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Biomedical subjects

B M Feldman

Publications and source records attributed to B M Feldman.

52 records · Page 3Linked to original sources

Sensitivity of the Systemic Lupus Erythematosus Disease Activity Index, British Isles Lupus Assessment Group Index, and Systemic Lupus Activity Measure in the evaluation of clinical change in childhood-onset systemic lupus erythematosus.

OBJECTIVE: To investigate whether 3 disease activity indices commonly used to evaluate systemic lupus erythematosus (SLE) in adults are sensitive to clinical change in children, and thus suitable for the use in the management of childhood-onset SLE. METHODS: Thirty-five SLE patients who were newly diagnosed between 1993 and 1997, had an age at onset of 6-16 years (26 female and 9 male), and were currently being followed up at The Hospital for Sick Children (followup of 9 months to 4 years) were reviewed. The SLEDAI (Systemic Lupus Erythematosus Disease Activity Index), BILAG (British Isles Lupus Assessment Group index), and SLAM (Systemic Lupus Activity Measure) were applied at up to 4 occasions during the disease course: at the time of diagnosis, 6 months postdiagnosis, at the time of a flare (a deterioration in clinical presentation or laboratory results requiring initiation or increase of either corticosteroids or "second-line" drugs), and 6 months postflare. The sensitivity of the 3 measures to change, as gauged by the effect size (ES), effect size index (ESI), standard response mean (SRM), responsiveness statistic (RS), and relative efficiency index (REI), were compared. RESULTS: All 3 tools were very sensitive to change in disease activity (ES >0.8, ESI >2.3, SRM >0.6, RS >0.86, REI >0.72), but were ranked differently depending on the statistic used for comparison. CONCLUSION: All 3 measures of disease activity are highly sensitive to clinical change in children; none showed an overall superiority. The SLEDAI, BILAG, and SLAM can all be used to study response to treatment in children with SLE.

Adult↗

Assessment of myocardial perfusion and function in childhood systemic lupus erythematosus.

OBJECTIVES: To determine the prevalence of abnormalities in myocardial perfusion or function in children with systemic lupus erythematosus (SLE), and describe potential factors that may predict their development. STUDY DESIGN: Patients (n = 40; 30 female) were enrolled through the Lupus Clinic at The Hospital for Sick Children between 1990 and 1992. Resting and exercise thallium myocardial perfusion scans, radionuclide angiography with multiple gated acquisition (MUGA), and resting M-mode and two-dimensional echocardiography were performed. RESULTS: All patients were free of symptoms, and none had a history of ischemic heart disease. Their median age was 15.9 years (range 10.5 to 19.8 years) at enrollment. Abnormalities of coronary perfusion were found in 5 (16%) of 31 patients (95% confidence interval: 3%, 29%) and included a large fixed perfusion defect in 1; 5 of 27 MUGA scans showed marginally low left ventricular ejection fractions at rest, whereas all had normal exercise responses. In the group with abnormal thallium scans, three of five patients had antiphospholipid antibodies detected, and two of four had an abnormal plasma lipid profile. This group tended to have a shorter disease duration and had received a lower cumulative dose of corticosteroids; these differences were not statistically significant compared with the group with normal scans. CONCLUSION: Asymptomatic abnormalities of myocardial perfusion occur in children with SLE and are more common than previously suspected. Patients with these abnormalities of myocardial perfusion may be predisposed to the previously recognized early-onset ischemic heart disease seen in adults with SLE.

Adolescent↗

Cyclosporine in the treatment of an unusual case of juvenile systemic sclerosis.

We describe a child who initially presented with juvenile dermatomyositis (JDM). Four years after the complete remission of JDM she developed systemic sclerosis without internal organ involvement. She was unresponsive to a combination of systemic corticosteroid therapy and pencillamine. The institution of cyclosporine induced a complete remission.

Adolescent↗

Development of validated disease activity and damage indices for the juvenile idiopathic inflammatory myopathies: I. Physician, parent, and patient global assessments. Juvenile Dermatomyositis Disease Activity Collaborative Study Group.

OBJECTIVE: To determine the reliability, content validity, and responsiveness of physician global assessments of disease activity and damage in the juvenile idiopathic inflammatory myopathies (IIM), and to investigate concordance among physician, parent, and patient global ratings. METHODS: Sixteen pediatric rheumatologists rated 10 juvenile IIM paper patient cases for global disease activity and damage, and assessed the importance of 51 clinical and laboratory parameters in formulating their global assessments. Then, 117 juvenile IIM patients were enrolled in a protocol to examine the relationship between Likert and visual analog scale global assessments, their sensitivity to change, and the comparability of physician, parent, and patient global ratings. RESULTS: Pediatric rheumatologists demonstrated excellent interrater reliability in their global assessments of juvenile IIM disease activity and damage (97.7% and 94.7% agreement among raters, respectively), and agreed on a core set of clinical parameters in formulating their judgments. Likert scale ratings correlated with those on a visual analog scale, and both were comparable in responsiveness (standardized response means -0.56 for disease activity, 0.02 [Likert] and 0.14 [visual analog] for damage, measured over 8 months). Parent global ratings of disease activity correlated with physician assessments, but were not colinear (Spearman's correlation [r] = 0.41-0.45). Patient global disease activity assessments correlated with those done by parents (r = 0.57-0.84) and physicians (r = 0.37-0.63), but demonstrated less responsiveness (standardized response means -0.21 and -0.12, respectively, over 8 months). CONCLUSION: Physician global assessments of juvenile IIM disease activity and damage demonstrated high interrater reliability and were shown to be comprehensive measures. Both physician and parent disease activity assessments should be considered valuable as quantitative measures for evaluating therapeutic responses in juvenile IIM patients.

Arthritis, Juvenile↗

General and local scleroderma in children and dermatomyositis and associated syndromes.

Scleroderma in children virtually always manifests in the form of localized disease, whereas adult scleroderma usually manifests as systemic disease. The recent literature on childhood scleroderma is limited almost exclusively to localized disease. The treatment of this disease remains a dilemma, especially with regard to which patients should be treated, how they should be treated, and how the treatment response should be monitored. Our current therapeutic approach is reviewed. The recent literature regarding juvenile dermatomyositis is discussed. New understanding of the pathobiology of this disease should lead to improved, less toxic treatments in the future.

Child↗

Seasonal onset of systemic-onset juvenile rheumatoid arthritis.

OBJECTIVE: This study was undertaken to investigate the recent finding of a seasonal difference in the onset of systemic-onset juvenile rheumatoid arthritis (SoJRA). We hypothesized that a seasonal onset pattern might implicate on infectious agent as a cause of SoJRA. METHODS: The date of onset was collected from the records of all patients with SoJRA from 1980 to 1992 at presentation to pediatric rheumatology clinics across Canada. The onset pattern of SoJRA was then compared with incidence data on viral infections obtained for the same period. RESULTS: Across Canada the onset of SoJRA was constant across the seasons. However, in the Prairie region there was a statistically significant seasonal pattern, with peaks in autumn and early spring. We could find no evidence that viral incidence correlated with disease incidence either throughout Canada or in the Prairie region. CONCLUSIONS: If a seasonal infectious agent causes SoJRA, then it is likely only one of several causes and may act only in certain regions. Future studies should be carried out in those areas where SoJRA does have a seasonal onset pattern.

Adolescent↗

Clinical significance of specific autoantibodies in juvenile dermatomyositis.

OBJECTIVE: To determine the prevalence and clinical association of myositis specific antibodies in an unselected group of patients with juvenile dermatomyositis (DM). METHODS: The sera of 42 subjects, representing an unselected group of patients from a single center, with juvenile DM and 7 others with idiopathic inflammatory myopathy (IIM) were examined for the presence of myositis specific antibodies by immunodiffusion against calf thymus extract and immunoprecipitation with HeLa extract. RESULTS: Of the subjects with juvenile DM, only 2 had evidence of antibodies specific to myositis (anti-Mi2). Three other patients with juvenile DM had defined autoantibodies not usually considered to be specific to myositis. Two of the 3 subjects had anti-PM-Scl; both developed features of scleroderma after the juvenile DM remitted. The 5 subjects with defined autoantibodies did not differ clinically from the remainder of the subjects with the exception of the late development of scleroderma features in 2. Fourteen other subjects with juvenile DM had unidentified bands on immunoprecipitation, which may represent as yet undiscovered myositis specific antibodies. No myositis specific antibodies were detected in any of the 7 subjects with other IIM syndromes. CONCLUSION: Based on our findings, we do not recommend routine clinical testing for these antibodies in children with typical juvenile DM. Further study of the unidentified bands seen in our subjects may lead to better understanding of the clinical groupings and etiopathogenesis of childhood myositis.

Adolescent↗

Measuring disability in juvenile dermatomyositis: validity of the childhood health assessment questionnaire.

OBJECTIVE: Physical disability is perhaps the most important outcome of juvenile dermatomyositis (JDM). No functional assessment tool has been validated for inflammatory myopathies either in children or adults. We studied the measurement properties of the Childhood Health Assessment Questionnaire (CHAQ) in children with JDM. METHODS: We studied 37 patients followed at the JDM clinic and compared the results obtained by the CHAQ to a global disease severity score and quantitative muscle strength testing measured by sphygmomanometry (construct validity). We also measured the reliability of the CHAQ and its responsiveness to clinical change. RESULTS: For the initial measurement of each subject, the correlation between disease severity and CHAQ was high [Spearman's correlation, (rs = 0.71, p < 0.002)]. Disability as measured by the CHAQ was inversely correlated with proximal muscle strength (hip abduction rs = -0.57, p < 0.002; shoulder abduction rs = -0.51, p < 0.01) but, as expected, less so with more distal muscle strength (knee extension rs = -0.40, p = 0.05; grip strength rs = -0.079, p > 0.20). The CHAQ was reliable in subjects who showed no clinical change in muscle strength (intraclass correlation coefficient = 0.87) and responsive to treatment induced clinical change (responsiveness coefficient = 0.90). CONCLUSION: The CHAQ can serve as a valid and sensitive tool in the evaluation of functional outcomes in JDM.

Adolescent↗

How should excess admission laboratory test utilization be curtailed? --paediatricians' preferences.

Routine admission casefinding tests are costly and rarely lead to changes in management. Strategies have been proposed to decrease them. However, it is unclear which tests are considered routine at admission. Our objectives were to clarify which tests are considered to be routine and to determine which methods of reducing test utilization are most acceptable. Sixty-five physicians from a paediatric teaching hospital were selected by random stratified sampling. A questionnaire was administered asking about the role of routine tests and preferences for 6 strategies to reduce test ordering. Sixty percent of respondents felt that more casefinding was occurring than should be, and 89% would agree to a trial of test reduction strategies. Just over half the respondents felt that hemoglobin determination and urinalysis were routine admission tests; fewer physicians listed a wide variety of other tests. Physicians who thought that all admissions should be screened were older, graduated earlier, and tended to be community based. Educational strategies to reduce testing were more acceptable than administrative ones. We conclude that many physicians would support the trial implementation of test reduction strategies in childrens' hospitals. Hospitals wishing to reduce excess admission casefinding should implement educational strategies, as physicians prefer these.

Child↗

Diagnostic use of B-cell alloantigen D8/17 in rheumatic chorea.

Two unrelated patients with a family history of rheumatic fever had isolated, acquired chorea. Both index cases, as well as affected family members, had increased expression of the rheumatic B-cell alloantigen D8/17. This test may help differentiate Sydenham chorea from lupus chorea.

B-Lymphocytes↗

Prevalence of nurse smokers and variables identified with successful and unsuccessful smoking cessation.

A mailed survey of 823 randomly selected registered nurses, constituting an 82% return rate, was conducted to determine the prevalence and quit rate of smoking related behaviors. Variables identified with successful quitters and continuing smokers also were investigated. The findings revealed that nurses have a decreased proportion of current smokers, an increased proportion of never smokers, and an increased quit smoking rate. The smoking cessation method used most frequently by successful quitters was "cold turkey".

Adult↗

The prevalence of hot flash and associated variables among perimenopausal women.

A telephone survey of a random sample of 594 perimenopausal women was done to study the prevalence of hot flashes, use of estrogen, age of menopause onset, and, among those subjects experiencing hot flashes, the frequency of occurrence and number of years of hot flashes. The prevalence of hot flashes was 88%. Surgical menopause women had a prevalence rate of 92% and had the highest estrogen utilization rate. The median age of onset for natural menopause women was 49 years. The frequency of occurrence and number of years of hot flash experience was variable across all groups.

Adolescent↗

Unilateral tuboovarian autoamputation.

Six cases of unilateral tuboovarian absence are reviewed. In four, the peritoneal cavity contained a separate ovoid structure. In two of these, the pathologist could identify the remnant as a phagocytized ovary. In a third patient, the ovoid body was seen fixed to the contralateral ovary during laparoscopy. Subsequently, it was identified radiologically as a calcific density. In the fourth case, an intact ovary was separated from the uterus and engulfed by omentum. If a total embryogenic error or selective dysgenesis of the urogenital fold occurs, ipsilateral anomalies usually involve adjacent structures of both the urinary and genital systems. Howerver, no anomalies of the uterus and urinary structures appeared evident in any reviewed case. Therefore, adnexal torsion with subsequent infarction necrosis and autoamputation represents the most likely explanation for this phenomenon.

Adult↗

Preference-based measurement of health-related quality of life (HRQL) in children with chronic musculoskeletal disorders (MSKDs).

BACKGROUND: Health-related quality of life can be measured by patients' health preferences (utilities or values). No method for measuring health state preferences has been standardized for children with arthritis or other musculoskeletal disorders (MSKDs). Such a method is needed for economic evaluations of current and new pediatric treatments. OBJECTIVES: 1) To assess the feasibility of utility measurements in children with MSKDs, 2) to test the validity of the Health Utility Index (HUI) for these children, 3) to assess whether rating scale values can be mathematically converted into meaningful standard gamble (SG) utilities, and 4) to study whether parents can act as proxies for their children with respect to health state preferences. METHODS: Eighty parents of children with MSKDs were consecutively sampled. Their children, if 8 years of age or older (n = 55), were studied concurrently. Utilities of current health states were obtained by using the SG and the HUI in random order. In addition, health state preferences were assessed using categorical and analog rating scales. Traditional nonutility measures of health status (the Childhood Health Assessment Questionnaire [CHAQ] and the Activities Scale for Kids [ASK]) were also completed. Intraclass correlation coefficients (ICCs) were calculated to assess concordance between the different utility measures and also between the ratings of the parents and their children. RESULTS: Children 8 years of age or older were able to express the strength of their health state preferences using the HUI and rating scales. Children older than 10 years of age were able to use the SG method. The health state utilities of the parents were higher than those of their children. The utilities varied widely depending on the elicitation method. The expected high agreement between the SG and the HUI was not found (ICC = 0.028 for parents, ICC = 0.016 for patients). Unlike the SG, the global utilities derived from the HUI agreed better with preferences derived from rating scales (ICC = 0.23-0.25) and correlated with traditional health status measures (with CHAQ, r = -0.56; with ASK, r = 0.46) both for parents and children. It was not possible to mathematically convert rating scale preferences into SG utilities. The SG utilities were unrelated to results from the rating scales, the CHAQ, and the ASK. Especially for parents, the SG utilities were very high, even when ratings of the other measures indicated poor health. CONCLUSIONS: Although it is possible to measure health utilities for children with MSKDs, the results are highly method dependent. The properties of the HUI in this population are more like those of the traditional health status measures rather than those of the SG. Preferences derived from rating scales, although easily performed, cannot readily be converted into SG utilities. Parents' ratings for their children are impaired by risk aversion.

Adolescent↗