http://www.ped-rheum.com The latest research articles published by Pediatric Rheumatology 2010-02-11T00:00:00Z This is an RSS newsfeed from BioMed Central It is intended to be used with an RSS reader. For more information about RSS newsfeeds from BioMed Central, visit http://www.biomedcentral.com/info/about/rss/ We report on pediatric patient with clinical and laboratory evidence of pancreatitis at onset of atypical Kawasaki disease (KD). In KD pancreatic inflammation was described previously, but clinical pancreatitis was rarely reported and its true incidence is unknown.In febrile pediatric patients suspected to have KD, but not fulfilling classical diagnostic criteria, signs of pancreatic inflammation may help in making correct diagnosis. Further analysis of cases with atypical KD developing pancreatitis may reveal if signs of pancreatic inflammation can be used as supportive diagnostic finding. http://www.ped-rheum.com/content/8/1/8 Dragan Prokic Goran Ristic Zoran Paunovic Srdjan Pasic Pediatric Rheumatology 2010, 8:8 2010-02-11T00:00:00Z doi:10.1186/1546-0096-8-8 Pediatric Rheumatology 1546-0096 8 8 2010-02-11T00:00:00Z XML ObjectivesTo examine the prescribing habits of NSAIDs among pediatric medical and surgical practitioners, and to examine concerns and barriers to their use. Methods: A sample of 1289 pediatricians, pediatric rheumatologists, sports medicine physicians, pediatric surgeons and pediatric orthopedic surgeons in the United States and Canada were sent an email link to a 22-question web-based survey. Results: 338 surveys (28%) were completed, 84 were undeliverable. Of all respondents, 164 (50%) had never prescribed a selective cyclooxygenase-2 (COX-2) NSAID. The most common reasons for ever prescribing an NSAID were musculoskeletal pain, soft-tissue injury, fever, arthritis, fracture, and headache. Compared to traditional NSAIDs, selective COX-2 NSAIDs were believed to be as safe (42%) or safer (24%); have equal (52%) to greater efficacy (20%) for pain; have equal (59%) to greater efficacy (15%) for inflammation; and have equal (39%) to improved (44%) tolerability. Pediatric rheumatologists reported significantly more frequent abdominal pain (81% vs. 23%), epistaxis (13% vs. 2%), easy bruising (64% vs. 8%), headaches (21% vs. 1%) and fatigue (12% vs. 1%) for traditional NSAIDs than for selective COX-2 NSAIDs. Prescribing habits of NSAIDs have changed since the voluntary withdrawal of rofecoxib and valdecoxib; 3% of pediatric rheumatologists reported giving fewer traditional NSAID prescriptions, and while 57% reported giving fewer selective COX-2 NSAIDs, 26% reported that they no longer prescribed these medications. Conclusions: Traditional and selective COX-2 NSAIDs were perceived as safe by pediatric specialists. The data were compared to the published pediatric safety literature. http://www.ped-rheum.com/content/8/1/7 Deborah Levy Lisa Imundo Pediatric Rheumatology 2010, 8:7 2010-02-04T00:00:00Z doi:10.1186/1546-0096-8-7 Pediatric Rheumatology 1546-0096 8 7 2010-02-04T00:00:00Z XML We report a 14-year-old girl with juvenile dermatomyositis (JDM) complicated by severe inflammatory calcinosis successfully treated with thalidomide. She was diagnosed as JDM when she was 4 years old after a few months of increasing lethargy, muscle pain, muscle weakness, and rash. During three months, clinical manifestations and abnormal laboratory findings were effectively treated with oral prednisolone. However, calcinosis was recognized 18 months after disease onset. Generalized calcinosis rapidly progressed with high fever, multiple skin/subcutaneous inflammatory lesions, and increased level of CRP. Fifty mg/day (1.3 mg/kg day) of oral thalidomide was given for the first four weeks, and then the dose was increased to 75 mg/day. Clinical manifestations subsided, and inflammatory markers had clearly improved. Frequent high fever and local severe pain with calcinosis were suppressed. The levels of FDP-E, IgG, and tryglyceride, which were all elevated before the thalidomide treatment, were gradually returned to the normal range. Over the 18 months of observation up to the present, she has had no inflammatory calcinosis, or needed any hospitalization, although established calcium deposits still remain. Her condition became painless, less extensive and less inflammatory with the CRP level below 3.08 mg/dL. Recent examination by whole-body 18F-FDG-PET-CT over the 15 months of thalidomide treatment demonstrated fewer hot spots around the subcutaneous calcified lesions. http://www.ped-rheum.com/content/8/1/6 Takako Miyamae Fumie Sano Remi Ozawa Tomoyuki Imagawa Yoshiaki Inayama Shumpei Yokota Pediatric Rheumatology 2010, 8:6 2010-02-04T00:00:00Z doi:10.1186/1546-0096-8-6 Pediatric Rheumatology 1546-0096 8 6 2010-02-04T00:00:00Z XML A 6 year old female with symptoms of small bowel obstruction underwent an exploratory laparotomy which revealed widespread evidence of inflammatory fibrotic adhesions involving the jejunal mesentery. In view of persistent growth failure, chronic anaemia, elevated acute phase reactants and imaging evidence of a diffuse progressive inflammatory process, the child was treated with corticosteroids and methotrexate with complete response. The literature on juvenile idiopathic sclerosing mesenteritis has been reviewed. http://www.ped-rheum.com/content/8/1/5 Vijay Viswanathan Kevin Murray Pediatric Rheumatology 2010, 8:5 2010-01-21T00:00:00Z doi:10.1186/1546-0096-8-5 Pediatric Rheumatology 1546-0096 8 5 2010-01-21T00:00:00Z XML A 12-year-old girl developed influenza B virus infection proven by typical symptoms and detection of the virus in a nasopharyngeal swab by culture and PCR. Two weeks later she developed an otherwise unexplained transient oligoarthritis of small joints of the left foot. Influenza viruses may be a hitherto underappreciated cause of a post-infectious arthritis. http://www.ped-rheum.com/content/8/1/4 Normi Bruck Manfred Gahr Frank Pessler Pediatric Rheumatology 2010, 8:4 2010-01-14T00:00:00Z doi:10.1186/1546-0096-8-4 Pediatric Rheumatology 1546-0096 8 4 2010-01-14T00:00:00Z XML ObjectiveTo determine the methods of anesthesia currently being used by pediatric rheumatologists when performing intra-articular corticosteroid injections (IACI).Study designA questionnaire was emailed to all members of the Childhood Arthritis & Rheumatology Research Alliance, a pediatric rheumatology research network in North America. The questionnaire consisted of 11 questions ranging from procedure technique, treatments prescribed for topical anesthesia and oral analgesia, and factors that might affect procedural pain. Results: Seventy-four of 161 physicians (46%) responded to the questionnaire. On average, each physician injected 33 children (median 25, range 1-160) and 43 joints (median 30, range 1-150) yearly. Local anesthesia was used in children on average ≥ 8 years (range 2-16 years), with general anesthesia being more frequently used for younger children. All respondents used local anesthesia. The most commonly used methods of local anesthesia were EMLA® cream plus subcutaneous lidocaine (58.8%), ethyl chloride spray only (39.7%), EMLA® cream only (33.8%), subcutaneous lidocaine only (25%), and lidocaine iontophoresis only (11.8%). Buffering of the lidocaine was routinely done only 7.4% of the time. Conclusion: Although pediatric rheumatologists in North America perform IACI on a large number of patients each year, a wide variety of methods are used to deliver local anesthesia with no accepted standard of care. More studies are needed to determine the optimal method of local anesthesia delivery to minimize pain associated with IACI. http://www.ped-rheum.com/content/8/1/3 Jennifer Weiss America Uribe Peter Malleson Yukiko Kimura Pediatric Rheumatology 2010, 8:3 2010-01-13T00:00:00Z doi:10.1186/1546-0096-8-3 Pediatric Rheumatology 1546-0096 8 3 2010-01-13T00:00:00Z XML Background: This study aimed to describe the proportion of patients with juvenile idiopathic arthritis (JIA) who had experienced an unsuccessful transfer from a pediatric rheumatology team to an adult rheumatologist and to compare the characteristics of those who achieved successful transfer to those who did not. Methods: We conducted a systematic chart review of all patients with JIA who attended their final Montreal Children's Hospital JIA clinic appointment between 1992 and 2005. We tracked these patients for the two years after transfer to an adult rheumatologist. We then compared characteristics of patients with successful and unsuccessful transfers of care. Variables pertaining to disease characteristics, disease severity and psychosocial factors were examined. Univariate analyses were performed to determine if any single factor was associated with the outcome of unsuccessful transfer of care. Results: 52% of patients fulfilled our criteria for unsuccessful transfer. Of the variables tested, an active joint count (AJC) of zero at last visit was associated with the outcome of unsuccessful transfer (OR = 2.67 (CI 1.16-6.16; p = 0.0199)). Conclusions: Despite the presence of a coordinated process of transfer from pediatric to adult health care for the majority of the patients in this study, there was a high rate of unsuccessful transfer and/or sustained follow up which is disheartening. We found that patients with less active disease at the time of transfer, as indicated by a lower AJC, were more likely to be lost to follow up. Recent literature suggests that even in the least severe categories of JIA, 50% of patients persist with active disease into adulthood. Thus educating all JIA patients about the possibility of disease flare in adulthood may improve their adherence to recommendations for sustained follow-up in the adult milieu. This may lead to improvement of longitudinal outcomes for all JIA patients. http://www.ped-rheum.com/content/8/1/2 Elizabeth Hazel Xun Zhang Ciaran Duffy Sarah Campillo Pediatric Rheumatology 2010, 8:2 2010-01-11T00:00:00Z doi:10.1186/1546-0096-8-2 Pediatric Rheumatology 1546-0096 8 2 2010-01-11T00:00:00Z XML Background: Therapy with mycophenolate mofetil (MMF) has become a valuable therapeutic option in children with autoimmune disease. MMF prescription in children with autoimmune diseases differs from that in transplant recipients in terms of different dosing regimen, and concomitant administration of other immunosuppressive medications. Recently, another formulation of the same active compound, mycophenolic acid (MPA), has become available as enteric-coated Mycophenolate Sodium (EC-MPS). Dosing and pharmacokinetics of EC-MPS in pediatric autoimmune disease have never been studied. Methods: We therefore performed a pilot study on 6 patients, who were treated with EC-MPS. All patients underwent 1-2 full 10-point pharmacokinetic (PK) profiles over a 12-hour dosing interval. We compared the results with that of 22 similar patients on MMF therapy. Results: Median EC-MPS dose was 724 mg/m2 (range 179-933 mg/m2). The MPA Area-Under-The-(Time-Concentration)-Curves (AUCs) on MMF and EC-MPS were comparable (54.4 mg × h/L on MMF and 44.0 mg × h/L on EC-MPS, n.s., Mann Whitney). After correcting for bioequivalence, the dose-normalized AUCs were also similar on both the formulations. However, PK profiles on EC-MPS were quite random, and time to maximum concentration varied from 30 minutes to 720 minutes. The concentration at six-hour correlated best with the AUC. This was different from a homogenous PK-profile on MPA. Conclusions: EC-MPS has a different PK profile from MMF. The data suggest that patients on EC-MPS must undergo a complete PK profile to assess adequate exposure. The 6-hour concentration provides an estimate of the exposure and should be targeted between 3-4 mg/L. http://www.ped-rheum.com/content/8/1/1 Guido Filler Ajay Sharma Deborah Levy Abeer Yasin Pediatric Rheumatology 2010, 8:1 2010-01-04T00:00:00Z doi:10.1186/1546-0096-8-1 Pediatric Rheumatology 1546-0096 8 1 2010-01-04T00:00:00Z XML PurposeWe investigated the etiology of acute hepatitis in three children with systemic Juvenile Idiopathic Arthritis (sJIA) taking Interleukin-1 receptor antagonist (IL1RA). Methods: Laboratory and clinical data for three children with sJIA diagnosed at ages 13 months to 8 years who developed acute hepatitis during treatment with IL1RA were reviewed for evidence of sJIA flare, infection, macrophage activation syndrome (MAS), malignancy, and drug reaction. Results: In all patients, hepatitis persisted despite cessation of known hepatotoxic drugs and in absence of known infectious triggers, until discontinuation of IL1RA. Liver biopsies had mixed inflammatory infiltrates with associated hepatocellular injury suggestive of an exogenous trigger. At the time of hepatitis, laboratory data and liver biopsies were not characteristic of MAS. In two patients, transaminitis resolved within one week of discontinuing IL1RA, the third improved dramatically in one month. Conclusions: Although sJIA symptoms improved significantly on IL1RA, it appeared that IL1RA contributed to the development of acute hepatitis. Hepatitis possibly occurred as a result of an altered immune response to a typical childhood infection while on IL1RA. Alternatively, hepatitis could have represented an atypical presentation of MAS in patients with sJIA taking IL1RA. Further investigation is warranted to determine how anti-IL1 therapies alter immune responsiveness to exogenous triggers in patients with immune dysfunction such as sJIA. Our patients suggest that close monitoring for hepatic and other toxicities is indicated when treating with IL1RA. http://www.ped-rheum.com/content/7/1/21 Scott Canna Jennifer Frankovich Gloria Higgins Michael Narkewicz S Russell Nash J Roger Hollister Jennifer Soep Leonard Dragone Pediatric Rheumatology 2009, 7:21 2009-12-22T00:00:00Z doi:10.1186/1546-0096-7-21 Pediatric Rheumatology 1546-0096 7 21 2009-12-22T00:00:00Z XML ObjectiveTo determine the population incidence of juvenile rheumatoid arthritis (JRA) in Quebec. Methods: We obtained data from Quebec's physician claims database. Incident cases were defined as having a visit for JRA in 2000, no visit in the previous 3 years, a confirmed diagnosis by an arthritis specialist, or having ≥ 2 visits to any physician for JRA, ≥ 2 months apart but within 2 years. Results: Cumulative incidence of JRA was 17.8/100,000. Mean age at diagnosis was 9.8 ± 4.6 years, 68% were female and more persons were diagnosed in winter. Subjects had a median of 10 medical visits over the first year. Conclusion: Our population based incidence estimate was similar to others. Children and adolescents with JRA are heavy users of medical care. Additional study of environmental or climate- related triggers may be warranted. http://www.ped-rheum.com/content/7/1/20 Debbie Ehrmann Feldman Sasha Bernatsky Michelle Houde Pediatric Rheumatology 2009, 7:20 2009-11-19T00:00:00Z doi:10.1186/1546-0096-7-20 Pediatric Rheumatology 1546-0096 7 20 2009-11-19T00:00:00Z XML