4 years). The mean total score was 14.5/20 (good functional outcome) for the 22 unilateral amputees and 7.2/20 (intermediate result) for the seven bilateral amputees. Previous studies concerning functional outcome with other contact casts (without a silicon liner with a bolt) had shown similar results. CONCLUSION: Despite its beneficial initial effect, early fitting by the ICEROSS system did not improve walking ability at the steady functional state, which is more linked to advanced age and comorbidities.

Bioenergetic comparison of a new energy-storing foot and SACH foot in traumatic below-knee vascular amputations.

In this study, the metabolic performances of a new energy-storing foot (Proteor) and of the solid-ankle cushion heel (SACH) are compared. Twelve patients with traumatic below-knee amputations (mean age: 50.0 +/- 19.9 years) and 12 patients with vascular below-knee amputations (mean age: 73 +/- 7 years) were studied. Oxygen uptake (VO2) was measured in all the subjects on a walkway at a self-selected velocity; only the subjects with traumatic amputation were tested on a level treadmill (progressive speed: 2.4-4 and 6 km/h), and then in two randomized trials: incline (+5%) and decline walking treadmill test at 4 km/h. Vascular explorations were done in the vascular patients: distal pressure measurements, pulse plethysmography, transcutaneous oxygen tension. Free walking was improved in subjects with traumatic amputation using the energy-storing foot (+6%), with a better bioenergetic efficiency (0.24 +/- 0.4mL/kg.m vs 0.22 +/- 0.04mL/kg.m). However, in subjects with vascular amputation, this foot did not produce an increased free velocity nor an improved energy cost. During the level treadmill test, the traumatic amputee subjects showed a decrease of energy expenditure with the new prosthetic foot, more significant at sufficient speed (4 km/h): 17.00 +/- 3.42 vs 14.67 +/- 2.05 mL/kg/min (p < .05). The same effect is shown during the incline (19.31 +/- 2.80 vs 16.79 +/- 2.32 mL/kg/min-p < .02) and decline walking tests (14.13 +/- 3.64 vs 11.81 +/- 1.54mL/kg/min-p < .02). There is no significant difference in cardiocirculatory effects between the two types of prosthetic foot. Despite a lower velocity, the subjects with vascular amputation exceed 70% of the maximal heart rate, with the cardiocirculatory factor being the main cause of walking restriction. The energy-storing foot should be reserved for active and fast walkers, whereas the SACH foot seems more suitable for elderly patients with amputation with a slow walk.

Muscle metabolism changes with training in the nonamputated limb after vascular amputation: interest of phosphorus 31 NMR spectroscopy.

OBJECTIVE: To determine by 31P nuclear magnetic resonance (NMR) spectroscopy the efficacy of training in improving aerobic metabolism of calf muscle in nonamputated limb after recent vascular amputation; to assess the possible associated microcirculatory changes; and to evaluate the need for noninvasive monitoring techniques during training in the nonamputated limb after recent vascular amputation. DESIGN: Prospective study, before and after training. Subjects served as their own controls and were compared with a control group. SETTING: Rehabilitation center of a university hospital. PATIENTS: Ten unilateral vascular amputated patients were included with ankle systolic index between 0.5 and 0.8 in the nonamputated limb, and 10 control subjects without cardiovascular disease or risk factors of atherosclerosis with ankle systolic index of >.95. INTERVENTION: Walking with prosthesis at self-selected velocity over increasing walking distance, arm training at a workload of 60% of a maximal arm test, and analytical exercises of the nonamputated leg (dynamic contractions against low resistance). Subjects received training as inpatients, 5 days a week. MAIN OUTCOME MEASURES: Before and after training, ankle systolic index, forefoot transcutaneous oxygen tension (TcPO2) and veno-arteriolar reflex, and digital plethysmography of the second toe with reactive hyperemia test were studied. Changes in calf muscle pH, phosphocreatine (PCr), and inorganic phosphate (Pi) were measured by 31P NMR spectroscopy at rest and during a plantar flexion-type incremental protocol. RESULTS: There was no significant difference in ankle systolic index (.63 +/- .10 vs .64 + .07) or in TcPO2 (42 +/- 11 vs 44 +/- 10mmHg), and there was reappearance of veno-arteriolar reflex in 3 cases, of a plethysmographic signal in 2 cases, and of the positivity of the reactive hyperemia test in 3 cases. No differences were found with 31P NMR spectroscopy at rest before and after training. At the same workload (1 watt) the difference of the ratio (PCr/(PCr + Pi)) of rest to effort (PCr depletion) was significantly increased in the amputated patients (.423 +/- .159 vs .145 +/- .058; p < .01). This difference of ratio was lower after training (.360 +/- .158 vs .423 +/- .159; p < .05). The pH was less acid between the two periods. CONCLUSION: Vascular monitoring with systolic index and TcPO2 is necessary to follow and to prevent serious ischemia of the nonamputated limb. Claudication is often not detected because of early exhaustion during walking. Training after recent vascular amputation improves the skeletal muscle oxidative capacity.

Healing of open stump wounds after vascular below-knee amputation: plaster cast socket with silicone sleeve versus elastic compression.

OBJECTIVE: To assess the effect of a plaster cast socket on the healing of open wounds and on temporary prosthesis fitting after below-knee amputation because of arterial occlusive disease. DESIGN: Randomized controlled trial. SETTING: Rehabilitation center, university hospital. PATIENTS: All included patients had undergone recent (in the previous 3 months) below-knee amputation because of arterial disease and initially had an open stump. Patients were randomly assigned to two groups of 28 subjects each. The sizes of the amputation scars were 8 to 24 cm2. Ischemia of the stump was eliminated as a probable cause of delayed wound healing by the inclusion criterion of transcutaneous oxygen tension (TcPO2) of >35 mmHg. The average age in group I (the experimental group) was 65.2 +/- 12.4 (SD) years and in group II (the control group) 66.8 +/- 10.8 years (not significant). INTERVENTION: A plaster cast (supracondylar-type) socket was fitted on the stumps of group I patients, interposed with a silicone sleeve. The patients were gradually trained to wear this cast for up to 5 hours a day. They were provided with elastic compression bandages for the remainder of the time. Patients in group II wore elastic compression bandages, which were only removed for dressing changes. MAIN OUTCOME MEASURES: Time required for stump healing, length of time between amputation and ability to walk wearing a contact socket, and length of hospital stay. RESULTS: Group I had a quicker average healing time (71.2 +/- 31.7 [SD] days compared to the control group's 96.8 +/- 54.9 days) and a shorter average length of hospital stay (99.8 +/- 22.4 days compared to the control group's 129.9 +/- 48.3 days). CONCLUSION: Use of a plaster cast socket leads to more rapid healing of the open stump and to a shorter hospitalization. If there is no stump ischemia, this plaster cast technique is safe.

Expression of the multidrug resistance glycoprotein 170 in the peripheral blood lymphocytes of rheumatoid arthritis patients. The percentage of lymphocytes expressing glycoprotein 170 is increased in patients treated with prednisolone.

The objective was to evaluate the expression of the multidrug resistance P-glycoprotein (P-gp) in peripheral blood lymphocytes (PBL) of patients with rheumatoid arthritis (RA). PBL from 68 RA patients and 44 controls were evaluated. RA patients had a mean disease duration of 10.7 yr, with a mean number of past resistances to DMARDs of 0.82, and were treated with NSAIDs (n = 34), DMARDs (n = 25) and prednisolone (n = 40). Fluorescence flow cytometry was used to assess P-gp membrane expression on PBL. In the RA group, the percentage of PBL expressing P-gp was higher in patients treated with prednisolone than in other patients [mean +/- S.D.: 10.7 +/- 15.8% vs 3.3 +/- 7.6%, P < 0.03, Student] and was not related to other therapies, age, sex, RA duration, number of past resistances to DMARDs, activity, ESR, CRP. The percentage of PBL expressing P-gp did not differ in RA and control groups, but was higher in the prednisolone-treated RA patients than in controls. Prednisolone could induce a rise in the percentage of PBL expressing P-gp. On the contrary, patients with a high percentage of PBL expressing P-gp could be more resistant to DMARDs and need prednisolone earlier. Further studies are needed to address this question and to evaluate the potential implication of P-gp in drug resistance in RA.

Effects of low-frequency electrical stimulation of quadriceps and calf muscles in patients with chronic heart failure.

PURPOSE: The aim of this preliminary study was to evaluate the effects of low-frequency electrical stimulation of quadriceps and calf muscles on global exercise capacities, skeletal muscle metabolism, calf muscle volume, and cardiac output in patients with chronic heart failure. METHODS: Fourteen patients with chronic heart failure (mean age of 56.4 years +/- 9.1 SD; mean radionuclide left ventricular ejection fraction of 22.3% +/- 8.8 SD) underwent 5 weeks (1 hour per day, 5 days per week) of low-frequency electrical stimulation of quadriceps and calf muscles. RESULTS: Low-frequency electrical stimulation was well tolerated. Exercise capacity and the calf muscles volumes increased significantly after rehabilitation in comparison with prior rehabilitation (the peak oxygen consumption increased from 17.2 mL/(kgmin) +/- 5.3 SD to 19.6 mL/(kgmin) +/- 5.9 SD; the anaerobic threshold increased from 12.3 mL/(kgmin) +/- 3.2 SD to 15.2 mL/(kgmin) +/- 3.3 SD; the 6-minute walking test increased from 419 m +/- 122 SD to 459 m +/- 114.3 SD; the gastrocnemius volume increased from 259.4 cm3 +/- 58 SD to 273.4 cm3 +/- 74 SD, and the soleus volume increased from 319 cm3 +/- 42.9 SD to 338 cm3 +/- 52.5 SD). The New York Heart Association class was improved after rehabilitation. The P-31 nuclear magnetic resonance spectroscopy of gastrocnemius muscle data were not significantly modified after rehabilitation, thereby inferring that no significant improvement of the muscle metabolism occurred. These data reinforce the hypothesis of an increased muscle mass during stimulation. It is noteworthy that the electrical stimulation did not increase cardiac output at any stage; an enormous asset in favor of this mode of rehabilitation. CONCLUSION: These results suggest that low-frequency muscular electrical stimulation is well tolerated, induces an increased exercise capacity in patients with chronic heart failure, without an undesirable increase in cardiac output.