http://www.ped-rheum.com The latest research articles published by Pediatric Rheumatology 2012-02-02T00:00:00Z Dr. Henrickson presents a thoughtful three part analysis of current challenges to pediatric rheumatologists regarding barriers to access to care for children with rheumatic disease and to achieving optimal clinical outcomes. 1-3 He then presents some possible solutions. He has initiated an important conversation that has the potential to impact the future of our discipline. http://www.ped-rheum.com/content/10/1/5 Carol Lindsley Pediatric Rheumatology 2012, null:5 2012-02-02T00:00:00Z doi:10.1186/1546-0096-10-5 /content/figures/1546-0096-10-5-toc.gif Pediatric Rheumatology 1546-0096 ${item.volume} 5 2012-02-02T00:00:00Z PDF Background: Teaching of motor skills is fundamental to physical therapy practice. In order to optimize the benefits of these teaching and training efforts, various forms of patient education material are developed and handed out to patients. One very important fact has been overlooked. While comparative effectiveness of various modes of instruction has been studied in adults, attention has not been paid to the fact that learning capabilities of children are different from that of adults. The intent of the present study is to compare the effectiveness of video and handout mode of instructions specifically on children. Methods: A total of 115 normal elementary-age children aged 10 to 12 years of age were studied. The children were randomized into two groups: A) the video group, and B) the handout group. The video group viewed the video for physical therapy exercises while the handout group was provided with paper handouts especially designed according to the readability of their age group. Results: Statistical analysis using the student's't' test showed that subjects of both the video and handout groups exhibited equal overall performance accuracy. There was no significant difference between the groups both in acquisition and retention accuracy tests. Conclusion: The findings of the present study suggest that if the readability and instructional principles applicable to different target age groups are strictly adhered to, then both video as well as handout modes of instructions result in similar feedback and memory recall in ten to twelve year-old children. Principles of readability related to the patient age are of utmost importance when designing the patient education material. These findings suggest that the less expensive handouts can be an effective instructional aid for teaching exercises to children with various neuromuscular, rheumatic, and orthopedics conditions and the most costly videotape techniques are not necessarily better. http://www.ped-rheum.com/content/10/1/4 Garima Gupta Stuti Sehgal Pediatric Rheumatology 2012, null:4 2012-01-30T00:00:00Z doi:10.1186/1546-0096-10-4 /content/figures/1546-0096-10-4-toc.gif Pediatric Rheumatology 1546-0096 ${item.volume} 4 2012-01-30T00:00:00Z PDF Background: Children with physical disabilities may have an increased risk for obesity and obesity might be a risk factor for inflammatory arthritis. The aims of this study were: to determine the prevalence of obesity in children and adolescents with juvenile idiopathic arthritis (JIA), and to examine the association between obesity and disease activity in this population. Findings: A cross-sectional analysis of all patients with JIA attending a pediatric rheumatology clinic, between October 2009 and September 2010, was performed. A linear regression model was used to explore the association between obesity and disease activity in patients with JIA. A total of 154 subjects were included in the analysis; median age was 10.6 years, 61% were female, and 88% were white. Obesity was found in 18%, 12% were overweight, and 3% were underweight. There was no association between obesity and JADAS-27 (Juvenile Arthritis Disease Activity Score 27), physician's assessment of disease activity, parent's assessment of child's well-being, erythrocyte sedimentation rate, number of active joints, or C-reactive protein (p-value range 0.10 to 0.95). Conclusions: Although 18% of patients with JIA were obese, we did not find an association between obesity and disease activity. As obesity confers an additional health risk in children with arthritis, addressing this co-morbidity should be a health priority in patients with JIA. Future studies are necessary to further explore potential associations between obesity, development of JIA, and disease activity. http://www.ped-rheum.com/content/10/1/3 Christina Pelajo Jorge Lopez-Benitez Laurie Miller Pediatric Rheumatology 2012, null:3 2012-01-12T00:00:00Z doi:10.1186/1546-0096-10-3 /content/figures/1546-0096-10-3-toc.gif Pediatric Rheumatology 1546-0096 ${item.volume} 3 2012-01-12T00:00:00Z PDF OBJECTIVEBehcet's is an idiopathic multi-organ syndrome, which may have onset during childhood. Vascular involvement is uncommon, with rarely reported coronary aneurysm formation. We present a case report of a teenager girl who developed recalcitrant life-threatening Behcet's vasculitis, involving both small and large venous and arterial systems including a giant coronary aneurysm.CASE REPORTDe-identified data were collected retrospectively in case report format. Although our sixteen year old female with Behcet's vasculitis had resolution of many arterial aneurysms, she had persistent venous thrombosis of large vessels, as well as persistent, giant arterial aneurysms requiring intra-arterial coiling of a lumbar artery and coronary bypass grafting despite intensive immunosuppression including glucocorticoids, cyclophosphamide, infliximab, methotrexate, azathioprine and intravenous immunoglobulin. Conclusions: Vascular manifestations may be seen in Behcet's syndrome, including asymptomatic coronary aneurysm, which may be refractory to immunosuppression and ultimately require surgical intervention. Increased awareness is essential for prompt diagnosis and management. http://www.ped-rheum.com/content/10/1/2 Philip Kahn Yusuf Yazici Michael Argilla Monvadi Srichai Deborah Levy Pediatric Rheumatology 2012, null:2 2012-01-06T00:00:00Z doi:10.1186/1546-0096-10-2 /content/figures/1546-0096-10-2-toc.gif Pediatric Rheumatology 1546-0096 ${item.volume} 2 2012-01-06T00:00:00Z PDF Juvenile polyarteritis nodosa (PAN) is a rare, necrotizing vasculitis, primarily affecting small to medium-sized muscular arteries. Cardiac involvement amongst patients with PAN is uncommon and reports of coronary artery aneurysms in juvenile PAN are exceedingly rare. We describe a 16 year old girl who presented with fever, arthritis and two giant coronary artery aneurysms, initially diagnosed as atypical Kawasaki disease and treated with IVIG and methylprednisolone. Her persistent fevers, arthritis, myalgias were refractory to treatment, and onset of a vasculitic rash suggested an alternative diagnosis. Based on angiographic abnormalities, polymyalgia, hypertension and skin involvement, this patient met criteria for juvenile PAN. She was treated with six months of intravenous cyclophosphamide and high dose corticosteroids for presumed PAN related coronary vasculitis. Maintenance therapy was continued with azathioprine and the patient currently remains without evidence of active vasculitis. She remains on anticoagulation for persistence of the aneurysms. This case illustrates a rare and unusual presentation of giant coronary artery aneurysms in the setting of juvenile PAN. http://www.ped-rheum.com/content/10/1/1 Therese Canares Dawn Wahezi Kanwal Farooqi Robert Pass Norman Ilowite Pediatric Rheumatology 2012, null:1 2012-01-05T00:00:00Z doi:10.1186/1546-0096-10-1 /content/figures/1546-0096-10-1-toc.gif Pediatric Rheumatology 1546-0096 ${item.volume} 1 2012-01-05T00:00:00Z XML Background: To investigate the prevalence of cumulative organ damage among Egyptian children with juvenile-onset systemic lupus erythematosus (jSLE) and the relationships between the organ damage and the demographic data, clinical variables, and disease activity. Methods: A total of 148 patients with jSLE have been followed in the pediatric rheumatology clinic and section at Cairo University. These patients were evaluated by retrospective chart review. The organ system damage due to SLE was measured using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). Risk factors for damage were also studied including demographic criteria as well as clinical and laboratory manifestations. Results: Overall, 43.9% of the patients had damage within a mean of 6.57 ± 3.59 years of disease diagnosis. Neuropsychiatric (NPS-21%) and renal (16.9%) system involvement were observed most frequently, followed by cardiovascular (11.5%), skin (9.5%), pulmonary (6.1%), and ocular (4.8%), with a mean SDI score of 0.93 ± 1.37. In our study, the presence of neuropsychiatric manifestations at diagnosis showed the strongest association with the presence of later disease damage.The number of SLE diagnostic criteria at presentation was strongly associated with the total SDI score, and the renal damage was significantly more prevalent in patients with age at disease diagnosis below 10 years of age. A higher mean disease duration was found in patients with musculoskeletal damage. Conclusion: We found that cumulative organ damage, as measured by the SDI, was present in 43.9% of Egyptian patients with juvenile-onset SLE. The damage was significantly more likely in patients who had more SLE diagnostic criteria at time of disease presentation and NPS manifestations at the time of diagnosis. http://www.ped-rheum.com/content/9/1/36 Samia Salah Hala Lotfy Abir Mokbel Ahmed Kaddah Nouran Fahmy Pediatric Rheumatology 2011, null:36 2011-12-09T00:00:00Z doi:10.1186/1546-0096-9-36 /content/figures/1546-0096-9-36-toc.gif Pediatric Rheumatology 1546-0096 ${item.volume} 36 2011-12-09T00:00:00Z XML Background: For adolescents with a diagnosis of lifelong chronic illness, mastery of self-management skills is a critical component of the transition to adult care. This study aims to examine self-reported medication adherence and self-care skills among adolescents with chronic rheumatic disease. Methods: Cross-sectional survey of 52 adolescent patients in the Pediatric Rheumatology Clinic at UCSF. Outcome measures were self-reported medication adherence, medication regimen knowledge and independence in health care tasks. Predictors of self-management included age, disease perception, self-care agency, demographics and self-reported health status. Bivariate associations were assessed using the Student's t-test, Wilcoxon rank sum test and Fisher exact test as appropriate. Independence in self-management tasks were compared between subjects age 13-16 and 17-20 using the chi-squared test. Results: Subjects were age 13-20 years (mean 15.9); 79% were female. Diagnoses included juvenile idiopathic arthritis (44%), lupus (35%), and other rheumatic conditions (21%). Mean disease duration was 5.3 years (SD 4.0). Fifty four percent reported perfect adherence to medications, 40% reported 1-2 missed doses per week, and 6% reported missing 3 or more doses. The most common reason for missing medications was forgetfulness. Among health care tasks, there was an age-related increase in ability to fill prescriptions, schedule appointments, arrange transportation, ask questions of doctors, manage insurance, and recognize symptoms of illness. Ability to take medications as directed, keep a calendar of appointments, and maintain a personal medical file did not improve with age. Conclusions: This study suggests that adolescents with chronic rheumatic disease may need additional support to achieve independence in self-management. http://www.ped-rheum.com/content/9/1/35 Erica Lawson Aimee Hersh Mark Applebaum Edward Yelin Megumi Okumura Emily von Scheven Pediatric Rheumatology 2011, null:35 2011-12-06T00:00:00Z doi:10.1186/1546-0096-9-35 /content/figures/1546-0096-9-35-toc.gif Pediatric Rheumatology 1546-0096 ${item.volume} 35 2011-12-06T00:00:00Z XML Uveitis treatment involves topical corticosteroids along with cycloplegic-mydriatics. Particularly severe cases may require systemic corticosteroids and immunosuppressive drugs. Vernal keratoconjunctivitis (VKC) treatment consists of a brief period of topical corticosteroids and/or cyclosporine. In patients refractory to traditional treatment, the use of 0.1% topical ophtalmic FK- 506 (tacrolimus) ointment has been occasionally reported.This is the first report of the coexistence of uveitis and VKC. The documented response to topical tacrolimus eyedrop of uveitis and VKC is also of interest, in particular since to our knowledge there are no published reports on its clinical use in uveitis. http://www.ped-rheum.com/content/9/1/34 Andrea Taddio Rolando Cimaz Roberto Caputo Cinzia de Libero Laura Di Grande Francesca Mori Gabriele Simonini Elio Novembre Neri Pucci Pediatric Rheumatology 2011, null:34 2011-11-02T00:00:00Z doi:10.1186/1546-0096-9-34 /content/figures/1546-0096-9-34-toc.gif Pediatric Rheumatology 1546-0096 ${item.volume} 34 2011-11-02T00:00:00Z XML Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with pulmonary hemorrhage is rare in childhood. Standard treatment includes corticosteroids and cyclophosphamide (CYC), which is associated with a high level of toxicity. We report a white female with ANCA positive pulmonary hemorrhage who was treated with cyclophosphamide (CYC) and rituximab (RTX) combination therapy. http://www.ped-rheum.com/content/9/1/33 Emily Baird Thomas Lehman Stefan Worgall Pediatric Rheumatology 2011, null:33 2011-10-27T00:00:00Z doi:10.1186/1546-0096-9-33 /content/figures/1546-0096-9-33-toc.gif Pediatric Rheumatology 1546-0096 ${item.volume} 33 2011-10-27T00:00:00Z XML There is a debate whether post-streptococcal reactive arthritis (PSRA) is a separate entity or a condition on the spectrum of acute rheumatic fever (ARF). We believe that PSRA is a distinct entity and in this paper we review the substantial differences between PSRA and ARF. We show how the demographic, clinical, genetic and treatment characteristics of PSRA differ from ARF. We review diagnostic criteria and regression formulas that attempt to classify patients with PSRA as opposed to ARF. The important implication of these findings may relate to the issue of prophylactic antibiotics after PSRA. However, future trials will be necessary to conclusively answer that question. http://www.ped-rheum.com/content/9/1/32 Yosef Uziel Liat Perl Judith Barash Philip Hashkes Pediatric Rheumatology 2011, null:32 2011-10-20T00:00:00Z doi:10.1186/1546-0096-9-32 /content/figures/1546-0096-9-32-toc.gif Pediatric Rheumatology 1546-0096 ${item.volume} 32 2011-10-20T00:00:00Z XML