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Introduction: Methotrexate (MTX) is the drug of choice for juvenile idiopathic arthritis. Its clinical efficacy is limited due to the development of adverse effects (AEs)., Patients and Methods: A retrospective observational study was conducted on the AEs associated with MTX therapy in children diagnosed with juvenile idiopathic arthritis followed-up in a tertiary hospital between 2008 and 2016., Results: The study included a total of 107 patients, of whom 71 (66.3%) were girls (66.3%). The median age at diagnosis was 6.4 years (IQR 3.1-12.4), with a median follow-up of 45.7 months (IQR 28.8-92.4). There were 48 patients (44.9%) with oligoarthritis, and 26 children (24.3%) with rheumatoid-factor negative polyarthritis. Of these, 52/107 (48.6%) developed AEs, with the most frequent being gastrointestinal symptoms (35.6%) and behavioural problems (35.6%). An age older than 6 years at the beginning of therapy increased the risk of developing AEs, both in the univariate (OR=3.5; 95% CI: 1.5-7.3) and multivariate (12% increase per year) analyses. The doses used, administration route, or International League of Associations for Rheumatology (ILAR) classification, were not associated with the development of AEs. Twenty children required a dosage or route of administration modification, which resolved the AE in 11 (55%) cases. MTX was interrupted due to the development of AEs in 37/107 patients (34.6%), mainly due to increased plasma transaminases (n=14, 37.8%), gastrointestinal symptoms (n=9, 24.3%) and behavioural problems (n=6, 16.3%)., Conclusions: MTX is the therapy of choice for patients with juvenile idiopathic arthritis, but 50% of the children develop some form of AE. Although the AEs are not severe, they lead to interruption of therapy in 35% of the children., (Copyright © 2019 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction: Synovial fluid (SF) analysis is an important tool for the diagnosis of patients with juvenile idiopathic arthritis (JIA)., Patients and Methods: A retrospective analysis was carried out of cytological features of SF samples obtained from patients with JIA during the period 2008-2016., Results: A total of 102 SF samples from 59 patients were analysed. JIA was more common in females (66%). The most frequent form was persistent oligoarticular JIA (52.5%). The median age at onset was 5 years (IQR 2.4-11.8). SF usually showed an inflammatory pattern (median white blood cells count 11,757/mm 3 ; IQR 4,543-18,800), with a predominance of polymorphonuclear (PMN) cells (61%; IQR 30-75). Eight patients (14%) had white blood cells counts of less than 2,000 cells/mm 3 , with predominance of mononuclear cells (80%), whereas 3 patients (5%) had white blood cells counts higher than 50,000 cells/mm 3 , with a predominance of PMN cells (90%). Synovial white blood cells count did not show significant differences among the different forms of JIA. The median synovial white blood cells count in ANA-positive patients was 20% lower than in ANA-negative (9,340 vs. 11,600/mm 3 ; P=.23). The proportion of PMN increased with increasing levels of ESR (P<.001) and/or CRP (P=.03). No significant correlation was found between JADAS-10 and synovial white blood cells count (P=.4). SF obtained from different joints in simultaneous arthrocentesis showed a significant correlation P=.001)., Conclusion: SF from JIA patients usually had inflammatory characteristics, although 19% of the patients showed white blood cells counts below 2,000cells/mm 3 or higher than 50,000cells/mm 3 . SF cell count was non-significantly lower in ANA-positive patients, and the proportion of PMN increased with increasing levels of ESR/CRP., (Copyright © 2019. Publicado por Elsevier España, S.L.U.)

Introduction: Non-bacterial chronic osteomyelitis (NBCO) is an autoinflammatory disease that presents with recurrent bouts of bone inflammation in the absence of microbiological isolation. It is a diagnosis of exclusion. Its treatment was classically based on the use of non-steroidal anti-inflammatory drugs (NSAIDs) and corticosteroids, although nowadays bisphosphonates or anti-tumour necrosis factor-α (anti-TNF) drugs are frequently used with good results. The objective of the study is to describe our experience in the diagnosis and treatment of patients with NBCO., Patients and Methods: Retrospective chart review of patients with NBCO followed up in a tertiary centre between 2008 and 2015., Results: A total of 7 patients with NBCO were recorded. Four were female and the median age was 10 years (IQR 2). The most common complaint was pain that interfered with sleep in 5 of the patients. Six patients had multifocal lesions at diagnosis. Bone biopsy demonstrated neutrophilic or lymphocytic infiltration and sclerosis in 6 patients. Four patients received antibiotics and NSAIDs without clinical response. Five received a short course of prednisone with an adequate control of symptoms, but only one of them maintained remission after corticosteroid suspension. Five patients received bisphosphonates with disease remission in 3 of them. The other 2 showed an inadequate response to pamidronate and were started on anti-TNF therapy (etanercept, infliximab or adalimumab), remaining asymptomatic at present., Conclusions: Our series, although limited, confirms the effectiveness and safety of bisphosphonate and anti-TNF therapy for children with NBCO., (Copyright © 2015 Asociación Española de Pediatría. Published by Elsevier España, S.L.U. All rights reserved.)

Musculoskeletal pain is a frequent complaint in pediatrics usually related to benign conditions. However, it may also represent the initial symptom of serious diseases such as infections, malignancies or orthopedic emergencies. We present the case of a child diagnosed with Burkitt's lymphoma whose initial presentation was a limp. This is, to the best of our knowledge, the first case reported in the literature with this type of debut. This case illustrates the importance of including neoplasms in the differential diagnosis of atypical musculoskeletal pain, since early diagnosis can significantly improve their prognosis., (Copyright © 2010 Elsevier España, S.L. All rights reserved.)

Musculoskeletal pain is a common complaint in paediatrics usually due to benign diseases. Nevertheless neoplasms, particularly acute leukaemia, must be considered in the differential diagnosis. During the last 9 months 4 of the 9 patients diagnosed with acute leukaemia at our hospital presented with a limp, arthralgias, lumbar or bony pain. We describe these cases and review the clinical and analytical parameters that help to differentiate benign pain from that associated with a malignant disease. The early detection of these processes may represent a significant improvement in their prognosis., (Copyright 2009 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.)

Introduction: Macrophage activation syndrome (MAS) is a severe complication of systemic juvenile idiopathic arthritis (sJIA)., Objective: To describe the clinical characteristics and outcome of patients diagnosed with MAS in Spanish pediatric rheumatology units., Patients and Method: A protocol for data collection was designed and distributed to pediatricians and rheumatologists attending children with rheumatic diseases., Results: Information was available from 31 patients (16 boys and 15 girls) who had 37 MAS episodes. Twenty-seven children had only one episode, three had two episodes and one had four episodes. The interval between episodes ranged from 1 to 33 months. The median age was 5.9 years (range 1-23). MAS was the initial manifestation of sJIA in nine patients. The most frequent symptom was fever (97%), followed by skin rash (49%), central nervous system dysfunction (41%), and gastrointestinal abnormalities (15%). Abnormal laboratory findings included thrombopenia (78%) and elevated levels of hepatic enzymes (70%). Hemophagocytosis was confirmed in 16 of 30 bone marrow samples evaluated, 15 with cyclosporine A and six with etoposide. All episodes but one were treated with steroids. One patient received a liver transplant before diagnosis. The mortality rate was 6.5% (2/31)., Conclusion: MAS is a severe, potentially lethal, complication of sJIA. The clinical and laboratory abnormalities characteristic of sJIA complicate its diagnosis. The earliest and most frequent findings were decreased in platelet count and elevation of hepatic enzymes. A high degree of suspicion as well as early diagnosis and prompt treatment are essential in this disease.

Objectives: (1) To determine the prevalence of joint hypermobility in children aged 4-14 years old without organic disease of the locomotor system; (2) to compare the prevalence of hypermobility in children with and without arthralgia and (3) to analyze the influence of certain variables on the development of arthralgia., Patients and Methods: We performed an observational study in a sample of children aged 4-14 years old living in Area 4 of the Community of Madrid (Spain). Joint hypermobility was evaluated using a goniometer. Hypermobility was defined using Beighton's criteria., Results: A total of 222 subjects were analyzed: 176 in the primary care setting and 46 in the emergency department of a referral hospital. Of the 222 children, 43 reported arthralgia. The prevalence of hypermobility (> or = 4 criteria) was 55 % (123/222), reaching 71 % (49/69) in children aged less than 8 years. No significant differences were found in the prevalence of hypermobility in children with and without arthralgia (65 % and 53 % respectively). Of the variables analyzed (age, sex, country of origin, primary care/emergency department setting) only differences in the absolute number of Beighton criteria present in children with and without arthralgia (4.34 +/- 2.47 and 3.48 +/- 2.35, p = 0.03) were detected, which disappeared when at least four criteria (definition of hypermobility) were required., Conclusions: Fifty-five percent of the population studied and 71 % of those younger than 8 years old met the criteria for joint hypermobility. In the sample analyzed, the presence of joint hypermobility did not seem to favor the development of arthralgias.

Objective: 1) To determine the number of clinic visits in primary care due to musculoskeletal pain in children > or = 3 years. 2) To describe the demographic characteristics of the population assessed for limb/back pain. 3) To characterize the etiology of musculoskeletal pain in a general pediatric clinic., Patients and Methods: A prospective lineal study was performed between October 30, 1996 and January 14, 1997 in two urban general pediatric clinics located in the city of Madrid (Spain). During the period of the study, all consultations related to musculoskeletal pain were registered in a protocol form. In addition, a registry of all clinic visits was maintained during the study., Results: During the study period, 43 clinic visits were due to musculoskeletal pain, representing 6.2% (43/692) of the clinic visits in children > or = 3 years of age (95% confidence interval 4.4-7.9%). The mean age was 9.8 years (+/- 3.2 years). These complaints, although statistically non-significant, were more frequent in children > or = 10 years old (53.4%) and in males (65.1%). The most frequent complaints were knee athralgias (30%), arthralgias of other joints (23%) and soft tissue pain (18%). These symptoms were usually due to overuse syndromes and normal skeletal growth variants., Conclusions: The increase of primary pediatric care age from 7 to 14 years of age is associated with an increase in the number of consultations related to musculoskeletal pain. This information should be kept in mind at the time of designing continuing medical education and training programs in pediatrics.

Authors report one pediatric patient with prolonged QT-interval syndrome. Importance of this syndrome is based on the possibility of syncopal episodes, due to serious arrhythmias, and sudden death. Therapeutics protocols and prophylactic treatment's indications. They are reviewed conclude that familial study is necessary in congenital type and that better therapeutical results are achieved with propranolol than with others drugs.

Report of a case of kinesic paroxysmal choreoathetosis in a family (as far as we know the first case of a family on record in Spanish) which responded very well to treatment with low doses of carbamazepine. The characteristics of this infrequent clinical entity are also discussed.

Reiter's Syndrome (RS) is not common in children and adolescents. From 1982 to 1987 we have seen 13 pediatric patients (8 male and 5 female) with clinical manifestations that characterized RS. Age of onset was 13 years. Clinical findings have been arthritis (100%) involving the knee and ankle joints, heel pain (84%), lumbosacral pain (38%). One to three weeks before joint involvement, six patients (46%) complained of urethritis, 5 (38%) had enteritis and two patients had both. 46% of the patients had HLA-B27 antigen. Radiologic findings were sacroileitis in five patients and calcaneal spurs. All patients were treated with non-steroidal antiinflammatory therapy and, in general, there was a clinical remission in 1-3 months. Nevertheless, after 4 years of follow up, 2 patients had acute relapsing arthritis and 4 had chronic arthropathy. Three patients have developed secondary ankylosing spondylitis. RS is not all that infrequent in pediatric patients with joint involvements if one looks for urethritis, enteritis, eye involvement and cutaneous manifestations. The clinical course is favorable, but there might be some chronic or relapsing cases. Some patients can developed secondary ankylosing spondylitis. For that reason a prolonged follow up of these patients is necessary.

We present the clinical data and the management of twelve patients with symptomatic pericardial effusion (PE). The etiology of PE was: Chronic renal failure, viral infection, cardiac surgery, juvenile rheumatoid arthritis and chronic myelocytic leukemia. Four cases were diagnosed as idiophatic. PE in childhood is usually asymptomatic. When symptoms are present they are non-specific and don't help to know the size of the effusion; therefore, it's necessary to practice an echocardiography to demostrate the presence of PE. The hemodynamic findings permit to diferentiate patients with and without cardiac tamponade. The treatment of first choice is aspirin. In patients with cardiac tamponade the treatment should be pericardiocentesis.