Joint hypermobility in children.
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Biomedical subjects
Publications and source records attributed to H A Bird.
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BACKGROUND: Together with visual information, awareness of limb position and movement is essential for limb coordination. A proprioceptive deficit has been demonstrated in a number of rheumatological disorders. There is a lack of a portable device for measuring hand proprioception in the field. METHODS: A compact portable device for measuring joint position sense in the metacarpophalangeal joint of the index finger of either hand was constructed. This device was manually operated and required the subject to match the position of the hidden finger with a surface-mounted silhouette. Reliability studies were performed over three consecutive days in 12 normal volunteers. RESULTS: Intrasubject variability [95% confidence interval (CI)] was 0.86 degrees (0.04-1.76) between days 1 and 2, and 1.23 degrees (1.04-3.50) between days 2 and 3. The intraclass correlation coefficient (95% CI) between all 3 days was 0.92 (0.85-0.96). Average proprioceptive error (95% CI) in the sample population was 5.72 degrees (1.23-10.2) over the 3 days. This value was 5.94 degrees , 5.79 degrees and 5.42 degrees on days 1, 2 and 3, respectively. No difference was found between sexes but dominant hands gave smaller errors (mean dominant error 5.11 degrees , mean non-dominant error 6.35 degrees ; t = -3.4, P = 0.002). CONCLUSIONS: This report describes a new portable device for measuring proprioception in the hand. Reproducibility was shown to be good on an individual and group basis. These results are promising and warrant larger age- and sex-related studies. The ease and portability of the device make it ideal for use in epidemiological studies of rheumatological disorders involving the hands, including joint hypermobility.
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OBJECTIVE: To compare the performance of the several different diagnostic criteria sets currently in use for polymyalgia rheumatica (PMR). METHODS: 213 patients attending eight rheumatological centres in eight different European countries were studied. All had recently been referred and were considered by the senior investigator at each centre, selected because of their experience in treatment of PMR, to have this condition. By use of a standard international proforma, the requisite diagnostic points in each criteria set were sought. Sensitivity for each criterion from each set was then calculated, as well as the sensitivity of each criteria set as a whole. RESULTS: Of four criteria sets compared, the Bird (1979) criteria performed best with a sensitivity of 99.5%, and the Hunder (1982) criteria second best, with sensitivity of 93.3%. These both performed significantly better than the two other criteria sets, though each of these was admittedly developed for rather specialised reasons. CONCLUSIONS: Although this study compares homogeneity, we suggest the Bird 1979 or Hunder 1982 criteria should be used whenever possible. Studies that have used alternative criteria may have less sensitivity in diagnosis.
OBJECTIVE: To develop a composite score for measurement of disease activity in polymyalgia rheumatica (PMR) and assess its internal and external validity. METHODS: A PMR activity score (AS) was designed and assessed for internal and external validity in two patient cohorts: 57 international patients evaluated primarily for development of the PMR-AS at baseline, weeks 4 and 24; and for validation, 24 Austrian patients assessed at baseline, week 4, and at a mean (SD) point of week 33.6 (24.5). The PMR-AS was calculated as: CRP (mg/dl)+VAS p (0-10)+VAS ph (0-10)+(MST (min)x0.1)+EUL (3-0); Cronbach's alpha was calculated. Factor analysis by linear regression was applied, and responses calculated on the basis of the PMR response criteria and the PMR-AS applied. PMR-AS values at different times were compared by paired t tests. RESULTS: Cronbach's alpha for the composite score was 0.91 and 0.88 in the two cohorts. Factor analysis showed that each single item contributed significantly to the total score and the relative weight of each item in both cohorts was equally distributed. Mean PMR-AS at baseline was 27.54 and 28.72, respectively, at week 4, 5.99 and 8.99, and at the final visit 5.35 and 5.92 (NS). PMR-AS values at baseline and at later visits were significantly different (p<0.0001). PMR-AS values <7 indicated low disease activity, 7-17 medium disease activity, and >17 high PMR activity. In a third control cohort the PMR-AS correlated highly with patient's global assessment, patient satisfaction, and ESR (p<0.001). CONCLUSION: The PMR-AS provides an easily applicable and valid tool for monitoring disease activity, and in combination with the PMR response criteria provides a better description of response.
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OBJECTIVE: To develop response criteria for polymyalgia rheumatica (PMR) for monitoring treatment and comparing alternative treatments regimens. METHODS: 76 patients, mean (SD) age 68.7 (7.7) years, were enrolled. Corticosteroids, and non-steroidal anti-inflammatory drugs (NSAIDs) were the only drugs allowed during the observation period. Erythrocyte sedimentation rate (ESR), C reactive protein (CRP), alpha(2) globulin, serum iron, pain, physician's global assessment (PGA), morning stiffness (MST), muscle tenderness (MT), myalgia, and the elevation of upper limbs (EUL) were determined regularly. The daily corticosteroid and NSAID doses as the corticosteroid response time were recorded. To ensure evaluation of an adequate number of patients (n = 57) week 24 was chosen for final analysis. RESULTS: ESR, CRP, alpha(2) globulin, pain, PGA, MST, myalgia, MT, and EUL showed significant improvement (p<0.0001) at week 24 compared with week 0. Multiple regression analysis showed that changes of ESR (p = 0.08), CRP (p = 0.41), alpha(2) globulin (p = 0.13), MST (p = 0.1), and MT (p = 0.07) were independent of pain, but myalgia (p<0.001) and EUL (p = 0.003) were pain dependent. Consequently, a core set of PMR response criteria, comprising ESR or CRP, pain, PGA, MST, and EUL was established. Assessment of treatment responses with this core set resulted in 90%, 70%, 50%, and 20% improvement in 31/57 (54%), 46/57 (81%), 51/57 (89%), and 54/57 (95%) of the patients, respectively. CONCLUSION: These PMR response criteria are a promising tool for better monitoring of disease activity and treatment in PMR. It is proposed that these criteria should be used in clinical trials in the near future to explore alternative treatment options for PMR.
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OBJECTIVE: To determine whether elastase-alpha1-proteinase inhibitor (E-alpha1PI) complex behaves as an acute phase reactant in inflammatory arthritis. METHODS: E-alpha1PI complex was measured in the plasma of 89 patients with different rheumatic diseases and 28 healthy controls. Correlations were sought between standard indices of disease activity as patients went into remission with the use of disease modifying antirheumatic drugs (DMARD). RESULTS: Compared with levels in 28 healthy controls, E-alpha1PI was significantly higher in 58 patients with rheumatoid arthritis (RA), 14 with ankylosing spondylitis, and 17 with noninflammatory rheumatoid disease. No significant correlations were found between E-alpha1PI and various laboratory and clinical variables of disease activity in RA before introduction of a DMARD. There was a slight decrease in E-alpha1PI over 24 weeks of treatment as other acute phase reactants fell significantly, although this did not reach significant levels. No correlations were found between intra-individual change of E-alpha1PI and different indices of disease activity from Week 0 to Week 24. CONCLUSION: E-alpha1PI complex levels, measured as an index of neutrophil activation, did not correlate with improvement in disease activity in inflammatory arthritis afforded by DMARD.
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The aim of this study was to determine the range of movement in gymnastic and dance populations. Sixty-five participants (41 females, 24 males; mean age 21.4 years) were assessed. The sample included dancers and gymnasts ranging from novice and club standard to international and professional status. Non-specialized physical education students acted as controls. Range of movement was measured at the shoulders, hips, lumbar spine and ankles using a Loebl hydrogoniometer, and inherent joint laxity was assessed using Beighton and coworkers' adaptation of the Carter and Wilkinson 9-point scale. The right and left sides of the body were assessed and measures of active and passive motion were recorded. A graded increase in laxity was observed from controls, through novice gymnasts, to dancers and finally international gymnasts. The greater laxity of females than males was also confirmed. Dancers and gymnasts had a greater passive range of movement in all joints, which was partly inherited and partly acquired. There was a large difference between their active and passive ranges, which appeared to render the joints unstable.
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Osteoarthritis is a multifactorial disease that is more common in the elderly than in younger individuals. Opinion is divided on whether it is a degenerative or inflammatory process. Most rheumatologists accept that the disease has an inflammatory component, particularly when it is complicated by crystal deposition. Nonsteroidal anti-inflammatory drugs (NSAIDs) are particularly toxic in the elderly. The geriatrician should use a more conservative approach to initial therapy, which should include patient education, physiotherapy and even the consideration of intra-articular steroid injections. If systemic drug therapy is still required, simple analgesics should usually be tried first. If NSAIDs are then required, they can be selected according to their chemical structure (which bears some relation to adverse effects) or half-life (which is more relevant to optimum prescribing). Propionic acid derivatives are well established and remain the NSAIDs of choice: agents with a short half-life, perhaps given in low dosages, are preferred. The use of topical formulations, which are more expensive, may avoid some adverse effects; however, drug effects are unlikely to remain truly localised. It may be prudent to provide gastroprotective therapy for some elderly patients. A full evaluation of the new generation of cyclooxygenase-2 inhibitors in osteoarthritis is still awaited.
The objective was to analyse whether the activity of the cytochrome P450 isoenzyme CYP3A4 is altered by disease activity of rheumatoid arthritis (RA). Urinary 6 beta-hydroxycortisol excretion, expressed as a fraction of the urinary creatinine output, was measured in 21 patients with RA treated with three different disease-modifying anti-rheumatic drugs (DMARDs) over 24 weeks. There were no correlations between urinary 6 beta-hydroxycortisol/creatinine (6 beta-OHC/Creat) ratio and measurements of disease activity such as plasma viscosity, Ritchie articular index and early morning stiffness. In addition, the three DMARDs sulphasalazine, sodium aurothiomalate and D-penicillamine, smoking and the intake of various CYP3A4 substrates had no consistent detectable effect on the 6 beta-OHC/Creat ratio. There is no evidence that the dosage of drugs metabolized by the CYP3A4 isoenzyme needs to be adjusted for disease activity in RA.
The production of superoxide by the peripheral blood neutrophils of 19 patients with active rheumatoid arthritis was measured during treatment with sulphasalazine (SASP). The response to drug treatment was determined by change in plasma viscosity, CRP, early morning stiffness and articular index over a 10-point scale. Of the 19 patients studied, eight were considered to have responded well to SASP and seven to have responded poorly or not at all. Over the treatment period, plateau levels of superoxide production fell in seven of the eight responders (P = 0.028) compared with a non-significant fall in 3/7 of the non-responder groups. The initial rate of superoxide production also fell in the responder group, but this was not statistically significant. Initial values in both the responder and non-responder groups were comparable with those seen for normal controls. Analysis of drug levels showed all patients to be compliant with drug treatment; however, drug levels and neutrophil activity were not correlated. Studies of the effect of SASP and sulphapyridine on superoxide production in vitro showed no difference between good and poor responders. These results suggest that there is no inherent difference between good and poor responders regarding the susceptibility of their neutrophils to SASP. SASP's action on neutrophils, therefore, appears not to be its main mechanism of disease-modifying activity in RA.
This study examined the factors influencing the use of complementary therapies in patients with fibromyalgia. A postal questionnaire was sent to 90 patients who had attended a rheumatology out-patient clinic in West Yorkshire for their diagnosis or treatment of fibromyalgia. Seventy-one percent of fibromyalgia patients had used or were using complementary therapies. Patients who were using complementary therapies were of a higher socio-economic group (p < 0.001). The most popular therapy was oral supplementation. The duration of complementary therapies ranged from 3 months to 26 years (median = 3). The number of therapies used by each patient ranged from 1 to 10 (median = 3). The duration of fibromyalgia was associated with both the duration of complementary therapies (p < 0.001) and the number of therapies used (p < 0.05). The most popular source of advice for the decision to use complementary therapies was from a magazine (40%). Patients using complementary therapies were less likely to be satisfied with their current hospital treatment and turned to complementary therapies in the chance of relief from their fibromyalgia. The relatively high cost and lack of information on complementary therapies appeared to dissuade those patients who chose not to use it.
Imaging is likely to play an increasingly important role in clinical trials of antirheumatic drugs. The need to evaluate efficacy of a drug quickly often precludes the use of radiological evaluation as an outcome measure, particularly in chronic arthritides. New imaging techniques with the refinement to detect differential early change, often between drugs of comparable potency, would be advantageous. However, the trend to set up large scale multicentre trials that have adequate statistical power for the detection of change mitigates against the use of expensive imaging techniques of restricted access. The majority of drug trials, therefore, still resort to conventional radiology. With the new generation of drugs that have specific actions, sensitivity in comparing changes in synovium, cartilage, bone and adjacent soft tissues would be of value.