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R M Laxer

Publications and source records attributed to R M Laxer.

At least 19 recordsLinked to original sources
PubMed2006

Tumour necrosis factor alpha inhibitors in the treatment of childhood uveitis.

OBJECTIVE: To describe the efficacy of anti-TNF-alpha agents in the treatment of childhood uveitis. METHODS: We performed a retrospective chart review of all children with uveitis treated with TNF-alpha blockers at The Hospital for Sick Children, Toronto. RESULTS: Twenty-one children with uveitis were treated with the anti-TNF-alpha agents etanercept (11 patients) and infliximab (13 patients), resulting in 24 treatment courses. All patients had persistently active uveitis despite treatment with at least one standard immunosuppressive drug before the start of anti-TNF-alpha therapy. Six of 21 patients (29%) had idiopathic uveitis. In the other 15 patients, the underlying disease was juvenile idiopathic arthritis in 12 (57%), Behçet disease in two (9%) and sarcoidosis in one (5%). Response to etanercept treatment was good in 27%, moderate in 27% and poor in 45% of patients. Response to infliximab treatment was good in 38%, moderate in 54% and poor in 8% of patients. The difference in the percentage of patients with a moderate or good response was statistically significant (P = 0.0481). We also observed a lower rate of complications, such as new-onset or worsening glaucoma or cataract in the infliximab-treated group. CONCLUSION: Anti-TNF-alpha treatment was beneficial in a high percentage of patients with childhood uveitis refractory to standard immunosuppressive treatment. Infliximab resulted in better clinical responses with less ocular complications than etanercept.

Adolescent↗
PubMed2006

Risk of new-onset uveitis in patients with juvenile idiopathic arthritis treated with anti-TNFalpha agents.

OBJECTIVE: To determine whether treatment with tumor necrosis factor alpha (TNFalpha)-blocking agents alters the incidence of new-onset uveitis in patients with juvenile idiopathic arthritis (JIA). STUDY DESIGN: Cohort study based on retrospective chart review. The charts of all 1109 patients with a diagnosis of JIA seen between January 1, 1996, and June 30, 2003, at our clinic were reviewed for diagnosis of uveitis and treatment with TNFalpha inhibitors. Cox regression analysis was performed with anti-TNFalpha treatment as a time-dependent covariate for risk of development of uveitis. RESULTS: We identified 70 patients treated with anti-TNFalpha without a prior diagnosis of uveitis. Two of these 70 patients (2.9%), both treated with etanercept, had development of new-onset uveitis during anti-TNFalpha therapy. One had juvenile psoriatic arthritis diagnosed 4.1 years before onset of uveitis. The other had extended oligoarticular JIA diagnosed 6.4 years before onset of uveitis. We found no statistically significant difference in the risk for development of uveitis between patients with or without anti-TNFalpha treatment. CONCLUSIONS: In our patients with JIA, anti-TNFalpha treatment did not alter the risk for development of new-onset uveitis. However, anti-TNFalpha therapy with etanercept did not prevent the development of uveitis in 2 patients.

Adolescent↗
PubMed2005

The effectiveness of treating juvenile dermatomyositis with methotrexate and aggressively tapered corticosteroids.

OBJECTIVE: Childhood dermatomyositis (DM) is often a chronic disease, lasting many years. It has traditionally been treated with long-term corticosteroid therapy; side effects are often seen. For more than a decade, methotrexate (MTX) has been safely used for the treatment of juvenile arthritis. Here, we report use of MTX as first-line therapy for DM, along with aggressively tapered corticosteroids, in an attempt to reduce treatment-related side effects. METHODS: We studied an inception cohort of 31 children with DM who were rigorously followed up in our myositis clinic, and compared them with a control group of 22 patients with incident cases of juvenile DM who received treatment just before we instituted a policy of first-line therapy with MTX. The mean starting dosage of MTX in the study group was 15 mg/m(2)/week. RESULTS: Both groups had similar improvement in strength and physical function; however, the median time during which patients in the study group received corticosteroids was 10 months, compared with 27 months for controls (P < 0.0001). As a result, the cumulative prednisone dose in the study group was approximately half that in the control group (7,574 mg versus 15,152 mg; P = 0.0006). The study group had greater height velocity during the first year of treatment and a smaller increase in the body mass index over the first 2 years. In the control group, the relative risk of cataracts developing was 1.95 (95% confidence interval 1.05-4.17). Side effects of MTX were rarely observed. CONCLUSION: Use of MTX in conjunction with an aggressively tapered course of prednisone may be as effective as traditional long-term corticosteroid therapy for children with DM, while decreasing the cumulative dose of corticosteroids.

Dermatomyositis↗
PubMed2004

Increased anticardiolipin antibody IgG titers do not predict recurrent stroke or TIA in children.

BACKGROUND: Increased anticardiolipin antibody (ACLA) immunoglobulin (Ig) G titers are commonly found in children with arterial ischemic stroke (AIS) or TIA (AIS/TIA). The associated risk of recurrent thromboembolism is unknown. OBJECTIVE: To determine the risk of recurrent thromboembolism associated with persistently increased ACLA titers of the IgG isotype in children with AIS/TIA. METHODS: The authors studied a cohort of children surviving first AIS/TIA tested by standardized ELISA for beta2-glycoprotein I-dependent ACLA of the IgG isotype. Children with ACLA titers >15 IgG phospholipid (GPL) units (per manufacturer's cutoff point) on more than two occasions > or =6 weeks apart were classified as ACLA-positive (ACLA+) and compared with ACLA-negative (ACLA-) children with respect to recurrent thromboembolic events (AIS/TIA, sinovenous thrombosis, and extracerebral thromboembolism). RESULTS: The authors recruited 34 ACLA+ children and 151 ACLA- children. Most ACLA+ children (30/34; 88%) had ACLA titers < or =40 GPL units. During the follow-up period (median duration, 2.8 years for ACLA+ children and 3.0 years for ACLA- children), AIS/TIA recurred in 26% of ACLA+ children and in 38% of ACLA- children; none developed sinovenous thrombosis or extracerebral thromboembolism. Based on survival analysis, this difference was nonsignificant (p = 0.54). Using binary partition evaluation, no titer criteria for ACLA positivity (range, 0 to 60 GPL units) predicted recurrent AIS/TIA. CONCLUSION: In children surviving arterial ischemic stroke/TIA, increased anticardiolipin antibody immunoglobulin G titers do not predict recurrent thromboembolism.

Antibodies, Anticardiolipin↗
PubMed2002

Clinical relevance of the risk factors for coronary artery inflammation in Kawasaki disease.

The objective of this study was to determine predictive factors in children with Kawasaki disease (KD) with which we could distinguish the patients with KD who are either at very low risk or at very high risk for coronary artery inflammation (i.e., either patients who do not need intravenous immunoglobulin treatment or patients in whom more aggressive or even experimental therapies should be considered). Prospectively collected demographic, clinical, and laboratory data on 344 patients treated for KD were correlated with the patients' echocardiographic findings. The parameters studied were age, sex, duration of the fever, erythrocyte sedimentation rate, hemoglobin, white blood cell count, platelet count, and serum albumin. These were examined both in bivariable comparisons and in multiple logistic regression models. Low serum albumin, age <1 year, and the duration of the fever prior to treatment were risk factors for coronary arteritis. In the multivariable models, their combined predictive value for coronary lesions was poor, especially when identifying the patients at a low risk for coronary artery lesions (CALs). In fact, 44 of 98 patients with CALs were falsely classified to the low-risk group. Ten of 14 patients younger than 1 year of age, who also had low serum albumin (<30 g/L), had echocardiographically verified CALs, and 7 (50%) had a definite coronary artery aneurysm. We could not distinguish a group at such a low risk that these patients could be left untreated. Young patients with low albumin run a very high risk for CALs.

Adolescent↗
PubMed2001

Inflammatory linear verrucous epidermal nevus and arthritis: a new association.

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare, chronic skin condition that begins in early childhood. We present two children with ILVEN and arthritis, a previously undescribed association. We discuss the relevance of this association and suggest appropriate management for this arthritis.

Antirheumatic Agents↗
PubMed2001

The arthritis of inflammatory childhood myositis syndromes.

OBJECTIVE: Arthritis has been an associated finding in juvenile dermatomyositis (JDM), but its prevalence, course, and response to therapy has not been well described. We investigated the frequency, course, and clinical and radiographic features in a large cohort of patients with JDM. METHODS: The charts of 94 patients with idiopathic myositis (1984-99) were reviewed: 80 JDM, 3 juvenile polymyositis (JPM), 5 amyopathic JDM, and 6 overlap myositis syndromes. Compiled data included demographics, clinical features, a detailed description of the arthritis, investigations (radiographs, autoantibodies), course, and response to therapy. All radiographs were independently reviewed by a single radiologist. RESULTS: Sixty-one percent (95% CI 50-72%) of patients with JDM had arthritis. The arthritis was reported a median 4.5 mo (range -73.6 to 76.6 mo) after the JDM onset. When compared to patients with no arthritis, the occurrence of arthritis was not significantly related to sex, race, positive antinuclear antibody or rheumatoid factor, calcinosis, nodules, vasculitis, or Raynaud's phenomenon. The initial involvement was pauciarticular in 67% and polyarticular in 33%. In the pauci group, asymptomatic knee effusions were the predominant finding (n = 19, 58%), and in 18 patients may have been the result of steroid therapy. Two patients evolved from a pauci onset to a polyarticular course. All responded to therapy (corticosteroids; 47 were taking other medications) with remission of the arthritis within a median of 2.0 mo (range 0.1-64.5 mo). However, the arthritis recurred in 39% as the corticosteroids were tapered. Four patients with JDM eventually required corticosteroid wrist injections, with resolution of the arthritis. The arthritis was nonerosive in all cases. No patient with JPM had arthritis. Three of 5 patients with amyopathic JDM and 4 of 6 with overlap myositis syndrome had a nonerosive polyarthritis. CONCLUSION: Nonerosive arthritis involving the knees, wrists, elbows, and fingers is a frequent manifestation of JDM and other idiopathic childhood myositis. The arthritis is seen early in the course of JDM and often responds to treatment. However, the arthritis may recur with tapering of corticosteroids despite remission of the JDM. In a significant proportion of JDM cases, arthritis is the major sequela and may warrant further medical therapy or intraarticular corticosteroid injections.

Adolescent↗
PubMed2001

Autologous stem cell transplantation for pediatric rheumatic diseases.

The National Institute of Allergy and Infectious Disease, National Institutes of Health, convened a workshop entitled The Next Step: Protocol Development for Autologous Stem Cell Transplantation for Pediatric Rheumatic Disease, June 2000, co-chaired by Drs. Karyl Barron and Carol Wallace. The goal of the workshop was to focus on the scientific rationale for stem cell transplantation therapy in the pediatric diseases, unique aspects of this therapy in the pediatric rheumatic diseases, transplantation issues and options, regulatory issues, and development of a DNA repository for these diseases.

Child↗
PubMed2000

Providing after-hours on-call clinical coverage in academic health sciences centres: the Hospital for Sick Children experience.

An increasing number of admissions of patients requiring complex and acute care coupled with a decreasing number of pediatric postgraduate trainees has caused a shortage of house staff available to provide after-hours on-call coverage in the Department of Pediatrics at Toronto's Hospital for Sick Children. The Clinical Assistant program created to deal with this problem was short on staff, did not provide adequate continuity of care and was becoming increasingly unaffordable. The Clinical Departmental Fellowship program was created to address the problem of after-hours clinical coverage. The program is aimed at qualified pediatricians seeking additional clinical or research training in one of the subspecialty divisions in the Department of Pediatrics. We describe the hiring process, job description and evolution of the program since its inception in 1996. This program has been mutually advantageous for the individual fellows and their sponsoring divisions as well as the Department of Pediatrics and the Hospital for Sick Children. We recommend the introduction of similar programs to other academic medical departments facing staff shortages.

Academic Medical Centers↗
PubMed2000

Early predictors of poor functional outcome in systemic-onset juvenile rheumatoid arthritis: a multicenter cohort study.

OBJECTIVE: To examine the ability of a previously described set of criteria to predict poor functional outcome in a large, multicenter cohort of children with systemic-onset juvenile rheumatoid arthritis (JRA). METHODS: All children who were diagnosed with systemic-onset JRA since 1980 at the Hospital for Sick Children (Toronto), since 1983 at the Isaac Walton Killam Hospital for Children (Halifax), and since 1981 at the Children's Hospital of Eastern Ontario (Ottawa) were evaluated. Patients were included in the study if they had been evaluated clinically within 6 months of diagnosis and had been followed up for at least 2 years. Patients were divided into 4 cohorts according to their length of followup: 2-4 years, 4-7 years, 7-10 years, and >10 years. Using previously described criteria for destructive arthritis in children with systemic-onset JRA, the patients were classified as either high risk or low risk for poor functional outcome based on the data from their 6-month visit. High-risk patients had active systemic disease (persistent fever or corticosteroid requirement for control of systemic disease) and a platelet count > or =600 x 10(9)/liter. Poor outcome was defined as moderate or severe disability (defined as a score of > or =0.75 on the Childhood Health Assessment Questionnaire) or disease-associated death. RESULTS: Among 122 eligible patients with systemic-onset JRA, we were able to contact 111 (91%) for outcome data. The mean followup period was 7.7 years (SD 3.7). The mean age at outcome assessment was 13.5 years (SD 5.3). There were 51 boys and 60 girls. Twenty-four patients (22%) had moderate-to-severe disability and 2 patients died; these 26 patients were considered to have had a poor outcome. We could determine risk classification for 104 patients. Twenty-four patients (23%) met the criteria for high risk at the 6-month visit. Overall, the risk of a poor functional outcome was significantly higher in the high-risk group (relative risk 3.3, 95% confidence interval [95% CI] 1.73-6.43, P = 0.0004). This risk was most marked in the cohort with > 10 years of followup (relative risk 4.3, 95% CI 1.82-10.29, P = 0.006). CONCLUSION: The presence of active systemic disease at 6 months, as characterized by fever or the need for corticosteroids, and thrombocytosis strongly predicted the development of a poor functional outcome in these patients. This was especially apparent with longterm followup. Our study validates the previously developed prognostic criteria for systemic-onset JRA.

Activities of Daily Living↗
PubMed2000

Methotrexate and corticosteroid therapy for pediatric localized scleroderma.

INTRODUCTION: Localized scleroderma (LS) can cause permanent disability, and there is no universally accepted effective treatment. Methotrexate (MTX) has been shown to be effective in the treatment of systemic sclerosis. OBJECTIVES: To determine the efficacy and tolerability of MTX and corticosteroid therapy in patients with LS. METHODS: MTX, 0.3 to 0.6 mg/kg per week, was given to 10 patients (6 girls, 4 boys; mean age, 6.8 years; mean disease duration before starting treatment, 4 years) with active LS. In addition, pulse intravenous methylprednisolone, 30 mg/kg for 3 days monthly for 3 months, was given to 9 patients at the initiation of therapy. RESULTS: One patient discontinued taking MTX after a month; the remaining 9 patients responded. The median time to response was 3 months (95% CI, 1.15-4.85). One responder discontinued taking MTX after a year because of leukopenia; the LS worsened within 2 months. In another patient LS flared up after 10 months and responded to an increased dose of MTX and intravenous methylprednisolone. At the last follow-up visit, all patients who continued to receive MTX therapy had inactive skin lesions. CONCLUSION: Treatment with MTX and corticosteroids appears to be effective in the treatment of LS and is generally well tolerated. A placebo-controlled study is necessary to confirm the efficacy of MTX therapy in LS.

Administration, Oral↗
PubMed2000

Children with morphea have normal self-perception.

We evaluated the self-esteem and quality of life of 47 children with morphea with the use of the Harter self-perception profile for children and Visual Analog Scale. Most children with morphea have normal self-worth and a high quality of life. Morphea, like some other childhood chronic illnesses, does not impair self-esteem.

Adolescent↗
PubMed2000

A bioethics education program in pediatric rheumatology.

The medical care of children by pediatric rheumatologists may be ethically very complex. Medical trainees and members of the multidisciplinary health care team must be sensitive to and knowledgeable about ethical issues relevant to the care of their patients, and capable of ethical reasoning. Members of the Bioethics Department and the Division of Rheumatology at the Hospital for Sick Children have cooperatively developed a Bioethics Education Program with a curriculum tailored to meet the needs of the health care team. The quarterly ethics seminars are case based and interdisciplinary. Topics addressed include the nature of the patient-practitioner relationship, research with child participants, the developing autonomy of the child, the child's "best interests," and consent

Bioethics↗
PubMed2000

Intravenous immunoglobulin therapy for juvenile dermatomyositis: efficacy and safety.

OBJECTIVE: To assess the efficacy of intravenous immunoglobulin (IVIG) for the treatment of juvenile dermatomyositis (JDM) in patients who were unresponsive to corticosteroids (steroid resistant or steroid dependent) or showed unacceptable toxicity. METHODS: A retrospective chart review of the course of all patients with JDM treated with IVIG who attended the Dermatomyositis Clinic at The Hospital for Sick Children, Toronto, Canada, from August 1986 to December 1996. RESULTS: Eighteen patients with JDM were treated with IVIG. Ten patients were taking additional 2nd line treatments, methotrexate, azathioprine, cyclosporine, and cyclophosphamide. The main indication for starting IVIG was the failure of steroid therapy to induce remission of JDM. Twelve patients showed clinical improvement with IVIG. In these patients, the corticosteroid dose was reduced by > 50% for > 3 months without clinical or biochemical flare. Nine of these 12 patients had IVIG alone as a 2nd line agent, whereas 3 patients were treated with additional agents. Six patients remained steroid dependent; they subsequently required multiple agents to induce remission of JDM. CONCLUSION: Most steroid dependent or steroid resistant patients in our clinic were able to markedly reduce their dose of corticosteroid with the addition of IVIG. Given the retrospective nature of our data and the fact that multiple agents were sometimes used together, it will be important to confirm these findings in a controlled trial.

Antirheumatic Agents↗
PubMed2000

Rheumatic disorders of the hand and wrist in childhood and adolescence.

Rheumatic diseases are common in the pediatric population. Because the hand surgeon is often the first specialist to whom children with rheumatic disease involving the upper extremity are referred, it is important they are aware of the wide variety of disorders that can present with joint complaints to facilitate prompt referral and treatment of these patients.

Adolescent↗