Biomedical subjects

Robert P Sundel

Publications and source records attributed to Robert P Sundel.

12 recordsLinked to original sources

PubMed2006

Patients with juvenile psoriatic arthritis comprise two distinct populations.

OBJECTIVE: Psoriatic arthritis (PsA) in children is clinically heterogeneous. We examined a large population of children with juvenile PsA for evidence of phenotypic clustering that could suggest the presence of distinct clinical entities. METHODS: We reviewed the medical records of 139 patients meeting the Vancouver criteria for juvenile PsA. To identify segregation into phenotypic groups, we compared younger patients with their older counterparts and subjected the whole population to 2-step cluster analysis. RESULTS: Among patients with juvenile PsA, the age at onset is biphasic, with peaks occurring at approximately 2 years of age and again in late childhood. Compared with children ages 5 years and older, younger patients are more likely to be female, exhibit dactylitis and small joint involvement, and express antinuclear antibodies. Progression to polyarticular disease (>or=5 joints) is more common in younger children, although joint involvement remains oligoarticular in the majority of children. In contrast, older patents tend to manifest enthesitis, axial joint disease, and persistent oligoarthritis. Uveitis is equally represented in both age groups. Despite a higher utilization of methotrexate therapy, younger patients required, on average, more than twice as long to achieve clinical remission (23 months versus 9.2 months; P = 0.044). Cluster analysis identified largely overlapping subgroups but suggested that the presence of dactylitis, rather than age, has the greatest capacity to predict essential features of the clinical phenotype. CONCLUSION: Juvenile PsA comprises 2 distinct populations of patients. Although the pathophysiologic correlate of this finding remains undefined, future studies should avoid the assumption that PsA in childhood constitutes a single etiologic entity.

Age of Onset↗

PubMed2005

Coronary artery dilation among patients presenting with systemic-onset juvenile idiopathic arthritis.

OBJECTIVE: To evaluate coronary artery diameters among patients presenting with systemic-onset juvenile idiopathic arthritis (SoJIA). METHODS: Fifty cases of SoJIA were reviewed. At the time of initial presentation with fever, 12 patients had echocardiograms that included a complete evaluation of the coronary arteries. A single reviewer measured the diameters of the left main, proximal left anterior descending, and proximal right coronary arteries. Body surface area-adjusted z scores were calculated with respect to a normative population. RESULTS: Coronary artery dilation (z score: >2) was observed for 5 of the 12 patients with SoJIA who had echocardiograms performed at the time of presentation with fever. No patient developed a coronary artery aneurysm, and all of the coronary artery z scores normalized within 4 months. Only 2 of the 5 patients with coronary artery z scores of >2 fulfilled the clinical criteria for Kawasaki disease, the most commonly recognized cause of coronary artery dilation among children. CONCLUSIONS: Children presenting with SoJIA may have coronary artery dilation similar to that observed for children with Kawasaki disease. These data suggest that the presence of coronary artery dilation on initial echocardiograms for patients with fever does not exclude the diagnosis of SoJIA.

Arthritis, Juvenile↗

PubMed2005

Vasculitis in children.

This article provides a general overview of vasculitis, situations in which the diagnosis should be considered, diagnostic methods, and therapeutic considerations. Details and treatments unique to specific vasculitides are also reviewed.

Child↗

PubMed2005

MRI features of Lyme arthritis in children.

OBJECTIVE: Oligoarthritis is the most common manifestation of late Lyme disease in children. Considerable overlap can occur in the clinical presentation of Lyme arthritis and acute septic arthritis. Early differentiation is critical, given the disparate therapeutic implications; Lyme arthritis is treated with outpatient oral antibiotics, while septic arthritis requires hospitalization, IV antibiotics, and, often, surgical drainage. We wanted to identify MRI features that may distinguish Lyme arthritis from septic arthritis in children. MATERIALS AND METHODS: Knee MR images in 11 children with Lyme arthritis and 7 with septic arthritis, with a mean age 10.6 years old and 11.7 years old, respectively, were reviewed by a radiologist blinded to the final diagnosis. Joint effusion size, synovial thickness, adenopathy, subcutaneous, marrow, and muscle edema on MRI; and clinical parameters including age, sex, fever, WBC, erythrocyte sedimentation rate, C-reactive protein, and joint fluid WBC in the two patient groups were compared using univariate and multivariate analyses. RESULTS: Subcutaneous edema was seen in all septic arthritis patients but in only one of 11 patients with Lyme arthritis (p < 0.01). Myositis and adenopathy were present in all Lyme arthritis patients and two of seven patients with septic arthritis (both p < 0.01). No significant difference was present in synovial thickness, marrow edema, or joint fluid size. There were no statistically significant differences in the clinical parameters assessed. CONCLUSION: Our results identified three MRI features, specifically, myositis, adenopathy, and lack of subcutaneous edema, that strongly suggest the diagnosis of Lyme arthritis rather than septic arthritis in children with acute inflammation of the knee. Awareness of these characteristic MRI features may avoid unnecessary invasive procedures and cost.

Arthritis, Infectious↗

PubMed2004

Thalidomide for severe systemic onset juvenile rheumatoid arthritis: A multicenter study.

Thirteen children with difficult systemic onset juvenile rheumatoid arthritis were treated with thalidomide. At 6 months, 11 of the 13 were able to reduce their use of prednisone ( P < .002), with a concurrent improvement in erythrocyte sedimentation rate ( P < .0001) and an increase in hemoglobin level ( P < 0.005). Juvenile rheumatoid arthritis improvement scores >/=50% were obtained by 10 of the 13 children.

Arthritis, Juvenile↗

PubMed2003

Corticosteroids in the initial treatment of Kawasaki disease: report of a randomized trial.

OBJECTIVE: We conducted a prospective randomized trial to determine whether the addition of corticosteroids to intravenous immunoglobulin (IVIG) might improve outcomes in Kawasaki disease (KD). STUDY DESIGN: Subjects were randomized to receive IVIG, 2 gm/kg over 10 hours, with or without pulsed-dose intravenous methylprednisolone (IVMP), 30 mg/kg. All patients received standard doses of aspirin (ASA). Groups were similar in baseline demographic and laboratory data. RESULTS: Patients in the IVMP plus ASA/IVIG group, compared with those in the ASA/IVIG alone group, had a shorter mean duration of fever >/=38.3 degrees C after initiation of therapy (1.0 +/- 1.3 vs 2.4 +/- 1.9 days, mean +/- SD, P =.012), shorter hospital stays (1.9 +/- 0.7 vs 3.3 +/- 2.1 days, P =.010), and at six weeks, lower mean erythrocyte sedimentation rate (11.1 +/- 5.7 vs 19.4 +/- 12.4, P =.027) and median c-reactive protein (0.03 vs 0.08, P =.011, Wilcoxon). No significant differences between treatment groups were noted in coronary dimensions, but statistical power was limited. IVMP was well tolerated; transient hypertension developed in one child, but it did not require treatment. CONCLUSIONS: Treatment of acute KD with IVMP plus ASA/IVIG, compared with ASA/IVIG alone, resulted in faster resolution of fever, more rapid improvement in markers of inflammation, and shorter length of hospitalization. Adverse effects were infrequent. Steroid therapy should be further assessed in a multicenter, placebo-blind trial.

Child↗

PubMed2003

Rituximab therapy for multisystem autoimmune diseases in pediatric patients.

OBJECTIVE: To evaluate the effects of rituximab (anti-CD20 monoclonal antibody) on the disease course in pediatric patients with multisystem autoimmune diseases. METHODS: Four patients with multisystem autoimmune diseases refractory to conventional immunosuppressive medications, each with central nervous system (CNS) involvement, were treated with four weekly infusions of rituximab. Their clinical and laboratory responses were evaluated. RESULTS: Each of the patients had improvement in clinical symptoms and laboratory parameters. One patient with autoimmune cytopenias and autoimmune CNS and peripheral nervous system disease had resolution of the cytopenias and marked improvement in neurologic symptoms; he currently receives no immunosuppressive medications. Two half-siblings with lymphoplasmacytic colitis, pulmonary nodules, and CNS disease had improvement of their symptoms. A fourth patient with chorea and seizures secondary to primary antiphospholipid antibody syndrome had improvement in fine and gross motor function and reduced seizure frequency. There were no serious adverse events. CONCLUSIONS: The biologic response modifier rituximab, designed to eliminate B lymphocytes, was safe and effective in four pediatric patients with multisystem autoimmune disorders. It appears to be beneficial in autoimmune conditions presumably mediated by a variety of B-cell-related mechanisms, and may decrease or eliminate the need for other immunosuppressive medications.

Adolescent↗

PubMed2003

Physical and psychosocial health in children who have had Kawasaki disease.

OBJECTIVE: The purpose of this study was to examine the physical and psychosocial well-being of children who have had Kawasaki disease (KD), including the influence of coronary artery status on health and health perceptions. METHODS: The Child Health Questionnaire (CHQ) measures overall physical and psychosocial well-being in children 5 to 18 years. To study the long-term impact of KD on overall health status, we mailed the CHQ to patients without a history of coronary artery abnormalities (normal group), with regressed aneurysms (regressed group), with current coronary aneurysms <8 mm (mild-moderate aneurysm group), and with giant aneurysms >or=8 mm (giant group). RESULTS: Of 201 questionnaires mailed, 174 were delivered and 110 (63%) were completed. Median age (range) at completion was 10.5 years (5.1-17.9 years) and at illness onset was 3.1 years (0.2-12 years). There were no significant differences in psychosocial summary scores in any of the Kawasaki groups when compared with the US population sample. Physical summary scores were also similar to the US population sample in the normal coronary, mild-moderate aneurysm, and regressed aneurysm groups. However, the giant aneurysm group had significantly lower physical summary scores compared with the US population sample. Among subscales, general health perceptions in the KD groups were lower than in the US population sample, reaching statistical significance in all but the mild to moderate aneurysm group. In addition, parents whose children have had KD reported a higher proportion of anxiety issues, allergies, and orthopedic/bone/joint issues in their children than did the general US population sample. We did not find any difference in the incidence of attentional, behavioral, or learning issues when compared with the US population sample. CONCLUSIONS: KD patients without coronary artery aneurysms were similar to the general population in their general physical and psychosocial health. However, the parents of children in all KD groups reported lower general health perceptions than parents in the US population sample, suggesting that long-term concerns about their children's health exist regardless of overall health status. In addition, children with giant coronary artery aneurysms had lower overall physical summary scores.

Adaptation, Psychological↗

PubMed2003

Raynaud's phenomenon in children: a retrospective review of 123 patients.

OBJECTIVE: To examine the clinical presentation and disease associations of Raynaud's phenomenon (RP) in children and adolescents. METHODS: A systematic retrospective chart review was conducted of 123 cases drawn from 2 computerized databases at the Children's Hospital of Boston. Participants aged <19 years with episodic reversible color changes in the extremities were examined. Case records were analyzed for clinical presentation, disease associations, and physical examination and laboratory findings. RESULTS: In contrast to the findings of smaller pediatric series reported to date, the large majority of our patients-approximately 70%-did not have a recognized underlying connective tissue disease. For both primary and secondary RP, approximately 80% of patients were female, and mean age of onset was similar in the 2 groups. Biphasic or triphasic color changes were less common than monophasic changes and were no more common in secondary than primary RP. Findings predictive of secondary RP were limited to the presence of antinuclear antibodies and abnormal nailfold capillaries. Antiphospholipid antibodies were found at some time in at least 21% of patients with both primary and secondary RP. CONCLUSIONS: RP in children, as in adults, principally affects girls and is frequently free of association with connective tissue disease. Antinuclear antibody positivity and abnormal nailfold capillaries correlate with secondary disease. Antiphospholipid antibodies are surprisingly common, a new finding of uncertain implications.

Adolescent↗

PubMed2002

Update on the treatment of Kawasaki disease in childhood.

Intravenous immunoglobulin (IVIG) treatment for Kawasaki disease (KD), first discovered almost 20 years ago, dramatically changed the management and prognosis of the condition. Although standard Japanese Ministry of Health criteria suggest that current treatment is more than 95% effective at preventing coronary artery changes, echocardiographic measurements adjusted for body size, imply a far higher incidence of coronary artery dilitation despite prompt therapy. If one also considers data on chronic alterations in endothelial function after KD, then more effective approaches to the management of acute and recurrent KD are needed. A variety of possible adjunct therapies--most notably high-dose corticosteroids--currently are being studied to determine whether better long-term outcomes may be achieved than with IVIG alone.

Abciximab↗

PubMed2002

Aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis.

BACKGROUND: Long-term consequences of juvenile dermatomyositis (JDM) include onset of calcinosis and subsequent functional impairment. Historic incidence of calcinosis has been reported between approximately 23% and 70%. Recent reports note improved outcome with high-dose steroids, yet the incidence of calcinosis has remained above 30%. OBJECTIVE: We attempted to determine whether rapid, aggressive disease management can prevent calcinosis and improve functional outcome. METHODS: Medical records of children with JDM managed at a pediatric medical center during a 10-year period were reviewed to determine (1) interval between onset of symptoms and diagnosis, (2) treatment modality, and (3) functional outcome and presence of calcinosis. RESULTS: A total of 21 female and 14 male subjects diagnosed with JDM met inclusion criteria, with a mean age of diagnosis of 7.6 +/- 3.9 years. Mean time from onset of symptoms to treatment was 6.6 +/- 8.2 months. Pulse intravenous methylprednisone (30 mg/kg daily) or high-dose prednisone was used in 31 of 35 patients. Patients who failed to respond within 6 weeks were started on a regimen of methotrexate (23/35). At follow-up, 5 patients had mild calcinosis (14%). Onset of calcinosis was associated with a longer time to diagnosis and treatment (30.6 vs 6 months, P =.003), a longer duration of elevated muscle enzymes (34 vs 12.6 months, P =.03), and longer disease duration (42.8 vs 22.2 months, P =.05). CONCLUSION: Stepwise, aggressive treatment directed at achieving rapid and complete control of muscle inflammation is highly successful in minimizing the long range sequelae of JDM, including calcinosis.

Calcinosis↗

PubMedDate not supplied

Fibroblastic rheumatism: case report and review of the literature.

A previously healthy 7-year-old boy presented with polyarthritis and skin lesions. Multiple, skin- to pink-colored, firm papules were noted on the periungual areas, hands, feet, and nose. There was swelling of the proximal interphalangeal joints, wrists, elbows, ankles, and knees. A skin biopsy specimen revealed dermal fibrosis and interspersed histiocytes and lymphocytes. These findings were consistent with fibroblastic rheumatism, a condition characterized by cutaneous nodules and a symmetric polyarthritis. He was treated with methotrexate and corticosteroids with improvement in the symptoms of his arthritis and skin lesions. This early treatment was beneficial in our patient.

Anti-Inflammatory Agents↗