Biomedical subjects

Brian M Feldman

Publications and source records attributed to Brian M Feldman.

32 records · Page 2Linked to original sources

PubMed2004

Favorable outcome in patients with renal involvement complicating macrophage activation syndrome in systemic onset juvenile rheumatoid arthritis.

Systemic-onset juvenile rheumatoid arthritis (SoJRA) constitutes about 10-20% of all JRA. However more than two-thirds of the mortality seen in JRA patients is accounted for by SoJRA. Macrophage activation syndrome (MAS), which can also be considered as a form of secondary hemophagocytic lymphohistiocytosis, is a major cause of morbidity and mortality in children with SoJRA. MAS is characterized by persistent high fever, pancytopenia, mild to serious derangements of liver cell function, encephalopathy, and disseminated intravascular coagulation. Renal involvement in MAS is a rarely recognized feature. In 2 recently reported case series of MAS in SoJRA, renal involvement appeared to be associated with poor prognosis. We describe 3 children with SoJRA who had renal involvement complicating MAS and had a favorable outcome.

Adolescent↗

PubMed2003

Preliminary core sets of measures for disease activity and damage assessment in juvenile systemic lupus erythematosus and juvenile dermatomyositis.

OBJECTIVE: To identify preliminary core sets of outcome variables for disease activity and damage assessment in juvenile systemic lupus erythematosus (JSLE) and juvenile dermatomyositis (JDM). METHODS: Two questionnaire surveys were mailed to 267 physicians from 46 different countries asking each member to select and rank the response variables used when assessing clinical response in patients with JSLE or JDM. Next, 40 paediatric rheumatologists from 34 countries met and, using the nominal group technique, selected the domains to be included in the disease activity and damage core sets for JSLE and JDM. RESULTS: A total of 41 response variables for JSLE and 37 response variables for JDM were selected and ranked through the questionnaire surveys. In the consensus conference, domains selected for both JSLE and JDM activity or damage core sets included the physician and parent/patient subjective assessments and a global score tool. Domains specific for JSLE activity were the immunological tests and the kidney function parameters. Concerning JDM, functional ability and muscle strength assessments were indicated for both activity and damage core sets, whereas serum muscle enzymes were included only in the activity core set. A specific paediatric domain called 'growth and development' was introduced in the disease damage core set for both diseases and the evaluation of health-related quality of life was advised in order to capture the influence of the disease on the patient lifestyle. CONCLUSIONS: We developed preliminary core sets of measures for disease activity and damage assessment in JSLE and JDM. The prospective validation of the core sets is in progress.

Child↗

PubMed2003

European Consensus Lupus Activity Measurement is sensitive to change in disease activity in childhood-onset systemic lupus erythematosus.

OBJECTIVES: To evaluate the European Consensus Lupus Activity Measurement (ECLAM) for responsiveness to change in disease activity when used in childhood-onset systemic lupus erythematosus (cSLE). To confirm the construct validity and to characterize the measurement properties of the ECLAM by assessing its ability to predict damage and steroid requirements. METHODS: The disease courses of 66 newly diagnosed cSLE patients were reviewed. The ECLAM and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) were scored for all clinic visits and hospitalizations. Damage was assessed at the end of the followup period using the Systemic Lupus International Collaboration Clinics/American College of Rheumatology Damage Index. Disease activity at the time of diagnosis, 6 months after diagnosis, at the time of first flare, and 6 months after first flare was used to estimate responsiveness of the measures. Responsiveness was measured by the effect size, the effect size index, the standardized response mean, and the relative efficiency index (REI). The measurement properties of the ECLAM and SLEDAI over time were examined by comparing the ability of both measures to predict damage and oral steroid requirement. RESULTS: The ECLAM and SLEDAI are both responsive to change in disease activity irrespective of the statistic used. The ECLAM is more sensitive than the SLEDAI using the REI (all >1.9). Cumulative disease activity as measured by the SLEDAI or the ECLAM are important predictors of damage. There are no statistically important differences between the 2 measures with regard to their ability to predict steroid requirements. CONCLUSIONS: The ECLAM has construct validity in cSLE and, like the SLEDAI, is highly sensitive to clinically important change in disease activity. The ECLAM may be more responsive. The quantitative properties of the 2 measures are very similar. The SLEDAI likely remains the preferable disease activity measure for cSLE given its overall measurement properties and ease of use.

Administration, Oral↗

PubMed2003

Use of complementary and alternative medicine by pediatric rheumatology patients.

OBJECTIVE: To assess how often the patients in The Hospital for Sick Children's rheumatology clinic use complementary and alternative medicine (CAM), and the type of CAM used. METHODS: Cross-sectional descriptive survey study in a tertiary care referral center. RESULTS: We found that 90 (64%) of 141 respondents used at least 1 form of CAM; 45 (50%) of these 90 used more than 1 form. Duration of illness was positively associated with the use of CAM; the difference in the mean length of illness between users (4.12 +/- 3.60 years) and nonusers (2.82 +/- 3.23 years) was 1.3 years (P = 0.04). Of the 41 respondents with more than 1 illness, 32 used CAM, compared with 57 of 96 who had only 1 illness (P = 0.036). CONCLUSION: Health care providers must be aware of their patients' possible use of CAM, especially those with more than 1 illness.

Arthritis↗

PubMed2003

Vitamin E: the evidence for multiple roles in cancer.

There is accumulating evidence that vitamin E may have different roles in the prevention and treatment of cancer. The purpose of this review is to summarize and evaluate this evidence and to suggest future avenues of research. A comprehensive literature review of vitamin E and cancer was conducted. Articles were organized into the following categories: 1) cancer prevention, 2) direct antineoplastic activity, 3) augmentation of chemotherapy effects, and 4) attenuation or treatment of chemotherapy toxicity. The evidence was systematically evaluated using guidelines developed by the U.S. Preventative Services Task Force. There is evidence to suggest that those individuals with higher serum vitamin E levels and those receiving vitamin E supplementation have a decreased risk of some cancers, including lung, prostate, stomach, and gastrointestinal carcinoma. However, these results differed depending on the study design and the population studied. There is insufficient evidence to support anticancer activity and attenuation of chemotherapy toxicity by vitamin E. Vitamin E is likely to be important in the prevention of some cancers. The therapeutic role of vitamin E is poorly understood. Further research will be required before routine use of vitamin E in patients with cancer can be advocated in the clinical setting.

Case-Control Studies↗

PubMed2003

Item weightings for the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Disease Damage Index using Rasch analysis do not lead to an important improvement.

OBJECTIVE: To develop Systemic Lupus International Collaborating Clinics/American College of Rheumatology Disease Damage Index (SLICC/ACR-DI) item weightings that improve the ability of the measure to predict patient mortality in systemic lupus erythematosus (SLE). METHODS: Disease damage was measured for 738 patients followed at the University of Toronto Lupus Clinic since diagnosis. Using Rasch analysis, item weightings were determined and tested for their ability to predict death in a logistic regression model. Receiver operating characteristic (ROC) curves were produced to compare the original and weighted scales' ability to discriminate patients that died during the followup period from those who remained alive. RESULTS: The average SLICC/ACR-DI score per patient was 1.66. In total, 138 of the patients died during a mean followup of 9.2 years. A Rasch analysis derived weighting scheme using weighted domain scores (SLICC/ACR-DI-weighted) was the best weighted scale, with item reliability = 94%, model mean square infit = 1.01 (STD = 0.05); model mean square outfit = 0.99 (STD = 0.3), separation 4.08. The SLICC/ACR-DI-weighted was modestly better than the SLICC/ACR-DI in discriminating patients who died from those who remained alive. Using standardized scores for comparability, the SLICC/ACR-DI-weighted was better in predicting patient death than the unweighted SLICC/ACR-DI [OR(death)(SLICC/ACR-DI-weighted) = 1.7 vs OR(death)(SLICC-ACR-DI) = 1.4; p < 0.005]. ROC curve analysis supports that the SLICC/ACR-DI-weighted was somewhat superior to the SLICC/ACR-DI for predicting mortality. CONCLUSION: In this test set, the SLICC/ACR-DI-weighted was modestly better in predicting death than the traditional unweighted SLICC/ACR-DI. However, the SLICC/ACR-DI-weighted is more difficult to apply and the weightings appear not to have provided a clinically relevant improvement of the SLICC-ACR-DI.

Adolescent↗

PubMed2002

Minimal difference in pain associated with change in quality of life in children with rheumatic disease.

OBJECTIVE: To establish the minimal change on a pain visual analog scale (VAS) associated with change in self-reported quality of life in pediatric rheumatology patients. METHODS: Subjects were a cohort of 533 pediatric rheumatology patients in Toronto. Pain and perceived quality of life were measured at 2 consecutive visits to a clinic. RESULTS: Among patients who rated quality of life at the second visit as "a little better" and "much better," the mean change in pain score on a 10-cm VAS was reduced by 0.82 and 1.45 cm, respectively. For those whose quality of life changed to "a little worse" and "much worse," the pain scores increased by 1.90 and 3.69 cm. CONCLUSION: Our results suggest that future studies of the assessment and treatment of pain in this population should aim for a minimum reduction in pain score of 0.82 cm on a 10-cm VAS to achieve clinical improvement in quality of life.

Adolescent↗

PubMed2002

Risk factors for damage in childhood-onset systemic lupus erythematosus: cumulative disease activity and medication use predict disease damage.

OBJECTIVE: The Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index measures damage in adult patients with systemic lupus erythematosus (SLE), but its usefulness in patients with childhood-onset SLE has not been examined. This study was conducted to evaluate the sensibility of the SLICC/ACR Damage Index, to investigate how cumulative disease activity is related to damage in childhood-onset SLE, and to identify other risk factors for damage in childhood-onset SLE. METHODS: Disease activity and damage in 66 patients with newly diagnosed childhood-onset SLE were assessed retrospectively, and information on potential risk factors for damage (age, race, sex, medications, duration of disease, hypertension, body mass index, antiphospholipid antibodies, kidney disease, acute thrombocytopenia) was obtained. In addition, a group of physicians was surveyed to establish the sensibility of the SLICC/ACR Damage Index in childhood-onset SLE. RESULTS: The SLICC/ACR Damage Index was found to have face, content, and construct validity when used in children. The mean SLICC/ACR Damage Index score of the patients was 1.76 (mean followup 3.3 years). Cumulative disease activity over time was the single best predictor of damage (R(2) = 0.30). Other, possibly important risk factors for damage were corticosteroid treatment, the presence of antiphospholipid antibodies, and acute thrombocytopenia. It was determined that immunosuppressive agents may be protective. CONCLUSION: The SLICC/ACR Damage Index, though useful in childhood-onset SLE, may benefit from the introduction of weightings and redefinition of some of the items. Ongoing disease activity leads to disease damage, and treatment should be prompt. Prolonged use of high-dose corticosteroids may further increase damage, but use of immunosuppressive agents may protect against disease damage; this latter finding may have potential implications for the treatment of childhood-onset SLE and deserves further study. The relationship between disease activity and concomitant use of medication also requires further investigation.

Adolescent↗

PubMed2002

Clinical features and outcomes of juvenile dermatomyositis and other childhood onset myositis syndromes.

The childhood myositis syndromes, primarily JDM, are relatively homogeneous diseases that have a good outcome in most cases, but are chronic, with poor outcomes, in a substantial number of cases. Because of the rarity of childhood myositis, and because there has not yet been a unified evidence-based approach to therapy, optimal treatment remains unknown. In clinic-based series the disease seems to be homogeneous but there are patients with rare clinical features, (e.g., cardiac, pulmonary, and neurologic disease), that impact prognosis. Racial, genetic, and other factors may lead to geographic variations in clinical presentations and outcomes, and perhaps further exploration of these influences will lead to a better understanding of the clinical features and outcomes seen in children. Our treatments are based on accumulated experience, but proper clinical trials have not been done. Ongoing registry studies, development of validated activity and damage assessment tools, large clinical trials, and continued investigation into the pathogenesis of the childhood myositis syndromes should lead to improved understanding and better treatments.

Algorithms↗

PubMed2002

Clinical assessment of conjunctival and episcleral vessel tortuosity in juvenile dermatomyositis.

PURPOSE: Conjunctival and episcleral vessel tortuosity are thought to be associated features of juvenile dermatomyositis. This study was conducted to determine the interobserver reliability of assessing normal and abnormal conjunctival and episcleral vessels from photographs of patients with and without juvenile dermatomyositis. METHODS: Color and red-free external ocular photographs of each eye of 28 children were assessed by 5 pediatric ophthalmologists from The Hospital for Sick Children. Fifteen of these pairs of photographs were of normal control eyes and 13 were from eyes of patients with juvenile dermatomyositis. On a standardized form each ophthalmologist was asked to determine whether both the conjunctival and episcleral vessels appeared normal or abnormal. Interobserver agreement was calculated by the weighted kappa statistic. The sensitivity and specificity of abnormal vessels for diagnosing juvenile dermatomyositis were also determined. RESULTS: Interobserver agreement for assessing conjunctival and episcleral vessels was 0.18 and -0.005, respectively. The average sensitivity and specificity of the evaluators for correctly assessing conjunctival vessels were 0.70 and 0.70, and for episcleral vessels, 0.67 and 0.47, respectively. CONCLUSIONS: There was low interobserver agreement in distinguishing between normal and abnormal eyes based on conjunctival and episcleral vessels. The sensitivity and specificity for identifying patients with juvenile dermatomyositis based on the appearance of vessels alone were relatively low. The appearance of conjunctival and episcleral vessels should be assessed in the context of other ocular and periocular findings of patients with juvenile dermatomyositis.

Adolescent↗

PubMed2002

Longterm anticoagulation is preferable for patients with antiphospholipid antibody syndrome. result of a decision analysis.

OBJECTIVE: Patients with antiphospholipid antibody syndrome (APS) have a high risk for rethrombosis. Anticoagulation with warfarin and aspirin reduces the frequency of recurrences. No universally accepted approach regarding the duration and intensity of antithrombotic therapy exists. We investigated the best antithrombotic regimen for patients with APS after the first deep venous thrombosis (DVT). METHODS: We identified 6 anticoagulation regimens used in such patients, the rates of morbidity and mortality associated with bleeding, and the rates of recurrent thrombosis associated with APS by literature search. A decision tree was developed and the expected risks and benefits of each anticoagulation regimen were assessed at 2 different time points: at one year and again 4 years after the initial thrombosis. RESULTS: Based on the decision analysis, longterm warfarin alone at an international normalization ratio (INR) between 3.0 and 4.0 had the highest expected utility of the 6 antithrombotic regimens, both one year and 4 years after the initial venous thrombotic event. Short term anticoagulation for only 6 months is less beneficial. Combination therapy of warfarin and aspirin (ASA) does not offer an improvement in the expected utility over warfarin alone. CONCLUSION: Although the applicability of this analysis to clinical decision-making is not entirely clear, patients with APS presenting with DVT appear to benefit from longterm warfarin (INR 3.0-4.0) that may be superior to warfarin (INR 2.0-3.0). Short term warfarin therapy seems to be less beneficial and the use of ASA does not offer a clear additional benefit. Randomized controlled trials are needed to provide a better basis for recommendations for the treatment APS.

Anticoagulants↗

PubMed2002

Differences in the profiles of circulating levels of soluble tumor necrosis factor receptors and interleukin 1 receptor antagonist reflect the heterogeneity of the subgroups of juvenile rheumatoid arthritis.

OBJECTIVE: To determine whether levels of soluble tumor necrosis factor receptor 55 (sTNFR55), sTNFR75, and interleukin 1 receptor antagonist (IL-1Ra) can differentiate different subtypes of juvenile rheumatoid arthritis (JRA), and to determine if the levels of these proteins correlate with disease activity. METHODS: Serum sTNFR (55 and 75) and IL-1Ra levels were measured by ELISA in 34 patients with JRA and these values were correlated with disease subtype and activity. RESULTS: Serum sTNFR55 levels were significantly elevated in patients with systemic onset JRA (SoJRA) (mean +/- 2 SD, 2.9 +/- 1.8 ng/ml) (p < or = 0.05) compared to rheumatoid factor positive (RF+) polyarticular JRA (2.1 +/- 0.6), RF-polyarticular JRA (1.5 +/- 0.6), and pauciarticular JRA (1.4 +/- 0.4). There was a trend for elevation of sTNFR75 levels in patients with SoJRA compared to other subtypes (p = 0.08). More patients had elevated levels of sTNFR75 than sTNFR55 (15 vs 7). This was true for all subsets (SoJRA 7 vs 5; polyarticular JRA 4 vs 2; and pauciarticular JRA 4 vs 0). In contrast to sTNFR, IL-1Ra levels were significantly elevated in RF+ polyarticular JRA compared to the other subgroups (p < or = 0.001). We found statistically significant Pearson correlations between (1) sTNFR75 and hemoglobin concentration: and (2) IL-1Ra and number of active joints and number of joints with effusions. CONCLUSION: The increased serum level of sTNF receptors in SoJRA suggests that TNF is likely more important than IL-1 in systemic inflammation and in particular in SoJRA. Conversely, IL-1 is likely more important in the inflammatory arthritis of JRA and in particular in the pathogenesis of RF+ polyarticular JRA. Our results suggest that cytokines have differing roles in JRA subtypes and likely reflect JRA subtype heterogeneity.

Adult↗

PubMedDate not supplied

The relationship between function, self-perception, and spinal deformity: Implications for treatment of scoliosis in children with spina bifida.

The purpose of this study was to determine the relationship of spinal deformity with physical function and self-perception in children with spina bifida. Ninety-eight eligible children with scoliosis and spina bifida were identified; 80 of them (82%) consented to participate. Spinal deformity was measured in many ways, including scoliosis, coronal balance, and pelvic obliquity. Measures of physical function included the Sitting Balance Scale, Jebsen Hand Scale, Hoffer Ambulation Scale, the Spine Bifida Spine Questionnaire, and the Activities Scale for Kids (ASK). Self-perception was determined with Harter's Self-Perception Profile. No relationship was found between spinal deformity and overall physical function (ASK). Of all aspects of spinal deformity, only coronal imbalance was significantly related to only one aspect of physical function (ie, sitting imbalance). No aspect of spinal deformity was related to self-perception. In conclusion, surgeons should be clear in their indications for surgery and recognize that in the short term the potential benefit of surgery may be, at best, to improve only sitting balance.

Activities of Daily Living↗

PubMedDate not supplied

e-Ouch: usability testing of an electronic chronic pain diary for adolescents with arthritis.

OBJECTIVES: The aim of this study was to evaluate the usability of the e-Ouch electronic chronic pain diary in adolescents with juvenile idiopathic arthritis. METHODS: A qualitative usability testing approach with semistructured, audiotaped interviews with two iterative cycles was used. A purposive sample of 10 adolescents per cycle was drawn from a rheumatology clinic in a university-affiliated pediatric tertiary care center. Participants were provided with a brief demonstration of the diary and then asked to use the diary "thinking aloud" to record the pain they experienced: (1) when they woke up that morning, (2) during that afternoon, and (3) from the previous evening. Adolescents were then asked a series of open-ended questions addressing ease of use of the diary. Qualitative thematic analysis was used to generate categories and emerging themes from interview data. RESULTS: All of the adolescents stated the e-Ouch diary was very easy to learn, use, and understand and was satisfying to complete. Participants took less than 9 minutes to complete all three of the diary entries with minimal errors. The usability evaluation revealed aspects of the interface that were suboptimal (eg, VAS slider) and impeded the performance of certain tasks. Adolescents generated ideas on how the diary interface could be improved. CONCLUSIONS: A multifaceted usability approach provided important insight regarding the use of technology by adolescents with arthritis and, more specifically, for understanding how adolescents can more effectively use an electronic chronic pain diary.

Adolescent↗