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Sample records for systemic juvenile idiopathic

  1. Idiopathic Pulmonary Hemosiderosis in a Child with Recurrent Macrophage Activation Syndrome Secondary to Systemic Juvenile Idiopathic Arthritis

    OpenAIRE

    Barut, Kenan; Sahin, Sezgin; Adrovic, Amra; Sen, Velat; Kasapcopur, Ozgur

    2017-01-01

    Macrophage activation syndrome, a severe complication of systemic juvenile idiopathic arthritis and other inflammatory diseases, represents one of the most important rheumatological emergencies. Delayed diagnosis could lead to life-threatening complications. Pulmonary hemosiderosis has been classically characterized by a triad of anemia, hemoptysis, and lung infiltrates on chest radiogram. Although the majority of patients of pulmonary hemosiderosis are considered idiopathic, secondary hemosi...

  2. Idiopathic Pulmonary Hemosiderosis in a Child with Recurrent Macrophage Activation Syndrome Secondary to Systemic Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Kenan Barut

    2017-01-01

    Full Text Available Macrophage activation syndrome, a severe complication of systemic juvenile idiopathic arthritis and other inflammatory diseases, represents one of the most important rheumatological emergencies. Delayed diagnosis could lead to life-threatening complications. Pulmonary hemosiderosis has been classically characterized by a triad of anemia, hemoptysis, and lung infiltrates on chest radiogram. Although the majority of patients of pulmonary hemosiderosis are considered idiopathic, secondary hemosiderosis associated with known diseases could be seen. In this case report, we aimed to present gradually increased pulmonary manifestations due to pulmonary hemosiderosis with recurrent macrophage activation syndrome attacks in a child with systemic juvenile idiopathic arthritis.

  3. CLINICAL CASE OF TOCILIZUMAB THERAPY IN A PATIENT WITH SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS

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    E. I. Alexeeva

    2013-01-01

    Full Text Available The article presents a case of successful application of a monoclonal antibodies drug to interleukin 6 receptors (tocilizumab at severe systemic juvenile idiopathic arthritis with the development of secondary hemophagocytic syndrome. Tocilizumab treatment secured a decrease in clinical and laboratory parameters of the disease activity, life quality improvement, systemic juvenile idiopathic arthritis and hemophagocytic syndrome remission and allowed avoiding the per os prescription of glucocorticoids.

  4. Clinical Case of Tocilizumab Use in a Patient with Systemic Juvenile Idiopathic Arthritis

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    Y. M. Spivakovskiy

    2015-01-01

    Full Text Available The article presents a case of using genetically engineered biopharmaceutical tocilizumab in a child with systemic juvenile idiopathic arthritis. On the initial stage, the treatment was characterized by resistance to high doses of glucocorticoids and cytostatic drugs. Successful termination of visceral and articular manifestations of systemic juvenile idiopathic arthritis and normalization of laboratory indicators of disease activity in the setting of use of interleukin 6 receptor blocker were described. We observed stable improvement of the child’s condition during a year-long follow-up in the setting of the selected anti-inflammatory therapy pattern. 

  5. Genetics Home Reference: juvenile idiopathic arthritis

    Science.gov (United States)

    ... Home Health Conditions Juvenile idiopathic arthritis Juvenile idiopathic arthritis Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Juvenile idiopathic arthritis refers to a group of conditions involving joint ...

  6. Macrophage activation syndrome in a patient with systemic onset of the juvenile idiopathic arthritis.

    Science.gov (United States)

    Jain, Deepak; Aggarwal, Hari K; Rao, Avinash; Mittal, Anshul; Jain, Promil

    2016-01-01

    Systemic onset juvenile idiopathic arthritis (sJIA) is defined as arthritis affecting one or more joint usually in the juvenile age group (< 16 years of age) with or preceded by fever of at least 2 weeks duration that is documented to be daily ("quotidian") for at least 3 days which may be associated with evanescent (non-fixed) erythematous rash or generalized lymph node enlargement or hepatomegaly/splenomegaly/both or serositis. Macrophage activation syndrome (MAS) is a life-threatening complication of sJIA marked by sudden onset of non-remitting high fever, profound depression in all three blood cell lines (i.e. leukopenia, anemia, and thrombocytopenia), hepatosplenomegaly, lymphadenopathy, and elevated serum liver enzyme levels. In children with systemic juvenile idiopathic arthritis, the clinical picture may mimic sepsis or an exacerbation of the underlying disease. We report a case of a 16-year-old female patient presenting with high grade fever with joint pains and generalized weakness which proved to be systemic onset juvenile idiopathic arthritis with macrophage activation syndrome after ruling out all other differential diagnoses and responded well to intravenous steroids.

  7. Juvenile idiopathic arthritis (JIA): a screening study to measure class II skeletal pattern, TMJ PDS and use of systemic corticosteroids.

    LENUS (Irish Health Repository)

    Mandall, Nicky A

    2010-03-01

    To screen patients with oligoarticular and polyarticular forms of Juvenile Idiopathic Arthritis (JIA) to determine (i) the severity of their class II skeletal pattern; (ii) temporomandibular joint signs and symptoms and (iii) use of systemic corticosteroids.

  8. [Macrophage activation syndrome in a patient with systemic juvenile idiopathic arthritis].

    Science.gov (United States)

    Tavares, Anna Carolina Faria Moreira Gomes; Ferreira, Gilda Aparecida; Guimarães, Luciano Junqueira; Guimarães, Raquel Rosa; Santos, Flávia Patrícia Sena Teixeira

    2015-01-01

    Machrophage activation syndrome (MAS) is a rare and potentially fatal disease, commonly associated with chronic rheumatic diseases, mainly juvenile idiopathic arthritis. It is included in the group of secondary forms of haemophagocytic syndrome, and other causes are lymphoproliferative diseases and infections. Its most important clinical and laboratorial manifestations are non-remitting fever, splenomegaly, bleeding, impairment of liver function, cytopenias, hypoalbuminemia, hypertriglyceridemia, hypofibrinogenemia and hyperferritinemia. The treatment needs to be started quickly, and the majority of cases have a good response with corticosteroids and cyclosporine. The Epstein-Barr virus is described as a possible trigger for many cases of MAS, especially in these patients in treatment with tumor necrosis factor (TNF) blockers. In these refractory cases, etoposide (VP16) should be administered, associated with corticosteroids and cyclosporine. Our objective is to describe a rare case of MAS probably due to EBV infection in a subject with systemic-onset juvenile idiopathic arthritis, which achieved complete remission of the disease after therapy guided by 2004-HLH protocol. Copyright © 2014 Elsevier Editora Ltda. All rights reserved.

  9. Are Bicipital Synovial Cysts in Children with Systemic Juvenile Idiopathic Arthritis still a Significant Clinical Challenge?

    DEFF Research Database (Denmark)

    Kyvsgaard, Nini; Herlin, Troels

    2015-01-01

    BACKGROUND: Large synovial cysts are rarely seen in juvenile idiopathic arthritis. When they do appear, they usually appear in the popliteal space (Baker’s cyst). Less commonly, they occur in the antecubital area or as bicipital synovial cysts. Bicipital synovial cysts present as a sudden-onset p...

  10. [Juvenile idiopathic epiretinal membrane].

    Science.gov (United States)

    Kontopoulou, K; Krause, S; Fili, S; Hayvazov, S; Schilling, H; Kohlhaas, M

    2016-07-01

    Idiopathic epiretinal membrane (iERM) is very rare in adolescent patients. The pathogenesis remains unclear although the role of hyalocytes is of major importance. The clinical features in young patients are different from those in older patients. We describe a case of iERM in a 15-year-old girl who presented with metamorphopsia of the right eye. This case report presents the basis for the decision for surgical treatment as well as the clinical features at follow-up examination 9 months after surgery.

  11. Inadequate dietary intake of children and adolescents with juvenile idiopathic arthritis and systemic lupus erythematosus.

    Science.gov (United States)

    Caetano, Michelle C; Ortiz, Thaís T; Terreri, Maria Teresa S R L A; Sarni, Roseli O S; Silva, Simone G L; Souza, Fabíola I S; Hilário, Maria Odete E

    2009-01-01

    To evaluate the dietary intake of children and adolescents with juvenile idiopathic arthritis (JIA) and juvenile systemic lupus erythematosus (JSLE) using a 24-hour diet recall and relating it to the patients' clinical and anthropometric characteristics and to the drugs used in their treatment. By means of a cross-sectional study, we assessed the 24-hour diet recalls of outpatients. Their nutritional status was classified according to the CDC (2000). The computer program NutWin UNIFESP-EPM was used for food intake calculation. The Recommended Dietary Allowances and the Brazilian food pyramid were used for quantitative and qualitative analysis. Median age was 12 years for JIA patients and 16.5 years for JSLE patients. Among the JIA patients, 37.5% had active disease, and among the JSLE patients, 68.2% showed Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) > 4. Malnutrition was found in 8.3 and 4.5% of the JIA and JSLE patients, respectively, and obesity was present in 16.7 and 18.2%. For JIA patients, the excessive intake of energy, protein, and lipids was 12.5, 75, and 31.3%, respectively. For JSLE patients, the excessive intake of energy, protein, and lipids was 13.6, 86.4, and 36.4%, respectively. Low intake of iron, zinc, and vitamin A was found in 29.2 and 50, 87.5 and 86.4, and 87.5 and 95.2% of the JIA and JSLE patients, respectively. There was not a significant association between intake, disease activity, and nutritional status. Patients with rheumatic diseases have inadequate dietary intake. There is excessive intake of lipids and proteins and low intake of micronutrients.

  12. A critical appraisal of radiographic scoring systems for assessment of juvenile idiopathic arthritis

    International Nuclear Information System (INIS)

    Doria, Andrea S.; Babyn, Paul S.; Feldman, Brian

    2006-01-01

    Assessing structural damage to joints over time is essential for evaluating the effectiveness of therapeutic interventions for patients with inflammatory arthritis. Although radiography is able to quantify joint damage, the changes found with conventional radiography early in the disease course are nonspecific, and late radiographic changes are often irreversible. Although many clinical trials on drug development for children still use radiographic scales as endpoints for the study, more specific therapies have been developed for juvenile idiopathic arthritis (JIA) that would enable imaging to ''fine-tune'' patients to placement into specific treatment algorithms. As a result, new imaging scales to identify early abnormalities are clearly needed. Many pediatric rheumatology centers around the world persistently apply adult-designed radiographic scoring systems to evaluate the progression of JIA. Few pediatric-targeted radiographic scales are available for assessment of progression of JIA in growing joints, and the clinimetric and psychometric properties of such scales have been poorly investigated. We present a critique to the evaluative, discriminative, and predictive roles of the van der Heijde modification of Sharp's radiographic method, a scale originally designed to assess damage to joints of adults with rheumatoid arthritis, when it is applied to a pediatric population. We discuss the advantages and drawbacks of this radiographic scoring system for assessing growing joints and the ability of MRI to overcome inadequacies of conventional radiography. (orig.)

  13. Algorithm development for corticosteroid management in systemic juvenile idiopathic arthritis trial using consensus methodology

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    Ilowite Norman T

    2012-08-01

    Full Text Available Abstract Background The management of background corticosteroid therapy in rheumatology clinical trials poses a major challenge. We describe the consensus methodology used to design an algorithm to standardize changes in corticosteroid dosing during the Randomized Placebo Phase Study of Rilonacept in Systemic Juvenile Idiopathic Arthritis Trial (RAPPORT. Methods The 20 RAPPORT site principal investigators (PIs and 4 topic specialists constituted an expert panel that participated in the consensus process. The panel used a modified Delphi Method consisting of an on-line questionnaire, followed by a one day face-to-face consensus conference. Consensus was defined as ≥ 75% agreement. For items deemed essential but when consensus on critical values was not achieved, simple majority vote drove the final decision. Results The panel identified criteria for initiating or increasing corticosteroids. These included the presence or development of anemia, myocarditis, pericarditis, pleuritis, peritonitis, and either complete or incomplete macrophage activation syndrome (MAS. The panel also identified criteria for tapering corticosteroids which included absence of fever for ≥ 3 days in the previous week, absence of poor physical functioning, and seven laboratory criteria. A tapering schedule was also defined. Conclusion The expert panel established consensus regarding corticosteroid management and an algorithm for steroid dosing that was well accepted and used by RAPPORT investigators. Developed specifically for the RAPPORT trial, further study of the algorithm is needed before recommendation for more general clinical use.

  14. Coronary artery abnormalities in children with systemic-onset juvenile idiopathic arthritis.

    Science.gov (United States)

    Lefèvre-Utile, Alain; Galeotti, Caroline; Koné-Paut, Isabelle

    2014-05-01

    Still's disease (Systemic-onset Juvenile Idiopathic Arthritis: SoJIA) is characterised by high-spiking daily fevers, arthritis and evanescent rashes. Diagnosis of Still's disease is often challenging. Infectious diseases and other inflammatory conditions, especially in young children, Kawasaki disease may look similar. Clinicians often rely on echocardiographic evidence of coronary artery abnormalities to differentiate between Kawasaki disease and Still's disease. Coronary artery dilation would typically favour the diagnosis of Kawasaki disease. We present four children with Still's disease and coronary artery abnormalities who were initially misdiagnosed as Kawasaki disease. The first patient had pericarditis and an irregular wall of the left coronary artery, without dilation on echocardiography. The second patient had a left coronary artery dilatation and a pericarditis. The third patient had thickened left coronary artery walls, and the fourth patient had a hyperechogenicity of the left and right coronary arteries. They received IVIG without success. The diagnosis of Still's disease was made secondary with evidence of persistent arthritis. All but one patient finally needed biologic treatments. Coronary abnormalities may be observed during various febrile conditions and do not exclude the diagnosis of Still's disease. Copyright © 2013 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  15. Exercise therapy in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Takken, T.; van Brussel, M.; Engelbert, R. H. H.; van der Net, J.; Kuis, W.; Helders, P. J. M.

    2008-01-01

    Exercise therapy is considered an important component of the treatment of arthritis. The efficacy of exercise therapy has been reviewed in adults with rheumatoid arthritis but not in children with juvenile idiopathic arthritis (JIA). To assess the effects of exercise therapy on functional ability,

  16. Acute Lymphoblastic Leukaemia presenting as Juvenile Idiopathic ...

    African Journals Online (AJOL)

    Background: Acute Lymphoblastic Leukaemia in children commonly presents with osteo articular manifestations that may mimic Juvenile Idiopathic Arthritis. This may create considerable diagnostic difficulty and lead to delay in commencing appropriate treatment. Case: An eight year old boy who presented with multiple ...

  17. Juvenile idiopathic arthritis and the temporomandibular joint ...

    African Journals Online (AJOL)

    Background: The temporomandibular joint (TMJ) is one of the most underdiagnosed and undertreated conditions of juvenile idiopathic arthritis (JIA) because its involvement is often asymptomatic and the joint is difficult to examine. Objectives: The aim of this study was to investigate clinical as well as magnetic resonance ...

  18. Features of renal dysfunction in children with systemic lupus erythematosus and juvenile idiopathic arthritis

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    L.F. Bogmat

    2017-09-01

    Full Text Available Background. In systemic autoimmune diseases of the connective tissue, kidney damage in some cases is a syndrome of the disease, and in others, it is a consequence of the underlying process. Objective: to establish the incidence and nature of violations of kidney function in children and adolescents with juvenile idiopathic arthritis (JIA and systemic lupus erythematosus (SLE, depending on the duration and activity of the disease. Materials and methods. 113 children aged 7 to 18 years were examined, of which 70 patients with JIA and 43 — with SLE. Distribution to the groups was carried out depending on the duration and activity of the disease. The status of kidney function was evaluated using hemorrhagic tests (serum creatinine level, determination of glomerular filtration rate. We have evaluated the level of proteinuria in the daily urine, as well as studied the concentration function of the kidneys by means of urine analysis according to Zimnytsky. Statistical processing was carried out using the statistical software package Statgra­phics 16.0. Results. Thus, in children with JIA, with the duration of the disease more than 3 years, there was a change in the renal function characterized as proteinuria, and a violation of glomerular filtration rate and a decrease in the concentration function of the kidneys. When studying the incidence of violations depending on the activity of the pathological process, it was established that in the group with 0 degree of activity, both glomerular filtration rate (25.0 % and concentration function of the kidneys (12.5 % decreased. In the group with I degree of activity, there was detected microproteinuria (4.4 %, violation of glomerular filtration rate (12.3 % and decreased renal concentration (6.6 %. In the group with II degree of activity, microproteinuria (14.2 % and a certain reduction in glomerular filtration rate (14.2 % were detected, and in the group with III degree of activity, almost all patients

  19. Experience in the use of tocilizumab in patient with systemic juvenile idiopathic arthritis and type 1 diabetes

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    E. A. Ligostaeva

    2016-01-01

    Full Text Available The article describes the experience of using tocilizumab in a patient with systemic juvenile idiopathic arthritis (JIA refractory to therapy with classical immunosuppressants, coupled with type 1 diabetes. Already after the first injection of tocilizumab there was a decrease in the severity of the disease’s systemic manifestations, pain in the affected joints and functional disorders. A 30% improvement by JADAS index, ACRPedi was reached. By the 8th week of therapy, proliferative changes in wrist joints and arthralgias reduced and the duration of morning stiffness decreased. After 3 months, JIA’s activity decreased (DAS 28 scale; erythrocyte sedimentation rate and serum concentrations of C-reactive protein decreased; hemoglobin concentration and the number of erythrocytes increased. After 6 months of treatment, clinical and laboratory remission (DAS 28 < 2.6 started. On the background of tocilizumab treatment, systemic manifestations of the disease stopped, laboratory and articular JIA’s activity decreased and there was no need for the introduction of glucocorticoids. There also has been a positive dynamics of glycemia, which allowed reducing the dose of insulin. There were no adverse events during treatment with tocilizumab. The disease’s activity on a visual analog scale has also decreased, as well as functional insufficiency by Children Heals Assessment Questionnaire.

  20. [Juvenile idiopathic arthritis and oral health].

    Science.gov (United States)

    Kobus, Agnieszka; Kierklo, Anna; Sielicka, Danuta; Szajda, Sławomir Dariusz

    2016-05-04

    Juvenile idiopathic arthritis (JIA) is the most common autoimmune inflammatory disease of connective tissue in children. It is characterized by progressive joint destruction which causes preserved changes in the musculoskeletal system. The literature describes fully clinical symptoms and radiological images in different subtypes of JIA. However, there is still a limited number of studies reporting on the medical condition of the oral cavity of ill children. JIA can affect hard and soft tissues of the oral cavity by: the general condition of the child's health, arthritis of the upper limbs, as the result of the pharmacotherapy, changes in secretion and composition of saliva, inflammation of the temporomandibular joint and facial deformity. The study summarizes the available literature on the condition of the teeth and periodontal and oral hygiene in the course of JIA. The presence of diverse factors that modify the oral cavity, such as facial growth, functioning of salivary glands, or the supervision and care provided by adults, prevents clear identification if JIA leads to severe dental caries and periodontal disease. Despite conflicting results in studies concerning the clinical oral status, individuals with JIA require special attention regarding disease prevention and maintenance of oral health.

  1. Management of Children with Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Viswanathan, Vijay; Murray, Kevin J

    2016-01-01

    Juvenile idiopathic arthritis (JIA) comprises a group of heterogeneous disorders of chronic arthritis in childhood and remains the commonest pediatric rheumatic disease associated with significant long-term morbidity. Advances in understanding of the pathogenesis, better definition of disease control/remission measures, and the arrival of biological agents have improved the outcomes remarkably. Methotrexate (Mtx) remains the first-line disease modifying (DMARD) therapy for most children with JIA due to its proven efficacy and safety. Sulphosalazine (SSz) (especially for enthesitis) and leflunomide may also have a secondary role. Tumor necrosis factor inhibitors (TNF-I), alone or in combination with Mtx have shown tremendous benefit in children with polyarticular JIA, enthesitis related arthritis (ERA) and psoriatic arthritis. Tocilizumab appears very efficacious in systemic arthritis and abatacept and tocilizumab also appear to benefit polyarticular JIA; the role of rituximab remains unclear, though clearly beneficial in adult RA. TNF-I with Mtx is also effective in uveitis associated with JIA. Biologicals have demonstrated an impressive safety record in children with JIA, although close monitoring for rare but potentially dangerous adverse events, such as tuberculosis and other infections; paradoxical development of additional autoimmune diseases; and possibly an increased risk of cancers is warranted.

  2. Carpal erosions in children with juvenile idiopathic arthritis: repeatability of a newly devised MR-scoring system

    Energy Technology Data Exchange (ETDEWEB)

    Boavida, Peter [Great Ormond Street Hospital for Children, Department of Radiology, London (United Kingdom); Lambot-Juhan, Karen [Hospital Necker Enfants Malades, Department of Radiology, Paris (France); Ording Mueller, Lil-Sofie [Oslo University Hospital, Department of Radiology, Oslo (Norway); Damasio, Beatrice; Malattia, Clara [Ospedale Pediatrico Gaslini, Department of Rheumatology, Genoa (Italy); Tanturri de Horatio, Laura [Ospedale Pediatrico Bambino Gesu, Department of Radiology, Rome (Italy); Owens, Catherine M. [Great Ormond Street Hospital for Children, Department of Radiology, London (United Kingdom); UCL, Institute of Child Health, London (United Kingdom); Rosendahl, Karen [Haukeland University Hospital, Department of Radiology, Bergen (Norway); University of Bergen, Department of Clinical Medicine, Bergen (Norway)

    2015-12-15

    Juvenile idiopathic arthritis (JIA) is characterized by synovial inflammation, with potential risk of developing progressive joint destruction. Personalized state-of-the-art treatment depends on valid markers for disease activity to monitor response; however, no such markers exist. To evaluate the reliability of scoring of carpal bone erosions on MR in children with JIA using two semi-quantitative scoring systems. A total of 1,236 carpal bones (91 MR wrist examinations) were scored twice by two independent pediatric musculoskeletal radiologists. Bony erosions were scored according to estimated bone volume loss using a 0-4 scale and a 0-10 scale. An aggregate erosion score comprising the sum total carpal bone volume loss was calculated for each examination. The 0-4 scoring system resulted in good intra-reader agreement and moderate to good inter-observer agreement in the assessment of individual bones. Fair and moderate agreement were achieved for inter-reader and intra-reader agreement, respectively, using the 0-10 scale. Intra- and particularly inter-reader aggregate score variability were much less favorable, with wide limits of agreement. Further analysis of erosive disease patterns compared with normal subjects is required, and to facilitate the development of an alternative means of quantifying disease. (orig.)

  3. Carpal erosions in children with juvenile idiopathic arthritis: repeatability of a newly devised MR-scoring system

    International Nuclear Information System (INIS)

    Boavida, Peter; Lambot-Juhan, Karen; Ording Mueller, Lil-Sofie; Damasio, Beatrice; Malattia, Clara; Tanturri de Horatio, Laura; Owens, Catherine M.; Rosendahl, Karen

    2015-01-01

    Juvenile idiopathic arthritis (JIA) is characterized by synovial inflammation, with potential risk of developing progressive joint destruction. Personalized state-of-the-art treatment depends on valid markers for disease activity to monitor response; however, no such markers exist. To evaluate the reliability of scoring of carpal bone erosions on MR in children with JIA using two semi-quantitative scoring systems. A total of 1,236 carpal bones (91 MR wrist examinations) were scored twice by two independent pediatric musculoskeletal radiologists. Bony erosions were scored according to estimated bone volume loss using a 0-4 scale and a 0-10 scale. An aggregate erosion score comprising the sum total carpal bone volume loss was calculated for each examination. The 0-4 scoring system resulted in good intra-reader agreement and moderate to good inter-observer agreement in the assessment of individual bones. Fair and moderate agreement were achieved for inter-reader and intra-reader agreement, respectively, using the 0-10 scale. Intra- and particularly inter-reader aggregate score variability were much less favorable, with wide limits of agreement. Further analysis of erosive disease patterns compared with normal subjects is required, and to facilitate the development of an alternative means of quantifying disease. (orig.)

  4. [Determination of 25(OH)D serum levels in children with systemic lupus erythematosus and juvenile idiopathic arthritis].

    Science.gov (United States)

    Rosiles, Vanessa Hernández; Salazar, Carolina Duarte; Velazquez, Rocío Maldonado; Ruiz, Rodolfo Rivas; Clark, Patricia

    It is well recognized that vitamin D has a direct effect in bone and muscle and has been associated as well with some rheumatologic diseases. Reports in children are scarce. The aim of this study was to determine the concentration level of 25(OH)D in a group of patients with systemic lupus erythematosus (SLE) and juvenile idiopathic arthritis (JIA) and compare them with healthy controls. Vitamin D (25(OH)D) was measured with isotope-dilution liquid chromatography-tandem mass spectrometry (ID-LC-MS/MS), PTH with immunoradiometric assay (IRMA), calcium, phosphorus and alkaline phosphatase by colorimetric assay in 37 patients with SLE, 37 patients with JIA and 79 healthy controls. Mean 25(OH)D concentration levels were as follows: SLE 18.9±7.92ng/ml, JIA 21.97±5.55ng/ml and 23.6±3.07ng/ml in healthy controls. There was a significant difference between SLE patients vs. healthy controls (p <0.05); 29.7% of SLE patients, 35.1% of JIA patients and 31.6% of healthy controls had deficient levels of vitamin D. One third of the total sample of children in this study had deficient levels of vitamin D. Patients with SLE presented a significant difference compared with healthy controls. Copyright © 2015 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.

  5. Síndrome de ativação macrofágica associada com artrite idiopática juvenil sistêmica Macrophage activation syndrome associated with systemic juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Clovis Artur A. Silva

    2004-12-01

    Full Text Available OBJETIVO: Descrever as características da síndrome de ativação macrofágica associada a artrite idiopática juvenil. DESCRIÇÃO DOS CASOS: Foram analisados retrospectivamente os prontuários de 462 pacientes com artrite idiopática juvenil. Destes, sete (1,5% pacientes desenvolveram síndrome de ativação macrofágica; todos tinham a forma sistêmica da doença. A mediana de idade de início da artrite idiopática juvenil foi de 3 anos e 10 meses, e a mediana do tempo de duração da artrite idiopática juvenil antes da síndrome de ativação macrofágica foi de 8 anos e 4 meses. Todos os pacientes apresentaram febre, icterícia, hepatoesplenomegalia, sangramentos, pancitopenia e elevação das enzimas hepáticas e dos tempos de coagulação e bilirrubina direta. Três casos apresentaram infecções associadas e um caso desenvolveu a síndrome de ativação macrofágica 2 semanas após a introdução de sulfasalazina. Três pacientes morreram. Proliferação macrofágica e hemofagocitose foram evidenciadas em cinco. A terapêutica da síndrome de ativação macrofágica incluiu pulsoterapia com metilprednisolona em todos, ciclosporina em três, plasmaférese em dois e gamaglobulina endovenosa em dois. COMENTÁRIOS: A síndrome de ativação macrofágica é uma complicação da artrite idiopática juvenil sistêmica com alta morbidade e mortalidade.OBJECTIVE: To describe the characteristics of macrophage activation syndrome associated with juvenile idiopathic arthritis. DESCRIPTION: This is a retrospective study involving 462 patients with juvenile idiopathic arthritis. Seven (1.5% of those patients suffered from systemic onset juvenile idiopathic arthritis and developed macrophage activation syndrome. The median age of the juvenile idiopathic arthritis onset was 3 years and 10 months and the median duration of juvenile idiopathic arthritis before macrophage activation syndrome was 8 years and 4 months. All of them presented with fever

  6. Catch-up growth during tocilizumab therapy for systemic juvenile idiopathic arthritis: results from a phase III trial.

    Science.gov (United States)

    De Benedetti, Fabrizio; Brunner, Hermine; Ruperto, Nicolino; Schneider, Rayfel; Xavier, Ricardo; Allen, Roger; Brown, Diane E; Chaitow, Jeffrey; Pardeo, Manuela; Espada, Graciela; Gerloni, Valeria; Myones, Barry L; Frane, James W; Wang, Jianmei; Lipman, Terri H; Bharucha, Kamal N; Martini, Alberto; Lovell, Daniel

    2015-03-01

    To investigate the impact of tocilizumab treatment on growth and growth-related laboratory parameters in patients with systemic juvenile idiopathic arthritis (JIA) enrolled in a phase III clinical trial. Patients with systemic JIA ages 2-17 years (n = 112) received tocilizumab in a 12-week, randomized, placebo-controlled period and a long-term open-label extension. Height velocity and standard deviation (SD) score; levels of insulin-like growth factor 1 (IGF-1), osteocalcin (OC), and C-telopeptide of type I collagen (CTX-I); and Juvenile Arthritis Disease Activity Score in 71 joints (JADAS-71) were measured in a post hoc analysis of 83 patients who never received growth hormone and did not reach Tanner stage 5 by the end of the first year of treatment. Patients had stunted growth at baseline (mean height SD score -2.2). During tocilizumab treatment, males (73%) and females (83%) experienced above-normal mean height velocities of 6.6 cm/year (P < 0.0001 versus World Health Organization norms). Mean height SD score increases during year 1 (0.29) and year 2 (0.31) were significant (both P < 0.0001). The mean SD score for IGF-1 levels increased significantly (-0.2 for year 1 and -0.1 for year 2 versus -1.0 at baseline; both P < 0.0001). Mean OC and CTX-I levels (both P < 0.0001) and the OC:CTX-I ratio (P = 0.014) significantly increased from baseline to year 2. In multiple regression analysis, first-year height velocity had a significant inverse relationship to JADAS-71 at year 1, age, mean glucocorticoid dosage during the year, and height SD score at baseline. Our findings indicate that during treatment with tocilizumab, patients with systemic JIA experience significant catch-up growth, normalization of IGF-1 levels, and bone balance improvement favoring bone formation. Copyright © 2015 by the American College of Rheumatology.

  7. EFFICACY OF ETANERCEPT IN TREATMENT OF VARIOUS TYPES OF JUVENILE IDIOPATHIC ARTHRITIS

    Directory of Open Access Journals (Sweden)

    O. Yu. Konopel'ko

    2013-01-01

    Full Text Available Aim: to assess efficacy and safety of etanercept in treatment of various types of juvenile idiopathic arthritis in children under conditions of real clinical practice. Patients and methods: 52 children were included into the study, among them 16 were with systemic and 36 with juvenile idiopathic arthritis without extra-articular involvement. Results: etanercept treatment was the most efficient in patients with systemic juvenile idiopathic arthritis without extra-articular involvement. In 6 and 12 months of the treatment 50 and 70% improvement according to the ACRpedi criteria were established in 31/36 (86% and 28/36 (78% of the patients, respectively. In 24 months in 5 (29% of 17 children remained in the study remission stage of the diseases was confirmed. Conclusions: etanercept treatment was not associated with significant unfavorable effects, which allows to recommend this drug for treatment of juvenile idiopathic arthritis without extra-articular involvent and resistant to standard anti-rheumatic therapy.

  8. Intravenous immunoglobulin therapy leading to dramatic improvement in a patient with systemic juvenile idiopathic arthritis and severe pericarditis resistant to steroid pulse therapy.

    Science.gov (United States)

    Aizawa-Yashiro, Tomomi; Oki, Eishin; Tsuruga, Kazushi; Nakahata, Tohru; Ito, Etsuro; Tanaka, Hiroshi

    2012-05-01

    A 7-year-old Japanese boy with a 4-month history of systemic juvenile idiopathic arthritis (s-JIA) experienced disease flare with spiking fever, exanthema and arthralgia. He then developed progressive dyspnea due to severe pericarditis, and proinflammatory hypercytokinemia was suspected. Methylprednisolone pulse therapy was ineffective and echocardiography showed massive pericardial effusion had persisted. Alternatively, subsequent intravenous immunoglobulin (IVIG) therapy resulted in dramatic resolution of the pericardial effusion, and his general condition significantly improved within a few days. This case report may lend further support the use of IVIG for selected patients with s-JIA and severe pericarditis.

  9. Biological agents in polyarticular juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Amarilyo, Gil; Tarp, Simon; Foeldvari, Ivan

    2016-01-01

    (nearly all exceptions were in agreement with the original study data). Serious AEs occurred very infrequently (0-8%) and an analysis was not possible. There were no differences for AEs when compared among biological agents or to placebo. CONCLUSION: There were no statistical differences among biological......BACKGROUND AND OBJECTIVE: Although various biological agents are in use for polyarticular juvenile idiopathic arthritis (pJIA), head-to-head trials comparing the efficacy and safety among them are lacking. We aimed to compare the efficacy and safety of biological agents in pJIA using all currently...... available randomized withdrawal trials (wRCTs). METHODS: A systematic search of MEDLINE, EMBASE, CENTRAL, and clinicaltrials.gov was performed. Eligible wRCTs: patients with pJIA where a biological agent was compared with another biological agent or placebo. Efficacy was evaluated using disease flare...

  10. Cardiac involvement in adult and juvenile idiopathic inflammatory myopathies

    DEFF Research Database (Denmark)

    Schwartz, TThomas W; Diederichsen, L. P.; Lundberg, Ingrid E.

    2016-01-01

    Idiopathic inflammatory myopathies (IIM) include the main subgroups polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and juvenile DM ( JDM). The mentioned subgroups are characterised by inflammation of skeletal muscles leading to muscle weakness and other organs can also...

  11. Towards Establishing a Standardized Magnetic Resonance Imaging Scoring System for Temporomandibular Joints in Juvenile Idiopathic Arthritis

    DEFF Research Database (Denmark)

    Tolend, Mirkamal A; Twilt, Marinka; Cron, Randy Q

    2017-01-01

    of a MRI scoring system for TMJs has important clinical utility in timely improvement of diagnosis, and serving as an outcome measure. We report on a multi-institutional collaboration towards developing a TMJ MRI scoring system for JIA. METHODS: Seven readers independently assessed MRI scans from 21...... patients (42 TMJs, age range 6-16y) using three existing MRI scoring systems from American, German, and Swiss institutions. Reliability scores, scoring system definitions and items were discussed among 10 JIA experts through two rounds of Delphi surveys, nominal group voting, and subsequent consensus...... meetings to create a novel TMJ MRI scoring system. RESULTS: Average-measure intraclass correlation coefficients (avICC) for the total scores of all three scoring systems were highly reliable at 0.96 each. Osteochondral items showed higher reliability than inflammatory items. An additive system was deemed...

  12. Efficacy and safety of biological agents for systemic juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Tarp, Simon; Amarilyo, Gil; Foeldvari, Ivan

    2016-01-01

    OBJECTIVE: To define the optimal biologic agent for systemic JIA (sJIA) based on safety and efficacy data from a randomized controlled trial (RCT). METHODS: Through a systematic literature search, sJIA RCTs evaluating biologic agents were identified. The primary efficacy outcome was defined as a 30......, this meta-analysis of short-term RCTs presents empirical evidence that canakinumab and tocilizumab are more effective than rilonacept. Biologic agents in sJIA seem safe and comparable with respect to SAE risk in the short term....

  13. "Immune Complexes in Juvenile Idiopathic Arthritis"

    Directory of Open Access Journals (Sweden)

    Terry Lynn Moore

    2016-05-01

    Full Text Available Abstract for invited review in Molecular Mechanisms of Immune Complex Pathophysiology thematic issue to be published in Frontiers in Immunology. Immune Complexes(ICin Juvenile Idiopathic Arthritis (JIA Terry L. Moore, MD, FAAP, FACR, MACR Professor of Internal Medicine,Pediatrics, and Molecular Biology and Immunology Director of Adult and Pediatric Rheumatology Saint Louis University School of Medicine Saint Louis, Missouri 631`04,USA Juvenile idiopathic arthritis (JIA reflects a group of clinically heterogeneous, autoimmune disorders in children characterized by chronic arthritis and hallmarked by elevated levels of circulating immune complexes (CICs and associated complement activation by-products in their sera. ICs have been detected in patients’ sera with JIA utilizing a variety of methods, including the anti-human IgM affinity column,C1q solid phase assay, polyethylene glycol precipitation, Staphylococcal Protein A separation method, anti-C1q/C3 affinity columns, and FcγRIII affinity method. As many as 75% of JIA patients have had IC detected in their sera. The CIC proteome in JIA patients has been examined to elucidate disease-associated proteins that are expressed in active disease. Evaluation of these IC s have shown the presence of multiple peptide fragments by SDS-PAGE and 2-DE. Subsequently, all isotypes of rheumatoid factor (RF, isotypes of anti-cyclic citrullinated (CCP peptide antibodies, IgG, C1q, C4, C3, and the membrane attack complex (MAC were detected in these IC. Complement activation and levels of IC correlate with disease activity in JIA, indicating their role in the pathophysiology of the disease. This review will summarize the existing literature and discuss the role of possible protein modification that participates in the generation of immune response. We will address the possible role of these events in the development of ectopic germinal centers that become the secondary site of plasma cell development in JIA. We

  14. The Myeloid-Related Proteins 8 and 14 Complex, a Novel Ligand of Toll-Like Receptor 4, and Interleukin-1 beta Form a Positive Feedback Mechanism in Systemic-Onset Juvenile Idiopathic Arthritis

    NARCIS (Netherlands)

    Frosch, Michael; Ahlmann, Martina; Vogl, Thomas; Wittkowski, Helmut; Wulffraat, Nico; Foell, Dirk; Roth, Johannes

    Objective. Fever of unknown origin is a diagnostic challenge in children, especially for differentiation of systemic-onset juvenile idiopathic arthritis (systemic-onset JIA) and infectious diseases. We undertook this study to analyze the relevance of myeloid-related proteins (MRPs) 8 and 14,

  15. [Optic neuritis in juvenile idiopathic arthritis patient].

    Science.gov (United States)

    Lourenço, Daniela M R; Buscatti, Izabel M; Lourenço, Benito; Monti, Fernanda C; Paz, José Albino; Silva, Clovis A

    2014-01-01

    Optic neuritis (ON) was rarely reported in juvenile idiopathic arthritis (JIA) patients, particularly in those under anti-tumor necrosis factor alpha blockage. However, to our knowledge, the prevalence of ON in JIA population has not been studied. Therefore, 5,793 patients were followed up at our University Hospital and 630 (11%) had JIA. One patient (0.15%) had ON and was reported herein. A 6-year-old male was diagnosed with extended oligoarticular JIA, and received naproxen and methotrexate subsequently replaced by leflunomide. At 11 years old, he was diagnosed with aseptic meningitis, followed by a partial motor seizure with secondary generalization. Brain magnetic resonance imaging (MRI) and electroencephalogram showed diffuse disorganization of the brain electric activity and leflunomide was suspended. Seven days later, the patient presented acute ocular pain, loss of acuity for color, blurred vision, photophobia, redness and short progressive visual loss in the right eye. A fundoscopic exam detected unilateral papilledema without retinal exudates. Orbital MRI suggested right ON. The anti-aquaporin 4 (anti-AQP4) antibody was negative. Pulse therapy with methylprednisolone was administered for five days, and subsequently with prednisone, he had clinical and laboratory improvement. In conclusion, a low prevalence of ON was observed in our JIA population. The absence of anti-AQP4 antibody and the normal brain MRI do not exclude the possibility of demyelinating disease associated with chronic arthritis. Therefore, rigorous follow up is required. Copyright © 2014 Elsevier Editora Ltda. All rights reserved.

  16. Imaging of juvenile idiopathic arthritis. Part II: Ultrasonography and MRI

    Directory of Open Access Journals (Sweden)

    Iwona Sudoł-Szopińska

    2016-09-01

    Full Text Available Juvenile idiopathic arthritis is the most common autoimmune systemic disease of the connective tissue affecting individuals in the developmental age. Radiography, which was described in the first part of this publication, is the standard modality in the assessment of this condition. Ultrasound and magnetic resonance imaging enable early detection of the disease which affects soft tissues, as well as bones. Ultrasound assessment involves: joint cavities, tendon sheaths and bursae for the presence of synovitis, intraand extraarticular fat tissue to visualize signs of inflammation, hyaline cartilage, cartilaginous epiphysis and subchondral bone to detect cysts and erosions, and ligaments, tendons and their entheses for signs of enthesopathies and tendinopathies. Magnetic resonance imaging is indicated in children with juvenile idiopathic arthritis for assessment of inflammation in peripheral joints, tendon sheaths and bursae, bone marrow involvement and identification of inflammatory lesions in whole-body MRI, particularly when the clinical picture is unclear. Also, MRI of the spine and spinal cord is used in order to diagnose synovial joint inflammation, bone marrow edema and spondylodiscitis as well as to assess their activity, location, and complications (spinal canal stenosis, subluxation, e.g. in the atlantoaxial region. This article discusses typical pathological changes seen on ultrasound and magnetic resonance imaging. The role of these two methods for disease monitoring, its identification in the pre-clinical stage and establishing its remission are also highlighted.

  17. Immunization With a Pneumococcal Polysaccharide Vaccine in Children With Juvenile Idiopathic Arthritis Without Systemic Manifestations: a Prospective Study

    Directory of Open Access Journals (Sweden)

    Ekaterina I. Alexeeva

    2017-01-01

    Full Text Available Background. Patients with juvenile idiopathic arthritis (JIA have an increased risk of being infected. Approximately half of all serious infections in children with JIA are associated with airway involvement.Objective. Our aim was to study the efficacy and safety of the pneumococcal 13-valent conjugate vaccine (PCV in children with JIA.Methods. In a prospective cohort study, 5 groups were formed:  children with JIA in the remission phase on methotrexate therapy  (group 1 or etanercept (group 2, with JIA in the active phase prior  to the appointment of methotrexate (group 3 or etanercept (group  4, control group (conditionally healthy children. 0.5 ml of the 13-valent PCV was administered once subcutaneously during therapy in patients in the remission phase or 3 weeks before the appointment  of methotrexate or etanercept in patients in the active phase. The  main study outcome was the proportion of patients with a protective  ( 40 mg/L level of specific anti-pneumococcal antibodies (anti-SPP IgG to Streptococcus pneumoniae 4 weeks after vaccination. In  addition, we assessed the incidence of infectious events before and  after vaccination as well as changes in the content of a high-sensitivity C-reactive protein, S100 protein, and post-vaccination period.Results. The study included 125 children. Four weeks after  vaccination, the protective level of anti-SPP IgG was established in  21 (84% patients in the 1st, 23 (92% in the 2nd, 22 (88% in the  3rd, 24 (96% in the 4th and 5th groups (p =1.0. Increase in the  concentration of S100 protein and high-sensitivity C-reactive protein  after vaccination was not noted. JIA exacerbation episodes were not  recorded in any patient. After immunization, the total number of infectious events decreased in all observed groups (p 0.001. Serious adverse events were not registered during the study.Conclusion. Vaccination with the 13-valent PCV in children with JIA  is highly effective

  18. Ultrasonography and color Doppler in juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Laurell, Louise; Court-Payen, Michel; Nielsen, Susan

    2011-01-01

    Background The ankle region is frequently involved in juvenile idiopathic arthritis (JIA) but difficult to examine clinically due to its anatomical complexity. The aim of the study was to evaluate the role of ultrasonography (US) of the ankle and midfoot (ankle region) in JIA. Doppler-US detected...

  19. Ultrasonography and color Doppler in juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Laurell, Louise; Court-Payen, Michel; Nielsen, Susan

    2012-01-01

    The wrist region is one of the most complex joints of the human body. It is prone to deformity and functional impairment in juvenile idiopathic arthritis (JIA), and is difficult to examine clinically. The aim of this study was to evaluate the role of ultrasonography (US) with Doppler in diagnosis...

  20. Imaging in juvenile idiopathic arthritis with a focus on ultrasonography

    DEFF Research Database (Denmark)

    Laurell, Louise; Court-Payen, Michel; Boesen, Mikael

    2013-01-01

    Early therapeutic intervention and use of new highly efficacious treatments have improved the outcome in many patients with juvenile idiopathic arthritis (JIA), but have also led to the need for more precise methods to evaluate disease activity. In adult rheumatology, numerous studies have establ...

  1. Exercise testing and fitness training in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Singh-Grewal, D.

    2010-01-01

    Juvenile Idiopathic Arthritis is the commonest rheumatic disease of childhood affecting 1:1000 children under the age of 16 years. Children with JIA have long been sidelined from physical activity due to active disease or irrational concerns that activity may in some way worsen disease. Children

  2. Exercise therapy in juvenile idiopathic arthritis: a Cochrane Review

    NARCIS (Netherlands)

    Takken, T.; van Brussel, M.; Engelbert, R. H. H.; van der Net, J.; Kuis, W.; Helders, P. J. M.

    2008-01-01

    Exercise therapy is considered an important component of the treatment of arthritis. The efficacy of exercise therapy has been reviewed in adults with rheumatoid arthritis but not in children with juvenile idiopathic arthritis (JIA). To assess the effects of exercise therapy on functional ability,

  3. Body composition in children with juvenile idiopathic arthritis: effect ...

    African Journals Online (AJOL)

    Introduction: the aim of this study was to evaluate the relationship between macronutrient intake, body composition (lean body mass and fat mass) and bone mineral content in Moroccan children with juvenile idiopathic arthritis (JIA). Methods: a cross-sectional study, conducted between May 2010 and June 2011, covering ...

  4. Juvenile idiopathic arthritis – an update on its diagnosis and ...

    African Journals Online (AJOL)

    Juvenile idiopathic arthritis (JIA) is the most common form of chronic arthritis in children and the most common cause of musculoskeletal disability in children. Early diagnosis may be challenging, but it is essential to ensure good outcomes. This review proposes an approach to the investigation and diagnosis of JIA.

  5. The family journey-to-diagnosis with systemic juvenile idiopathic arthritis: a cross-sectional study of the changing social media presence

    Directory of Open Access Journals (Sweden)

    Modica RF

    2016-05-01

    Full Text Available Renee F Modica,1 Kathleen Graham Lomax,2 Pamela Batzel,3 Leah Shapardanis,3 Kimberly Compton Katzer,3 Melissa E Elder1 1Division of Pediatric Rheumatology, Immunology and Infectious Diseases, University of Florida, Gainesville, FL, USA; 2Immunology and Dermatology Medical Affairs, Novartis Pharmaceuticals Corporation, East Hanover, USA; 3Treato, Princeton, NJ, USA Background: Children with systemic juvenile idiopathic arthritis (SJIA often encounter a delay between symptom onset and disease diagnosis, partly due to the broad differential of fever and lack of symptom recognition by providers. Families often seek multiple medical opinions and post on social media about their frustrations. This linguistic analysis observed the changing language patterns and social media posting behaviors of parents in the time leading to, during, and after SJIA diagnosis. Methods: Public social media sites were manually reviewed by a linguistic team to evaluate posts about SJIA from US-based parents. Results: A total of 3,979 posts between July 2001 and January 2015 were reviewed from 108 sites. Pre-SJIA diagnosis parents sought answers and shared status updates on social media, focusing primarily on the following three site types: alternative/natural lifestyle forums (39%, Facebook (27%, and disease-specific forums (17%. Posts during early prediagnosis phases were characterized by expressive language showing confidence in health care providers and trust in parental instincts. At later prediagnosis stages, parents continued to use social media, but the posts demonstrated increased frustration with delays in diagnosis and gaps in communication with providers. More objective symptom descriptions and a greatly reduced child-centered emotional focus were observed as parents shifted into caregiving roles. Once the diagnosis of SJIA was confirmed, parents used straightforward, less expressive language, and Facebook (47% to make "announcement" posts and increased their

  6. Analysis of the Juvenile Idiopathic Arthritis Immunization Schedule

    Directory of Open Access Journals (Sweden)

    L. S. Namazova-Baranova

    2016-01-01

    Full Text Available Background: The connection between vaccination and autoimmune diseases (and rheumatic pathology in particular is still a subject of discussions. When discussing the possibility of vaccinating rheumatic patients we should take into account the ultra high dangers that infectious diseases pose for such patients, including those that can be prevented by vaccination. We should also take into account the experience of using various vaccine types in rheumatic patients, which illustrates of their high safety profile.Objective: Our aim was to study the immunization schedule in children with juvenile idiopathic arthritis.Methods: The evaluation of vaccine history and other anamnestic data in juvenile idiopathic arthritis patients was based on individual medical records (individual child’s card/preventive vaccination certificate, as well as questionnaires filled by mothers.Results: It has been determined that a significant proportion of children with vaccination schedule deviations are juvenile idiopathic arthritis patients. Almost one in four children with a confirmed rheumatic diagnosis has not been immunized against the major vaccine-preventable diseases. In one non-vaccinated group, there was a case of juvenile arthritis onset after recovering from measles. A small number of patient mothers connects the manifestation of rheumatic diseases with vaccination.Conclusion: Violations of vaccination status in JIA patients require corrections according to the results of clinical studies and the recommendations of international experts.

  7. Therapeutic Potential of Interferon-γ and Its Antagonists in Autoinflammation: Lessons from Murine Models of Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome

    Directory of Open Access Journals (Sweden)

    Anneleen Avau

    2015-11-01

    Full Text Available Interferon-γ (IFN-γ affects immune responses in a complex fashion. Its immunostimulatory actions, such as macrophage activation and induction of T helper 1-type responsiveness, are widely acknowledged, however, as documented by a large body of literature, IFN-γ has also the potential to temper inflammatory processes via other pathways. In autoimmune and autoinflammatory disorders, IFN-γ can either play a disease-enforcing role or act as protective agent, depending on the nature of the disease. In animal models of any particular autoimmune disease, certain changes in the induction procedure can reverse the net outcome of introduction or ablation of IFN-γ. Here, we review the role of endogenous IFN-γ in inflammatory disorders and related murine models, with a focus on systemic juvenile idiopathic arthritis (sJIA and macrophage activation syndrome (MAS. In particular, we discuss our recent findings in a mouse model of sJIA, in which endogenous IFN-γ acts as a regulatory agent, and compare with results from mouse models of MAS. Also, we elaborate on the complexity in the activity of IFN-γ and the resulting difficulty of predicting its value or that of its antagonists as treatment option.

  8. Concurrence of Juvenile Idiopathic Arthritis and Multiple Sclerosis

    Directory of Open Access Journals (Sweden)

    Ben Abdelghani Kaouther

    2011-01-01

    Full Text Available We report a 21-year-old female patient known to have Juvenile idiopathic arthritis (JIA who later developed multiple sclerosis (MS. The disease was documented on the brain and cerebral magnetic resonance imaging (MRI and the visual evoked potential. Our case emphasizes the need to evaluate the symptoms and brain MRI carefully. The concurrence of MS and JIA is uncommon. The possible relationship between the 2 diseases was discussed.

  9. Clinical and Biochemical Characteristics of Children with Juvenile Idiopathic Arthritis

    International Nuclear Information System (INIS)

    Ahmed, S.; Ali, S. R.; Ishaque, S.

    2014-01-01

    Objective: To determine the clinical and biochemical characteristics of children with Juvenile Idiopathic Arthritis (JIA) at a tertiary care centre in Karachi, Pakistan. Study Design: A descriptive study. Place and Duration of Study: Paediatric Rheumatology Clinic of The Aga Khan University Hospital (AKUH), Karachi, from January 2008 to December 2011. Methodology: Clinical and laboratory profile and outcome of children less than 15 years of age attending the Paediatric Rheumatology Clinic of the Aga Khan University, Karachi with the diagnosis of Juvenile Idiopathic Arthritis according to International League against Rheumatism were studied. These children were classified into different types of JIA; their clinical and laboratory characteristics, response to therapy and outcome was evaluated. Results: Sixty eight patients satisfying the criteria of International League against Rheumatism (ILAR) for Juvenile Idiopathic Arthritis were enrolled during the study period of four consecutive years, their age ranged from 9 months to 15 years. Mean age at onset was 6.45 +- 4.03 years while mean age at diagnosis was 7.60 +- 3.93 years. Polyarticular was the most predominant subtype with 37 (54%) patients, out of these, 9 (24%) were rheumatoid factor positive. An almost equal gender predisposition was observed. Fever and arthritis were the most common presenting symptoms, with only 2 patients presenting with uveitis. Conclusion: The clinico-biochemical characteristics of JIA at the study centre showed a pattern distinct with early onset of disease, high frequency of polyarticular type and a higher rheumatoid factor (QRA) and ANA positivity in girls. (author)

  10. Challenges in the management of juvenile idiopathic arthritis with etanercept

    Directory of Open Access Journals (Sweden)

    Clare E Pain

    2009-03-01

    Full Text Available Clare E Pain, Liza J McCannAlder Hey Children’s NHS Foundation Trust, Eaton Road, Liverpool, UKAbstract: Biologic agents have been designed with the help of immunological studies to target particular areas of the immune system which are thought to play a role in the pathogenesis of disease. Etanercept is a soluble anti-tumor necrosis factor alpha (TNF-α agent licensed for the treatment of active poly-articular juvenile idiopathic arthritis (JIA in children aged 4 to 17 years who have failed to respond to methotrexate alone, or who have been intolerant of methotrexate. The safety and efficacy of etanercept in this patient group has been established by one randomized controlled trial and several longitudinal studies. This, together with the fact that until recently etanercept was the only anti-TNF licensed in JIA, has made it the most common first choice biologic for many clinicians. However, there are still many unanswered questions about etanercept, including its efficacy and safety in different subtypes of JIA, in children under 4 years of age and in those with uveitis. There are still concerns about the long term safety of TNF antagonists in the pediatric age group and unanswered questions about increased risks of malignancy and infection. Although adult studies are useful to improve understanding of these risks, they are not a substitute for good quality pediatric research and follow-up studies. Adult trials often include greater numbers of patients. However, they evaluate a different population and drug behavior may vary in children due to differences in metabolism, growth and impact on a developing immune system. In addition, rheumatoid arthritis is a different disease than JIA. Clinicians need to carefully weigh up the risk benefit ratio of anti-TNF use in children with JIA and push for robust clinical trials to address the questions that remain unanswered. This article summarizes the evidence available for use of etanercept in children

  11. Púrpura trombocitopênica imunológica como manifestação inicial de lúpus eritematoso sistêmico juvenil Idiopathic thrombocytopenic purpura as initial manifestation of juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Josefina Aparecida Pellegrini Braga

    2003-12-01

    salientar a importância da determinação de auto-anticorpos para lúpus eritematoso sistêmico nas crianças com a forma crônica desta patologia.Patients with Idiopathic Thrombocytopenic Purpura (ITP present a high trend to develop Systemic Lupus Erythematosus (SLE, especially those with chronic presentation. Some authors observed that female gender, older patients and familial history of autoimmune disease in patients with Idiopathic Thrombocytopenic Purpura are factors that lead to increased susceptibility for the development of Systemic Lupus Erythematosus. Based on these facts, we decided to study 5 children with chronic Idiopathic Thrombocytopenic Purpura and late Systemic Lupus Erythematosus. In this paper, we describe the clinical and laboratorial features of 5 female children with Idiopathic Thrombocytopenic Purpura that later developed Systemic Lupus Erythematosus. All patients were girls, 3 Caucasian, with Idiopathic Thrombocytopenic Purpura onset age ranged between 6 years and 3 months and 12 years and 1 month (mean - 9 years and 2 months. The age at Systemic Lupus Erythematosus diagnosis ranged between 8 years and 13 years and 8 months (mean - 10 years and 10 months. Though, the gap between Idiopathic Thrombocytopenic Purpura and Systemic Lupus Erythematosus diagnosis ranged between 11 months and 2 years and 9 months (mean - 1 year and 10 months. All patients presented chronic Idiopathic Thrombocytopenic Purpura (thrombocytopenia lasts longer than 6 months. The Systemic Lupus Erythematosus classification criteria were (in decreasing frequency: malar erythema and positivity of ANA in 5 patients; arthritis, hematological (thrombocytopenia and immunological alterations (positivity of anti-DNA in 4 patients; photosensitivity and positivity of anti-cardiolipin in 3 patients. Other manifestations included oral ulcers, renal involvement, leukopenia and auto-immune hemolytic anemia, serositis (pericarditis, neurological involvement and positivity of anti-Sm antibody

  12. Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis

    Directory of Open Access Journals (Sweden)

    İlknur Tuğal-Tutkun

    2016-04-01

    Full Text Available Pediatric uveitis may be a serious health problem because of the lifetime burden of vision loss due to severe complications if the problem is not adequately treated. Juvenile idiopathic arthritis (JIA-associated uveitis is characterized by insidious onset and potentially blinding chronic anterior uveitis. Periodic ophthalmologic screening is of utmost importance for early diagnosis of uveitis. Early diagnosis and proper immunomodulatory treatment are essential for good visual prognosis. The goal of treatment is to achieve enduring drug-free remission. The choice of therapeutic regimen needs to be tailored to each individual case. One must keep in mind that patients under immunomodulatory treatment should be monitored closely due to possible side effects. Local and systemic corticosteroids have long been the mainstay of therapy; however, long-term corticosteroid therapy should be avoided due to serious side effects. Steroid-sparing agents in the treatment of JIA-associated uveitis include antimetabolites and biologic agents in refractory cases. Among the various immunomodulatory agents, methotrexate is generally the first choice, as it has a well-established safety and efficacy profile in pediatric cases and does not appear to increase the risk of cancer. Other classic immunomodulators that may also be used in combination with methotrexate include azathioprine, mycophenolate mofetil, and cyclosporin A. Biologic agents, primarily tumor necrosis factor alpha inhibitors including infliximab or adalimumab, should be considered in cases of treatment failure with classic immunomodulatory agents.

  13. The family journey-to-diagnosis with systemic juvenile idiopathic arthritis: a cross-sectional study of the changing social media presence.

    Science.gov (United States)

    Modica, Renee F; Lomax, Kathleen Graham; Batzel, Pamela; Shapardanis, Leah; Katzer, Kimberly Compton; Elder, Melissa E

    2016-01-01

    Children with systemic juvenile idiopathic arthritis (SJIA) often encounter a delay between symptom onset and disease diagnosis, partly due to the broad differential of fever and lack of symptom recognition by providers. Families often seek multiple medical opinions and post on social media about their frustrations. This linguistic analysis observed the changing language patterns and social media posting behaviors of parents in the time leading to, during, and after SJIA diagnosis. Public social media sites were manually reviewed by a linguistic team to evaluate posts about SJIA from US-based parents. A total of 3,979 posts between July 2001 and January 2015 were reviewed from 108 sites. Pre-SJIA diagnosis parents sought answers and shared status updates on social media, focusing primarily on the following three site types: alternative/natural lifestyle forums (39%), Facebook (27%), and disease-specific forums (17%). Posts during early prediagnosis phases were characterized by expressive language showing confidence in health care providers and trust in parental instincts. At later prediagnosis stages, parents continued to use social media, but the posts demonstrated increased frustration with delays in diagnosis and gaps in communication with providers. More objective symptom descriptions and a greatly reduced child-centered emotional focus were observed as parents shifted into caregiving roles. Once the diagnosis of SJIA was confirmed, parents used straightforward, less expressive language, and Facebook (47%) to make "announcement" posts and increased their use of SJIA websites (30%). With treatment initiation, the posts demonstrated a slow return of expressive language and an increased parental understanding of the "new normal". Parents use different language styles, frames of reference, and websites before and after SJIA diagnosis. Gaps in parent-provider communication, especially before diagnosis, and their new roles as caregivers lead to parental use of social

  14. Evaluation of Fitness and the Balance Levels of Children with a Diagnosis of Juvenile Idiopathic Arthritis: A Pilot Study.

    Science.gov (United States)

    Patti, Antonino; Maggio, Maria Cristina; Corsello, Giovanni; Messina, Giuseppe; Iovane, Angelo; Palma, Antonio

    2017-07-19

    Background: Juvenile idiopathic arthritis is a main cause of physical disability and has high economic costs for society. The purpose of this study was to assess the fitness levels and the postural and balance deficits with a specific test battery. Methods: Fifty-six subjects were enrolled in this study. Thirty-nine healthy subjects were included in the control group and seventeen in the juvenile idiopathic arthritis group. All subjects were evaluated using a posturography system. The fitness level was evaluated with a battery of tests (Abalakov test, sit-up test, hand grip test, backsaver sit and reach, the toe touch test). An unpaired t -test was used to determine differences. Pearson's correlation coefficient was used to evaluate the correlation between the tests. Results: The battery of tests demonstrated that subjects in the juvenile idiopathic arthritis group have lower fitness levels compared to the control group. The juvenile idiopathic arthritis group showed low postural control with respect to the control group. Pearson analysis of the juvenile idiopathic arthritis group data showed significant correlations between variables. Pearson's results from the control group data showed a similar trend. Conclusions: The results suggest that the battery of tests used could be an appropriate tool. However, we highlight that these conclusions need to be supported by other studies with a larger population scale.

  15. Evaluation of Fitness and the Balance Levels of Children with a Diagnosis of Juvenile Idiopathic Arthritis: A Pilot Study

    Directory of Open Access Journals (Sweden)

    Antonino Patti

    2017-07-01

    Full Text Available Background: Juvenile idiopathic arthritis is a main cause of physical disability and has high economic costs for society. The purpose of this study was to assess the fitness levels and the postural and balance deficits with a specific test battery. Methods: Fifty-six subjects were enrolled in this study. Thirty-nine healthy subjects were included in the control group and seventeen in the juvenile idiopathic arthritis group. All subjects were evaluated using a posturography system. The fitness level was evaluated with a battery of tests (Abalakov test, sit-up test, hand grip test, backsaver sit and reach, the toe touch test. An unpaired t-test was used to determine differences. Pearson’s correlation coefficient was used to evaluate the correlation between the tests. Results: The battery of tests demonstrated that subjects in the juvenile idiopathic arthritis group have lower fitness levels compared to the control group. The juvenile idiopathic arthritis group showed low postural control with respect to the control group. Pearson analysis of the juvenile idiopathic arthritis group data showed significant correlations between variables. Pearson’s results from the control group data showed a similar trend. Conclusions: The results suggest that the battery of tests used could be an appropriate tool. However, we highlight that these conclusions need to be supported by other studies with a larger population scale.

  16. Juvenile idiopathic arthritis outcome and prognosis according to catamnesis evaluation

    Directory of Open Access Journals (Sweden)

    O V Semenova

    2005-01-01

    Full Text Available Objective. To study outcome and prognosis in pts with juvenile idiopathic arthritis (JIA. Material and methods. 239 JIA pts with disease duration of 10 years and more were analyzed. 80 children and adolescents before 18 years old (66 girls and 14 boys were included in group 1. Arthritis clinical and laboratory activity, radiological stage and functional status according to Steinbroker and CHAQ questionnaire were assessed. 159 grown up pts (109 female and 50 male suffering from JIA from childhood were included in group 2. They were examined using Stanford Health Assessment Questionnaire (HAQ and a specially developed social status questionnaire. Results. Half pts of group 1 had recurrence of the disease during examination but activity in most cases did not exceed stage I or 2 (54% and 31% respectively. Joint destructive changes were revealed in 50% of pts. 80% of pts had radiological signs of secondary osteoarthritis. Amyloidosis was revealed in 2 from 27 pts with systemic type of JIA. 68% of pts had 1 or 2 functional class. 1/3 of pts did not have functional limitations (CHAQ=0. 13% of pts had maximal disability with CHAQ ranging from 2,1 to 3,0 (pts with polyarticular and systemic types of JIA. Most grown up pts (59% considered their health as good. Substantial part of them worked or learned. 10 pts did not worked because of the disease (1,5%. 61% of pts did not have functional limitations (HAQ=0. 32% of pts were disabled. Most of them had 3 or 2 disability degree and had possibility to work. Conclusion. Substantial part of pts with longstanding JIA have stabilization of the disease or remission. Recurrent course of the disease was characterized by decrease of activity. Most children and grown ups with longstanding disease have relatively benign functional outcome. Pts with polyarticular and systemic types of JIA require especial attention because they have maximal risk of joint destruction with severe disability.

  17. MRI assessment of tenosynovitis in children with juvenile idiopathic arthritis: inter- and intra-observer variability

    International Nuclear Information System (INIS)

    Lambot, Karen; Brunelle, Francis; Boavida, Peter; Damasio, Maria Beatrice; Tanturri de Horatio, Laura; Barbuti, Domenico; Desgranges, Marie; Bader-Meunier, Brigitte; Quartier, Pierre; Malattia, Clara; Bracaglia, Claudia; Ording Mueller, Lil-Sofie; Elie, Caroline; Rosendahl, Karen

    2013-01-01

    There is sparse knowledge about grading tenosynovitis using MRI. The purpose of this study was to assess the reliability of a tenosynovitis MRI scoring system in juvenile idiopathic arthritis. Children with juvenile idiopathic arthritis and wrist involvement were enrolled in two paediatric centres, from October 2006 to January 2010. The extensor (compartments II, IV and VI) and flexor tendons were assessed for the presence of tenosynovitis on T1-weighted postcontrast fat-saturated MR images and were scored from 0 (normal) to 2 (moderate to severe) by two observers independently. Intra- and interobserver agreement was assessed. Ninety children (age range: 5-18.5 years) were included, of whom 34 had tenosynovitis involving extensors and 28 had tenosynovitis involving flexors. A total of 360 tendon areas were analysed, of which 114 had tenosynovitis (86/270 extensors and 28/90 flexors). Intra-reader 1 agreement was excellent for the extensors (k = 0.82-0.91) and for the flexors (k = 0.85); intra-reader 2 agreement was moderate to good for the extensors (k = 0.51-0.72) and good for the flexors (k = 0.64). Inter-reader agreement was good for the extensors (k = 0.69-0.73) and moderate for the flexors (k = 0.49). The proposed MRI scoring system for the assessment of wrist tenosynovitis in juvenile idiopathic arthritis appears feasible with an observer agreement sufficient for clinical use. (orig.)

  18. MRI assessment of tenosynovitis in children with juvenile idiopathic arthritis: inter- and intra-observer variability

    Energy Technology Data Exchange (ETDEWEB)

    Lambot, Karen; Brunelle, Francis [Hopital Necker-Enfants Malades, Department of Paediatric Radiology, Paris (France); Boavida, Peter [Great Ormond Street Hospital, Department of Radiology, London (United Kingdom); Damasio, Maria Beatrice [Ospedale Pediatrico Gaslini, Department of Radiology, Genoa (Italy); Tanturri de Horatio, Laura; Barbuti, Domenico [Ospedale Pediatrico Bambino Gesu, Department of Radiology, Rome (Italy); Desgranges, Marie; Bader-Meunier, Brigitte; Quartier, Pierre [Hopital Necker-Enfants Malades, Department of Paediatric Immunology, Hematology and Rheumatology, APHP French Reference Center ' ' Arthrites juveniles' ' , Paris (France); Malattia, Clara [University of Genoa, Department of Paediatrics, Genoa (Italy); Bracaglia, Claudia [Ospedale Pediatrico Bambino Gesu, Department of Paediatrics, Rome (Italy); Ording Mueller, Lil-Sofie [Great Ormond Street Hospital, Department of Radiology, London (United Kingdom); University Hospital of North Norway, Department of Radiology, Tromsoe (Norway); Elie, Caroline [Paris Descartes University, Department of Biostatistics, Hopital Necker-Enfants Malades, Paris (France); Rosendahl, Karen [Great Ormond Street Hospital, Department of Radiology, London (United Kingdom); Haukeland University Hospital, Department of Radiology, Bergen (Norway)

    2013-07-15

    There is sparse knowledge about grading tenosynovitis using MRI. The purpose of this study was to assess the reliability of a tenosynovitis MRI scoring system in juvenile idiopathic arthritis. Children with juvenile idiopathic arthritis and wrist involvement were enrolled in two paediatric centres, from October 2006 to January 2010. The extensor (compartments II, IV and VI) and flexor tendons were assessed for the presence of tenosynovitis on T1-weighted postcontrast fat-saturated MR images and were scored from 0 (normal) to 2 (moderate to severe) by two observers independently. Intra- and interobserver agreement was assessed. Ninety children (age range: 5-18.5 years) were included, of whom 34 had tenosynovitis involving extensors and 28 had tenosynovitis involving flexors. A total of 360 tendon areas were analysed, of which 114 had tenosynovitis (86/270 extensors and 28/90 flexors). Intra-reader 1 agreement was excellent for the extensors (k = 0.82-0.91) and for the flexors (k = 0.85); intra-reader 2 agreement was moderate to good for the extensors (k = 0.51-0.72) and good for the flexors (k = 0.64). Inter-reader agreement was good for the extensors (k = 0.69-0.73) and moderate for the flexors (k = 0.49). The proposed MRI scoring system for the assessment of wrist tenosynovitis in juvenile idiopathic arthritis appears feasible with an observer agreement sufficient for clinical use. (orig.)

  19. Effects of Combined Resistive Underwater Exercises and Interferential Current Therapy in Patients with Juvenile Idiopathic Arthritis: A Randomized Controlled Trial.

    Science.gov (United States)

    Elnaggar, Ragab K; Elshafey, Mohammed A

    2016-02-01

    The objective of this study was to verify the effects of combined resistive underwater exercises and interferential current on the peak torque of the quadriceps and hamstrings and pain levels in patients with juvenile idiopathic arthritis. This is a randomized controlled study; 30 children with polyarticular juvenile idiopathic arthritis were randomly distributed into two groups: the control group (n = 15) received the traditional physical therapy program and the study group (n = 15) received resistive underwater exercises and interferential current therapy. Peak torque of the quadriceps and hamstrings and pain levels were evaluated before treatment, 1 mo later, and after 3 mos using the HUMAC NORM, CSMI Testing and Rehabilitation Isokinetic System and visual analog scale, respectively. In the control group, all measures showed significant differences (P interferential current therapy is a potentially valuable treatment for patients with juvenile idiopathic arthritis.

  20. Skin Manifestations of Rheumatoid Arthritis, Juvenile Idiopathic Arthritis, and Spondyloarthritides.

    Science.gov (United States)

    Chua-Aguilera, Carolyn Jean; Möller, Burkhard; Yawalkar, Nikhil

    2017-12-01

    Extra-articular manifestations of rheumatoid arthritis, juvenile idiopathic arthritis, and various spondyloarthritides including psoriatic arthritis, ankylosing spondylitis, reactive arthritis, and inflammatory bowel disease-associated spondyloarthritis often involve the skin and may occur before or after diagnosis of these rheumatic diseases. Cutaneous manifestations encompass a wide range of reactions that may have a notable negative impact not only on the physical but especially on the emotional and psychosocial well-being of these patients. Several cutaneous manifestations have been related to rheumatoid arthritis such as subcutaneous nodules including classical rheumatoid nodules, accelerated rheumatoid nodulosis, and rheumatoid nodulosis; vascular disorders like rheumatoid vasculitis, livedo racemosa, and Raynaud's phenomenon; and neutrophilic and/or granulomatous diseases like pyoderma gangrenosum, Sweet's syndrome, rheumatoid neutrophilic dermatitis, interstitial granulomatous dermatitis with arthritis, as well as palisaded neutrophilic and granulomatous dermatitis. In juvenile idiopathic arthritis, the main cutaneous manifestations include an evanescent rash, rheumatoid nodules, as well as plaque and guttate psoriasis. Plaque psoriasis is also the main skin disease involved in spondyloarthritides. Furthermore, other forms of psoriasis including guttate, inverse, erythrodermic, pustular, and particularly nail psoriasis may also occur. In addition, a variety of drug-induced skin reactions may also appear in these diseases. Early recognition and understanding of these different dermatologic manifestations together with an interdisciplinary approach are often needed to optimize management of these diseases.

  1. US Evaluation of Juvenile Idiopathic Arthritis and Osteoarticular Infection.

    Science.gov (United States)

    Nguyen, Jie C; Lee, Kenneth S; Thapa, Mahesh M; Rosas, Humberto G

    2017-01-01

    Juvenile idiopathic arthritis (JIA) and osteoarticular infection can cause nonspecific articular and periarticular complaints in children. Although contrast material-enhanced magnetic resonance imaging is the reference standard imaging modality, musculoskeletal ultrasonography (US) is emerging as an important adjunct imaging modality that can provide valuable information relatively quickly without use of radiation or the need for sedation. However, diagnostic accuracy requires a systemic approach, familiarity with various US techniques, and an understanding of maturation-related changes. Specifically, the use of dynamic, Doppler, and/or multifocal US assessments can help confirm sites of disease, monitor therapy response, and guide interventions. In patients with JIA, ongoing synovial inflammation can lead to articular and periarticular changes, including synovitis, tenosynovitis, cartilage damage, bone changes, and enthesopathy. Although these findings can manifest in adult patients with rheumatoid arthritis, important differences and pitfalls exist because of the unique changes associated with an immature and maturing skeleton. In patients who are clinically suspected of having osteoarticular infection, the inability of US to evaluate the bone marrow decreases its sensitivity. Therefore, the US findings should be interpreted with caution because juxtacortical inflammation is suggestive, but neither sensitive nor specific, for underlying osteomyelitis. Similarly, the absence of a joint effusion makes septic arthritis extremely unlikely but not impossible. US findings of JIA and osteoarticular infection often overlap. Although certain clinical scenarios, laboratory findings, and imaging appearances can favor one diagnosis over the other, fluid analysis may still be required for definitive diagnosis and optimal treatment. US is the preferred modality for fluid aspiration and administering intra-articular corticosteroid therapy. © RSNA, 2017.

  2. Long-term outcome in patients with juvenile idiopathic arthritis.

    Science.gov (United States)

    Minden, Kirsten; Niewerth, Martina; Listing, Joachim; Biedermann, Thomas; Bollow, Matthias; Schöntube, Monika; Zink, Angela

    2002-09-01

    To describe the long-term outcome of juvenile idiopathic arthritis (JIA). All patients with JIA referred to a pediatric rheumatology center between 1978 and 1988 were identified and invited to undergo an assessment. Patients with JIA from a population-based cohort from East Berlin were included. The outcome assessment considered changes in body function and structure (e.g., mortality, joint abnormalities, disease activity), activities at the individual level (Health Assessment Questionnaire), and participation in society (e.g., mobility, educational and vocational background). Of 260 eligible patients, 215 (83%) were evaluated. Subtypes of JIA at disease onset included oligoarthritis (40%), polyarthritis (14%), systemic arthritis (14%), psoriatic arthritis (1%), enthesitis-related arthritis (15%), and other arthritis (16%). Followup was conducted after a median of 16.5 years. No deaths occurred in this cohort. At followup, approximately half of the patients had active disease and/or changes in body structures to a variable extent. Approximately one-third of patients rated themselves as being functionally limited. Patients demonstrated good social integration: few mobility problems were reported, and the educational achievements of patients were higher and their rate of unemployment was lower compared with the age-matched population. No significant differences in outcome were found between the population-based and the referral-based cohorts. Even though approximately half of the JIA patients had more or less distinctive changes in body function and/or structure after a disease duration of >15 years, fewer than 10% were severely disabled or handicapped. Because JIA often persists into adulthood, long-term followup and care are necessary.

  3. Juvenile Idiopathic Arthritis in the Era of International Cooperation.

    Science.gov (United States)

    Uziel, Yosef

    2017-01-30

    Juvenile idiopathic arthritis (JIA) is the most common chronic disease of childhood. Improved understanding of its pathogenesis has led to international cooperation in clinical studies. Multicenter, international collaborations and research facilitate rapid enrollment of enough patients to enable a variety of studies, including those of epidemiology, diagnostic and classification criteria, genetic disease predisposition, pathogenesis, outcomes, and treatment protocols. In the last 20 years, the vision of the Pediatric Rheumatology International Trial Organization (PRINTO) has become a reality of worldwide collaboration in pediatric rheumatology research, including North American and European research groups. Major advances have been made in treating systemic JIA and its main complication, macrophage-activating syndrome (MAS). Single Hub and Access Point to Pediatric Rheumatology in Europe (SHARE) is a project of the European Society of Pediatric Rheumatology with the goal of improving clinical care. Based on evidence in the scientific literature, position papers regarding optimal clinical approaches and care have been published. Formal, validated assessment tools to evaluate response to treatment have been developed. Recommendations have been established to encourage international research collaborations, especially in light of major advances achieved in the genetics of pediatric rheumatologic diseases and the need to share biological samples among different countries and continents. Every participating country has disease information available for patients and families. Additionally, educational programs and updated syllabi for pediatric rheumatology have been written to promote similar, high-level academic training in different countries. These efforts have resulted in significant improvements in treatment and in patient prognosis. However, improved cooperation is needed to enhance research with biological and genetic samples. The Israeli Research Group for

  4. Tocilizumab in systemic juvenile idiopathic arthritis in a real-world clinical setting: results from 1 year of postmarketing surveillance follow-up of 417 patients in Japan.

    Science.gov (United States)

    Yokota, Shumpei; Itoh, Yasuhiko; Morio, Tomohiro; Origasa, Hideki; Sumitomo, Naokata; Tomobe, Minako; Tanaka, Kunihiko; Minota, Seiji

    2016-09-01

    To evaluate the safety and effectiveness of tocilizumab (TCZ) in patients with systemic juvenile idiopathic arthritis (sJIA) in real-world clinical settings in Japan. Paediatric patients with sJIA initiating TCZ between April 2008 and February 2012 and those previously enrolled in clinical trials who initiated TCZ before April 2008 were enrolled in a Japanese registry surveillance programme. Safety and effectiveness parameters were collected for 52 weeks. Of 417 patients enrolled, mean age was 11.2 years and 48.0% were female. TCZ exposure was 407.0 patient-years (PYs). Baseline corticosteroid use was higher than in clinical trials. Rates of total adverse events (AEs) and serious AEs (SAEs) were 224.3/100 PYs and 54.5/100 PYs, respectively, with SAEs higher than previously reported. The most frequent AEs and SAEs were infections and infestations (69.8/100 PYs and 18.2/100 PYs, respectively). 74 serious infections occurred in 55 patients (18.2/100 PYs); higher than previously reported. 26 macrophage activation syndrome events were reported in 24 patients (6.4/100 PYs). Fever and rash symptoms improved from baseline to week 52 (54.6% to 5.6% and 43.0% to 5.6%, respectively). At 4 weeks, 8 weeks and 52 weeks, 90.5%, 96.2% and 99.0% of patients achieved normal C reactive protein levels (<0.3 mg/dL), respectively. These first real-world data demonstrated that TCZ was well tolerated, with acceptable safety and effectiveness in patients with sJIA. Higher incidences of SAEs and serious infections may be due to differences, such as corticosteroid use and concomitant diseases, between patient populations enrolled in previously reported clinical trials and this study. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  5. Tocilizumab: The evidence for its place in the treatment of juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Herlin, Troels

    2010-01-01

    INTRODUCTION: Juvenile idiopathic arthritis (JIA) is one of the most common chronic diseases with childhood onset. It comprises different subtypes of which the systemic onset subtype is often resistant to treatment. With the advent of biological treatment with tumor necrosis factor-alpha (TNFalpha......)-inhibitors, the clinical outcome of JIA has improved considerably, but only for subtypes other than systemic JIA. Substantial evidence shows that the proinflammatory cytokine interleukin-6 (IL-6) plays a pivotal role in systemic JIA. The blockage of IL-6 action by tocilizumab, a humanized anti-IL-6-receptor monoclonal...

  6. Idiopathic juvenile osteoporosis: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Ahmet Imerci

    2015-01-01

    Conclusion: With this report, we would like to raise awareness about a possible association of persistent fractures with this rare metabolic disorder, Idiopathic Juvenile Osteoporosis, which should be included in differential diagnosis of patients with persistent appendicular skeleton fractures.

  7. Advances in the treatment of polyarticular juvenile idiopathic arthritis

    Science.gov (United States)

    Webb, Kate; Wedderburn, Lucy R.

    2015-01-01

    Purpose of review To review recent advances in the management strategies of polyarticular course juvenile idiopathic arthritis (JIA) and identify unanswered questions and avenues for further research. Recent findings There is evidence for an early, aggressive, treat-to-target approach for polyarticular JIA. Clinical disease activity criteria have been recently defined and validated, including criteria for inactive disease and the juvenile arthritis disease activity score (JADAS). There is a need for evidence-based, defined disease targets and biomarkers for prediction of response, including targets for remission induction, and guidelines on drug withdrawal. Recent treatment consensus plans and guidelines are discussed and compared, including the 2015 NHS England clinical policy statement, the 2014 Childhood Arthritis and Rheumatology Research Alliance (CARRA) treatment plans and the 2011 American College of Rheumatology (ACR) guidelines. Evidence for new agents such as tocilizumab, rituximab, golimumab, ustekinumab, certolizumab and tofacitinib is promising: the recent clinical trials are summarized here. Stratification of individual patient treatment remains a goal, and predictive biomarkers have been shown to predict success in the withdrawal of methotrexate therapy. Summary There are promising advances in the treatment approaches, disease activity criteria, clinical guidelines, pharmaceutical choices and individually stratified therapy choices for polyarticular JIA. PMID:26147756

  8. Developing and Evaluating JIApp: Acceptability and Usability of a Smartphone App System to Improve Self-Management in Young People With Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Cai, Ran A; Beste, Dominik; Chaplin, Hema; Varakliotis, Socrates; Suffield, Linda; Josephs, Francesca; Sen, Debajit; Wedderburn, Lucy R; Ioannou, Yiannakis; Hailes, Stephen; Eleftheriou, Despina

    2017-08-15

    Flare-ups in juvenile idiopathic arthritis (JIA) are characterized by joint pain and swelling and often accompanied with fatigue, negative emotions, and reduced participation in activities. To minimize the impact of JIA on the physical and psychosocial development and well-being of young people (YP), it is essential to regularly monitor disease activity and side effects, as well as to support self-management such as adherence to treatment plans and engagement in general health-promoting behaviors. Smartphone technology has the potential to engage YP with their health care through convenient self-monitoring and easy access to information. In addition, having a more accurate summary of self-reported fluctuations in symptoms, behaviors, and psychosocial problems can help both YP and health care professionals (HCPs) better understand the patient's condition, identify barriers to self-management, and assess treatment effectiveness and additional health care needs. No comprehensive smartphone app has yet been developed in collaboration with YP with JIA, their parents, and HCPs involved in their care. The objective of this study was to design, develop, and evaluate the acceptability and usability of JIApp, a self-management smartphone app system for YP with JIA and HCPs. We used a qualitative, user-centered design approach involving YP, parents, and HCPs from the rheumatology team. The study was conducted in three phases: (1) phase I focused on developing consensus on the features, content, and design of the app; (2) phase II was used for further refining and evaluating the app prototype; and (3) phase III focused on usability testing of the app. The interview transcripts were analyzed using qualitative content analysis. A total of 29 YP (aged 10-23, median age 17) with JIA, 7 parents, and 21 HCPs were interviewed. Major themes identified as the ones that helped inform app development in phase I were: (1) remote monitoring of symptoms, well-being, and activities; (2

  9. SpineCor treatment for Juvenile Idiopathic Scoliosis: SOSORT award 2010 winner.

    Science.gov (United States)

    Coillard, Christine; Circo, Alin B; Rivard, Charles H

    2010-11-10

    Juvenile idiopathic scoliosis is a condition used to describe patients who are least 4 years of age but younger than 10 when the deformity is first identified. In these patients, the condition is usually progressive and those that are diagnosed at five years or younger have a high chance of progression to a large curve, with additional pulmonary and cardiac complications. The main form of conservative treatment for juvenile scoliosis is the use of a bracing system. This prospective interventional study was conducted to evaluate the effectiveness of the Dynamic SpineCor orthosis for juvenile idiopathic scoliosis as well as to evaluate the stability of the spine after the weaning point. For this study, 150 juvenile patients were treated by the SpineCor orthosis between 1993 and 2009. Of these, 67 patients had a definite outcome and 83 are still actively being treated. To determine the effectiveness of the brace the OUTCOME criteria recommended by the SRS was used. The results from our study showed that of the 67 patients with a definite outcome, 32.9% corrected their Cobb angle by at least 5° and 10.5% had a stabilization of their Cobb angle. Within the patients with a definite outcome, 37.3% of patients where recommended for surgery before authorized end of treatment. For this group of patients, surgery was postponed. Looking at the stability of the curves 2 years after the end of the treatment, we found 12.5% of the patients continued their correction without the brace being used and 71.4% remained stable. From our study we can clearly see that the effectiveness of the SpineCor orthosis in obtaining and maintaining the neuromuscular integration of the corrective movement can be achieved effectively for juvenile patients. Over 75% of all patients that finished the treatment had remained stable with a few continuing to correct their Cobb angle after the use of the SpineCor orthosis was discontinued. Our conclusion from this study is that the SpineCor orthosis is a

  10. SpineCor treatment for Juvenile Idiopathic Scoliosis: SOSORT award 2010 winner

    Directory of Open Access Journals (Sweden)

    Circo Alin B

    2010-11-01

    Full Text Available Introduction Juvenile idiopathic scoliosis is a condition used to describe patients who are least 4 years of age but younger than 10 when the deformity is first identified. In these patients, the condition is usually progressive and those that are diagnosed at five years or younger have a high chance of progression to a large curve, with additional pulmonary and cardiac complications. The main form of conservative treatment for juvenile scoliosis is the use of a bracing system. This prospective interventional study was conducted to evaluate the effectiveness of the Dynamic SpineCor orthosis for juvenile idiopathic scoliosis as well as to evaluate the stability of the spine after the weaning point. Material and Methods For this study, 150 juvenile patients were treated by the SpineCor orthosis between 1993 and 2009. Of these, 67 patients had a definite outcome and 83 are still actively being treated. To determine the effectiveness of the brace the OUTCOME criteria recommended by the SRS was used. Results The results from our study showed that of the 67 patients with a definite outcome, 32.9% corrected their Cobb angle by at least 5° and 10.5% had a stabilization of their Cobb angle. Within the patients with a definite outcome, 37.3% of patients where recommended for surgery before authorized end of treatment. For this group of patients, surgery was postponed. Looking at the stability of the curves 2 years after the end of the treatment, we found 12.5% of the patients continued their correction without the brace being used and 71.4% remained stable. Discussion From our study we can clearly see that the effectiveness of the SpineCor orthosis in obtaining and maintaining the neuromuscular integration of the corrective movement can be achieved effectively for juvenile patients. Over 75% of all patients that finished the treatment had remained stable with a few continuing to correct their Cobb angle after the use of the SpineCor orthosis was

  11. Immunogenicity, safety and tolerability of a bivalent human papillomavirus vaccine in adolescents with juvenile idiopathic arthritis.

    Science.gov (United States)

    Esposito, Susanna; Corona, Fabrizia; Barzon, Luisa; Cuoco, Federica; Squarzon, Laura; Marcati, Giorgia; Torcoletti, Marta; Gambino, Monia; Palù, Giorgio; Principi, Nicola

    2014-11-01

    To evaluate the immunogenicity, safety and tolerability of the bivalent HPV vaccine in female patients with juvenile idiopathic arthritis (JIA). Twenty-one patients with JIA aged 12-25 years and 21 healthy controls were enrolled and received three doses of the bivalent HPV vaccine. All of the subjects were seronegative at baseline and seroconverted after the scheduled doses. The JIA patients showed significantly lower HPV16 neutralising antibody titres than controls 1 month after the administration of the third dose (p HPV18 neutralising antibody titres. Local and systemic reactions were similarly frequent in the patients and controls, and there were no significant changes in 27-joint juvenile arthritis disease activity score or laboratory tests. The bivalent HPV vaccine is safe in patients with stable JIA and regardless of the use of medications the vaccine assures an adequate degree of protection for a certain time.

  12. Polyarticular juvenile idiopathic arthritis – epidemiology and management approaches

    Directory of Open Access Journals (Sweden)

    Oberle EJ

    2014-10-01

    Full Text Available Edward J Oberle, Julia G Harris, James W VerbskyDepartment of Pediatrics, Division of Rheumatology, Medical College of Wisconsin, Milwaukee, WI, USAAbstract: Juvenile idiopathic arthritis (JIA is a group of disorders characterized by arthritis persisting for at least 6 weeks with onset before the age of 16 years. Within this cluster of conditions, the polyarticular form (involving more than four joints within the first 6 months is further divided based on the presence of rheumatoid factor. Children with polyarticular JIA pose unique diagnostic and therapeutic challenges compared to children with involvement of fewer joints. Polyarticular JIA patients tend to have a more refractory course and therefore are at increased risk for joint damage, resulting in poorer functional outcomes and decreased quality of life. Although the ability to treat this disorder continues to improve, especially with the advent of biologic agents, there is still much about the epidemiology and pathogenesis of polyarticular JIA that is unknown. The epidemiology of polyarticular JIA varies worldwide with a vast difference in reported cases between different global regions as well as within individual countries. Several genetic risk loci have been identified conferring increased susceptibility to JIA, many within the human leukocyte antigen region. Beyond the genome, environmental factors also seem to contribute to the etiology of polyarticular JIA. This review article will focus on the epidemiology and current treatments of polyarticular JIA and briefly discuss genetic and environmental influences on the pathogenesis of JIA as well as new and emerging therapies.Keywords: juvenile arthritis, polyarticular, epidemiology, treatment, rheumatology

  13. Juvenile idiopathic inflammatory myopathies: the value of magnetic resonance imaging in the detection of muscle involvement

    Directory of Open Access Journals (Sweden)

    Maria Odete Esteves Hilário

    2000-03-01

    Full Text Available CONTEXT: One of the major current challenges related to juvenile idiopathic inflammatory myopathy is the search for highly sensitive and specific non-invasive methods for diagnosis as well as for follow-up. OBJECTIVES: The aim of our study was to describe typical magnetic resonance imaging findings and to investigate the usefulness of this method in detecting active muscle disease in juvenile dermatomyositis and juvenile systemic lupus erythematosus patients. DESIGN: Transverse study, blinded assessment. SETTING: University referral unit (Pediatric Rheumatology section, Department of Pediatrics, Universidade Federal de São Paulo / Escola Paulista de Medicina. SAMPLE: Thirteen patients (9 girls with dermatomyositis, as well as 13 patients (12 girls with juvenile systemic lupus erythematosus and 10 normal children (5 girls, were enrolled in the study. MAIN MEASUREMENTS: Qualitative and quantitative analyses of gluteus maximus, quadriceps, adductors and flexors were performed and evaluated by two radiologists, blinded to all clinical information. Spin-echo in T1, DP, T2 and IR was used in all MRI images. RESULTS: The different muscle groups presented non-uniform involvement in the patients. The patients with dermatomyositis presented acute and chronic muscular alterations, while those with lupus presented only chronic myopathy, especially atrophy. In the dermatomyositis group, the major alterations were found in the gluteus and flexor regions (signal intensity and fat replacement. The signal intensity was increased in all acute myopathies. CONCLUSION: The qualitative and quantitative resonance analyses are useful in detecting clinically active disease in patients with dermatomyositis.

  14. Macrophages – silent enemies in juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Joanna Świdrowska-Jaros

    2016-07-01

    Full Text Available The inflammatory response by secretion of cytokines and other mediators is postulated as one of the most significant factors in the pathophysiology of juvenile idiopathic arthritis (JIA. The effect of macrophage action depends on the type of their activation. Classically activated macrophages (M1 are responsible for release of molecules crucial for joint inflammation. Alternatively activated macrophages (M2 may recognize self antigens by scavenger receptors and induce the immunological reaction leading to autoimmune diseases such as JIA. Molecules essential for JIA pathophysiology include: TNF-α, the production of which precedes synovial inflammation in rheumatoid arthritis; IL-1 as a key mediator of synovial damage; chemotactic factors for macrophages IL-8 and MCP-1; IL6, the level of which correlates with the radiological joint damage; MIF, promoting the secretion of TNF-α and IL-6; CCL20 and HIF, significant for the hypoxic synovial environment in JIA; GM-CSF, stimulating the production of macrophages; and IL-18, crucial for NK cell functions. Recognition of the role of macrophages creates the potential for a new therapeutic approach.

  15. Audiovestibular function in patients with juvenile idiopathic arthritis (JIA).

    Science.gov (United States)

    El-Gharib, Amani Mohamed; El-Barbary, Amal Mohamad; Aboelhawa, Marwa Ahmed; Elkholy, Radwa Mostafa

    2016-10-01

    It was found that JIR children had potential sensory neural hearing loss and vestibular affection. Therefore, this study recommends: early complete audiologic evaluation of JIA child followed by regular follow-up, including TOAEs, extended high-frequency audiometry, and VNG. This follow-up is important for preliminary diagnosis and management in order to prevent the negative impact of hearing loss on a child's life. The aim of this study was to assess hearing in children with Juvenile idiopathic arthritis (JIA) and compare them with a healthy control group. In addition to conventional audiometry, extended high-frequency audiometry and Transient otoacoustic emission (TOAEs) were used. This study also tried to investigate the vestibular function in JIR children by videonystagmography (VNG). The study group comprised of 28 children with JIR and 28 healthy children. All subjects were examined audiologically using basic audiological evaluation, high-frequency audiometry, TOAEs, and VNG. Children with JIR had apparent normal peripheral hearing in conventional audiometry; sub-clinical sensory neural hearing loss was detected. This sub-clinical hearing loss appeared in statistically significant difference between them and normal in high-frequency audiometry and TOAEs. VNG test results showed affected tracking and second tests.

  16. Surgical Treatment of Dentofacial Deformities Caused by Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Stoor, Patricia; Hodzic, Zlatan; Arte, Sirpa

    2018-01-01

    The purpose of our retrospective study was to evaluate the results of orthognathic treatment, distraction osteogenesis, and/or prosthetic reconstruction of the temporomandibular joints in patients with juvenile idiopathic arthritis (JIA).Twelve patients with severely affected temporomandibular joints (TMJs) and reduced ramus height were treated with mandibular advancement with orthognathic surgery (11) and additional bilateral or unilateral mandibular ramus distraction (3) or additional bilateral or unilateral prosthetic reconstruction of the TMJ (3). One patient was treated surgically with bilateral TMJ prosthetic reconstruction only. The patients were followed up clinically and radiologically with emphasis on healing, TMJ function, stability of the occlusion, skeletal stability, and facial appearance for an average of 2.3 years after the final surgery. The mean mandibular advancement was 10.1 mm. The mean relapse at pogonion was 2.1 mm, which represents 20.8% of the surgical advancement. The occlusion was stable in 11/12 patients. The TMJ function was good and the facial esthetics improved in all patients. Orthognathic treatment and mandibular ramus distraction osteogenesis provide beneficial lengthening of the mandibular body in JIA patients with asymptomatic and stabile condyles. In adult patients with relapse of the disease or postoperative condylar relapse prosthetic total joint replacement is a reliable and safe alternative.

  17. Nocardia brasiliensis infection mimicking juvenile idiopathic arthritis in a 4-year-old girl.

    Science.gov (United States)

    Kapur, Nitin; Adib, Navid; Grimwood, Keith

    2013-11-01

    Nocardia are ubiquitous environmental saprophytes that cause pneumonia and disseminated disease in immunocompromised patients. They can also cause localized cutaneous and soft tissue infections in healthy people after direct percutaneous inoculation. Nocardia arthritis is rare in both forms of the disease. Here we present the first published case of a child with septic arthritis caused by N brasiliensis. Importantly, this otherwise well 4-year-old girl had no known history of trauma but presented with transient cutaneous lesions and a 6-week history of arthritis involving the right fourth digit proximal interphalangeal joint without accompanying fever or raised systemic inflammatory markers. She received a diagnosis of juvenile idiopathic arthritis and underwent antiinflammatory and immunosuppressant therapy. After 2 months she developed frank septic arthritis, which necessitated a surgical joint washout, from which an intraoperative swab grew N brasiliensis. The patient received 6 months of high-dose trimethoprim-sulfamethoxazole and remains well more than 4 years after treatment. This unusual case highlights the importance of considering an indolent infection from slow-growing organisms, including Nocardia, when diagnosing the oligoarthritis subtype of juvenile idiopathic arthritis. This is especially relevant when a single joint is involved and response to antiinflammatory therapy is suboptimal because antiinflammatory agents may mask evolving signs of infection.

  18. Medication adherence in patients with juvenile idiopathic arthritis.

    Science.gov (United States)

    Adriano, Liana Silveira; de França Fonteles, Marta Maria; de Fátima Menezes Azevedo, Maria; Beserra, Milena Pontes Portela; Romero, Nirla Rodrigues

    The aim of this study was to investigate pharmacological treatment adherence of patients with juvenile idiopathic arthritis, attended in an outpatient pharmacy at a tertiary hospital in northeastern Brazil. The analysis of adherence was performed along with caregivers, through a structured questionnaire based on Morisky, Green and Levine, which enabled the categorization of adherence in "highest", "moderate" or "low" grades, and through evaluating medication dispensing registers, which classified the act of getting medications at the pharmacy as "regular" or "irregular". Drug Related Problems (DRP) were identified through the narrative of caregivers and classified according to the Second Granada Consensus. Then, a pharmaceutical orientation chart with information about the therapeutic regimen was applied, in order to function as a guide for issues that influenced adherence. A total of 43 patients was included, with a mean age of 11.12 years, and 65.1% (n=28) were female. Applying the questionnaire, it was found "highest" adherence in 46.5% (n=20) patients, "moderate" adherence in 48.8% (n=21), and "low" adherence in 4.7% (n=2). Through an analysis of the medication dispensing registers, a lower level of adherence was observed: only 25.6% (n=11) of the participants received "regularly" the medications. Twenty-six DRP was identified, and 84.6% (n=22) were classified as real. There were no significant associations between socio-demographic variables and adherence, although some caregivers have reported difficulty in accessing the medicines and in understanding the treatment. Our findings showed problems in the adherence process related to inattention, forgetfulness and irregularity in getting medicines, reinforcing the need for the development of strategies to facilitate a better understanding of treatment and to ensure adherence. Copyright © 2016 Elsevier Editora Ltda. All rights reserved.

  19. Intravoxel incoherent motion magnetic resonance imaging of the knee joint in children with juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Hilbert, Fabian; Sauer, Alexander; Koestler, Herbert [University Hospital Wuerzburg, Department of Diagnostic and Interventional Radiology, Wuerzburg (Germany); Holl-Wieden, Annette [University Hospital Wuerzburg, Department of Paediatrics, Wuerzburg (Germany); Neubauer, Henning [University Hospital Wuerzburg, Department of Diagnostic and Interventional Radiology, Wuerzburg (Germany); University Hospital Ulm, Department of Diagnostic and Interventional Radiology, Ulm (Germany)

    2017-05-15

    MRI of synovitis relies on use of a gadolinium-based contrast agent. Diffusion-weighted MRI (DWI) visualises thickened synovium but is of limited use in the presence of joint effusion. To investigate the feasibility and diagnostic accuracy of diffusion-weighted MRI with intravoxel incoherent motion (IVIM) for diagnosing synovitis in the knee joint of children with juvenile idiopathic arthritis. Twelve consecutive children with confirmed or suspected juvenile idiopathic arthritis (10 girls, median age 11 years) underwent MRI with contrast-enhanced T1-weighted imaging and DWI at 1.5 T. Read-out segmented multi-shot DWI was acquired at b values of 0 s/mm{sup 2}, 200 s/mm{sup 2}, 400 s/mm{sup 2} and 800 s/mm{sup 2}. We calculated the IVIM parameters perfusion fraction (f) and tissue diffusion coefficient (D). Diffusion-weighted images at b=800 s/mm{sup 2}, f parameter maps and post-contrast T1-weighted images were retrospectively assessed by two independent readers for synovitis using the Juvenile Arthritis MRI Scoring system. Seven (58%) children showed synovial hypertrophy on contrast-enhanced imaging. Diagnostic ratings for synovitis on DWI and on f maps were fully consistent with contrast-enhanced imaging, the diagnostic reference. Two children had equivocal low-confidence assessments on DWI. Median f was 6.7±2.0% for synovitis, 2.1±1.2% for effusion, 5.0±1.0% for muscle and 10.6±5.7% for popliteal lymph nodes. Diagnostic confidence was higher based on f maps in three (25%) children and lower in one child (8%), as compared to DWI. DWI with IVIM reliably visualises synovitis of the knee joint. Perfusion fraction maps differentiate thickened synovium from joint effusion and hence increase diagnostic confidence. (orig.)

  20. Substance use and sexual function in juvenile idiopathic arthritis.

    Science.gov (United States)

    van Weelden, Marlon; Lourenço, Benito; Viola, Gabriela R; Aikawa, Nadia E; Queiroz, Lígia B; Silva, Clovis A

    2016-01-01

    To evaluate alcohol/tobacco/illicit drug use and sexual function in adolescent juvenile idiopathic arthritis (JIA) and healthy controls. 174 adolescents with pediatric rheumatic diseases were selected. A cross-sectional study with 54 JIA patients and 35 controls included demographic/anthropometric data and puberty markers assessments, physician-conducted CRAFFT (car/relax/alone/forget/friends/trouble) screen tool for substance abuse/dependence high risk and a questionnaire that evaluated sexual function, bullying and alcohol/tobacco/illicit drug use. Clinical/laboratorial data and treatment were also assessed in JIA. The median current age was similar between JIA patients and controls [15(10-19) vs. 15(12-18) years, p=0.506]. Frequencies of alcohol/tobacco/illicit drug use were high and similar in both JIA and controls (43% vs. 46%, p=0.829). However, age at alcohol onset was significantly higher in those with JIA [15(11-18) vs. 14(7-18) years, p=0.032], particularly in polyarticular onset (p=0.040). High risk for substance abuse/dependence (CRAFFT score≥2) was found in both groups (13% vs. 15%, p=1.000), likewise bullying (p=0.088). Further analysis of JIA patients regarding alcohol/tobacco/illicit drug use showed that the median current age [17(14-19) vs. 13(10-19)years, p<0.001] and education years [11(6-13) vs. 7(3-12)years, p<0.001] were significant higher in those that used substances. Sexual activity was significantly higher in the former group (48% vs. 7%, p<0.001). A positive correlation was evidenced between CRAFFT score and current age in JIA patients (p=0.032, r=+0.296). A high risk for substance abuse/dependence was observed in both JIA and controls. JIA substance users were more likely to have sexual intercourse. Therefore, routine screening is suggested in all visits of JIA adolescents. Copyright © 2016 Elsevier Editora Ltda. All rights reserved.

  1. Access to pediatric rheumatology care for Juvenile Idiopathic Arthritis in the United Arab Emirates.

    Science.gov (United States)

    Khawaja, Khulood; Al-Maini, Mustafa

    2017-05-16

    This study looks at access to care for Juvenile Idiopathic Arthritis through pediatric rheumatology in the UAE, as an example of multi-ethnic society. Patients with a diagnosis of Juvenile idiopathic arthritis were identified through the hospital electronic medical records system from January 1st 2011 to December 31st 2014. All residents of the United Arab Emirates hold an Emirates identity card. We divided our patients into two groups: Emirati-Emirates, who are native Emirati children and hold the Emirati nationality, as stated on their Emirates identity card, and who therefore have full, comprehensive access to free medical care; and non-Emirati-Emirates, who represent other nationalities, as stated on their Emirates identity card. The primary objective of this study is to look at access to care for Juvenile idiopathic arthritis through pediatric rheumatology in the two groups. The secondary objective is to look at the effect of having multiple types of healthcare insurance coverage on access to biologics. A retrospective review was carried out. Sixty-six patients with JIA identified: 33 Emirates and 33 non-Emirates. For Emirates, the mean time from onset to first appointment with pediatric rheumatologist and diagnosis is 9 months (range: 1-48), and for non-Emirates is 12.4 months (range: 1-96). Among the Emirates, 10 patients are currently on biologic with methotrexate. Among the non-Emirates, 15 are on biologic with methotrexate. Among the Emirates, 12 are currently in remission while on treatment, as are 10 non-Emirates. Regarding disability, one Emirati patient has blindness secondary to noncompliance while under previous treatment. One Non-Emirati developed joint deformities due to periods of noncompliance and no follow up. Delay in presentation to pediatric rheumatology has been identified as an important factor in our population, which is multi-cultural and multi-ethnic. Type of health care insurance cover did not affect number of patients getting

  2. Periodontal and hematological characteristics associated with aggressive periodontitis, juvenile idiopathic arthritis, and rheumatoid arthritis

    DEFF Research Database (Denmark)

    Poulsen, Anne Havemose; Westergaard, Jytte; Stoltze, Kaj

    2006-01-01

    Periodontitis shares several clinical and pathogenic characteristics with chronic arthritis, and there is some degree of coexistence. The aims of this study were to elucidate whether patients with localized aggressive periodontitis (LAgP), generalized aggressive periodontitis (GAgP), juvenile idi...... idiopathic arthritis (JIA), and rheumatoid arthritis (RA) share periodontal and hematological characteristics distinguishing them from individuals free of diseases....

  3. The enigma of uveitis in juvenile idiopathic arthritis : Genetic, immunologic and clinical aspects

    NARCIS (Netherlands)

    Haasnoot, A.J.W.

    2018-01-01

    Uveitis associated with juvenile idiopathic arthritis (JIA) is a sight-threatening inflammation of the eye, affecting up to 30% of children with JIA. Despite the fact that the association of uveitis in JIA was already described by Ohm in 1910 and has a life-long impact, it remains a poorly

  4. Safety of measles, mumps and rubella vaccination in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Heijstek, Marloes W; Pileggi, Gecilmara C S; Zonneveld-Huijssoon, Evelien; Armbrust, Wineke; Hoppenreijs, Esther P A H; Uiterwaal, Cuno S P M; Kuis, Wietse; Wulffraat, Nico M

    2007-01-01

    Objective: To assess the effect of measles, mumps and rubella (MMR) vaccination on disease activity in children with juvenile idiopathic arthritis (JIA). Methods: A retrospective observational multicentre cohort study was performed in 314 patients with JIA, born between 1989 and 1996. Disease

  5. Idiopathic juvenile osteoporosis: A case report and review of the literature.

    Science.gov (United States)

    Imerci, Ahmet; Canbek, Umut; Haghari, Sema; Sürer, Levent; Kocak, Muge

    2015-01-01

    Idiopathic Juvenile Osteoporosis is an uncommon condition that has few case reports in the literature. Reported series indicate that it is a condition classically accompanying vertebral and metaphyseal fractures during the immediate pre-puberty years but that seems to develop naturally during puberty. Current clinical treatment is complicated because of lack of understanding on the origins of Idiopathic Juvenile Osteoporosis. The 13-year-old female patient with no former complaints had pain in her left hip while walking 2 years ago. Excluding the secondary osteoporosis reasons, the patient was diagnosed with Idiopathic Juvenile Osteoporosis and after the medical treatment she was followed-up. The patient was subjected to a rehabilitation program for muscle weakness. She had difficulty in walking as a result of prolonged immobilization. At the end of a two-year treatment, significant improvement was achieved in muscle strength in the extremities, walking distance, and posture. With this report, we would like to raise awareness about a possible association of persistent fractures with this rare metabolic disorder, Idiopathic Juvenile Osteoporosis, which should be included in differential diagnosis of patients with persistent appendicular skeleton fractures. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.

  6. Anti-cyclic citrullinated peptide (anti-CCP) antibodies in children with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    van Rossum, Marion; van Soesbergen, Renée; de Kort, Sandra; ten Cate, Rebecca; Zwinderman, Aeilko H.; de Jong, Ben; Dijkmans, Ben; van Venrooij, Walther J.

    2003-01-01

    Objective. To determine if anti-cyclic citrullinated peptide antibodies (anti-CCP) can be detected in sera of patients with juvenile idiopathic arthritis (JIA) and if they can be used to identify patients with a more destructive course of disease. Methods. One hundred serum samples of 71 patients

  7. Prediction of Methotrexate Intolerance in Juvenile Idiopathic Arthritis: A prospective, observational cohort study

    NARCIS (Netherlands)

    E.H.P.V. Dijkhuizen (Evert H.P.V.); M.B. C̈alasan (M. Bulatovic̈); S. Pluijm (Saskia); M.C.F.J.D. Rotte (Maurits C.F.J.D.); S.J. Vastert (Sebastiaan); S.S.M. Kamphuis (Sylvia); R.D. Jonge (Robert D.); N.M. Wulffraat (Nico)

    2015-01-01

    textabstractBackground: Methotrexate (MTX) is an effective and safe drug in the treatment of juvenile idiopathic arthritis (JIA). Despite its safety, MTX-related gastrointestinal adverse effects before and after MTX administration, termed MTX intolerance, occur frequently, leading to non-compliance

  8. Increasing feasibility and patient comfort of MRI in children with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Hemke, Robert; van Veenendaal, Mira; Kuijpers, Taco W.; van Rossum, Marion A. J.; Maas, Mario

    2012-01-01

    MRI is the most sensitive imaging modality in juvenile idiopathic arthritis (JIA), but has practical limitations. Optimizing the scanning protocol is, therefore, necessary to increase feasibility and patient comfort. To determine the feasibility of bilateral non-contrast-enhanced open-bore MRI of

  9. Aerobic and anaerobic exercise capacity in children with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    van Brussel, M.; Lelieveld, O. T. H. M.; van der Net, J.; Engelbert, R. H. H.; Helders, P. J. M.; Takken, T.

    2007-01-01

    To compare the aerobic and anaerobic exercise capacity of children with juvenile idiopathic arthritis (JIA) with healthy controls, to determine if there were differences based on disease onset type, and to examine the relationship between aerobic and anaerobic exercise capacity in children with JIA.

  10. Aerobic and anaerobic exercise capacity in children with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    van Brussel, Marco; Lelieveld, O T H M; van der Net, J; Engelbert, R H H; Helders, P J M; Takken, T

    2007-01-01

    Objective. To compare the aerobic and anaerobic exercise capacity of children with juvenile idiopathic arthritis (JIA) with healthy controls, to determine if there were differences based on disease onset type, and to examine the relationship between aerobic and anaerobic exercise capacity in

  11. Aerobic and anaerobic exercise capacity in adolescents with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Lelieveld, Otto; van Brussel, Marco; Takken, Tim; van Weert, Ellen; van Leeuwen, Miek A.; Armbrust, Wineke

    2007-01-01

    OBJECTIVE: To examine the aerobic and anaerobic exercise capacity in adolescents with juvenile idiopathic arthritis (JIA) compared with age- and sex-matched healthy individuals, and to assess associations between disease-related variables and aerobic and anaerobic exercise capacity. METHODS: Of 25

  12. Measuring Physical Activity in Juvenile Idiopathic Arthritis : Activity Diary Versus Accelerometer

    NARCIS (Netherlands)

    Armbrust, Wineke; Bos, G J F Joyce; Geertzen, Jan H B; Sauer, Pieter J J; Dijkstra, Pieter U; Lelieveld, Otto T H M

    2017-01-01

    OBJECTIVE: (1) To determine convergent validity of an activity diary (AD) and accelerometer (Actical brand/Phillips-Respironics) in measuring physical activity (PA) in children with juvenile idiopathic arthritis (JIA). (2) To determine how many days give reliable results. (3) To analyze effects of

  13. Joint cartilage thickness and automated determination of bone age and bone health in juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Twilt, Marinka; Pradsgaard, Dan; Spannow, Anne Helene

    2017-01-01

    BACKGROUND: BoneXpert is an automated method to calculate bone maturation and bone health index (BHI) in children with juvenile idiopathic arthritis (JIA). Cartilage thickness can also be seen as an indicator for bone health and arthritis damage. The objective of this study was to evaluate...

  14. High prevalence of methotrexate intolerance in juvenile idiopathic arthritis : development and validation of a methotrexate intolerance severity score

    NARCIS (Netherlands)

    Bulatović, Maja; Heijstek, Marloes W; Verkaaik, Marleen; van Dijkhuizen, E H Pieter; Armbrust, Wineke; Hoppenreijs, Esther P A; Kamphuis, Sylvia; Kuis, Wietse; Egberts, Toine C G; Sinnema, Gerben; Rademaker, Carin M A; Wulffraat, Nico M

    OBJECTIVE: To design and validate a new questionnaire for identifying patients with methotrexate (MTX) intolerance, and to determine the prevalence of MTX intolerance in patients with juvenile idiopathic arthritis (JIA) using this questionnaire. METHODS: The MTX Intolerance Severity Score (MISS)

  15. High prevalence of methotrexate intolerance in juvenile idiopathic arthritis: development and validation of a methotrexate intolerance severity score

    NARCIS (Netherlands)

    Bulatovic, M.; Heijstek, M.W.; Verkaaik, M.; Dijkhuizen, E.H. van; Armbrust, W.; Hoppenreijs, E.P.A.H.; Kamphuis, S.; Kuis, W.; Egberts, T.C.; Sinnema, G.; Rademaker, C.M.A.; Wulffraat, N.M.

    2011-01-01

    OBJECTIVE: To design and validate a new questionnaire for identifying patients with methotrexate (MTX) intolerance, and to determine the prevalence of MTX intolerance in patients with juvenile idiopathic arthritis (JIA) using this questionnaire. METHODS: The MTX Intolerance Severity Score (MISS)

  16. Predictors of poor response to methotrexate in polyarticular-course juvenile idiopathic arthritis: analysis of the PRINTO methotrexate trial

    NARCIS (Netherlands)

    Vilca, Iris; Munitis, Pablo Garcia; Pistorio, Angela; Ravelli, Angelo; Buoncompagni, Antonella; Bica, Blanca; Campos, Lucia; Häfner, Renate; Hofer, Michael; Ozen, Seza; Huemer, Christian; Bae, Sang Cheol; Sztajnbok, Flavio; Arguedas, Olga; Foeldvari, Ivan; Huppertz, Hans Iko; Gamir, Maria Luz; Magnusson, Bo; Dressler, Frank; Uziel, Yosef; van Rossum, Marion A. J.; Hollingworth, Peter; Cawkwell, Gail; Martini, Alberto; Ruperto, Nicolino

    2010-01-01

    Objectives To determine whether baseline demographic, clinical, articular and laboratory variables predict methotrexate (MTX) poor response in polyarticular-course juvenile idiopathic arthritis. Methods Patients newly treated for 6 months with MTX enrolled in the Paediatric Rheumatology

  17. Cytokine profiles in peripheral blood and whole blood cell cultures associated with aggressive periodontitis, juvenile idiopathic arthritis, and rheumatoid arthritis

    DEFF Research Database (Denmark)

    Poulsen, Anne Havemose; Sørensen, Lars Korsbaek; Stoltze, Kaj

    2005-01-01

    Cytokines play a key role in the pathogenesis of inflammatory diseases. An obvious question is whether patients with aggressive periodontitis, juvenile idiopathic arthritis, or rheumatoid arthritis share blood cytokine profiles distinguishing them from individuals free of disease....

  18. Physical Exercise and Physical Activity for Children and Adolescents With Juvenile Idiopathic Arthritis: A Literature Review.

    Science.gov (United States)

    Catania, Hilenia; Fortini, Viola; Cimaz, Rolando

    2017-07-01

    This is a review of studies that investigate the efficacy of exercise therapy and physical activity for children with juvenile idiopathic arthritis since the 2008 Cochrane Review. Studies were identified that investigated the use of physical activity and exercise therapy in the treatment of children and adolescents with juvenile idiopathic arthritis, excluding the Cochrane Review. Two reviewers individually analyzed the results of the search to determine the eligibility of studies. The randomized controlled trial study designs were evaluated using PEDro scales. Recent literature supports the importance of the combination of strengthening, stretching, proprioceptive, and balance exercises and activities in water. Many of the reviewed studies proposed an intensive program of physical activity and exercise therapy, conducted 3 times a week for 12 weeks.

  19. Multicentric osteolysis with nodulosis and arthropathy (MONA) with cardiac malformation, mimicking polyarticular juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Castberg, Filip Christian; Kjaergaard, Susanne; Mosig, Rebecca A

    2013-01-01

    The 'vanishing bone' syndrome multicentric osteolysis with nodulosis and arthropathy (MONA) is a rare chronic skeleton disorder caused by matrix metalloproteinase 2 (MMP2) deficiency, mimicking erosive polyarticular juvenile idiopathic arthritis. MONA is characterised by facial dysmorphism...... six also had congenital cardiac malformations. Despite treatment attempts of our patient with methotrexate, eternacept and prednisolone, serial X-ray studies documented continuous severe bone degeneration. Conclusion: The case documents the natural history of MONA and establishes a link between MMP2...

  20. POSSIBLE PARENTERAL METHOTREXATE APPLICATION IN THE TREATMENT OF JUVENILE IDIOPATHIC ARTHRITIS

    Directory of Open Access Journals (Sweden)

    Yu. М. Spivakovsky

    2014-01-01

    Full Text Available The article describes a case of using methotrexate for subcutaneous injection in a rated dose of 12.5 mg / m2 of body surface per week when treating a child with juvenile idiopathic arthritis. When switching to the parenteral route of administration, methotrexate ensured the development of joint syndrome remission, normalization of laboratory indices of disease activity, and improvement of joint functions. After 6 months the inactive disease stage was detected, and after 12 months — remission.

  1. The correlation between the Childhood Health Assessment Questionnaire and disease activity in juvenile idiopathic arthritis.

    Science.gov (United States)

    Sontichai, Watchareewan; Vilaiyuk, Soamarat

    2018-03-24

    The Childhood Health Assessment Questionnaire (CHAQ) has been adapted from the Stanford Health Assessment Questionnaire for assessing functional ability in children. The present study aimed to determine the correlation between CHAQ and disease activity in juvenile idiopathic arthritis (JIA) during active and inactive disease. JIA patients in the Pediatric Department, Ramathibodi Hospital, between January 2011 and December 2013, were included in the study. The CHAQ disability index (DI) and disease activity variables, including active and limited joint count, erythrocyte sedimentation rate, patient's global assessment (PtGA), physician's global assessment (PGA) and 27-joint Juvenile Arthritis Disease Activity Score (JADAS27), were collected from medical records for each patient over six visits. At each visit, each patient was classified as having either active or inactive disease. The correlations between CHAQ-DI and disease activity variables were analysed using Spearman's correlation. The classification of 139 JIA patients consisted of enthesitis-related arthritis (30.9%), systemic JIA (28.1%), oligoarthritis (16.5%), rheumatoid factor (RF)-negative polyarthritis (15.1%), RF-positive polyarthritis (6.5%) and undifferentiated arthritis (2.9%). Out of 812 patient visits, 606 were in active disease and 206 were in inactive disease. RF-negative polyarthritis had the highest CHAQ-DI (0.39 ± 0.66), while oligoarthritis had the lowest (0.20 ± 0.32). There was a good correlation between CHAQ-DI and JADAS27, PGA and PtGA in all JIA subtypes (p < 0.05) during active disease, but a poor correlation between CHAQ-DI and disease activity variables during inactive disease. CHAQ-DI had a good correlation with disease activity during active disease but a poor correlation during inactive disease. Therefore, CHAQ is only useful for assessing functional ability during active disease. Copyright © 2018 John Wiley & Sons, Ltd.

  2. Comics as an educational tool for children with juvenile idiopathic arthritis.

    Science.gov (United States)

    Mendelson, Amir; Rabinowicz, Noa; Reis, Yonit; Amarilyo, Gil; Harel, Liora; Hashkes, Philip J; Uziel, Yosef

    2017-09-02

    This study examined whether the comic book Neta and the Medikidz Explain JIA would improve disease-related knowledge and treatment adherence among patients with juvenile idiopathic arthritis (JIA). In this prospective cohort study, JIA patients answered 20 multiple-choice knowledge questions about their disease, before and after reading the comic book. Demographic, clinical, health-related quality of life and adherence data were recorded and correlated to the responses. We studied 61 patients with a mean age of 14 ± 3.3 (range 8-18) years, 67% female, 83% Jewish and 17% non-Jewish. Thirty-nine percent had oligoarthritis, 13% systemic, 32% polyarthritis 11% psoriatic and 5% enthesitis-related type JIA. The disease was active in 46%, 40% were treated with biologics/disease modifying anti-rheumatic drugs, and 34% were in remission on medication. Among the 53 patients who completed before and after quizzes, average score increased from 63 to 80% (P comic book. Twenty-seven patients who also completed the quiz 1 year after the first reading retained their knowledge (79%). We did not find a statistically significant correlation between knowledge and age, sex, disease subtype, or Child Health Questionnaire quality of life scores. Adherence to medication use, physical therapy and rheumatology clinic visits were high at baseline; thus, these did not change after reading the comic. The comic booklet Neta and the Medikidz Explain JIA is a good educational tool for increasing disease-related knowledge in children with JIA.

  3. SIAE Rare Variants in Juvenile Idiopathic Arthritis and Primary Antibody Deficiencies

    Directory of Open Access Journals (Sweden)

    Eirini Sevdali

    2017-01-01

    Full Text Available Sialic acid acetylesterase (SIAE deficiency was suggested to lower the levels of ligands for sialic acid-binding immunoglobulin-like receptors, decreasing the threshold for B-cell activation. In humans, studies of rare heterozygous loss-of-function mutations in SIAE gene in common autoimmune diseases, including juvenile idiopathic arthritis (JIA, yielded inconsistent results. Considering the distinct pathogenesis of the two main subtypes of JIA, autoinflammatory systemic (sJIA and autoimmune oligo/polyarticular (aJIA, and a predisposition to autoimmunity displayed by patients and families with primary antibody deficiencies (PADs, the aim of our study was to analyze whether SIAE rare variants are associated with both the phenotype of JIA and the autoimmunity risk in families with PADs. A cohort of 69 patients with JIA, 117 healthy children, 54 patients, and family members with PADs were enrolled in the study. Three novel SIAE variants (p.Q343P, p.Y495X, and c.1320+33T>C were found only in patients with aJIA but interestingly also in their healthy relatives without autoimmunity, while none of PAD patients or their relatives carried SIAE defects. Our results show that SIAE rare variants are not causative of autoimmunity as single defects.

  4. Time to treatment as an important factor for the response to methotrexate in juvenile idiopathic arthritis.

    Science.gov (United States)

    Albers, H M; Wessels, J A M; van der Straaten, R J H M; Brinkman, D M C; Suijlekom-Smit, L W A; Kamphuis, S S M; Girschick, H J; Wouters, C; Schilham, M W; le Cessie, S; Huizinga, T W J; Ten Cate, R; Guchelaar, H J

    2009-01-15

    Methotrexate (MTX) is the most commonly used disease-modifying antirheumatic drug in juvenile idiopathic arthritis (JIA). Currently, individual response to MTX cannot be reliably predicted. Identification of clinical and genetic factors that influence the response to MTX could be helpful in realizing the optimal treatment for individual patients. A cohort of 128 JIA patients treated with MTX were studied retrospectively. Eleven clinical parameters and genotypes of 6 single nucleotide polymorphisms in 5 genes related to the mechanism of action of MTX were compared between MTX responders and nonresponders using a multivariate regression analysis. The time from diagnosis to start of MTX treatment, physician's global assessment at baseline, and the starting dose were significantly associated with the response to MTX at 6 months after initiation. Patients with a shorter time from diagnosis to start of MTX and a higher disease activity according to the physician but with a lower MTX dose showed an increased response. The effect of the starting dose on MTX response seemed to be mainly due to the influence of the systemic JIA subtype. The time from diagnosis to start of MTX treatment and physician's global assessment at baseline were highly correlated. Therefore, the precise effect size of each independent variable could not be determined. In children with JIA, the time from diagnosis to start of MTX appears to be an important factor for MTX response. Our results suggest that an earlier start of MTX treatment will lead to an increased response.

  5. [Two pairs of brothers with juvenile idiopathic arthritis (JIA): case reports].

    Science.gov (United States)

    Robazzi, Teresa Cristina M V; Rios, Gabriela; Castro, Catarina

    2015-01-01

    This is a case report of juvenile idiopathic arthritis (JIA) in two pairs of brothers followed in the department of pediatric rheumatology, Universidade Federal da Bahia. Genetic involvement in JIA pathogenesis is clear and the risk of recurrence among siblings supports this contribution. An important landmark of this discovery involves the acknowledgment of major histocompatibility complex (MHC) polymorphism contribution to JIA development susceptibility. Despite many advances, the numerous available studies cannot explain several implicit mechanisms in JIA pathogenesis yet. Copyright © 2012 Elsevier Editora Ltda. All rights reserved.

  6. X-linked agammaglobulinemia combined with juvenile idiopathic arthritis and invasive Klebsiella pneumoniae polyarticular septic arthritis.

    Science.gov (United States)

    Zhu, Zaihua; Kang, Yuli; Lin, Zhenlang; Huang, Yanjing; Lv, Huoyang; Li, Yasong

    2015-02-01

    X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disease caused by mutations in the Bruton's tyrosine kinase (BTK) gene. XLA can also present in combination with juvenile idiopathic arthritis (JIA), the major chronic rheumatologic disease in children. We report herein the first known case of a juvenile patient diagnosed with XLA combined with JIA that later developed into invasive Klebsiella pneumoniae polyarticular septic polyarthritis. An additional comprehensive review of XLA combined with JIA and invasive K. pneumoniae septic arthritis is also presented. XLA was identified by the detection of BTK mutations while the diagnosis of JIA was established by clinical and laboratory assessments. Septic arthritis caused by invasive K. pneumoniae was confirmed by culturing of the synovia and gene detection of the isolates. Invasive K. pneumoniae infections can not only result in liver abscesses but also septic arthritis, although this is rare. XLA combined with JIA may contribute to invasive K. pneumoniae infection.

  7. Measurement of blood calprotectin (MRP-8/MRP-14) levels in patients with juvenile idiopathic arthritis.

    Science.gov (United States)

    Bojko, Jaryna

    2017-01-01

    The aim of the investigation was to compare blood calprotectin (MRP8/14, S100A 8/9) levels in patients with systemic-onset, polyarticular, RF-negative and oligoarticular subtypes of juvenile idiopathic arthritis (JIA), and to explore links between blood calprotectin levels and clinical and laboratory markers of JIA activity. Measurement of calprotectin in blood serum was performed in 160 patients with JIA followed up at Lviv Regional Council Public Institution "Western-Ukrainian Specialised Children's Medical Centre". Seventeen patients with systemic-onset JIA (sJIA) and 49 patients with other JIA subtypes (RF-negative polyarthritis and oligoarthritis) in the active phase of the disease were included in this study. Determination of calprotectin levels in blood serum was performed using EK-MRP8/14 Buhlmann Calprotectin reagents (Buhlmann, Switzerland) by the ELISA method. The results of the investigations showed that blood calprotectin levels were higher in patients with systemic-onset subtype of the disease (median 13,800 ng/ml), and differed significantly from levels in healthy children (median 1,800 ng/ml, p = 0.00002), levels in patients with articular subtypes of JIA (median 2,700 ng/ml, p = 0.000008), and patients with RF-negative polyarthritis (median 3,800 ng/ml, p = 0.003226) and oligoarthritis (median 2,500 ng/ml, p = 0.000009). The highest blood calprotectin levels were found in patients with newly diagnosed sJIA, the median being 32,500 ng/ml (range: 13,800-177,000 ng/ml). Direct correlations were found between blood calprotectin and JADAS 27 activity score ( p = 0.000009), ESR ( p = 0.000079) and CRP ( p = 0.000058). Blood calprotectin level is one of the measures that can be used to confirm the diagnosis of sJIA and to monitor the disease activity and therapy effectiveness.

  8. [Comparison of the Disease Activity Score-28 and Juvenile Arthritis Disease Activity Score in the juvenile idiopathic arthritis].

    Science.gov (United States)

    Capela, Renata Campos; Corrente, José Eduardo; Magalhães, Claudia Saad

    2015-01-01

    the assessment of the activity of rheumatoid arthritis and juvenile idiopathic arthritis is made by means of different tools, respectively DAS-28 and JADAS. To compare DAS-28 and JADAS with scores of 71, 27 and 10 joint counts in juvenile idiopathic arthritis. A secondary analysis of a phase III placebo-controlled trial, testing safety and efficacy of abatacept was conducted in 8 patients with 178 assessment visits. Joint count scores for active and limited joints, physician's and parents' global assessment by 0-10cm Visual Analog Scale, and erythrocyte sedimentation rate normalized to 0-10 scale, in all visits. The comparison among the activity indices in different observations was made through Anova or adjusted gamma model. The paired observations between DAS-28 and JADAS 71, 27 and 10, respectively, were analyzed by linear regression. There were significant differences among individual measures, except for ESR, in the first four months of biological treatment, when five of the eight patients reached ACR-Pedi 30, with improvement. The indices of DAS-28, JADAS 71, 27 and 10 also showed significant difference during follow-up. Linear regression adjusted model between DAS-28 and JADAS resulted in mathematical formulas for conversion: [DAS-28=0.0709 (JADAS 71)+1.267] (R(2)=0.49); [DAS-28=0.084 (JADAS 27) +1.7404] (R(2)=0.47) and [DAS-28=0.1129 (JADAS-10) +1.5748] (R(2)=0.50). The conversion of scores of DAS-28 and JADAS 71, 27 and 10 for this mathematical model would allow equivalent application of both in adolescents with arthritis. Copyright © 2014 Elsevier Editora Ltda. All rights reserved.

  9. Changes of Platelet Indices in Juvenile Idiopathic Arthritis in Acute Phase and After Two Months Treatment.

    Science.gov (United States)

    Vakili, Marjan; Ziaee, Vahid; Moradinejad, Mohammad Hassan; Raeeskarami, Seyed Reza; Kompani, Farzad; Rahamooz, Tayebeh

    2016-06-01

    Various indices have been raised as predictors of activity and severity of juvenile idiopathic arthritis. This study was conducted to investigate the changes of platelet indices in acute phase and two months after treatment in these patients. In a cohort study, platelet count, mean platelet volume (MPV), platelet distribution width (PDW), plateletcrit (PCT) were evaluated in children referred to children's medical center, Tehran due to juvenile idiopathic arthritis from March 2013 to March 2014 during the acute phase and two months after standard treatment. The statistical data were analyzed by SPSS 19 software, and the significance level was set as P acute phase and reached 395418.2 ± 119601.6 two months after treatment (P = 0.01). The mean ± SD PCT in the acute phase of various subtypes of the disease was 0.32 ± 0.11, which reached 0.29 ± 0.10 after treatment (P = 0.09). However, the PDW range in different subtypes of the disease reached 13.4 ± 8.0 from 13.9 ± 2.9 and MPV reached 8.7 ± 0.9 from 8.8 ± 1.1 after treatment, but they were not significantly different from the results in the acute phase (P = 0.5). Platelet count is one of the most remarkable indices in JIA. Evaluation of PCT can also help determine the severity of the inflammatory process in the follow-up and treatment process.

  10. Anti-TNF therapy for juvenile idiopathic arthritis-related uveitis

    Directory of Open Access Journals (Sweden)

    Semeraro F

    2014-03-01

    Full Text Available Francesco Semeraro,1 Barbara Arcidiacono,2 Giuseppe Nascimbeni,1 Martina Angi,1 Barbara Parolini,2 Ciro Costagliola31Eye Clinic, Department of Neurological Sciences and Vision, University of Brescia, Brescia, Italy; 2Department of Ophthalmology, S. Anna Hospital, Brescia, Italy; 3Eye Clinic, Department of Health Sciences, University of Molise, Campobasso, ItalyAbstract: Juvenile idiopathic arthritis-related uveitis is the most common type of uveitis in childhood and one of the main causes of visual impairment in children. The introduction of biological treatment has widened the range of therapeutic options for children with uveitis refractory to standard nonbiologic immunosuppressants. Data from clinical trials suggest that both adalimumab and infliximab have demonstrated effectiveness and safety in open-label studies, although no large, randomized, controlled trials have been reported so far. The role of etanercept in treating juvenile idiopathic arthritis-related uveitis is not yet well defined. In our experience, anti-tumor necrosis factor therapy has been shown to be more effective than steroids and/or methotrexate in treating uveitis. Up to now, tumor necrosis factor blocking compounds have been reserved for the treatment of the most severe cases of refractory uveitis, and larger prospective clinical trials are required in order to better assess the safety of these new compounds.Keywords: adalimumab, etanercept, infliximab

  11. Exposure to animals and risk of oligoarticular juvenile idiopathic arthritis: a multicenter case-control study

    Directory of Open Access Journals (Sweden)

    Michels Hartmut

    2010-04-01

    Full Text Available Abstract Background An inverse association between early contact with microbial compounds and respiratory allergies is well established. The protective effect of infant contact with animals was also shown for inflammatory bowel disease (IBD and systemic lupus erythematosus (SLE. We aimed to test the association between animal contact in infancy and oligoarticular juvenile idiopathic arthritis (OA JIA. Methods Parents of children with OA JIA registered at the Hospital for Pediatric Rheumatology in Garmisch-Partenkirchen were asked to complete a questionnaire. Children who underwent strabismus surgery at six referral centers for ophthalmology served as controls. Children age 6 to 18 years born in Germany without malformations were included (238 cases; response 89% and 832 controls; response 86%. Data were analyzed using logistic regression models after adjusting for potential confounders. Results Neither place of living (urban vs. rural area, living on a farm, nor regular farm animal (adjusted odds ratio 0.79; 95% confidence interval 0.42-1.47 or pet contact (0.79; 0.55-1.14 during infancy were clearly related to case status. Allergic rhinitis was inversely related to OA JIA (0.57; 0.34-0.95. Neither place of living (urban vs. rural area, living on a farm, nor regular farm animal (adjusted odds ratio 0.79; 95% confidence interval 0.42-1.47 or pet contact (0.79; 0.55-1.14 during infancy were related to case status. Allergic rhinitis was inversely related to OA JIA (0.57; 0.34-0.95. Conclusions Contact with farm environments in infancy might not be associated with OA JIA. This finding is consistent with previous findings for diabetes mellitus type 1 but contradicts results for IBD and SLE.

  12. Distribution pattern of MRI abnormalities within the knee and wrist of juvenile idiopathic arthritis patients: signature of disease activity

    NARCIS (Netherlands)

    Nusman, Charlotte M.; Hemke, Robert; Schonenberg, Dieneke; Dolman, Koert M.; van Rossum, Marion A. J.; van den Berg, J. Merlijn; Kuijpers, Taco W.; Maas, Mario

    2014-01-01

    The aim of this study in clinically active juvenile idiopathic arthritis (JIA) was to assess the frequency and distribution pattern of synovitis as hallmark of disease and additional soft-tissue and bony abnormalities on MRI in the knee and wrist as two target joints. MRI datasets of 153 clinically

  13. Internet Program for Physical Activity and Exercise Capacity in Children With Juvenile Idiopathic Arthritis : A Multicenter Randomized Controlled Trial

    NARCIS (Netherlands)

    Armbrust, Wineke; Bos, G.J.F. Joyce; Wulffraat, Nico M; van Brussel, Marco; Cappon, Jeannette; Dijkstra, Pieter U; Geertzen, Jan H B; Legger, G Elizabeth; van Rossum, Marion A J; Sauer, Pieter J J; Lelieveld, Otto T H M

    Objective. To determine the effects of Rheumates@Work, an internet-based program supplemented with 4 group sessions, aimed at improving physical activity, exercise capacity, health-related quality of life (HRQoL), and participation in children with juvenile idiopathic arthritis. Methods. Patients

  14. Temporomandibular joint involvement in Juvenile Idiopathic Arthritis : reliability and validity of a screening protocol for the rheumatologist

    NARCIS (Netherlands)

    Steenks, Michel H.; Giancane, G; de Leeuw, Rob R. J.; Bronkhorst, Ewald M.; van Es, Robert J. J.; Koole, Ron; van Bruggen, H. Willemijn; Wulffraat, NM

    2015-01-01

    Background: In Juvenile Idiopathic Arthritis (JIA) the temporomandibular joint (TMJ) can be involved leading to pain, dysfunction and growth disturbances of the mandible and associated structures. There may be value to a three minute screening protocol allowing the rheumatologist to detect TMJ

  15. Temporomandibular joint involvement in Juvenile Idiopathic Arthritis: reliability and validity of a screening protocol for the rheumatologist.

    NARCIS (Netherlands)

    Steenks, M.H.; Giancane, G.; Leeuw, R.R. de; Bronkhorst, E.M.; Es, R.J. van; Koole, R.A.; Bruggen, H.W. van; Wulffraat, N.M.

    2015-01-01

    BACKGROUND: In Juvenile Idiopathic Arthritis (JIA) the temporomandibular joint (TMJ) can be involved leading to pain, dysfunction and growth disturbances of the mandible and associated structures. There may be value to a three minute screening protocol allowing the rheumatologist to detect TMJ

  16. EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups: a methodology report

    NARCIS (Netherlands)

    Bottai, Matteo; Tjärnlund, Anna; Santoni, Giola; Werth, Victoria P.; Pilkington, Clarissa; de Visser, Marianne; Alfredsson, Lars; Amato, Anthony A.; Barohn, Richard J.; Liang, Matthew H.; Singh, Jasvinder A.; Aggarwal, Rohit; Arnardottir, Snjolaug; Chinoy, Hector; Cooper, Robert G.; Danko, Katalin; Dimachkie, Mazen M.; Feldman, Brian M.; García-de la Torre, Ignacio; Gordon, Patrick; Hayashi, Taichi; Katz, James D.; Kohsaka, Hitoshi; Lachenbruch, Peter A.; Lang, Bianca A.; Li, Yuhui; Oddis, Chester V.; Olesinka, Marzena; Reed, Ann M.; Rutkowska-Sak, Lidia; Sanner, Helga; Selva-O'Callaghan, Albert; Wook Song, Yeong; Vencovsky, Jiri; Ytterberg, Steven R.; Miller, Frederick W.; Rider, Lisa G.; Lundberg, Ingrid E.; Amoruso, Maria; Andersson, Helena; Bayat, Nastaran; Bhansing, Kavish J.; Bucher, Sara; Champbell, Richard; Charles-Schoeman, Christina; Chaudhry, Vinay; Christopher-Stine, Lisa; Chung, Lorinda; Cronin, Mary; Curry, Theresa; Dahlbom, Kathe; Distler, Oliver; Efthimiou, Petros; van Engelen, Baziel G. M.; Faiq, Abdullah; Farhadi, Payam Noroozi; Fiorentino, David; Hengstman, Gerald; Hoogendijk, Jessica; Huber, Adam; Kataoka, Hiroshi; Katsumata, Yasuhiro; Kim, Susan; Kong-Rosario, Michelle; Kontzias, Apostolos; Krol, Petra; Kurita, Takashi; Li, Zhan-Guo; Lindvall, Björn; Linklater, Helen; Maillard, Sue; Mamyrova, Gulnara; Mantegazza, Renato; Marder, Galina S.; Nagahashi Marie, Suely Kazue; Mathiesen, Pernille; Mavragani, Clio P.; McHugh, Neil J.; Michaels, Mimi; Mohammed, Reem; Morgan, Gabrielle; Moser, David W.; Moutsopoulos, Haralampos M.

    2017-01-01

    To describe the methodology used to develop new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIMs) and their major subgroups. An international, multidisciplinary group of myositis experts produced a set of 93 potentially relevant variables to be tested for

  17. Dynamic contrast-enhanced magnetic resonance imaging of the wrist in children with juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Nusman, Charlotte M. [Emma Children' s Hospital, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Academic Medical Center, Amsterdam (Netherlands); Academic Medical Center, Department of Radiology, Amsterdam (Netherlands); Lavini, Cristina; Hemke, Robert; Caan, Matthan W.A.; Maas, Mario [Academic Medical Center, Department of Radiology, Amsterdam (Netherlands); Schonenberg-Meinema, Dieneke; Berg, J.M. van den; Kuijpers, Taco W. [Emma Children' s Hospital, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Academic Medical Center, Amsterdam (Netherlands); Dolman, Koert M. [Sint Lucas Andreas Hospital, Department of Pediatrics, Amsterdam (Netherlands); Reade Institute location Jan van Breemen, Department of Pediatric Rheumatology, Amsterdam (Netherlands); Rossum, Marion A.J. van [Reade Institute location Jan van Breemen, Department of Pediatric Rheumatology, Amsterdam (Netherlands); Emma Children' s Hospital, Department of Pediatrics, Academic Medical Center, Amsterdam (Netherlands)

    2017-02-15

    Dynamic contrast-enhanced MRI provides information on the heterogeneity of the synovium, the primary target of disease in children with juvenile idiopathic arthritis (JIA). To evaluate the feasibility of dynamic contrast-enhanced MRI in the wrist of children with JIA using conventional descriptive measures and time-intensity-curve shape analysis. To explore the association between enhancement characteristics and clinical disease status. Thirty-two children with JIA and wrist involvement underwent dynamic contrast-enhanced MRI with movement-registration and were classified using validated criteria as clinically active (n = 27) or inactive (n = 5). Outcome measures included descriptive parameters and the classification into time-intensity-curve shapes, which represent the patterns of signal intensity change over time. Differences in dynamic contrast-enhanced MRI outcome measures between clinically active and clinically inactive disease were analyzed and correlation with the Juvenile Arthritis Disease Activity Score was determined. Comprehensive evaluation of disease status was technically feasible and the quality of the dynamic dataset was improved by movement registration. The conventional descriptive measure maximum enhancement differed significantly between clinically active and inactive disease (P = 0.019), whereas time-intensity-curve shape analysis showed no differences. Juvenile Arthritis Disease Activity Score correlated moderately with enhancing volume (P = 0.484). Dynamic contrast-enhanced MRI is a promising biomarker for evaluating disease status in children with JIA and wrist involvement. Conventional descriptive dynamic contrast-enhanced MRI measures are better associated with clinically active disease than time-intensity-curve shape analysis. (orig.)

  18. Juvenil idiopatisk arthritis

    DEFF Research Database (Denmark)

    Herlin, Troels

    2002-01-01

    The new classification of juvenile idiopathic arthritis (JIA) is described in this review. Clinical characteristics divide JIA in to subtypes: systemic, oligoarticular (persistent and extended type), RF-positive and--negative polyarticular, enthesitis-related arthritis and psoriatic arthritis...

  19. Participation in Leisure Activities by Children and Adolescents with Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Cavallo, Sabrina; Majnemer, Annette; Duffy, Ciarán M; Feldman, Debbie Ehrmann

    2015-09-01

    To describe leisure activities of children and adolescents with juvenile idiopathic arthritis (JIA) in terms of diversity, intensity, and enjoyment, and to identify potential determinants. One hundred seven children and adolescents aged 8-17 years diagnosed with JIA and their families participated in this cross-sectional study. Participants answered the Children's Assessment of Participation and Enjoyment, which measures involvement in leisure (recreation, active physical, social, skill-based, self-improvement). Disease characteristics and sociodemographic factors were abstracted from the child's medical file. In terms of intensity, individuals with JIA were more often engaged in informal rather than formal leisure activities [t(106) = 45.5, p leisure activities in children and adolescents with arthritis may allow healthcare professionals to assess children's health needs with more precision and promote a healthier lifestyle.

  20. Long-term follow-up of 246 adults with juvenile idiopathic arthritis: education and employment.

    Science.gov (United States)

    Packham, J C; Hall, M A

    2002-12-01

    To examine the levels of education and employment in adults with juvenile idiopathic arthritis (JIA) compared with their siblings and national figures. Two hundred and forty-six adults identified with long-standing JIA had an average disease duration of 28.3 yr. Educational achievements and employment status were recorded and comparative data obtained from siblings and the National Office for Statistics, UK. The effects, incidence and nature of discrimination in the workplace were also explored. Across all levels of education the study group achieved significantly better results than both the national average and their siblings. In contrast, the rate of unemployment in the patient group was more than twice that in the national population. The percentage of patients who had encountered discrimination in the workplace was 25.1.

  1. The Role of Gender in Juvenile Idiopathic Arthritis-Associated Uveitis

    Directory of Open Access Journals (Sweden)

    Ahmadreza Moradi

    2014-01-01

    Full Text Available Uveitis is a common complication of juvenile idiopathic arthritis (JIA affecting up to 30% of patients with JIA. Although the typical bilateral chronic anterior uveitis associated with the persistent and extended oligoarticular and polyarticular, rheumatoid factor negative variants of JIA occurs predominantly in girls, boys may be more commonly affected in the HLA-B27 positive, enthesitis variant of JIA. While female gender has been associated with the development of the chronic anterior uveitis in children with JIA, the clinical course of JIA-associated uveitis may be worse in boys than in girls. The purpose of this paper is to review the available published literature to determine the role of gender in the clinical presentation and outcomes of patients with JIA-associated uveitis.

  2. [Presence of riziform bodies in a patient with juvenile idiopathic arthritis: case report and literature review.

    Science.gov (United States)

    Campos, Leonardo Rodrigues; Sztajnbok, Fernanda Cardoso das Neves; Galvão, Stélio; Lessa, Marise de Araújo; Aymoré, Ierecê Lins; Sztajnbok, Flavio

    2014-10-23

    Riziform bodies are structures formed by fibrin and cells that can be found in the synovial fluid or attached to the synovium, and have this denomination due to its rice grain-like appearance. They have already been described in several diseases such as tuberculous arthritis, rheumatoid arthritis, and rarely in juvenile idiopathic arthritis (JIA). This is the case of a boy with a 4-month course of chronic monoarthritis of the left knee, with family history of sarcoidosis in which diagnostic investigation showed the presence of these riziform bodies in the synovial biopsy. Diagnostic investigation ruled out sarcoidosis, tuberculosis and malignancies, establishing the diagnosis of JIA. Our objective was to describe what we believe is the 9th case reported on the presence of riziform bodies in JIA, which are probably underdiagnosed, and should be considered mainly in cases of severe arthritis of difficult medical treatment. Copyright © 2014 Elsevier Editora Ltda. All rights reserved.

  3. [Magnetic resonance imaging in juvenile idiopathic arthritis: peculiarities of imaging children].

    Science.gov (United States)

    Navallas, M; Rebollo Polo, M; Riaza, L; Muchart López, J; Maristany, T

    2013-09-01

    The term juvenile idiopathic arthritis (JIA) encompasses a heterogeneous group of arthritides with no known cause that begin before the age of 16 years and persist for at least 6 weeks. In recent decades, imaging techniques have acquired a fundamental role in the diagnosis and follow-up of JIA, owing to the unification of the different criteria for classification, which has strengthened the research in this field, and to the development of disease-modifying antirheumatic drugs. In this article, we briefly explain what JIA is. Moreover, we describe the role and limitations of plain-film radiography, ultrasonography, and magnetic resonance imaging (MRI). Finally, we review the MRI protocol and findings, and we comment on the differential diagnosis. Copyright © 2012 SERAM. Published by Elsevier Espana. All rights reserved.

  4. Accelerometry-based monitoring of daily physical activity in children with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Nørgaard, M; Twilt, M; Andersen, L B

    2015-01-01

    Objectives: Juvenile idiopathic arthritis (JIA) may cause functional impairment, reduced participation in physical activity (PA) and, over time, physical deconditioning. The aim of this study was to objectively monitor daily free-living PA in 10-16-year-old children with JIA using accelerometry...... with regard to disease activity and physical variables and to compare the data with those from healthy age- and gender-matched controls.Method: Patients underwent an evaluation of disease activity, functional ability, physical capacity, and pain. Accelerometer monitoring was assessed using the GT1M Acti......Graph. Normative data from two major studies on PA in Danish schoolchildren were used for comparison.Results: Data of accelerometry were available for 61 JIA patients and 2055 healthy controls. Of the JIA patients, 57% showed below-average values of maximal physical capacity (fitness level). JIA patients showed...

  5. Ankle arthritis predicts polyarticular disease course and unfavourable outcome in children with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Esbjörnsson, Anna-Clara; Aalto, Kristiina; Broström, Eva W

    2015-01-01

    OBJECTIVES: To evaluate the occurrence, clinical characteristics and prognostic factors associated with ankle arthritis in children with juvenile idiopathic arthritis (JIA). METHODS: 440 children with JIA were followed for eight years in a prospective Nordic population-based cohort study. Data...... on remission was available for 427 of these children. Occurrence of clinically assessed ankle arthritis was analysed in relation to JIA category, clinical characteristics and remission data eight years after disease onset. RESULTS: In 440 children with JIA, 251 (57%) experienced ankle arthritis during...... the first eight years of disease. Ankle arthritis was least common in the persistent oligoarticular category (25%) and most common in children with extended oligoarticular (83%) and polyarticular RF-negative (85%) JIA. Children who developed ankle arthritis during the first year of disease were younger...

  6. Clinical Observation of Employment of Umbilical Cord Derived Mesenchymal Stem Cell for Juvenile Idiopathic Arthritis Therapy

    Directory of Open Access Journals (Sweden)

    Liming Wang

    2016-01-01

    Full Text Available Juvenile idiopathic arthritis (JIA, known as Juvenile rheumatoid arthritis, is the most common type of arthritis in children aged under 17. It may cause sequelae due to lack of effective treatment. The goal of this study is to explore the therapeutic effect of umbilical cord mesenchymal stem cells (UC-MSCs for JIA. Ten JIA patients were treated with UC-MSCs and received second infusion three months later. Some key values such as 28-joint disease activity score (DAS28, TNF-α, IL-6, and regulatory T cells (Tregs were evaluated. Data were collected at 3 months and 6 months after first treatment. DAS28 score of 10 patients was between 2.6 and 3.2 at three months after infusion. WBC, ESR, and CRP were significantly decreased while Tregs were remarkably increased and IL-6 and TNF-α were declined. Similar changes of above values were found after 6 months. At the same time, the amount of NSAIDS and steroid usage in patients was reduced. However, no significant changes were found comparing the data from 3 and 6 months. These results suggest that UC-MSCs can reduce inflammatory cytokines, improve immune network effects, adjust immune tolerance, and effectively alleviate the symptoms and they might provide a safe and novel approach for JIA treatment.

  7. [The temporomandibular joint in juvenile idiopathic arthritis: what radiologists need to look for on magnetic resonance imaging].

    Science.gov (United States)

    De La Hoz Polo, M; Navallas, M

    2014-01-01

    The term "juvenile idiopathic arthritis" (JIA) encompasses a group of arthritis of unknown cause with onset before the age of 16 years that last for at least 6 weeks. The prevalence of temporomandibular joint involvement in published series ranges from 17% to 87%. Temporomandibular joint involvement is difficult to detect clinically, so imaging plays a key role in diagnosis and monitoring treatment. MRI is the technique of choice for the study of arthritis of the temporomandibular joint because it is the most sensitive technique for detecting acute synovitis and bone edema. Power Doppler ultrasonography can also detect active synovitis by showing the hypervascularization of the inflamed synovial membrane, but it cannot identify bone edema. This article describes the MRI technique for evaluating the temporomandibular joint in patients with juvenile idiopathic arthritis, defines the parameters to look for, and illustrates the main findings. Copyright © 2013 SERAM. Published by Elsevier Espana. All rights reserved.

  8. Peripheral blood mononuclear cell responses to heat shock proteins and their derived synthetic peptides in juvenile idiopathic arthritis patients

    Czech Academy of Sciences Publication Activity Database

    Sedláčková, L.; Velek, Jiří; Vavřincová, P.; Hromadníková, I.

    2006-01-01

    Roč. 55, č. 4 (2006), s. 153-159 ISSN 1023-3830 Grant - others:Transeurope(XE) QLK3-2002-01936; Transnet(XE) MRTN-CT-2004-512253 Institutional research plan: CEZ:AV0Z40550506 Keywords : heat shock proteins * proliferative response * juvenile idiopathic arthritis * Hsp-derived synthetic peptides Subject RIV: CC - Organic Chemistry Impact factor: 1.485, year: 2006

  9. What are the immunological consequences of long-term use of biological therapies for juvenile idiopathic arthritis?

    OpenAIRE

    Swart, Joost F; de Roock, Sytze; Wulffraat, Nico M

    2013-01-01

    This review summarizes the immunological consequences of biological therapies used in juvenile idiopathic arthritis (JIA). For every frequently used biological agent the characteristics are clearly specified (molecular target, isotype, registered indication for JIA, route of administration, half-life, contraindication, very common side effects, expected time of response and average cost in the first year). The emphasis of this review is on the immunological side effects that have been encount...

  10. The many shades of enhancement: timing of post-gadolinium images strongly influences the scoring of juvenile idiopathic arthritis wrist involvement on MRI

    Energy Technology Data Exchange (ETDEWEB)

    Rieter, Jasper F.M.M.; Nusman, Charlotte M.; Hemke, Robert; Maas, Mario [University of Amsterdam, Department of Radiology, Academic Medical Center, Amsterdam (Netherlands); Tanturri de Horatio, Laura [Ospedale Pediatrico Bambino Gesu, Department of Radiology, Rome (Italy); Ording Mueller, Lil-Sofie [Oslo University Hospital, Department of Radiology, Oslo (Norway); Avenarius, Derk F.M. [Faculty of Health Sciences at the University of Tromsoe, Tromsoe (Norway); Rossum, Marion A.J. van [University of Amsterdam, Department of Radiology, Academic Medical Center, Amsterdam (Netherlands); Emma Children' s Hospital, Amsterdam (Netherlands); Malattia, Clara [Ospedale Pediatrico Gaslini, Department of Paediatrics, Genoa (Italy); Rosendahl, Karen [Haukeland University Hospital, Radiology Department, Section of Pediatric Radiology, Bergen (Norway); University of Bergen, Department of Clinical Medicine, K1, Bergen (Norway)

    2016-10-15

    Potential long-term side effects of treatment for juvenile idiopathic arthritis are concerning. This has necessitated accurate tools, such as MRI, to monitor treatment response and allow for personalized therapy. To examine the extent to which timing of post-contrast MR images influences the scoring of inflammatory change in the wrist in children with juvenile idiopathic arthritis. We studied two sets of post-contrast 3-D gradient echo MRI series of the wrist in 34 children with juvenile idiopathic arthritis. These images were obtained immediately after administration of intravenous contrast material and again after approximately 10 min. The dataset was drawn from a prospective multicenter project conducted 2006-2010. We assessed five wrist locations for synovial enhancement, effusion and overall inflammation. Examinations were scored by one radiologist in two sessions - the first was based on the early post-contrast images, and the later session, for which the previous findings were masked, was based on the later post-contrast images. Fifty-two of the 170 locations (30.6%) received a higher synovial enhancement score based on the late post-contrast images as compared to the early images. Sixty of the 170 (35%) locations received a higher total inflammation score. The mean scores of synovial enhancement and total inflammation were significantly higher when based on the late post-contrast images as compared to the early post-contrast images. An MRI-based scoring system for the presence and degree of synovitis should be based on a standardized MR-protocol with a fixed interval between intravenous contrast injection and post-contrast images. (orig.)

  11. High rate of serious infection in juvenile idiopathic arthritis patients under biologic therapy in a real-life setting.

    Science.gov (United States)

    Brunelli, Juliana Barbosa; Schmidt, Ana Renata; Sallum, Adriana Maluf Elias; Goldenstein-Schainberg, Claudia; Bonfá, Eloisa; Silva, Clovis A; Aikawa, Nádia Emi

    2018-03-01

    To assess the rate of serious and/or opportunistic infections in juvenile idiopathic arthritis (JIA) patients from a single tertiary center under biologic therapy and to identify possible risk factors associated to these complications. A total of 107 JIA patients followed at the biologic therapy center of our tertiary university hospital using a standardized electronic database protocol including demographic data, clinical and laboratorial findings and treatment at baseline and at the moment of infection. Opportunistic infections included tuberculosis, herpes zoster and systemic mycosis. A total of 398 patient-yrs(py) were included. The median time of biologic exposure was 3.0 years (0.15-11.5). We observed 35 serious/opportunistic infectious events in 27 (25%) patients: 31(88.6%) were serious infections and four (11.4%) opportunistic infections. Serious/opportunistic infections rates were 10.6/100py for ETN, 10.9/100py for ADA, 2.6/100py for ABA and 14.8/100py for TCZ. Comparison of 27 patients with and 80 without infection showed a higher frequency of systemic-onset JIA, lower age at biologic therapy initiation and a history of previous serious infection (p biologic therapy in a real-life setting. Systemic-onset JIA, lower age at biologic therapy start and history of previous serious infections were important risk factors for these complications. Also, higher rates of severe infections comparing to the former studies was possibly due to elevated MTX doses in our patients.

  12. Prediction of methotrexate intolerance in juvenile idiopathic arthritis: a prospective, observational cohort study.

    Science.gov (United States)

    van Dijkhuizen, Evert Hendrik Pieter; Bulatović Ćalasan, Maja; Pluijm, Saskia M F; de Rotte, Maurits C F J; Vastert, Sebastiaan J; Kamphuis, Sylvia; de Jonge, Robert; Wulffraat, Nico M

    2015-01-01

    Methotrexate (MTX) is an effective and safe drug in the treatment of juvenile idiopathic arthritis (JIA). Despite its safety, MTX-related gastrointestinal adverse effects before and after MTX administration, termed MTX intolerance, occur frequently, leading to non-compliance and potentially premature MTX termination. The aim of this study was to construct a risk model to predict MTX intolerance. In a prospective JIA cohort, clinical variables and single nucleotide polymorphisms were determined at MTX start. The Methotrexate Intolerance Severity Score was employed to measure MTX intolerance in the first year of treatment. MTX intolerance was most prevalent at 6 or 12 months after MTX start, which was defined as the outcome for the prediction model. The model was developed in 152 patients using multivariable logistic regression analysis and subsequently internally validated using bootstrapping. The prediction model included the following predictors: JIA category, antinuclear antibody, parent/patient assessment of pain, Juvenile Arthritis Disease Activity Score-27, thrombocytes, alanine aminotransferase and creatinine. The model classified 77.5% of patients correctly, and 66.7% of patients after internal validation by bootstrapping. The lowest predicted risk of MTX intolerance was 18.9% and the highest predicted risk was 85.9%. The prediction model was transformed into a risk score (range 0-17). At a cut-off of ≥6, sensitivity was 82.0%, specificity 56.1%, positive predictive value was 58.7% and negative predictive value 80.4%. This clinical prediction model showed moderate predictive power to detect MTX intolerance. To develop into a clinically usable tool, it should be validated in an independent cohort and updated with new predictors. Such an easy-to-use tool could then assist clinicians in identifying patients at risk to develop MTX intolerance, and in turn to monitor them closely and intervene timely in order to prevent the development of MTX intolerance

  13. US findings of metacarpophalangeal joints in children with idiopathic juvenile arthritis

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    Karmazyn, Boaz [Riley Hospital for Children, Radiology, Indianapolis, IN (United States); Bowyer, Suzanne L.; Murphy Schmidt, Kara; Ballinger, Susan H.; Beam, Thuy T. [Indiana University, Pediatric Rheumatology, Indianapolis, IN (United States); Buckwalter, Kenneth [University Hospital, Radiology, Indianapolis, IN (United States); Ying, Jun [University of Cincinnati, Biostatistics, Institute for the Study of Health, Cincinnati, OH (United States)

    2007-05-15

    Juvenile idiopathic arthritis (JIA) is the most common cause of chronic arthritis in children, with frequent involvement of the metacarpophalangeal joints (MCPJ). To compare US findings with those of radiography and clinical examination. All MCPJs in 20 children with JIA (17 females, median age 9.7 years, range 3.6 to 16.8 years) were evaluated clinically and imaged with gray-scale and color Doppler US, and 90 MCPJs were also imaged radiographically. Each MCPJ was graded on physical examination from 0 (normal) to 4 (severe) by the patient's rheumatologist. US demonstrated abnormalities in 64 of 200 MCPJs (32.0%), including pannus vascularity and/or tenosynovitis in 55 joints (27.5%) (pannus vascularity in 43, tenosynovitis in 40) and bone destruction in 25 joints (12.5%). Overall, US abnormalities and physical examination scores were significantly associated (P < 0.001). However, interobserver agreement between US and clinical evaluation was poor (kappa 0.1) and between US and radiography was only fair (kappa 0.4). US of the MCPJ in children with JIA can demonstrate cartilage thinning, bone erosions, and pannus vascularity. Abnormal US findings are significantly correlated with severity of disease as evaluated clinically. (orig.)

  14. Psychological profile in children and adolescents with severe course Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Russo, Emanuela; Trevisi, E; Zulian, F; Battaglia, M A; Viel, D; Facchin, D; Chiusso, A; Martinuzzi, A

    2012-01-01

    Juvenile Idiopathic Arthritis (JIA) is the most common chronic pediatric rheumatic disease. It is recognized that only reliance on clinical signs of disease outcome is inadequate for understanding the impact of illness and its treatment on child's life and functioning. There is a need for a multidisciplinary and holistic approach to children with arthritis which considers both physical and emotional functioning. This study investigated the psychosocial functioning of children and adolescent with JIA and the disease-related changes in their family. The sample consisted of 33 hospitalized patients, aged 6-16 years. Both parents and the children were given a number of questionnaire to fill out. Clinical information was extracted from the interviews. Self-reported psychological functioning (depression, anxiety, and behavior) was not different from the normal population; however significant psychological suffering was detected by the clinical interview. Children and adolescents with JIA do not show overt psychopathology by structured assessment; nevertheless a more clinically oriented holistic approach confirms JIA as a disrupting event causing relevant changes in the quality of life of the affected families.

  15. Contrast-enhanced MRI features in the early diagnosis of Juvenile Idiopathic Arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Hemke, Robert; Maas, Mario [University of Amsterdam, Department of Radiology Academic Medical Center, Amsterdam (Netherlands); Kuijpers, Taco W.; Schonenberg-Meinema, Dieneke [University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Nusman, Charlotte M. [University of Amsterdam, Department of Radiology Academic Medical Center, Amsterdam (Netherlands); University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Rossum, Marion A.J. van; Berg, J.M. van den [University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Department of Pediatric Rheumatology, Reade, Amsterdam (Netherlands); Dolman, Koert M. [Department of Pediatric Rheumatology, Reade, Amsterdam (Netherlands); St. Lucas Andreas Hospital, Department of Pediatrics, Amsterdam (Netherlands)

    2015-11-15

    To determine whether clinical, laboratory or Magnetic Resonance Imaging (MRI) measures differentiate Juvenile Idiopathic Arthritis (JIA) from other forms of active childhood arthritis. We prospectively collected data of 80 treatment-naive patients clinically suspected of JIA with active non-infectious arthritis of (at least) one knee for <12 months duration. Upon presentation patients underwent clinical and laboratory assessments and contrast-enhanced MRI. MRI was not used as a diagnostic criterion. Forty-four (55 %) patients were clinically diagnosed with JIA, whereas in 36 (45 %) patients the diagnosis of JIA was discarded on clinical or laboratory findings. MRI-based synovitis was present in 27 (61.4 %) JIA patients and in 7 (19.4 %) non-JIA patients (P < 0.001). Five factors (male gender, physician's global assessment of overall disease activity, joints with limited range of motion, HLA-B27, MRI-based synovitis) were associated with the onset of JIA. In multivariate analysis MRI-based synovitis proved to be independently associated with JIA (OR 6.58, 95 % CI 2.36-18.33). In patients with MRI-based synovitis, the RR of having JIA was 3.16 (95 % CI 1.6-6.4). The presence of MRI-based synovitis is associated with the clinical onset of JIA. Physical examination could be supported by MRI, particularly to contribute in the early differentiation of different forms of non-infectious childhood arthritis. (orig.)

  16. Juvenile idiopathic arthritis and athletic participation: are we adequately preparing for sports integration?

    Science.gov (United States)

    Taxter, Alysha; Foss, Kim Barber; Melson, Paula; Ford, Kevin R; Shaffer, Michael; Myer, Gregory D

    2012-09-01

    Children with juvenile idiopathic arthritis (JIA) now have well-controlled disease due to improved therapies and management strategies. Children with JIA are more active than in the past and often participate in dynamic, high-loading sports. Standard measures of disease control include examination findings, laboratory values, and patient-directed surveys. However, these standards do not address the subtle deficits in biomechanics and neuromuscular control, which could place affected joints at higher risk for injury. Currently, there are limited evidence-based guidelines to structure conditioning recommendations as to the fitness and mechanics needed to provide safe integration into sports in this population; therefore, tools that objectively measure function with high accuracy and precision may be warranted. Previous work using 3-dimensional motion analysis demonstrated usefulness in guiding physical therapy treatment to correct these deficits. The use of a multidisciplinary team, including physical therapy, rheumatology, and sports medicine, is crucial for preparing these children to return to play. We suggest that the child transition into a sport preparatory-conditioning program to address any underlying deficits. A pediatric exercise specialist who is sensitive to the needs of this population can work with a physical therapist to then appropriately integrate the child safely into sport. Encouraging an active lifestyle is vital to the management of JIA and does not worsen the symptoms associated with childhood arthritis.

  17. Infrapatellar bursitis in children with juvenile idiopathic arthritis: a case series.

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    Alqanatish, Jubran T; Petty, Ross E; Houghton, Kristin M; Guzman, Jaime; Tucker, Lori B; Cabral, David A; Cairns, Robyn A

    2011-02-01

    Children with juvenile idiopathic arthritis (JIA) may infrequently present with localized anterior knee pain or swelling, in addition to generalize knee pain induced by JIA. We report five cases of deep infrapatellar bursitis in children with JIA. The clinical features, radiological findings, management, and outcome of five children with JIA and deep infrapatellar bursitis are reviewed. Three boys and two girls with a mean age of 9.8 years (range 6-14 years) were reviewed. Four children had persistent oligoarticular JIA, and one child had extended oligoarticular JIA. The presentation of deep infrapatellar bursitis was variable. In only one patient was the bursal swelling painful. Knee magnetic resonance imaging (MRI) was performed in four patients and demonstrated coexistent knee joint synovitis in three. Treatment included targeted corticosteroid injections into the deep infrapatellar bursa in two cases with complete resolution. One case was treated with corticosteroid injection by an outside health care provider with poor clinical response. Two cases are being treated with non-steroidal anti-inflammatory drugs and methotrexate. Deep infrapatellar bursitis can occur as an isolated finding or concurrently with knee joint synovitis in patients with JIA. Awareness of this entity is important because direct injection of the bursa may be needed for treatment, as the bursa does not communicate with the knee joint. Furthermore, when bursitis is suspected in JIA, MRI can be helpful to confirm the diagnosis, detect concurrent knee joint synovitis, and exclude other pathologies.

  18. Sleep disturbances and neurobehavioral functioning in children with and without juvenile idiopathic arthritis.

    Science.gov (United States)

    Ward, Teresa M; Ringold, Sarah; Metz, Jonika; Archbold, Kristen; Lentz, Martha; Wallace, Carol A; Landis, Carol A

    2011-07-01

    To compare sleep disturbances and neurobehavioral function in children with juvenile idiopathic arthritis (JIA) to age- and sex-matched control children. Children (n = 116) ages 6-11 years with (n = 70) and without (n = 46) JIA and their parents participated. Parents completed questionnaires on sleep habits, sleep behavior, and school competence of their children; children completed computerized neurobehavioral performance tests. Compared to control children, children with JIA had a statistically significant (P sleep disturbance score and higher scores on 6 of 8 subscales (all P Sleep Habits Questionnaire (CSHQ). There were no group differences on neurobehavioral performance test scores. However, regardless of group, children with an overall CSHQ score above an established cutoff for clinically significant sleep disturbances had slower mean simple reaction time (t = -2.2, P sleep disturbance score predicted reaction time (P sleep disturbances, but performed as well as control children on a series of standardized computer tests of neurobehavioral performance. Children with more disturbed sleep had slower reaction times. Copyright © 2011 by the American College of Rheumatology.

  19. Increasing feasibility and patient comfort of MRI in children with juvenile idiopathic arthritis

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    Hemke, Robert [Academic Medical Center, Department of Radiology, Amsterdam (Netherlands); Emma Children' s Hospital AMC, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Amsterdam (Netherlands); Veenendaal, Mira van; Kuijpers, Taco W. [Emma Children' s Hospital AMC, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Amsterdam (Netherlands); Rossum, Marion A.J. van [Emma Children' s Hospital AMC, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Amsterdam (Netherlands); Jan van Breemen Institute, Department of Pediatric Rheumatology, Amsterdam (Netherlands); Maas, Mario [Academic Medical Center, Department of Radiology, Amsterdam (Netherlands)

    2012-04-15

    MRI is the most sensitive imaging modality in juvenile idiopathic arthritis (JIA), but has practical limitations. Optimizing the scanning protocol is, therefore, necessary to increase feasibility and patient comfort. To determine the feasibility of bilateral non-contrast-enhanced open-bore MRI of knees and to assess the presence of literature-based MRI features in unsedated children with JIA. Children were classified into two clinical subgroups: active arthritis (group 1; n = 29) and inactive disease (group 2; n = 18). MRI features were evaluated using a literature-based score, comprising synovial hypertrophy, cartilage lesions, bone erosions, bone marrow changes, infrapatellar fat pad heterogeneity, effusion, tendinopathy and popliteal lymphadenopathy. The MRI examination was successfully completed in all 47 children. No scan was excluded due to poor image quality. Synovial hypertrophy was more frequent in group 1 (36.2%), but was also seen in 19.4% of the knees in group 2. Infrapatellar fat pad heterogeneity was more prevalent in group 2 (86.1%; P = 0.008). Reproducibility of the score was good (Cohen kappa, 0.49-0.96). Bilateral non-contrast-enhanced open-bore knee MRI is feasible in the assessment of disease activity in unsedated children with JIA. Signs differing among children with active and inactive disease include infrapatellar fat pad heterogeneity and synovial hypertrophy. (orig.)

  20. Assessment of the Body Composition and Parameters of the Cardiovascular Risk in Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Ewa Jednacz

    2015-01-01

    Full Text Available The study was aimed to evaluate cardiovascular risk parameters, body mass index (BMI centiles for sex and age, and body fat percentage using the electric bioimpedance method in children with juvenile idiopathic arthritis (JIA. 30 children with JIA participated in the study. A control group included 20 children. Patients were well matched for the age and sex. The body mass and body fat percentage were determined using the segmental body composition analyser; the BMI centiles were determined. All patients had the following parameters determined: lipid profile, hsCRP, homocysteine, and IL-6. The intima media thickness (IMT was measured. Patients with JIA had significantly lower body weight, BMI, and the BMI centile compared to the control group. The IL-6 levels were significantly higher in patients with JIA compared to the control group. There were no differences between two groups with regard to the lipid profile, % content of the fat tissue, homocysteine levels, hsCRP, and IMT. Further studies are necessary to search for reasons for lower BMI and BMI centile in children with JIA and to attempt to answer the question of whether lower BMI increases the cardiovascular risk in these patients, similarly as in patients with rheumatoid arthritis (RA.

  1. Gene Expression Deconvolution for Uncovering Molecular Signatures in Response to Therapy in Juvenile Idiopathic Arthritis.

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    Ang Cui

    Full Text Available Gene expression-based signatures help identify pathways relevant to diseases and treatments, but are challenging to construct when there is a diversity of disease mechanisms and treatments in patients with complex diseases. To overcome this challenge, we present a new application of an in silico gene expression deconvolution method, ISOpure-S1, and apply it to identify a common gene expression signature corresponding to response to treatment in 33 juvenile idiopathic arthritis (JIA patients. Using pre- and post-treatment gene expression profiles only, we found a gene expression signature that significantly correlated with a reduction in the number of joints with active arthritis, a measure of clinical outcome (Spearman rho = 0.44, p = 0.040, Bonferroni correction. This signature may be associated with a decrease in T-cells, monocytes, neutrophils and platelets. The products of most differentially expressed genes include known biomarkers for JIA such as major histocompatibility complexes and interleukins, as well as novel biomarkers including α-defensins. This method is readily applicable to expression datasets of other complex diseases to uncover shared mechanistic patterns in heterogeneous samples.

  2. Accelerometry-based monitoring of daily physical activity in children with juvenile idiopathic arthritis.

    Science.gov (United States)

    Nørgaard, M; Twilt, M; Andersen, L B; Herlin, T

    2016-01-01

    Juvenile idiopathic arthritis (JIA) may cause functional impairment, reduced participation in physical activity (PA) and, over time, physical deconditioning. The aim of this study was to objectively monitor daily free-living PA in 10-16-year-old children with JIA using accelerometry with regard to disease activity and physical variables and to compare the data with those from healthy age- and gender-matched controls. Patients underwent an evaluation of disease activity, functional ability, physical capacity, and pain. Accelerometer monitoring was assessed using the GT1M ActiGraph. Normative data from two major studies on PA in Danish schoolchildren were used for comparison. Data of accelerometry were available for 61 JIA patients and 2055 healthy controls. Of the JIA patients, 57% showed below-average values of maximal physical capacity (fitness level). JIA patients showed low disease activity and pain and were physically well functioning. Accelerometer counts were lower in JIA patients than in controls. Accelerometer measurements were negatively correlated with disease activity, erythrocyte sedimentation rate (ESR), and number of joints with swelling and/or limited range of motion (ROM). No correlation was found between PA and pain scores, functional ability, and hypermobility. Patients with involvement of ankles or hips demonstrated significantly lower levels of PA. Children with JIA are less physically active and have lower physical capacity and fitness than their age- and gender-matched healthy peers despite good disease control. The involvement of hips or ankles is associated with lower PA.

  3. Growth retardation and functional disability in patients with juvenile idiopathic arthritis

    International Nuclear Information System (INIS)

    Saeed, S.; Zammurrad, S.; Rasheed, U.

    2017-01-01

    To determine the frequency of growth retardation and functional disability in patients with juvenile idiopathic arthritis (JIA). Methods: This descriptive cross sectional study was carried out at the Department of Rheumatology, Pakistan Institute of Medical Sciences, Islamabad from March 2016 to December 2016. 92 patients with JIA were included in the study. Height and weight of each patient was recorded and BMI calculated. Functional Disability was determined by calculating the CHAQ Score, while disease activity was estimated through JADAS 27 score. Growth Retardation was defined as height, weight and/or BMI below the 3rd centile according to the CDC growth charts. A cut off of ?1 for CHAQ Score was used to define severe functional disability. Results: Out of 92 patients, there were 47 male (51.1%) and 45 (48.9%) females. Height, weight and BMI below 3rd centile was observed in 30(32.6%), 49(53.3%) and 41(44.6%) patients, respectively. The overall frequency of growth retardation was 64.1% (n=59) and severe functional disability (CHAQ Score ?1) was seen in 32.6% (n=30) of patients. Conclusions: Growth retardation was seen in more than half of patients with JIA and a significant proportion had severe functional disability. Severe functional disability was seen only in patients with active disease. (author)

  4. Temporomandibular joint involvement in juvenile idiopathic arthritis: treatment with an orthodontic appliance

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    M. Gattinara

    2011-09-01

    Full Text Available Introduction and purpose: About 65% of children suffering from juvenile idiopathic arthritis (JIA shows a more or less marked involvement of temporo-mandibular joint (TMJ with altered mandibular growth, resorption of the condyles, occlusary instability, reduced chewing ability and facial dysmorphia. The purpose of our study is to prevent and to treat the progressive evolution of JIA on craniofacial growth and morphology with a functional appliance; surgery should be considered only in so far as the adequacy of TMJ movement is concemed. Methods: From 1992 until now 72 children with proved JIA and TMJ involvement have been treated (50 females, 22 males, aged 6 to 16 years old. TMJ involvement was bilateral in 61% and unilateral in 39% of patients. A diagnostic workup was carried out involving tomograms of TMJ and cephalometric radiograph and analysis. The authors used a bimaxillary activator in the attempt to modify the unfavourable growth pattern and provide a gradual ante-rotation of the jaw. Results: Almost all JIA patients showed satisfactory long term results, easing of pain, reduced skeletal discrepancy, increased function and good facial profile. Conclusions: The long term results of this study indicate that orthopaedic therapy might control the vicious circle of the malocclusion in children with JIA, preventing exacerbation of mandibular clockwise rotation. Surgical intervention for the improvement of TMJ function should be considered only if a severe restricted state is imminent.

  5. EXPERIENCE OF TREATMENT OF THE TWINS SICK WITH JUVENILE IDIOPATHIC ARTHRITIS BY ABATACEPT

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    V. A. Kel'tsev

    2014-01-01

    Full Text Available The case of clinical observation over twin brothers (boys E. and N suffering juvenile idiopathic arthritis is presented in the article. Boys fell ill with a difference in 1 year and 5 months at the age of 1 year and 9 months and 3 years 2 months, respectively. The disease proceeded wavily with the periods of remissions and exacerbations. Resistant articulate syndrome developed in July and November, 2009, respectively. Both children received NSAID, methotrexate combination in a dose of 10 mg/m2/week and sulphasalazine in a dose of 20 mg/kg of body weight per day. To the boy E. it was also prescripted prednisolon in a dose of 10 mg per day. Duration of antirheumatic therapy made 7 and 3 months, respectively. The therapy with a blocker of T-lymphocytes costimulation, abatacept, was initiated for the twins due to the inefficiency of treatment by immunodepressants and prednisolon at the boy E. In 6 weeks of the treatment 50% improvement, in 24 weeks — 90% improvement by criteria of the American College of Rheumatologists (ACRpedi were registered. The undesirable phenomena weren’t observed.

  6. Variation in dentofacial morphology and occlusion in juvenile idiopathic arthritis subjects: a case-control study.

    Science.gov (United States)

    Hu, Yuqian; Billiau, An D; Verdonck, An; Wouters, Carine; Carels, Carine

    2009-02-01

    Juvenile idiopathic arthritis (JIA) can severely disturb facial growth and affect occlusal development. In this case-control study, facial, functional, and occlusal characteristics of 100 JIA patients (35 males and 65 females; age range: 1.7-19.4 years) comprising all subtypes classified according to the revised classification criteria of the International League of Associations for Rheumatology (ILAR) were studied. They were compared with a mixed orthodontic control group (n=32; 12 males and 20 females) and with a Class II division 1 malocclusion group (n=19; eight males and 11 females). The JIA patients and controls were evaluated using clinical assessment, dental pantomograms, lateral cephalograms (LCGs), and dental casts. Compared with the age- and gender-matched mixed orthodontic controls, JIA patients showed a significantly greater prevalence of anterior open bites (AOBs; Pplane (Pplane, the gonial and the interincisal angles, or total face height. From this case-control study, it can be concluded that although JIA patients share occlusal characteristics with non-JIA patients with a Class II division 1 malocclusion, they are different with regard to the prevalence of condylar lesions and AOBs, as well as SNA and SNB angles and LAFH.

  7. Periodontal and hematological characteristics associated with aggressive periodontitis, juvenile idiopathic arthritis, and rheumatoid arthritis.

    Science.gov (United States)

    Havemose-Poulsen, Anne; Westergaard, Jytte; Stoltze, Kaj; Skjødt, Henrik; Danneskiold-Samsøe, Bente; Locht, Henning; Bendtzen, Klaus; Holmstrup, Palle

    2006-02-01

    Periodontitis shares several clinical and pathogenic characteristics with chronic arthritis, and there is some degree of coexistence. The aims of this study were to elucidate whether patients with localized aggressive periodontitis (LAgP), generalized aggressive periodontitis (GAgP), juvenile idiopathic arthritis (JIA), and rheumatoid arthritis (RA) share periodontal and hematological characteristics distinguishing them from individuals free of diseases. The study population consisted of white adults (or=4 mm, CAL>or=2 mm, and ABL>or=2 mm compared to controls. The percentage of sites with CAL>or=2 mm significantly correlated with the levels of IgM-RF and IgA-RF. Missing teeth in JIA and RA patients were not lost due to periodontitis. Patients with GAgP showed higher levels of leukocytes, including neutrophils, and CRP compared to controls. In part, JIA and RA patients showed similar results. Young adults with RA may develop periodontal destruction, and these patients require professional attention. Both differences and similarities in periodontal and hematological variables were seen in individuals with periodontitis, JIA, and RA.

  8. Influence of etanercept on leptin and ghrelin secretion in children with juvenile idiopathic arthritis.

    Science.gov (United States)

    Maciejewska-Paszek, Izabela; Grochowska-Niedworok, Elżbieta; Siwiec, Andrzej; Gruenpeter, Anna; Dul, Lechosław; Irzyniec, Tomasz

    2017-04-01

    Objective To assess possible changes in leptin and ghrelin secretion due to etanercept in juvenile idiopathic arthritis (JIA). Methods 50 patients with JIA and 16 age-matched controls were enrolled into this prospective, cross-sectional study. Serum leptin, total and acyl ghrelin were measured in addition to white blood cell (WBC) and lymphocyte counts. Results 25 patients received etanercept and 25 conventional therapies (including methotrexate) for JIA. There was no difference between treatment and control groups in leptin or ghrelin levels and no evidence of a relationship between leptin and ghrelin in patients with JIA. In all children with JIA there was a correlation between leptin and body mass index (BMI). However, compared with children in the conventional treatment group, children in the etanercept group showed a positive correlation between total ghrelin and BMI and those with a low BMI showed a negative correlation between acyl ghrelin and BMI. Conclusion No differences in leptin and ghrelin concentrations were found when patients with JIA and controls were compared or when patients who received etanercept were compared with those who received conventional treatment for JIA.

  9. Contrast-enhanced MRI features in the early diagnosis of Juvenile Idiopathic Arthritis

    International Nuclear Information System (INIS)

    Hemke, Robert; Maas, Mario; Kuijpers, Taco W.; Schonenberg-Meinema, Dieneke; Nusman, Charlotte M.; Rossum, Marion A.J. van; Berg, J.M. van den; Dolman, Koert M.

    2015-01-01

    To determine whether clinical, laboratory or Magnetic Resonance Imaging (MRI) measures differentiate Juvenile Idiopathic Arthritis (JIA) from other forms of active childhood arthritis. We prospectively collected data of 80 treatment-naive patients clinically suspected of JIA with active non-infectious arthritis of (at least) one knee for <12 months duration. Upon presentation patients underwent clinical and laboratory assessments and contrast-enhanced MRI. MRI was not used as a diagnostic criterion. Forty-four (55 %) patients were clinically diagnosed with JIA, whereas in 36 (45 %) patients the diagnosis of JIA was discarded on clinical or laboratory findings. MRI-based synovitis was present in 27 (61.4 %) JIA patients and in 7 (19.4 %) non-JIA patients (P < 0.001). Five factors (male gender, physician's global assessment of overall disease activity, joints with limited range of motion, HLA-B27, MRI-based synovitis) were associated with the onset of JIA. In multivariate analysis MRI-based synovitis proved to be independently associated with JIA (OR 6.58, 95 % CI 2.36-18.33). In patients with MRI-based synovitis, the RR of having JIA was 3.16 (95 % CI 1.6-6.4). The presence of MRI-based synovitis is associated with the clinical onset of JIA. Physical examination could be supported by MRI, particularly to contribute in the early differentiation of different forms of non-infectious childhood arthritis. (orig.)

  10. The importance of an ophthalmologic examination in patients with juvenile idiopathic arthritis.

    Science.gov (United States)

    Rodríguez-García, Alejandro

    2015-01-01

    Uveitis occurs within the first year of arthritis onset in 73% of patients with juvenile idiopathic arthritis (JIA) considered at risk. The intraocular inflammation is characterized by an insidious onset and a silent and chronic clinical course capable of producing significant visual loss due to complications such as: cataract formation, secondary glaucoma, maculopathy and optic neuropathy. The absence of initial signs and symptoms, along with a deficient ophthalmic monitoring produce a delay in diagnosis with serious consequences. It has been estimated that 47% of JIA patients at risk for developing uveitis are legally blind (20/200 or worse) at least in one eye at the time of their first visit to the ophthalmologist. To reduce ocular complications and improve their visual outcome, it is necessary that rheumatologists refer all patients recently diagnosed (within the first month) with JIA for an ophthalmic evaluation, and maintain periodical follow-up visits based on classification and risk category of the disease. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

  11. Principles on the use of imaging studies in the diagnosis and management of juvenile idiopathic arthritis

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    N. T. Vatutin

    2015-01-01

    Full Text Available The paper presents the basic principles on the use of imaging studies in the diagnosis and management of juvenile idiopathic arthritis (JIA, which have been elaborated by the European League Against Rheumatism (EULAR jointly with the Pediatric Rheumatology European Society (PreS. These principles will render certain assistance to practitioners in diagnosing and treating patients with JIA. Undoubtedly, there have been no answers to many questions so far. This primarily applies to the necessity of understanding the standards for the possibility to interpret the pathological process, to harmonize the respective MRI protocols, to identify bone marrow edema, erosions, and synovitis, and to determine the suitability of these or those examinations in order to reveal changes in individual joints. There are considerable conceptual differences in approaches to diagnostic techniques in adults and children. The EULAR-PReS experts assume that some studies may be impracticable or economically inaccessible and this may hinder their introduction into clinical practice. However, most techniques, including ultrasonography, are quite affordable. The practical application of these techniques certainly requires a high professionalism of specialists in functional diagnosis and other instrumental studies.

  12. A commentary on TREAT: The trial of early aggressive drug therapy in juvenile idiopathic arthritis

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    Baildam Eileen

    2012-06-01

    Full Text Available Abstract Polyarticular juvenile idiopathic arthritis (JIA is a category of JIA where multiple joints are affected by chronic inflammation, and where serious and lasting damage to joints is the expected natural history in untreated disease. There is evidence of response to disease-modifying antirheumatic and biologic drugs, but little evidence of permanent remission from any of the existing therapeutic trials. The TREAT trial by Wallace et al., recently published in Arthritis and Rheumatism, used a collaborative multicenter approach to studying early aggressive treatment of polyarticular JIA in an attempt to achieve full clinical inactive disease after 6 months of treatment. The study's main finding that the earlier in the disease course that treatment is started, the better the chance of disease control, has provided evidence that there is a 'window of opportunity' for treating JIA as there is in adult rheumatoid arthritis (RA. The study provides both a platform and an impetus for concentrating future treatment trials on early rather than established disease and investigating a standard of starting treatment within 10 to 12 weeks.

  13. Dynamics of body composition and bone in patients with juvenile idiopathic arthritis treated with growth hormone.

    Science.gov (United States)

    Bechtold, Susanne; Ripperger, Peter; Dalla Pozza, Robert; Roth, Johannes; Häfner, Renate; Michels, Hartmut; Schwarz, Hans Peter

    2010-01-01

    GH has a positive impact on growth, bone, and muscle development. The objectives of this study were to demonstrate the effects of GH treatment on regional body composition and bone geometry at final height in patients with juvenile idiopathic arthritis (JIA). In this longitudinal study, parameters of bone mineral density and geometry as well as muscle and fat cross-sectional area (CSA) in the nondominant forearm were recorded using peripheral quantitative computed tomography at yearly intervals until final height in 12 patients (seven females) receiving GH treatment. Data at final height were compared with 13 patients (nine females) with JIA not treated with GH. Patients were treated with GH for a mean of 5.35 +/- 0.7 yr. Correcting for height, total bone CSA (+0.89 +/- 0.5 sd) and muscle CSA (+1.14 +/- 0.6 sd) increased significantly and normalized at final height. Compared with JIA patients without GH at final height, there was a significantly higher muscle CSA and a lower fat CSA in GH-treated patients. Additionally, in relation to total bone CSA, there was significantly more cortical and less marrow CSA in boys with GH treatment. During GH treatment, there was a significant increase and normalization of total bone and muscle CSA at final height. In accordance with an anabolic effect of GH, fat mass stabilized at the lower limit of healthy children. At final height, cortical and marrow CSA, relative to total bone CSA, were normalized in GH-treated patients.

  14. Síndrome metabólica e artrite idiopática juvenil Metabolic syndrome and juvenile idiopathic arthritis

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    Clarisse de Almeida Zanette

    2010-04-01

    Full Text Available A Artrite Idiopática Juvenil (AIJ é a artropatia crônica mais prevalente na infância e na adolescência. A prevalência da síndrome metabólica, assim como da obesidade, vem apresentando rápido aumento, atingindo todas as faixas etárias, inclusive a infância. A síndrome metabólica é caracterizada por um conjunto de riscos para doença cardiovascular e diabetes melito tipo 2, abrangendo adiposidade abdominal, resistência à insulina, dislipidemias e hipertensão arterial sistêmica. Além desses componentes, a inflamação tem sido reconhecida cada vez mais como um fator importante na síndrome metabólica e na obesidade, e pacientes com doenças caracterizadas por processos inflamatórios crônicos, como a AIJ, poderiam representar grupos de risco especiais. Os glicocorticoides são utilizados rotineiramente no controle da inflamação da AIJ, em doses elevadas e com uso prolongado. O uso crônico do glicocorticoide pode induzir resistência à insulina, hipertensão arterial sistêmica e obesidade, aumentando o risco de desenvolver síndrome metabólica. O objetivo deste artigo é revisar a literatura sobre a prevalência dos diversos componentes da síndrome metabólica em pacientes com AIJ. Observamos que, nesses pacientes, os dados sobre síndrome metabólica e seus componentes são muito escassos e mais estudos se fazem necessários, tendo em vista o potencial impacto no aumento do risco de doença cardiovascular.Juvenile idiopathic arthritis (JIA is the most prevalent chronic arthropathy in childhood and adolescence. The prevalence of metabolic syndrome, as well as obesity, is increasing rapidly in all age groups, including children. Metabolic syndrome is defined as a cluster of risk factors for cardiovascular disease and type 2 diabetes mellitus, including abdominal obesity, insulin resistance, dyslipidemia and hypertension. Besides those components, inflammation has been increasingly considered as a significant component of

  15. Síndrome da ativação do macrófago em paciente com artrite idiopática juvenil poliarticular Macrophage activation syndrome in a patient with polyarticular juvenile idiopathic arthritis

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    Acir Rachid

    2004-10-01

    Full Text Available A linfohistiocitose hemofagocítica caracteriza-se por ativação e proliferação excessiva de linfócitos e macrófagos. Quando associada à artrite idiopática juvenil é também conhecida por "síndrome de ativação do macrófago", sendo uma complicação potencialmente fatal desta doença. Apresentamos o caso de uma mulher de 26 anos portadora de artrite idiopática juvenil (poliartrite, fator reumatóide negativo, com diagnóstico aos 13 anos, em uso de antiinflamatórios não esteroidais (diclofenaco, nimesulide. Admitida com quadro de resposta inflamatória sistêmica, febre, linfonodomegalia, esplenomegalia, anemia, trombocitopenia, hipofibrinogenemia, hiperferritinemia, hipertrigliceridemia e achados de hematofagocitose na medula óssea. Os autores discutem aspectos relacionados com a patogênese, diagnóstico e tratamento desta doença pouco conhecida.Hemophagocytic lymphohistiocytosis is characterized by massive lymphocyte and macrophage activation and proliferation. When observed in association with juvenile idiopathic arthritis it is also called "macrophage activation syndrome" being a potentially lethal complication of this disease. We report the case of a 26 years old woman with juvenile idiopathic arthritis (polyarthritis, rheumatoid factor negative since 13 years old, receiving nonsteroidal anti-inflammatory drugs (diclofenac, nimesulide. She was admitted with systemic inflammatory response, fever, lymph node enlargement, splenomegaly, anemia, thrombocytopenia, hypofibrinogenemia, hyperferritinemia, hypertriglyceridemia and bone marrow hemophagocytosis. Aspects related to pathogenesis, diagnosis and treatment of this little known disease are discussed.

  16. Contrast-enhanced MRI compared with the physical examination in the evaluation of disease activity in juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Hemke, Robert; Maas, Mario [Academic Medical Centre, University of Amsterdam, Department of Radiology, Amsterdam (Netherlands); Veenendaal, Mira van; Kuijpers, Taco W. [University of Amsterdam, Department of Paediatric Haematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Dolman, Koert M. [Department of Paediatric Rheumatology, Amsterdam (Netherlands); St. Lucas Andreas Hospital, Department of Paediatrics, Amsterdam (Netherlands); Rossum, Marion A.J. van; Berg, J.M. van den [University of Amsterdam, Department of Paediatric Haematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Department of Paediatric Rheumatology, Amsterdam (Netherlands)

    2014-02-15

    To assess the value of magnetic resonance imaging (MRI) in discriminating between active and inactive juvenile idiopathic arthritis (JIA) patients and to compare physical examination outcomes with MRI outcomes in the assessment of disease status in JIA patients. Consecutive JIA patients with knee involvement were prospectively studied using an open-bore MRI. Imaging findings from 146 JIA patients were analysed (59.6 % female; mean age, 12.9 years). Patients were classified as clinically active or inactive. MRI features were evaluated using the JAMRIS system, comprising validated scores for synovial hypertrophy, bone marrow oedema, cartilage lesions and bone erosions. Inter-reader reliability was good for all MRI features (intra-class correlation coefficient [ICC] = 0.87-0.94). No differences were found between the two groups regarding MRI scores of bone marrow oedema, cartilage lesions or bone erosions. Synovial hypertrophy scores differed significantly between groups (P = 0.016). Nonetheless, synovial hypertrophy was also present in 14 JIA patients (35.9 %) with clinically inactive disease. Of JIA patients considered clinically active, 48.6 % showed no signs of MRI-based synovitis. MRI can discriminate between clinically active and inactive JIA patients. However, physical examination is neither very sensitive nor specific in evaluating JIA disease activity compared with MRI. Subclinical synovitis was present in >35 % of presumed clinically inactive patients. (orig.)

  17. Rituximab use in young adults diagnosed with juvenile idiopathic arthritis unresponsive to conventional treatment: report of 6 cases.

    Science.gov (United States)

    Sakamoto, Ana Paula; Pinheiro, Marcelo M; Barbosa, Cássia Maria Passarelli Lupoli; Fraga, Melissa Mariti; Len, Claudio Arnaldo; Terreri, Maria Teresa

    2015-01-01

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. Without an effective therapy, patients may progress quickly to functional disability. Recently, depletion of B cells emerged as a new approach for the treatment of autoimmune diseases, including JIA. We describe six cases of JIA patients followed at a referral center for Rheumatology and Pediatric Rheumatology, submitted to treatment with rituximab (RTX) after refractoriness to three anti-TNF agents. Patients received RTX cycles with two infusions every six months. Response to treatment was assessed by DAS28, HAQ/CHAQ, and an overall assessment by the doctor and the patient. Of our six patients, four were girls (mean age at onset of disease: 6.1 years; mean disease evolution time: 15.1 years; mean age upon receiving RTX: 21.6 years). Four patients belonged to polyarticular subtype (1 rheumatoid factor [RF]-negative, 3 FR-positive), a patient with systemic JIA subtype with a polyarticular course and arthritis related to enthesitis. Of our six patients, five responded to treatment; and during the course of 12 months, the clinical response was maintained, although not sustained. However, discontinuation by infusion reactions caused the withdrawal of RTX in two patients. The use of RTX in JIA is restricted to cases refractory to other biological agents and, even considering that this study was held in a small number of advanced patients, RTX proved to be an effective therapeutic option. Copyright © 2015 Elsevier Editora Ltda. All rights reserved.

  18. MRI of the wrist in juvenile idiopathic arthritis: erosions or normal variants? A prospective case-control study

    International Nuclear Information System (INIS)

    Ording Muller, Lil-Sofie; Boavida, Peter; Avenarius, Derk; Eldevik, Odd Petter; Damasio, Beatrice; Malattia, Clara; Lambot-Juhan, Karen; Tanturri, Laura; Owens, Catherine M.; Rosendahl, Karen

    2013-01-01

    Bony depressions at the wrist resembling erosions are frequently seen on MRI in healthy children. The accuracy of MRI in detecting early bony destruction is therefore questionable. We compared findings on MRI of the wrist in healthy children and those with juvenile idiopathic arthritis (JIA) to investigate markers for true disease. We compared the number and localisation of bony depressions at the wrist in 85 healthy children and 68 children with JIA, ages 5-15 years. The size of the wrist was assessed from a radiograph of the wrist performed on the same day as the MRI. No significant difference in the number of bony depressions in the carpal bones was seen between healthy children and children with JIA at any age. Depressions are found in similar locations in the two groups, except for a few sites, where bony depressions were seen exclusively in the JIA group, particularly at the CMC joints. The wrist was significantly smaller in children with JIA (P < 0.001). Using adult scoring systems and standard MR sequences in the assessment of bone destruction in children may lead to overstaging or understaging of disease. At present, standard MRI sequences cannot easily be used for assessment of early signs of erosions in children. (orig.)

  19. Role of vitamin D receptor (VDR gene polymorphism in the pathogenesis of juvenile idiopathic arthritis: Theoretical and practical aspects

    Directory of Open Access Journals (Sweden)

    M. M. Kostik

    2014-01-01

    Full Text Available Juvenile idiopathic arthritis (JIA is a chronic inflammatory joint disease associated with impaired immune system performance. The specific features of JIA may be genetically determined.Objective: to assess JIA activity in children with vitamin D receptor (VDR gene ApaI and BsmI polymorphism genotypes.Subjects and methods. The investigation enrolled 71 patients with JIA. When included in the investigation, all the patients were in an active state of disease. JIA activity was assessed using the most commonly used clinical and laboratory indicators, including the Ritchie articular index (RAI, JADAS10, JADAS27, JADAS71, CDAI, DAS, and DAS28. Molecular genetic studies determined VDR gene ApaI and BsmI polymorphisms by polymerase chain reaction, followed by restriction analysis.Results. The boys who were carriers of a bb BsmI polymorphic marker in the VDR gene had a significantly higher activity of JIA measured by RAI (p=0.03, DAS (p<0.05, JADAS10 (p=0.04, JADAS27 (p=0.04, and JADAS71 (p=0.04 than those who were carriers of B allele (BB + Bb genotypes.Conclusion. The carriage of the VDR gene bb BsmI genotype of the polymorphic marker is associated with high JIA activity, which may be regarded as a marker of poor prognosis in boys with JIA.

  20. REFORMATIONS IN ZIMBABWE'S JUVENILE JUSTICE SYSTEM

    African Journals Online (AJOL)

    Mugumbate

    1996-05-23

    May 23, 1996 ... inconsistent parenting practices, poverty, school-related problems and exposure to delinquent peers and community and societal problems. This paper discusses the recent reforms in the juvenile justice system in Zimbabwe and elaborates on the role of social work practice in the field of juvenile justice.

  1. Reformations in Zimbabwe's juvenile justice system | Ruparanganda ...

    African Journals Online (AJOL)

    Children in conflict with the law are often stigmatized and shunned by society as they are perceived as a threat to society. Historically, Zimbabwe's juvenile justice system has been retributive and focused on punishing the juvenile offender. As a result, it has been criticised from a number of viewpoints, including the need to ...

  2. Transforming Growth Factor-Beta (TGF-β Signaling in Paravertebral Muscles in Juvenile and Adolescent Idiopathic Scoliosis

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    Roman Nowak

    2014-01-01

    Full Text Available Most researchers agree that idiopathic scoliosis (IS is a multifactorial disease influenced by complex genetic and environmental factors. The onset of the spinal deformity that determines the natural course of the disease, usually occurs in the juvenile or adolescent period. Transforming growth factors β (TGF-βs and their receptors, TGFBRs, may be considered as candidate genes related to IS susceptibility and natural history. This study explores the transcriptional profile of TGF-βs, TGFBRs, and TGF-β responsive genes in the paravertebral muscles of patients with juvenile and adolescent idiopathic scoliosis (JIS and AIS, resp.. Muscle specimens were harvested intraoperatively and grouped according to the side of the curve and the age of scoliosis onset. The results of microarray and qRT-PCR analysis confirmed significantly higher transcript abundances of TGF-β2, TGF-β3, and TGFBR2 in samples from the curve concavity of AIS patients, suggesting a difference in TGF-β signaling in the pathogenesis of juvenile and adolescent curves. Analysis of TGF-β responsive genes in the transcriptomes of patients with AIS suggested overrepresentation of the genes localized in the extracellular region of curve concavity: LTBP3, LTBP4, ITGB4, and ITGB5. This finding suggests the extracellular region of paravertebral muscles as an interesting target for future molecular research into AIS pathogenesis.

  3. Transforming Growth Factor-Beta (TGF-β) Signaling in Paravertebral Muscles in Juvenile and Adolescent Idiopathic Scoliosis

    Science.gov (United States)

    Kwiecien, Magdalena

    2014-01-01

    Most researchers agree that idiopathic scoliosis (IS) is a multifactorial disease influenced by complex genetic and environmental factors. The onset of the spinal deformity that determines the natural course of the disease, usually occurs in the juvenile or adolescent period. Transforming growth factors β (TGF-βs) and their receptors, TGFBRs, may be considered as candidate genes related to IS susceptibility and natural history. This study explores the transcriptional profile of TGF-βs, TGFBRs, and TGF-β responsive genes in the paravertebral muscles of patients with juvenile and adolescent idiopathic scoliosis (JIS and AIS, resp.). Muscle specimens were harvested intraoperatively and grouped according to the side of the curve and the age of scoliosis onset. The results of microarray and qRT-PCR analysis confirmed significantly higher transcript abundances of TGF-β2, TGF-β3, and TGFBR2 in samples from the curve concavity of AIS patients, suggesting a difference in TGF-β signaling in the pathogenesis of juvenile and adolescent curves. Analysis of TGF-β responsive genes in the transcriptomes of patients with AIS suggested overrepresentation of the genes localized in the extracellular region of curve concavity: LTBP3, LTBP4, ITGB4, and ITGB5. This finding suggests the extracellular region of paravertebral muscles as an interesting target for future molecular research into AIS pathogenesis. PMID:25313366

  4. Physical activity, functional ability, and disease activity in children and adolescents with juvenile idiopathic arthritis.

    Science.gov (United States)

    Gueddari, S; Amine, B; Rostom, S; Badri, D; Mawani, N; Ezzahri, M; Moussa, F; Shyen, S; Abouqal, R; Chkirat, B; Hajjaj-Hassouni, N

    2014-09-01

    Juvenile idiopathic arthritis (JIA) is a chronic condition known to cause pain-related complications in youth and affect children's physical functioning. There is no data in Arabic children with JIA about the impact of illness upon their physical activity. The objective of this study was to explore physical activity (PA) in children and adolescents with JIA compared with a healthy population and to examine associations between PA, functional ability, and disease activity. Our study included patients with JIA and group control aged between 8 and 17 years. The diagnosis was used according to the International League of Association of Rheumatology (ILAR) criteria 2001. Sociodemographic data and clinical features were collected. Physical activity level and energy expenditure were assessed with a 1-day activity diary and the metabolic equivalent (MET), respectively. Functional ability was assessed with the Moroccan version of the Childhood Health Assessment Questionnaire (CHAQ). Disease activity was measured using the Juvenile Arthritis Disease Activity Score (JADAS). Fifty patients and 50 controls were included (mean ± SD age 11.5 ± 3.3 and 10.5 ± 3.8 years, respectively; p = 0.49) with masculine predominance n = 30 (59.6 %) and n = 29 (58 %), respectively (p = 0.26). The median disease duration was 4.3 years (2-5). The median analog scale (VAS) pain was 20 (10-40). Fourteen patients (28 %) had an active disease. Patient population consisted in majority of oligoarticular arthritis (28 %), 14 patients. The mean of energy expenditure and physical activity were significantly higher in the JIA group. The JIA group spent more time in bed and less time on moderate to vigorous PA than the control group. There is no significant relationship between PA, functional ability, and disease activity. Our study suggests that children and adolescents with JIA have low PA levels and are at risk of losing the benefits of PA. Low PA is not related to

  5. Outcome in adults with juvenile idiopathic arthritis: a quality of life study.

    Science.gov (United States)

    Foster, Helen E; Marshall, Nicola; Myers, Andrea; Dunkley, Pippa; Griffiths, Ian D

    2003-03-01

    To evaluate quality of life (QOL) in adults with juvenile idiopathic arthritis (JIA), using validated measures of functional disability and generic health status, and to quantify their educational attainment and employment status. The adult rheumatology departmental database was used to identify patients. Functional disability and generic health status/QOL were assessed by the Health Assessment Questionnaire (HAQ) and the Short Form 36-item health profile (SF-36), respectively. Educational achievement and employment status were assessed by questionnaire. Complete data were available for 82 of the 101 patients identified. The median age of patients was 30 years, and the median disease duration was 21 years. No deaths were recorded. All subtypes of JIA were represented. Thirty-nine percent of patients had active disease (based on the physician global assessment scale score). The median HAQ score was 1.125 (range 0-3). SF-36 scores for bodily pain, general health, physical functioning, vitality, emotion, and social isolation were significantly worse in patients compared with controls, and this trend increased with increasing age of the patients and disease duration. The SF-36 mental summation scores of patients were low compared with those of controls, for all subtypes of JIA, and this finding was independent of the degree of functional disability (by HAQ and SF-36 physical summation scores). The educational attainment of patients was comparable to that of local controls, but unemployment rates for patients were 3-fold higher than those for controls. This is the largest study in which the SF-36 was used to assess generic health status and QOL in adults with JIA. Many patients had active disease in adulthood, and although the physical outcome of adults with JIA is relatively good, a profound effect on generic health status and QOL was demonstrated for all types of JIA. Furthermore, despite excellent educational attainment, there was a high rate of unemployment among

  6. Development and testing of reduced joint counts in juvenile idiopathic arthritis.

    Science.gov (United States)

    Bazso, Anna; Consolaro, Alessandro; Ruperto, Nicolino; Pistorio, Angela; Viola, Stefania; Magni-Manzoni, Silvia; Malattia, Clara; Buoncompagni, Antonella; Loy, Anna; Martini, Alberto; Ravelli, Angelo

    2009-01-01

    To develop and test reduced joint counts in children with juvenile idiopathic arthritis (JIA). Four reduced joint counts including 45, 35, 27, and 10 joints were devised by a panel of experienced pediatric rheumatologists, who selected the joints to be included based on the ease of technical assessment, functional relevance, and frequency of involvement. Three large samples of patients with JIA (total n=4353) who had a detailed joint assessment available were used to develop and test reduced joint counts. Performance of reduced counts was examined by comparing their Spearman correlation with the standard (i.e., complete) joint count. Construct validity was evaluated by calculating Spearman correlation with other JIA outcome measures. Responsiveness to clinical change was determined through the standardized response mean (SRM). Spearman correlations of reduced joint counts with the whole joint count and with the other JIA outcome measures were comparable, revealing that they had similar ability to serve as surrogate for the whole joint count and construct validity. Responsiveness to clinical change was also comparable across reduced counts (SRM 0.83-1.09 for active joint counts and 0.63-0.81 for restricted joint counts). Based on these results and considering the relative feasibility of the different counts, the 27-joint reduced count is proposed for use in JIA. This joint count includes the cervical spine and the elbow, wrist, metacarpophalangeal (from first to third), proximal interphalangeal, hip, knee, and ankle joints. Reduced joint counts appear to be as reliable as standard joint counts in assessment of the severity of joint disease and its change over time in children with JIA.

  7. How do parents of children with juvenile idiopathic arthritis (JIA perceive their therapies?

    Directory of Open Access Journals (Sweden)

    Nageswaran Savithri

    2008-06-01

    Full Text Available Abstract Background Complementary and alternative medical (CAM therapies are commonly used by pediatric patients with chronic medical conditions. Little is known about parents' perceptions of these therapies. This study describes the views of parents of patients with juvenile idiopathic arthritis (JIA regarding conventional and CAM therapies. Methods Parents of children with JIA seen at a pediatric rheumatology clinic were surveyed between June 1 and July 31, 2007. Questionnaires asked about patients' use of over 75 therapies in the past 30 days, their perceived helpfulness (0 = not helpful; 3 = very helpful, perceived side effects (0 = none; 3 = severe, and whether each therapy would be recommended to other patients with JIA (Yes, No, Not sure. Results Questionnaires were returned by 52/76 (68% parents; patients' average age was 10.9 years and 87% were Caucasian. Medications were used by 45 (88% patients; heat (67% and extra rest (54% were also commonly used. CAM therapies were used by 48 (92%, e.g., massage (54%, vitamins and other supplements (54%, avoiding foods that worsened pain (35% and stress management techniques (33%. Among the therapies rated by 3 or more parents, those that scored 2.5 or higher on helpfulness were: biologic medications, methotrexate, naproxen, wheelchairs, orthotics, heat, vitamins C and D, music, support groups and prayer. CAM therapies had 0 median side effects and parents would recommend many of them to other families. Conclusion JIA patients use diverse therapies. Parents report that many CAM therapies are helpful and would recommend them to other parents. These data can be used in counseling patients and guiding future research.

  8. Intra-articular injection in patients with juvenile idiopathic arthritis: factors associated with a good response.

    Science.gov (United States)

    Cunha, Ana Luiza Garcia; Miotto E Silva, Vanessa Bugni; Osaku, Fabiane Mitie; Niemxeski, Luísa Brasil; Furtado, Rita Nely Vilar; Natour, Jamil; Sande, Maria Teresa de; Terreri, Lemos Ramos Ascensão

    Intra-articular injection of corticosteroids (IIC) for treatment of patients with juvenile idiopathic arthritis (JIA) is increasingly used in Pediatric Rheumatology. To describe the clinical course of patients undergoing IIC in our Pediatric Rheumatology Unit. Retrospective study of patients with JIA undergoing IIC from January 2008 to December 2012, with a minimum follow-up of six months after the injection. Good response to IIC was set as the presence of inactivity on the infiltrated joint by at least six months. Eighty-eight patients underwent a total of 165 IICs. Of these, 75% were girls and 35.2% had persistent oligoarticular JIA. The mean age at diagnosis was 6.8 years, and when IIC was carried out, 12.2 years. Regarding patients, younger age at diagnosis (p=0.037) and the occurrence of uveitis in the course of the disease (p=0.015) were associated with good response to IIC. From 165 IICs, 63% had a good response and joints remained inactive for a median of 18.1 months. The type of joint injection (p=0.001), lesser values stated in the overall visual analog scale by the physician (p=0.015) and by parents/patient (p=0.01) have been associated with a good response to IIC. Nine adverse events (5.4%) were observed. In our study, more than half of the joints showed a good response to IIC. Younger patients at diagnosis and uveitis during the course of the disease had good response to IIC. Knees, wrists and elbows were the joints that best responded to IIC. IIC proved to be a safe procedure. Copyright © 2016 Elsevier Editora Ltda. All rights reserved.

  9. High rates of unsuccessful transfer to adult care among young adults with juvenile idiopathic arthritis

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    Duffy Ciarán M

    2010-01-01

    Full Text Available Abstract 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.

  10. Association between vitamin D deficiency and disease activity in juvenile idiopathic arthritis.

    Science.gov (United States)

    Çomak, Elif; Doğan, Çağla Serpil; Uslu-Gökçeoğlu, Arife; Akbaş, Halide; Özdem, Sabahat; Koyun, Mustafa; Akman, Sema

    2014-01-01

    Vitamin D has been shown to have immunomodulatory and anti-inflammatory properties in addition to its well-established role in the maintenance of mineral homeostasis and bone health. The aims of this study were to evaluate vitamin D status in patients with juvenile idiopathic arthritis (JIA), and also to examine whether there is an association between serum levels of 25-hydroxyvitamin D [25(OH)D] and disease activity in JIA. Children with JIA who had an outpatient visit between March and April 2011 were evaluated retrospectively. Clinical and laboratory findings and vitamin D levels were evaluated. Disease activity was calculated using JADAS-27. Serum vitamin D levels were measured using high-performance liquid chromatography (HPLC). A total of 47 patients, 29 (61.7%) of them girls, with a mean age of 9.3±3.9 years and a median follow-up period of 28 months, were included in the study. The mean serum vitamin D level of all patients was 17.7±11.6 ng/ml. Vitamin D insufficiency (serum vitamin D: 15-20 ng/ml) and deficiency (serum vitamin D level 20 ng/ml). There was a significant negative correlation between vitamin D levels and disease activity (p=0.01, r=-0,37). The mean JADAS-27 score was significantly higher in patients with 25(OH)D levels 15 ng/ml (p = 0.003). We suggest that vitamin D deficiency may be a possible modifiable risk factor affecting disease activity in JIA.

  11. [Low bone mineral density in juvenile idiopathic arthritis: Prevalence and related factors].

    Science.gov (United States)

    Galindo Zavala, Rocío; Núñez Cuadros, Esmeralda; Martín Pedraz, Laura; Díaz-Cordovés Rego, Gisela; Sierra Salinas, Carlos; Urda Cardona, Antonio

    2017-10-01

    Height adjustment is currently recommended for Z-score bone mineral density (BMD) assessed by dual energy X-ray absorptiometry. At present there are no studies that evaluate the prevalence of low BMD in paediatric patients with Juvenile Idiopathic Arthritis (JIA) in Spain following current recommendations. To evaluate low BMD in JIA in paediatric patients with JIA in Spain following the latest recommendations, as well as to assess associated factors. Observational cross-sectional study of Spanish JIA patients from 5 to 16 years-old, followed-up in a Paediatric Rheumatology Unit between July 2014 and July 2015. Anthropometric, clinical and treatment data were recorded. Dual energy X-ray absorptiometry, and bone metabolism parameters were collected, and a completed diet and exercise questionnaire was obtained. A total of 92 children participated. The population prevalence estimation of low BMD was less than 5% (95% CI). A significant positive correlation was found in the multiple linear regression analysis between the body mass index percentile (B: 0.021; P<.001) and lean mass index (B: 0.0002; P=.012), and BMD Z-score adjusted for height (Z-SAH). A significant negative correlation was found between fat mass index (B: -0.0001; P=.018) and serum type I collagen N-propeptide (B: -0,0006; P=.036) and Z-SAH. Low BMD prevalence in JIA patients in our population is low. An adequate nutritional status and the prevalence of lean over fat mass seem to promote the acquisition of bone mass. Those JIA patients with lower BMD could be subjected to an increase of bone turnover. Copyright © 2016 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  12. The relationship between physical activity levels and pain in children with juvenile idiopathic arthritis.

    Science.gov (United States)

    Limenis, Elizaveta; Grosbein, Haddas A; Feldman, Brian M

    2014-02-01

    Pain and reduced physical activity levels are common in children with juvenile idiopathic arthritis (JIA). Currently, there is no consensus about the role of physical activity in managing pain in JIA. The purpose of our study was to assess the relationship between physical activity level and pain in children ages 11 to 18 years with JIA. A random sample of 50 patients with JIA were approached by mailed questionnaires. Physical activity was determined using the Physical Activity Questionnaire (PAQ). Pain measures included the Numerical Rating Scale (pain severity), SUPER-KIDZ body diagram (number of painful areas), and the Child Activities Limitations Inventory-21 (pain interference). Generalized linear models were used to assess the relationship between physical activity and pain, as well as the roles of sex and age. The response rate was 84%. Thirty-four respondents completed the questionnaire package. The median age was 15 years. The mean PAQ score was 2.16/5. Physical activity declines with increasing age in youth with JIA (r = 0.53, p = 0.0014). Lower physical activity is associated with greater pain interference (r = 0.39, p = 0.0217) and more severe pain (r = 0.35, p = 0.0422). Children with JIA report significantly less activity than healthy children based on PAQ scores, with physical activity declining throughout adolescence. Physical activity is inversely related to pain interference and severity in children with JIA. Our findings suggest that physical activity interventions may play an important role in the management of pain in JIA.

  13. Efficacy of rehabilitation therapy on hospital stage of treatment of patients with juvenile idiopathic arthritis

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    T A Shelepina

    2008-01-01

    Full Text Available Objective. To assess efficacy of hospital stage of treatment including rehabilitation methods in pts with juvenile idiopathic arthritis (JIA evaluating changes of some measures of functional status at admission and at discharge. Material and methods. 56 pts with different variants of JIA admitted to the pediatric department of the Institute of Rheumatology of RAMS and treated with medicaments and rehabilitation methods. Correction of anti-inflammatory therapy influencing functional status (NSAIDs dose increase, intra-articular injections of glucocorticoids was done in 36 pts. In the rest of pts treatment with DMARDs was changed. Mean duration of hospital stay was 20 days. At admission and at discharge following measures were recorded: pain on VAS, dynamometry, 25 m walking time, contracture angle and amplitude of movement of joint causing disability and treated with rehabilitation methods (mean summated measures were assessed, localization was not considered. Results. Significant change of pain (29,04 mm+2,9 mm vs 9,92 mm±l,6 mm, contracture angle (19,33o±l 1,12 о vs 10,33 о ±8,34 о and movement amplitude (71,5 о ±38,52 о vs 90,71 о ±38,52 о. Significant improvement (movement amplitude increase from 68,6±42,4 to 85,3±41,0 and angle of deformation decrease from 15±5,8 to 8,5±7,4 was achieved in 20 pts without correction of treatment influencing functional status. Conclusion. Functional status of pts with JIA improved as a result of complex treatment with medicaments and active rehabilitation methods. Importance of rehabilitation treatment in pts with JIA during hospital stay was shown.

  14. Transitional care in clinical networks for young people with juvenile idiopathic arthritis: current situation and challenges.

    Science.gov (United States)

    Cruikshank, Mary; Foster, Helen E; Stewart, Jane; Davidson, Joyce E; Rapley, Tim

    2016-04-01

    Clinical networks for paediatric and adolescent rheumatology are evolving, and their effect and role in the transition process between paediatric and adult services are unknown. We therefore explored the experiences of those involved to try and understand this further. Health professionals, young people with juvenile idiopathic arthritis and their families were recruited via five national health service paediatric and adolescent rheumatology specialist centres and networks across the UK. Seventy participants took part in focus groups and one-to-one interviews. Data was analysed using coding, memoing and mapping techniques to identify features of transitional services across the sector. Variation and inequities in transitional care exist. Although transition services in networks are evolving, development has lagged behind other areas with network establishment focusing more on access to paediatric rheumatology multidisciplinary teams. Challenges include workforce shortfalls, differences in service priorities, standards and healthcare infrastructures, and managing the legacy of historic encounters. Providing equitable high-quality clinically effective services for transition across the UK has a long way to go. There is a call from within the sector for more protected time, staff and resources to develop transition roles and services, as well as streamlining of local referral pathways between paediatric and adult healthcare services. In addition, there is a need to support professionals in developing their understanding of transitional care in clinical networks, particularly around service design, organisational change and the interpersonal skills required for collaborative working. Key messages • Transitional care in clinical networks requires collaborative working and an effective interface with paediatric and adult rheumatology.• Professional centrism and historic encounters may affect collaborative relationships within clinical networks.• Education

  15. Ultrasonography and color Doppler of proximal gluteal enthesitis in juvenile idiopathic arthritis: a descriptive study

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    Thomsen Carsten

    2011-08-01

    Full Text Available Abstract Background The presence of enthesitis (insertional inflammation in patients with juvenile idiopathic arthritis (JIA is difficult to establish clinically and may influence classification and treatment of the disease. We used ultrasonography (US and color Doppler (CD imaging to detect enthesitis at the small and deep-seated proximal insertion of the gluteus medius fascia on the posterior iliac crest where clinical diagnosis is difficult. The findings in JIA patients were compared with those obtained in healthy controls and with the patients' MRI results. Methods Seventy-six proximal gluteus medius insertions were studied clinically (tenderness to palpation of the posterior iliac crest and by US and CD (echogenicity, thickness, hyperemia in 38 patients with JIA and in 38 healthy controls, respectively (median age 13 years, range 7-18 years. In addition, an additional MRI examination of the sacroiliac joints and iliac crests was performed in all patients. Results In patients with focal, palpable tenderness, US detected decreased echogenicity of the entheses in 53% of the iliac crests (bilateral in 37% and unilateral in 32%. US also revealed significantly thicker entheses in JIA patients compared to healthy controls (p Conclusions According to US, the gluteus medius insertion was thicker in JIA patients than in controls, and it was hypoechoic (enthesitis in about half of the patients. These findings may represent chronic, inactive disease in some of the patients, because there was only limited Doppler flow and MRI contrast enhancement. The present study indicates that US can be useful as an adjunct to clinical examination for improved assessment of enthesitis in JIA. This may influence disease classification, ambition to treat, and choice of treatment regimen.

  16. Overlap of disease susceptibility loci for rheumatoid arthritis and juvenile idiopathic arthritis

    Science.gov (United States)

    Hinks, Anne; Eyre, Steve; Ke, Xiayi; Barton, Anne; Martin, Paul; Flynn, Edward; Packham, Jon; Worthington, Jane; Thomson, Wendy

    2010-01-01

    Background Genome-wide association studies (GWAS) have been extremely successful in the search for susceptibility risk factors for complex genetic autoimmune diseases. As more studies are published, evidence is emerging of considerable overlap of loci between these diseases. In juvenile idiopathic arthritis (JIA), another complex genetic autoimmune disease, the strategy of using information from autoimmune disease GWAS or candidate gene studies to help in the search for novel JIA susceptibility loci has been successful, with confirmed association with two genes, PTPN22 and IL2RA. Rheumatoid arthritis (RA) is an autoimmune disease that shares similar clinical and pathological features with JIA and, therefore, recently identified confirmed RA susceptibility loci are also excellent JIA candidate loci. Objective To determine the overlap of disease susceptibility loci for RA and JIA. Methods Fifteen single nucleotide polymorphisms (SNPs) at nine RA-associated loci were genotyped in Caucasian patients with JIA (n=1054) and controls (n=3531) and tested for association with JIA. Allele and genotype frequencies were compared between cases and controls using the genetic analysis software, PLINK. Results Two JIA susceptibility loci were identified, one of which was a novel JIA association (STAT4) and the second confirmed previously published associations of the TRAF1/C5 locus with JIA. Weak evidence of association of JIA with three additional loci (Chr6q23, KIF5A and PRKCQ) was also obtained, which warrants further investigation. Conclusion All these loci are good candidates in view of the known pathogenesis of JIA, as genes within these regions (TRAF1, STAT4, TNFAIP3, PRKCQ) are known to be involved in T-cell receptor signalling or activation pathways. PMID:19674979

  17. Increased prevalence of dental caries and poor oral hygiene in juvenile idiopathic arthritis.

    Science.gov (United States)

    Welbury, R R; Thomason, J M; Fitzgerald, J L; Steen, I N; Marshall, N J; Foster, H E

    2003-12-01

    Recent decades have seen a trend to treat juvenile idiopathic arthritis (JIA) with increasing immunosuppression to improve the long-term outcome. Poor oral hygiene and dental decay cause significant morbidity, and patients with chronic disease (who may be further immunocompromised by treatment) are at greater risk. This study investigated patients with JIA using standard measures of oral health. One hundred and forty-nine patients with JIA were included. The children were attending a regional paediatric rheumatology service and the adults were attending an adult rheumatology clinic. Random age- and sex-matched healthy controls were recruited from a dental teaching hospital. The structured dental examination included standard epidemiological indices of oral hygiene (gingival index, plaque index, oral cleanliness index) and dental decay [DMFT (decayed, missing or filled teeth) index]. JIA patients, at all ages, had increased levels of dental decay and poor oral hygiene. This increased level of decay was statistically significant in the patients aged 0-11 yr. Significant levels of untreated caries and increased levels of missing teeth were found in JIA, suggesting that patients with JIA had less restorative dental treatment, with tooth extraction often the chosen option for the treatment of dental decay. This is the largest study of oral health in JIA and is cross-sectional with non-diseased controls. It shows significantly increased levels of poor oral hygiene and dental decay in patients with JIA. The high levels of untreated dental decay suggest barriers to dental care. These results emphasize the role of regular dental care in the multidisciplinary management of JIA.

  18. Regular Aerobic Training Combined with Range of Motion Exercises in Juvenile Idiopathic Arthritis

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    Mine Doğru Apti

    2014-01-01

    Full Text Available Objective. To assess the effects of regular aerobic training combined with range of motion (ROM exercises on aerobic capacity, quality of life, and function in children with juvenile idiopathic arthritis (JIA. Methods. Thirty patients with JIA and 20 healthy age-matched controls (mean age ± SD, 11.3 ± 2.4 versus 11.0 ± 2.3, resp.; P>0.05 were included. All patients performed aerobic walking (4 days a week for 8 weeks and active and passive ROM exercises of involved joints. All patients completed the childhood health assessment questionnaire (CHAQ and the child health questionnaire. ROM measurements of joints were performed by using universal goniometer. Aerobic capacity was determined by measuring peak oxygen uptake (VO2peak during an incremental treadmill test. Results. Peak oxygen uptake and exercise duration were significantly lower in JIA group than in controls (32.5 ± 6.6 versus 35.9 ± 5.8 and 13.9 ± 1.9 versus 15.0 ± 2.0, resp.; P<0.05 for both. Eight-week combined exercise program significantly improved exercise parameters of JIA patients (baseline versus postexercise VO2peak and exercise duration, 32.5 ± 6.6 to 35.3 ± 7.9 and 13.9 ± 1.9 to 16.3 ± 2.2, resp.; P<0.001 for both. Exercise intervention significantly improved CHAQ scores in JIA patients (0.77 ± 0.61 to 0.20 ± 0.28, P<0.001. Conclusion. We suggest that regular aerobic exercise combined with ROM exercises may be an important part of treatment in patients with JIA.

  19. Radiographic and MR Imaging Findings of the Spine after Bisphosphonate Treatment, in a Child with Idiopathic Juvenile Osteoporosis

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    Olympia Papakonstantinou

    2015-01-01

    Full Text Available Bisphosphonates are employed with increasing frequency in various pediatric disorders, mainly associated with osteoporosis. After cessation of bisphosphonate treatment in children, skeletal radiologic changes have been documented including dense metaphyseal lines of the long bones and “bone in bone” appearance of the vertebrae. However, the evolution of these radiographic changes has not been fully explored. We describe the MR imaging appearance of the spine that, to our knowledge, has not been previously addressed in a child with idiopathic juvenile osteoporosis who had received bisphosphonates and emphasize the evolution of the radiographic findings of the spine and pelvis over a four-year period.

  20. Long-term results after Boston brace treatment in late-onset juvenile and adolescent idiopathic scoliosis

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    Gunderson Ragnhild

    2011-08-01

    Full Text Available Abstract Background It is recommended that research in patients with idiopathic scoliosis should focus on short- and long-term patient-centred outcome. The aim of the present study was to evaluate outcome in patients with late-onset juvenile or adolescent idiopathic scoliosis 16 years or more after Boston brace treatment. Methods 272 (78% of 360 patients, 251 (92% women, responded to follow-up examination at a mean of 24.7 (range 16 - 32 years after Boston brace treatment. Fifty-eight (21% patients had late-onset juvenile and 214 had adolescent idiopathic scoliosis. All patients had clinical and radiological examination and answered a standardised questionnaire including work status, demographics, General Function Score (GFS (100 - worst possible and Oswestry Disability Index (ODI (100 - worst possible, EuroQol (EQ-5D (1 - best possible, EQ-VAS (100 - best possible, and Scoliosis Research Society - 22 (SRS - 22 (5 - best possible. Results The mean age at follow-up was 40.4 (31-48 years. The prebrace major curve was in average 33.2 (20 - 57°. At weaning and at the last follow-up the corresponding values were 28.3 (1 - 58° and 32.5 (7 - 80°, respectively. Curve development was similar in patients with late-onset juvenile and adolescent start. The prebrace curve increased > 5° in 31% and decreased > 5° in 26%. Twenty-five patients had surgery. Those who did not attend follow-up (n = 88 had a lower mean curve at weaning: 25.4 (6-53°. Work status was 76% full-time and 10% part-time. Eighty-seven percent had delivered a baby, 50% had pain in pregnancy. The mean (SD GFS was 7.4 (10.8, ODI 9.3 (11.0, EQ-5D 0.82 (0.2, EQ-VAS 77.6 (17.8, SRS-22: pain 4.1 (0.8, mental health 4.1 (0.6, self-image 3.7 (0.7, function 4.0 (0.6, satisfaction with treatment 3.7 (1.0. Surgical patients had significantly reduced scores for SRS-physical function and self-image, and patients with curves ≥ 45° had reduced self-image. Conclusion Long-term results were

  1. Reabilitação em artrite idiopática juvenil Rehabilitation in juvenile idiopathic arthritis

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    Vanessa Cristina Bueno

    2007-06-01

    adequadamente os diversos tipos de exercícios.INTRODUCTION AND AIMS: juvenile idiopathic arthritis (JIA may cause permanent physical disabilities in children and adolescents. This study aimed to describe the several kinds of rehabilitation procedures, ranging from evaluation to prescription of exercises, as well as the elaboration of a practical rehabilitation guide for JIA patients. SOURCES OF DATA: the research was based on data from Medline and Lilacs. The opinion of experts working on the Pediatric Rheumatology service from Lar Escola São Francisco and Universidade Federal de São Paulo was considered on the debate of several topics. SUMMARY: JIA patients may present pain and limitation of joint movement thereby leading to decrease in physical capacity, affecting both aerobic and anaerobic activities. In addition to the joint compromise, cardiac and autonomic dysfunctions collaborate on this process, impairing sport and everyday activities. The American College of Rheumatology recommends 30-minute activity with moderate intensity, two to three times weekly. Hydrotherapy is associated to treatment adherence, besides helping in decreasing pain perception and adding to cope with daily activities. Other rehabilitation modalities, such as massage, education, joint protection, energy conservation, and splints are also considered in the present review. CONCLUSION: there are few studies in the literature focusing on rehabilitation in children with JIA. Particularly, there is a lack of studies concerning aspects of adequate prescription of exercises, weight-bearing, number of series and repetitions, as well as the best choice regarding ground or water activity. We believe that additional information is needed in order to improve the physical care to these patients.

  2. Evaluation of classification systems for nonspecific idiopathic orbital inflammation

    NARCIS (Netherlands)

    Bijlsma, Ward R.; van 't Hullenaar, Fleur C.; Mourits, Maarten P.; Kalmann, Rachel

    2012-01-01

    To systematically analyze existing classification systems for idiopathic orbital inflammation (IOI) and propose and test a new best practice classification system. A systematic literature search was conducted to find all studies that described and applied a classification system for IOI.

  3. Juvenile idiopathic arthritis patients and their skeletal status: possible role of vitamin D receptor gene polymorphism.

    Science.gov (United States)

    Kostik, M M; Smirnov, A M; Demin, G S; Scheplyagina, L A; Larionova, V I

    2014-01-01

    We evaluated bone mineralization and metabolism changes related to vitamin D receptor (VDR) polymorphic genotypes in children with juvenile idiopathic arthritis. One hundred and ninety eight children (82 boys and 116 girls) were included in our study. Bone mineral density (BMD) was measured by lumbar spine DXA. Osteocalcin, CTX, parathyroid hormone, total and ionized calcium, inorganic phosphate, total alkaline phosphatase activity was utilized for assessment of bone metabolism. Molecular testing: TaqI (rs731236) and Cdx2 (rs11568820) polymorphisms of VDR were detected by RFLP. No differences in TaqI and Cdx2 haplotypes, genotypes and alleles distribution related with normal and low BMD (Zscore 90th percentile) had the highest frequency of allele A-contained genotypes (GA+AA) of Cdx2 VDR (p = 0.009). Girls with TT TaqI VDR, who never been treated by glucocorticoides had lower BMD-Zscore than C allele carriers (TT = -0.94SD [IQR: -2.1;-0.5], TC+CC = -0.62SD [IQR: -1.26;0.39], p = 0.03). Girls with Tanner I with TT had higher total and ionized Ca level than carriers of C allele (Ca: TT = 2.43 ± 0.15 mmol/l, TC+CC = 2.28 ± 0.2 mmol/l, p = 0.024; Ca(2+): TT = 1.15 ± 0.08 mmol/l, TC+CC = 1.06 ± 0.13 mmol/l, p = 0.026). Presence of TT genotype negatively correlated with BMD-Zscore (r = -0.28, p = 0.04), and positively with frequency of LBMD (r = 0.3, p = 0.037). Boy with GG Cdx2 genotype had lower total Ca (GG = 2.3 ± 0.17 mmol/l, GA+AA = 2.43 ± 0.17 mmol/l, p = 0.004) compare with carriers of A allele. Pubertal boys (Tanner IV-V) with GG had higher CTX (GG = 1.75 ± 0.11 ng/ml, GA+AA = 1.06 ± 0.07 ng/ml, p = 0.04. TT genotype of TaqI and GG genotype of Cdx2 VDR is a negative factor impact bone mineralization metabolism and linear growth.

  4. Bone mineral density, T- and Z-scores in young men with juvenile idiopathic arthritis

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    V.V. Povoroznyuk

    2017-10-01

    Full Text Available Background. Juvenile Idiopathic Arthritis (JIA is a term used to classify a group of heterogeneous pediatric rheumatic diseases. Many of these conditions persist through adulthood. Presence of chronic inflammatory disease along with a glucocorticoid treatment is the risk factor of osteoporosis in young adult males. The purpose was to study the bone mineral density (BMD, T- and Z-scores in young adult males with JIA. Materials and methods. The study included 50 patients aged 19–25 years, divided into two groups: I — 25 apparently healthy young males; ІІ — 25 young men from different regions of Ukraine with a history of JIA in childhood, regardless of the presence or absence of active inflammation at the time of the observation. Two-energy X-ray densitometry (Prodigy, GE Lunar, Madison, USA was performed at the D.F. Chebotarev Institute of Ge­rontology, Ukrainian Scientific-Medical Centre for the Problems of Osteoporosis, together with analysis of BMD, T- and Z-scores at different skeletal areas. Results. Young men with JIA and healthy individuals did not differ in age, height, weight and body mass index. Four patients with JIA (16 % had fractures, while in the control group, there were no fractures. Negative impact of the JIA on the BMD was found in group II compared to group I. Lumbar spine BMD in group II was lower (p < 0.01 than in healthy subjects, as well as the Z-score (p < 0.001 in the L1-L4 lumbar spine region. BMD, T- and Z-scores in femoral neck region were lower in group II than in group I (p < 0.001; p < 0.001; p < 0.01, respectively. Significant differences between the two groups were found in total body BMD (p < 0.001, T-score (p < 0.01, Z-score (p < 0.05. Patients with JIA had lower BMD (p < 0.01 and T-score (p < 0.05 of the ultradistal radius. Decrease of BMD up to the level of osteopenia (Z-score < –2 SD was found in 20 % patients at the level of L1-L4 lumbar spine, in 8 % — at femoral neck, in 12 % — at total

  5. Selecting magnetic resonance imaging (MRI) outcome measures for juvenile idiopathic arthritis (JIA) clinical trials: first report of the MRI in JIA special interest group.

    Science.gov (United States)

    Hemke, Robert; Doria, Andrea S; Tzaribachev, Nikolay; Maas, Mario; van der Heijde, Désirée M F M; van Rossum, Marion A J

    2014-02-01

    Recent advances in magnetic resonance imaging (MRI) techniques have substantially improved the evaluation of joint pathologies in juvenile idiopathic arthritis (JIA). Because of the current availability of highly effective antirheumatic therapies and the unique and useful features of MRI, there is a growing need for an accurate and reproducible MRI assessment scoring system for JIA, such as the rheumatoid arthritis MRI Scoring (RAMRIS) for patients with rheumatoid arthritis (RA). To effectively evaluate the efficacy of treatment in clinical research trials, we need to develop and validate scoring methods to accurately measure joint outcomes, standardize imaging protocols for data acquisition and interpretation, and create imaging atlases to differentiate physiologic and pathologic joint findings in childhood and adolescence. Such a standardized, validated, JIA-MRI scoring method could be used as an outcome measure in clinical trials.

  6. Promoting physical activity in children with juvenile idiopathic arthritis through an internet-based program: Results of a pilot randomised controlled trial

    NARCIS (Netherlands)

    Lelieveld, O.; Armbrust, W.; Geertzen, J.; De Graaf, I.; Van Leeuwen, M.; Sauer, P.; Van Weert, E.; Bouma, J.

    2011-01-01

    Purpose: Patients with juvenile idiopathic arthritis (JIA) are less physically active than healthy peers. Therefore we developed an internet-based intervention to improve physical activity (PA). The aim of the study was to examine the effectiveness of the program in improving PA. Relevance: Evidence

  7. Subcutaneous golimumab for children with active polyarticular-course juvenile idiopathic arthritis : results of a multicentre, double-blind, randomised-withdrawal trial

    NARCIS (Netherlands)

    Brunner, Hermine I; Ruperto, Nicolino; Tzaribachev, Nikolay; Horneff, Gerd; Chasnyk, Vyacheslav G.; Panaviene, Violeta Vladislava; Abud-Mendoza, Carlos; Reiff, Andreas; Alexeeva, Ekaterina; Rubio-Pérez, Nadina; Keltsev, Vladimir; Kingsbury, Daniel J.; Del Rocio Maldonado Velázquez, Maria; Nikishina, Irina; Silverman, Earl D.; Joos, Rik; Smolewska, Elzbieta; Bandeira, Márcia; Minden, Kirsten; van Royen-Kerkhof, Annet; Emminger, Wolfgang; Foeldvari, Ivan; Lauwerys, Bernard R.; Sztajnbok, Flavio; Gilmer, Keith E.; Xu, Zhenhua; Leu, Jocelyn H.; Kim, Lilianne; Lamberth, Sarah L.; Loza, Matthew J.; Lovell, Daniel J.; Martini, Alberto

    2018-01-01

    OBJECTIVE: This report aims to determine the safety, pharmacokinetics (PK) and efficacy of subcutaneous golimumab in active polyarticular-course juvenile idiopathic arthritis (polyJIA). METHODS: In this three-part randomised double-blinded placebo-controlled withdrawal trial, all patients received

  8. Short term effectiveness and experiences of a peer guided web-based self-management intervention for young adults with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    J. Ammerlaan (Judy); H. van Os-Medendorp (Harmieke); de Boer-Nijhof, N. (Nienke); Scholtus, L. (Lieske); A.A. Kruize (A.); P.A. van Pelt (Philomine); B.J. Prakken (Berent); J.W.J. Bijlsma (Hans)

    2017-01-01

    textabstractBackground: A web-based self-management intervention guided by peer-trainers was developed to support young adults' self-management in coping with Juvenile Idiopathic Arthritis (JIA). To investigate its effectiveness, a randomized controlled trial (RCT) was conducted. In addition, the

  9. The many shades of enhancement: timing of post-gadolinium images strongly influences the scoring of juvenile idiopathic arthritis wrist involvement on MRI

    NARCIS (Netherlands)

    Rieter, Jasper F. M. M.; de Horatio, Laura Tanturri; Nusman, Charlotte M.; Müller, Lil-Sofie Ording; Hemke, Robert; Avenarius, Derk F. M.; van Rossum, Marion A. J.; Malattia, Clara; Maas, Mario; Rosendahl, Karen

    2016-01-01

    Potential long-term side effects of treatment for juvenile idiopathic arthritis are concerning. This has necessitated accurate tools, such as MRI, to monitor treatment response and allow for personalized therapy. To examine the extent to which timing of post-contrast MR images influences the scoring

  10. Ultrasonography and color Doppler in juvenile idiopathic arthritis: diagnosis and follow-up of ultrasound-guided steroid injection in the ankle region. A descriptive interventional study

    DEFF Research Database (Denmark)

    Laurell, Louise; Court-Payen, Michel; Nielsen, Susan

    2011-01-01

    The ankle region is frequently involved in juvenile idiopathic arthritis (JIA) but difficult to examine clinically due to its anatomical complexity. The aim of the study was to evaluate the role of ultrasonography (US) of the ankle and midfoot (ankle region) in JIA. Doppler-US detected synovial h...

  11. Promoting physical activity in children with juvenile idiopathic arthritis through an internet-based program : results of a pilot randomized controlled trial

    NARCIS (Netherlands)

    Lelieveld, Otto; Armbrust, Wineke; Geertzen, Jan H. B.; de Graaf, Inez; van Leeuwen, Miek A.; Sauer, Pieter J. J.; van Weert, Ellen; Bouma, Jelte

    OBJECTIVE: Patients with juvenile idiopathic arthritis (JIA) are less physically active than healthy peers. Therefore, we developed an Internet-based intervention to improve physical activity (PA). The aim of this study was to examine the effectiveness of the program in improving PA. METHODS: PA was

  12. Feasibility of a Website and a Hospital-Based Online Portal for Young Adults With Juvenile Idiopathic Arthritis : Views and Experiences of Patients

    NARCIS (Netherlands)

    Ammerlaan, Judy Jw; Scholtus, Lieske W; Drossaert, Constance Hc; van Os-Medendorp, Harmieke; Prakken, Berent; Kruize, Aike A; Bijlsma, Johannes JW

    2015-01-01

    BACKGROUND: To improve knowledge and to encourage active involvement of young adults with juvenile idiopathic arthritis (JIA), an informative website with written and video information and an online portal with access to the personal medical record, self-monitoring, and e-consult functionalities

  13. Feasibility of a Website and a Hospital-Based Online Portal for Young Adults With Juvenile Idiopathic Arthritis: Views and Experiences of Patients

    NARCIS (Netherlands)

    Ammerlaan, Judy J.W.; Scholtus, Lieske W.; Drossaert, Constance H.C.; van Os-Medendorp, Harmieke; Prakken, Berent; Kruize, Aike A.; Bijlsma, Johannes J.W.

    2015-01-01

    Background: To improve knowledge and to encourage active involvement of young adults with juvenile idiopathic arthritis (JIA), an informative website with written and video information and an online portal with access to the personal medical record, self-monitoring, and e-consult functionalities

  14. Disk abnormality coexists with any degree of synovial and osseous abnormality in the temporomandibular joints of children with juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Kirkhus, Eva; Smith, Hans-Joergen [Oslo University Hospital, Rikshospitalet, Department of Radiology and Nuclear Medicine, Oslo (Norway); University of Oslo, Institute of Clinical Medicine, Oslo (Norway); Arvidsson, Linda Z.; Larheim, Tore A. [University of Oslo, Department of Maxillofacial Radiology, Institute of Clinical Dentistry, Oslo (Norway); Flatoe, Berit; Hetlevik, Siri O. [Oslo University Hospital, Rikshospitalet, Department of Rheumatology, Oslo (Norway); University of Oslo, Institute of Clinical Medicine, Oslo (Norway)

    2016-03-15

    MRI manifestation of temporomandibular joint arthritis is frequently reported in children with juvenile idiopathic arthritis. However, little attention has been paid to temporomandibular joint disk abnormalities. To assess combinations of MRI findings in the symptomatic temporomandibular joint in children with juvenile idiopathic arthritis with focus on disk abnormalities. This was a retrospective study of 46 patients with juvenile idiopathic arthritis, mean age 12 years (range: 5-17 years). Mean disease duration was 70 months (standard deviation: 61 months). MR images of 92 temporomandibular joints were scored for thickness of abnormally enhancing synovium (synovitis), joint effusion, bone marrow oedema, abnormal bone shape, bone erosion and disk abnormalities. The 92 temporomandibular joints were categorized as A: No synovitis and normal bone shape (30/92; 33%), B: Synovitis and normal bone shape (14/92: 15%), C: Synovitis and abnormal bone shape (38/92; 41%) and D: No synovitis but abnormal bone shape (10/92; 11%). Thirty-six of the 46 patients (78%) had synovitis and 33/46 (72%) had abnormal bone shape, most frequently in combination (30/46; 65%). Disk abnormalities (flat disk, fragmented disk, adherent disk and displaced disk) were found in 29/46 patients (63%). Disk abnormalities were found in all categories of juvenile idiopathic arthritis involved temporomandibular joints (B: 8/14 [57%]; C: 25/38 [66%] and D: 7/10 [70%]). Disk displacement was found in half of the joints (7/14) in category B. Synovitis was most pronounced in this category. Disk abnormalities were frequent. Disk displacement also occurred in joints with early temporomandibular joint arthritis, i.e., with normal bone shape. Other disk abnormalities were found in joints with bone abnormalities. Attention should be paid to disk abnormalities both in early and long-standing temporomandibular joint arthritis in children with juvenile idiopathic arthritis. (orig.)

  15. Studies on physical performance and functional ability in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Takken, T.

    2003-01-01

    There is a growing interest in the physical training possibilities of children with juvenile arthritis. In the first Chapter a brief introduction on physical fitness and physical training is given including an overview of the existing studies in juvenile arthritis patients. In Chapter 2, we

  16. Consensus in pediatric rheumatology: part I - criteria definition of inactive disease and remission in juvenile idiopathic arthritis / juvenile rheumatoid arthritis

    OpenAIRE

    Machado, Claudia [UNESP; Ruperto, Nicolino

    2005-01-01

    Não há critérios universalmente aceitos para a remissão clínica em artrite idiopática juvenil/artrite reumatóide juvenil (AIJ/ARJ). OBJETIVO: formar consenso sobre estes critérios. MÉTODOS: foi utilizado um inquérito pelo método Delphi para reunir os critérios vigentes e utilizados por especialistas em reumatologia pediátrica (RP) no mundo todo. A análise dos resultados constituiu a base para uma Consensus Conference utilizando a nominal group technique (NGT) para alcançar o consenso nas ques...

  17. Systemic Mastocytosis Associated with Chronic Idiopathic Myelofibrosis

    Science.gov (United States)

    Sotlar, Karl; Bache, Anja; Stellmacher, Florian; Bültmann, Burkhard; Valent, Peter; Horny, Hans-Peter

    2008-01-01

    In ∼20 to 30% of patients with systemic mastocytosis (SM), an associated clonal hematological nonmast cell lineage disorder (AHNMD) is diagnosed. Although SM may be considered to be closely related to the myeloproliferative disorders (MPDs), it is unknown whether JAK2V617F+ MPD may occur as AHNMD in patients with SM. We here describe five patients with SM and co-existing chronic idiopathic myelofibrosis (SM-CIMF). In five of five patients, we detected the SM-related KIT mutation D816V, and in four of five patients, the MPD-related JAK2 mutation V617F. Surprisingly, JAK2V617F was found not only in the AHMMD component of the disease but also in microdissected mast cells in all four JAK2V617F-positive cases. Conversely, in two of the five patients, KITD816V was found not only in neoplastic mast cells but also in microdissected CD15+ neoplastic myeloid cells. Control experiments showed that 10 indolent SM patients without associated MPD did not carry the JAK2 mutation V617F and that 15 CIMF patients without SM did not carry the KIT mutation D816V. Altogether, these data suggest that KITD816V+ SM can co-exist with JAK2V617F+ CIMF and that, in some of these SM-CIMF cases, the two mutations are present in the neoplastic cells of both disease components. PMID:18165278

  18. Motor system hyperconnectivity in juvenile myoclonic epilepsy: a cognitive functional magnetic resonance imaging study.

    Science.gov (United States)

    Vollmar, Christian; O'Muircheartaigh, Jonathan; Barker, Gareth J; Symms, Mark R; Thompson, Pamela; Kumari, Veena; Duncan, John S; Janz, Dieter; Richardson, Mark P; Koepp, Matthias J

    2011-06-01

    Juvenile myoclonic epilepsy is the most frequent idiopathic generalized epilepsy syndrome. It is characterized by predominant myoclonic jerks of upper limbs, often provoked by cognitive activities, and typically responsive to treatment with sodium valproate. Neurophysiological, neuropsychological and imaging studies in juvenile myoclonic epilepsy have consistently pointed towards subtle abnormalities in the medial frontal lobes. Using functional magnetic resonance imaging with an executive frontal lobe paradigm, we investigated cortical activation patterns and interaction between cortical regions in 30 patients with juvenile myoclonic epilepsy and 26 healthy controls. With increasing cognitive demand, patients showed increasing coactivation of the primary motor cortex and supplementary motor area. This effect was stronger in patients still suffering from seizures, and was not seen in healthy controls. Patients with juvenile myoclonic epilepsy showed increased functional connectivity between the motor system and frontoparietal cognitive networks. Furthermore, we found impaired deactivation of the default mode network during cognitive tasks with persistent activation in medial frontal and central regions in patients. Coactivation in the motor cortex and supplementary motor area with increasing cognitive load and increased functional coupling between the motor system and cognitive networks provide an explanation how cognitive effort can cause myoclonic jerks in juvenile myoclonic epilepsy. The supplementary motor area represents the anatomical link between these two functional systems, and our findings may be the functional correlate of previously described structural abnormalities in the medial frontal lobe in juvenile myoclonic epilepsy.

  19. Idiopathic Juvenile Osteoporosis: Clinical Experience from a Single Centre and Screening of LRP5 and LRP6 Genes.

    Science.gov (United States)

    Franceschi, Roberto; Vincenzi, Monica; Camilot, Marta; Antoniazzi, Franco; Freemont, Anthony J; Adams, Judith E; Laine, Christine; Makitie, Outi; Mughal, M Zulf

    2015-06-01

    We report clinical findings, bone mineral density (BMD) and bone biopsy data in ten children with features of classic idiopathic juvenile osteoporosis (IJO). We also screened the patients for mutations in LRP5 and LRP6. We found low BMD in the lumbar spine, the hip and distal radius. In the spine and distal radius, the reduction in BMD was more marked in the trabecular compartment. Biopsy confirmed that the trabecular compartment is more severely involved with reduction in bone formation and increase in bone resorption. No mutations in LRP5 and LRP6 could be identified. IJO is likely to be a heterogeneous bone disorder, and next-generation genomic sequencing studies may help reveal causative genes.

  20. The Effects of Orthopedic Manual Physical Therapy in the Management of Juvenile Idiopathic Arthritis: A Case Report.

    Science.gov (United States)

    Lantz, Justin M; Joshi, Shruti; OʼHearn, Michael

    Juvenile idiopathic arthritis (JIA) is a cause of disability in childhood. Little research exists concerning physical therapy management, and no evidence exists for orthopedic manual physical therapy (OMPT) for JIA. The purpose of this case report is to describe the use of OMPT in combination with therapeutic exercise in the successful treatment of a child with oligoarticular JIA. A 6-year-old girl with oligoarticular JIA presented with elbow pain and stiffness interfering with function. Treatment consisted of OMPT in combination with therapeutic exercise and a home exercise program. Improvements were demonstrated in elbow range of motion, pain, Childhood Health Assessment Questionnaire, Pediatric Outcomes Data Collection Instrument, and the Patient Specific Functional Scale. Orthopedic manual physical therapy may be considered as a treatment of a child with JIA. We provide evidence that OMPT may be considered as a treatment of a child with JIA.

  1. Reduced articular cartilage thickness in joints without a history of active arthritis in children with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Pradsgaard, Dan Østergaard; Spannow, Anne Helene; Heuck, Carsten

    Background: The functional disability experienced in juvenile idiopathic arthritis is primarily caused by degeneration of the osteocartilaginous structures due to the inflammatory process in the synovium. It is therefore essential for evaluating the therapeutic efficacy to closely monitor...... in joint cartilage thickness (Cth) between healthy children and JIA children measured by US (1). But are there any differences in Cth measured by US between healthy children and joints without a history of activity among JIA children’s. Aim: To investigate a possible effect of the inflammatory process...... on joints never directly affected by arthritic activity during the history of the child’s disease course. Furthermore we wanted to compare joint cartilage thickness within the JIA group in joints with or without a history of activity. Methods: We included 95 Danish JIA children. Age, mean (range) 10...

  2. Relationship of pain coping strategies and pain specific beliefs to pain experience in children with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Thastum, Mikael; Herlin, Troels; Zachariae, R.

    2005-01-01

    . The parents completed the Childhood Health Assessment Questionnaire (CHAQ). Second order principal component analyses were conducted in order to reduce the number of independent variables. Regression analyses of the dependent measure were performed. The use of coping strategies and health beliefs were...... contribution to the prediction of pain after controlling for other variables. Significant differences were found between the scores of high pain patients and the rest of the group for the health belief subscale of disability (mean +/- SD 2.0 +/- 0.6 and 1.2 +/- 0.7, respectively), and for the health belief......OBJECTIVE: To examine whether pain-specific beliefs and coping strategies of patients with juvenile idiopathic arthritis (JIA) independently predict their reported pain, while controlling for relevant demographic variables, disease activity, and parent-rated disability. To compare use of pain...

  3. Contrast-enhanced MRI of the knee in children unaffected by clinical arthritis compared to clinically active juvenile idiopathic arthritis patients

    Energy Technology Data Exchange (ETDEWEB)

    Nusman, Charlotte M.; Hemke, Robert [University of Amsterdam, Department of Radiology, Academic Medical Center, Amsterdam (Netherlands); University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Benninga, Marc A.; Kindermann, Angelika [University of Amsterdam, Department of Pediatric Gastroenterology, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Schonenberg-Meinema, Dieneke; Berg, J.M. van den; Kuijpers, Taco W. [University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Rossum, Marion A.J. van [University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Reade, Department of Pediatric Rheumatology, Amsterdam (Netherlands); Maas, Mario [University of Amsterdam, Department of Radiology, Academic Medical Center, Amsterdam (Netherlands)

    2016-04-15

    To evaluate enhancing synovial thickness upon contrast-enhanced magnetic resonance imaging (MRI) of the knee in children unaffected by clinical arthritis compared with clinically active juvenile idiopathic arthritis (JIA) patients. A secondary objective was optimization of the scoring method based on maximizing differences on MRI between these groups. Twenty-five children without history of joint complaints nor any clinical signs of joint inflammation were age/sex-matched with 25 clinically active JIA patients with arthritis of at least one knee. Two trained radiologists, blinded for clinical status, independently evaluated location and extent of enhancing synovial thickness with the validated Juvenile Arthritis MRI Scoring system (JAMRIS) on contrast-enhanced axial fat-saturated T1-weighted MRI of the knee. Enhancing synovium (≥2 mm) was present in 13 (52 %) unaffected children. Using the total JAMRIS score for synovial thickening, no significant difference was found between unaffected children and active JIA patients (p = 0.091). Additional weighting of synovial thickening at the JIA-specific locations enabled more sensitive discrimination (p = 0.011). Mild synovial thickening is commonly present in the knee of children unaffected by clinical arthritis. The infrapatellar and cruciate ligament synovial involvement were specific for JIA, which - in a revised JAMRIS - increases the ability to discriminate between JIA and unaffected children. (orig.)

  4. Autoimmune hepatitis and juvenile systemic lupus erythematosus

    NARCIS (Netherlands)

    Deen, M. E. J.; Porta, G.; Fiorot, F. J.; Campos, L. M. A.; Sallum, A. M. E.; Silva, C. A. A.

    Juvenile systemic lupus erythematosus (JSLE) and autoimmune hepatitis (AIH) are both autoimmune disorders that are rare in children and have a widespread clinical manifestation. A few case reports have shown a JSLE-AIH associated disorder. To our knowledge, this is the first study that

  5. PReS-FINAL-2148: Rheumates@work a cognitive behavioural internet based intervention promoting physical activity in children with juvenile idiopathic arthritis: Preliminary results of a randomized clinical trail

    NARCIS (Netherlands)

    Bos, J; Armbrust, W.; Geertzen, J.; Sauer, P.; Dijkstra, P.; Van Brussel, M.; Cappon, J.; Lelieveld, O.

    2013-01-01

    Introduction: Juvenile Idiopathic Arthritis (JIA) is a chronic disease in which periods of active inflammation alternate with periods of inactive disease in an unpredictable way. Although impairments are most pronounced in children with disease activity, deficits like fatigue, decreased physical

  6. Effects of the use of growth hormone in children and adolescents with juvenile idiopathic arthritis: a systematic review.

    Science.gov (United States)

    Frittoli, Renan Bazuco; Longhi, Barbara Sugui; Silva, Amanda Meireles; Filho, Antônio de Azevedo Barros; Monteiro, Maria Ângela Reis de Góes; Appenzeller, Simone

    Children with juvenile idiopathic arthritis (JIA) often have impaired growth and short stature. There is evidence that the therapeutic use of growth hormone (GH) is useful and safe in these patients. To analyze the effects of GH use in patients with JIA. A systematic review of the literature over the last 18 years in Medline and Embase databases. The criteria were analyzed independently by the researchers. We used the following keywords: "growth hormone", "arthritis, juvenile", "arthritis, rheumatoid", "child" and "adolescent". Among the 192 identified articles, 20 corresponded to the inclusion criteria. Seventeen longitudinal studies and 3 case reports were found. Most studies analyzed observed increased growth, muscle mass and bone mass using GH. Adverse effects observed were glucose intolerance, diabetes, bone deformities, osteonecrosis, reactivation of the disease and low final height. The majority of studies reported positive effects after the therapeutic use of GH, but some variability in response to treatment was observed. The combination of growth hormone with other drugs seems to be a good option. Copyright © 2016. Published by Elsevier Editora Ltda.

  7. Clinical responsiveness of self-report functional assessment measures for children with juvenile idiopathic arthritis undergoing intraarticular corticosteroid injections.

    Science.gov (United States)

    Brown, G Ted; Wright, F Virginia; Lang, Bianca A; Birdi, Nina; Oen, Kim; Stephens, Derek; McComas, Joan; Feldman, Brian M

    2005-12-15

    The Childhood Health Assessment Questionnaire (CHAQ), Juvenile Arthritis Functional Assessment Report (JAFAR), and Juvenile Arthritis Functional Status Index (JASI) are widely used functional measures for juvenile idiopathic arthritis (JIA) that differ in content, format, and completion time. We compared the responsiveness and child-parent agreement of the JAFAR, CHAQ, and JASI in a prospective, multicenter study. Children and adolescents from 5 rheumatology centers were enrolled. Subjects were about to undergo therapy (intraarticular corticosteroid injections [IAS] and methotrexate or hip surgery (MTX/hip]) expected to produce a functional improvement. All subjects were studied before the intervention and at 6 weeks and 6 months posttreatment. At each study visit, the 3 measures were administered in randomized, balanced order to both parents and children. A total of 92 subjects (mean age 12.8 years) were enrolled in the study, 74 of which were in the IAS group. The responsiveness of all 3 measures was moderate to strong. The standardized response mean at 6 weeks for the IAS group on the JAFAR, CHAQ, and JASI was 0.41 (95% confidence interval [95% CI] 0.18, 0.64), 0.70 (95% CI 0.47, 0.93), and 0.36 (95% CI 0.13, 0.59), respectively. The CHAQ was somewhat more responsive to change at 6 weeks (IAS group: relative efficiency 0.34 [JAFAR], 0.27 [JASI]), but less responsive at 6 months (MTX/hip group: relative efficiency 5.1 [JAFAR], 3.9 [JASI]). All 3 questionnaires showed acceptable parent-child agreement, and overall, there were few differences between the 3 questionnaires. The functional outcome measures currently used for JIA are all adequately responsive for use in trials or in the clinic setting. The choice of which measure to use should therefore be based on the time available for completion, the intended clinical/research use, and the depth of content required.

  8. Protocol for the Foot in Juvenile Idiopathic Arthritis trial (FiJIA: a randomised controlled trial of an integrated foot care programme for foot problems in JIA

    Directory of Open Access Journals (Sweden)

    Hendry Gordon J

    2009-06-01

    Full Text Available Abstract Background Foot and ankle problems are a common but relatively neglected manifestation of juvenile idiopathic arthritis. Studies of medical and non-medical interventions have shown that clinical outcome measures can be improved. However existing data has been drawn from small non-randomised clinical studies of single interventions that appear to under-represent the adult population suffering from juvenile idiopathic arthritis. To date, no evidence of combined therapies or integrated care for juvenile idiopathic arthritis patients with foot and ankle problems exists. Methods/design An exploratory phase II non-pharmacological randomised controlled trial where patients including young children, adolescents and adults with juvenile idiopathic arthritis and associated foot/ankle problems will be randomised to receive integrated podiatric care via a new foot care programme, or to receive standard podiatry care. Sixty patients (30 in each arm including children, adolescents and adults diagnosed with juvenile idiopathic arthritis who satisfy the inclusion and exclusion criteria will be recruited from 2 outpatient centres of paediatric and adult rheumatology respectively. Participants will be randomised by process of minimisation using the Minim software package. The primary outcome measure is the foot related impairment measured by the Juvenile Arthritis Disability Index questionnaire's impairment domain at 6 and 12 months, with secondary outcomes including disease activity score, foot deformity score, active/limited foot joint counts, spatio-temporal and plantar-pressure gait parameters, health related quality of life and semi-quantitative ultrasonography score for inflammatory foot lesions. The new foot care programme will comprise rapid assessment and investigation, targeted treatment, with detailed outcome assessment and follow-up at minimum intervals of 3 months. Data will be collected at baseline, 6 months and 12 months from baseline

  9. Idiopathic systemic AA-amyloidosis in a skunk (Mephitis mephitis).

    Science.gov (United States)

    Elhensheri, Mohamed; Linke, Reinhold P; Blankenburg, Anja; Beineke, Andreas

    2012-03-01

    This report describes a case of systemic amyloidosis in a captive striped skunk. At necropsy, bilateral alopecia, as well as reno-, hepato-, and splenomegaly were present. Congo red staining and immunohistochemistry revealed depositions of AA-amyloid in different organs. The lack of a predisposing disease is suggestive of idiopathic systemic AA-amyloidosis.

  10. How common is clinically inactive disease in a prospective cohort of patients with juvenile idiopathic arthritis? The importance of definition.

    Science.gov (United States)

    Shoop-Worrall, Stephanie J W; Verstappen, Suzanne M M; Baildam, Eileen; Chieng, Alice; Davidson, Joyce; Foster, Helen; Ioannou, Yiannis; McErlane, Flora; Wedderburn, Lucy R; Thomson, Wendy; Hyrich, Kimme L

    2017-08-01

    Many criteria for clinically inactive disease (CID) and minimal disease activity (MDA) have been proposed for juvenile idiopathic arthritis (JIA). It is not known to what degree each of these criteria overlap within a single patient cohort. This study aimed to compare the frequency of MDA and CID across different criteria in a cohort of children with JIA at 1 year following presentation. The Childhood Arthritis Prospective Study recruits children at initial presentation to paediatric or adolescent rheumatology in seven UK centres. Children recruited between October 2001 and December 2013 were included. The proportions of children with CID and MDA at 1 year were calculated using four investigator-defined and eight published composite criteria. Missing data were accounted for using multiple imputation under different assumptions. In a cohort of 1415 children and adolescents, 67% patients had no active joints at 1 year. Between 48% and 61% achieved MDA and between 25% and 38% achieved CID using published criteria. Overlap between criteria varied. Of 922 patients in MDA by either the original composite criteria, Juvenile Arthritis Disease Activity Score (JADAS) or clinical JADAS cut-offs, 68% were classified as in MDA by all 3 criteria. Similarly, 44% of 633 children with CID defined by either Wallace's preliminary criteria or the JADAS cut-off were in CID according to both criteria. In a large JIA prospective inception cohort, a majority of patients have evidence of persistent disease activity after 1 year. Published criteria to capture MDA and CID do not always identify the same groups of patients. This has significant implications when defining and applying treat-to-target strategies. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  11. Síndrome de ativação macrofágica em pacientes com artrite idiopática juvenil Macrophage activation syndrome in patients with juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Rogério do Prado

    2004-10-01

    Full Text Available A síndrome de ativação macrofágica (SAM é uma complicação rara das doenças reumáticas crônicas, particularmente a artrite idiopática juvenil (AIJ de início sistêmico. Este processo pode ser desencadeado por agentes infecciosos virais e bacterianos, neoplásicos, drogas antiinflamatórias não esteroidais ou drogas modificadoras da doença, mudanças abruptas das medicações e doenças reumáticas. O quadro clínico inicia-se com irritação do sistema nervoso central, acompanhado de falências hepática e renal, além de pancitopenia. Relatamos três casos de pacientes com AIJ do nosso serviço que desenvolveram SAM com descrição das características clínicas, evolutivas e de tratamento.The macrophage activation syndrome (MAS is an uncommon complication of chronic rheumatic diseases, specially systemic onset juvenile idiopathic arthritis (JIA. It can be triggered by infectious (viral or bacterial or malignant diseases, non-steroidal anti-inflammatory or disease modified anti-rheumatic drugs, changes in the therapy and rheumatic diseases. The clinical features present at the onset are related mainly with central nervous system involvement, hepatic and renal failure and pancytopenia. We describe the clinical, evolutive features and treatment of three patients with JIA that developed MAS.

  12. Diffusion-weighted imaging for assessment of synovial inflammation in juvenile idiopathic arthritis. A promising imaging biomarker as an alternative to gadolinium-based contrast agents

    Energy Technology Data Exchange (ETDEWEB)

    Barendregt, Anouk M.; Gulik, E.C. van [Academic Medical Center/University of Amsterdam, Department of Radiology, Amsterdam (Netherlands); Academic Medical Center/University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital, Amsterdam (Netherlands); Lavini, Cristina; Nusman, Charlotte M.; Hemke, Robert; Maas, Mario [Academic Medical Center/University of Amsterdam, Department of Radiology, Amsterdam (Netherlands); Berg, J.M. van den; Schonenberg-Meinema, Dieneke; Kuijpers, Taco W. [Academic Medical Center/University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital, Amsterdam (Netherlands); Dolman, Koert M. [Reade, Department of Pediatric Rheumatology, Amsterdam (Netherlands); Onze Lieve Vrouwe Gasthuis West, Department of Pediatric Rheumatology, Amsterdam (Netherlands); Onze Lieve Vrouwe Gasthuis Oost, Department of Pediatric Rheumatology, Amsterdam (Netherlands)

    2017-11-15

    To compare dynamic-contrast-enhanced MRI (DCE) and diffusion-weighted imaging (DWI) in quantifying synovial inflammation in juvenile idiopathic arthritis (JIA). Regions of interest (ROI) were drawn in the synovium of JIA patients on T1 DCE and T2 DWI, followed by extraction of the maximum enhancement (ME), maximum initial slope (MIS), time to peak (TTP), % of different time intensity curve shapes (TIC) and apparent diffusion coefficient (ADC) of the ROIs. Mann-Whitney-U test was used for comparing parameters between MRI-active and -inactive patients (defined by the juvenile arthritis MRI scoring system). Spearman's rank was used to analyse the correlation between DCE and DWI. Thirty-five JIA patients (18 MRI active and 17 MRI inactive) were included. Median age was 13.1 years and 71% were female. ME, MIS, TTP, % TIC 5 and ADC were significantly different in MRI-active versus MRI-inactive JIA with median ADC 1.49 x 10{sup -3} mm{sup 2}/s in MRI-active and 1.25 x 10{sup -3} mm{sup 2}/s in MRI-inactive JIA, p = 0.001, 95% confidence interval of difference in medians =0.11-0.53 x 10{sup -3} mm{sup 2}/s. ADC correlated to ME, MIS and TIC 5 shapes (r = 0.62, r = 0.45, r = -0.51, respectively, all p < 0.05). Similar to DCE parameters, DWI-derived ADC is significantly different in MRI-active JIA as compared to MRI-inactive JIA. The non-invasiveness of DWI combined with its possibility to detect synovial inflammation shows the potential of DWI. (orig.)

  13. Diffusion-weighted imaging for assessment of synovial inflammation in juvenile idiopathic arthritis. A promising imaging biomarker as an alternative to gadolinium-based contrast agents

    International Nuclear Information System (INIS)

    Barendregt, Anouk M.; Gulik, E.C. van; Lavini, Cristina; Nusman, Charlotte M.; Hemke, Robert; Maas, Mario; Berg, J.M. van den; Schonenberg-Meinema, Dieneke; Kuijpers, Taco W.; Dolman, Koert M.

    2017-01-01

    To compare dynamic-contrast-enhanced MRI (DCE) and diffusion-weighted imaging (DWI) in quantifying synovial inflammation in juvenile idiopathic arthritis (JIA). Regions of interest (ROI) were drawn in the synovium of JIA patients on T1 DCE and T2 DWI, followed by extraction of the maximum enhancement (ME), maximum initial slope (MIS), time to peak (TTP), % of different time intensity curve shapes (TIC) and apparent diffusion coefficient (ADC) of the ROIs. Mann-Whitney-U test was used for comparing parameters between MRI-active and -inactive patients (defined by the juvenile arthritis MRI scoring system). Spearman's rank was used to analyse the correlation between DCE and DWI. Thirty-five JIA patients (18 MRI active and 17 MRI inactive) were included. Median age was 13.1 years and 71% were female. ME, MIS, TTP, % TIC 5 and ADC were significantly different in MRI-active versus MRI-inactive JIA with median ADC 1.49 x 10 -3 mm 2 /s in MRI-active and 1.25 x 10 -3 mm 2 /s in MRI-inactive JIA, p = 0.001, 95% confidence interval of difference in medians =0.11-0.53 x 10 -3 mm 2 /s. ADC correlated to ME, MIS and TIC 5 shapes (r = 0.62, r = 0.45, r = -0.51, respectively, all p < 0.05). Similar to DCE parameters, DWI-derived ADC is significantly different in MRI-active JIA as compared to MRI-inactive JIA. The non-invasiveness of DWI combined with its possibility to detect synovial inflammation shows the potential of DWI. (orig.)

  14. Effects of Juvenile Idiopathic Arthritis on Kinematics and Kinetics of the Lower Extremities Call for Consequences in Physical Activities Recommendations

    Directory of Open Access Journals (Sweden)

    M. Hartmann

    2010-01-01

    Full Text Available Juvenile idiopathic arthritis (JIA patients (n=36 with symmetrical polyarticular joint involvement of the lower extremities and healthy controls (n=20 were compared concerning differences in kinematic, kinetic, and spatio-temporal parameters with 3D gait analysis. The aims of this study were to quantify the differences in gait between JIA patients and healthy controls and to provide data for more detailed sport activities recommendations. JIA-patients showed reduced walking speed and step length, strongly anterior tilted pelvis, reduced maximum hip extension, reduced knee extension during single support phase and reduced plantar flexion in push off. Additionally the roll-off procedure of the foot was slightly decelerated. The reduced push off motion in the ankle was confirmed by lower peaks in ankle moment and power. The gait of JIA-patients can be explained as a crouch-like gait with hyperflexion in hip and knee joints and less plantar flexion in the ankle. A preventive mobility workout would be recommendable to reduce these restrictions in the future. Advisable are sports with emphasis on extension in hip, knee, and ankle plantar flexion.

  15. A recurring rollercoaster ride: a qualitative study of the emotional experiences of parents of children with juvenile idiopathic arthritis.

    Science.gov (United States)

    Gómez-Ramírez, Oralia; Gibbon, Michele; Berard, Roberta; Jurencak, Roman; Green, Jayne; Tucker, Lori; Shiff, Natalie; Guzman, Jaime

    2016-03-09

    Despite the wealth of clinical research carried out in children with juvenile idiopathic arthritis (JIA), little is known about the emotional experiences of their parents. This article describes the predominant emotional experiences reported by parents of children with JIA in two Canadian cities. Research participants included 15 experienced parents and 8 novice parents (team following a four-step qualitative analytical process. Parents of children with JIA experienced recurrent mixed negative and positive emotions that varied over time. Between disease onset and diagnosis, mounting anxiety, fear and confusion were the predominant emotions. Shortly after diagnosis there were shock, disbelief, and fear, with a sense of having being blindsided by the disease. At times of disease quiescence there was hope and gratitude, but also fatigue and frustration with ongoing treatment and fear of flares. During periods of increasing or ongoing symptoms there was admiration and sympathy for the courageous way children coped with JIA, as well as sorrow and frustration for ongoing pain and limitations. There were at times, frustration and indignation with peers and teachers unable to understand the child's fluctuations in physical activity and schoolwork. Throughout the disease, parents felt an underlying anxiety and powerlessness. Parents of children with JIA described complex emotional journeys akin to the recurring ups and downs of rollercoaster rides, instead of ordered emotional phases ending in resolution. This has implications for healthcare providers who need to be aware of the complexity of these emotional journeys to support parents more effectively, thereby helping improve patient outcomes.

  16. Multidisciplinary approach in the management of absolute trismus with bilateral temporomandibular joint replacements for a patient with juvenile idiopathic arthritis.

    Science.gov (United States)

    Fanaras, Nikolaos; Parry, Nicholas S; Matthews, N Shaun

    2014-11-01

    Juvenile idiopathic arthritis (JIA) is an exclusion diagnosis that gathers together all forms of arthritis that begin before the age of 16 years, persist for more than 6 weeks and are of unknown origin. We present the case of a 42 year old woman with a 20 year history of absolute trismus, secondary to bilateral temporomandibular joint (TMJ) ankylosis caused by JIA. The trismus resulted in grossly compromised oral hygiene and limited the patient to a semi-solid diet. JIA also affected her neck leading to a severe cervico-thoracic kyphosis. The patient who had been wheelchair bound developed severe lymphoedema of both lower limbs, complicating the pre-operative work up further. This particularly challenging case required input from specialists in anaesthetics, neurosurgery, special care dentistry, intensive care and maxillofacial surgery. Treatment consisted of ankylosis release, dental clearance and bilateral alloplastic replacement of her TMJs with custom implants. A full range of hinge movement and good functional outcome was achieved. This case presents the multidisciplinary approach to a severely compromised patient and illustrates the pre-, intra- and postoperative management of bilateral TMJ ankylosis with bespoke implants. Copyright © 2014 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

  17. Genetic Predictors of Poor Prognosis in Portuguese Patients with Juvenile Idiopathic Arthritis: Data from Reuma.pt

    Science.gov (United States)

    Mourão, Ana Filipa; Santos, Maria José; Mendonça, Sílvia; Oliveira-Ramos, Filipa; Salgado, Manuel; Estanqueiro, Paula; Melo-Gomes, José; Martins, Fernando; Lopes, Ana; Bettencourt, Bruno Filipe; Bruges-Armas, Jácome; Costa, José; Furtado, Carolina; Figueira, Ricardo; Brito, Iva; Branco, Jaime; Fonseca, João Eurico; Canhão, Helena

    2015-01-01

    Introduction. This study aimed to assess the genetic determinants of poor outcome in Portuguese patients with juvenile idiopathic arthritis (JIA). Methods. Our study was conducted in Reuma.pt, the Rheumatic Diseases Portuguese Register, which includes patients with JIA. We collected prospectively patient and disease characteristics and a blood sample for DNA analysis. Poor prognosis was defined as CHAQ/HAQ >0.75 at the last visit and/or the treatment with biological therapy. A selected panel of single nucleotide polymorphisms (SNPs) associated with susceptibility was studied to verify if there was association with poor prognosis. Results. Of the 812 patients with JIA registered in Reuma.pt, 267 had a blood sample and registered information used to define “poor prognosis.” In univariate analysis, we found significant associations with poor prognosis for allele A of TNFA1P3/20 rs6920220, allele G of TRAF1/C5 rs3761847, and allele G of PTPN2 rs7234029. In multivariate models, the associations with TRAF1/C5 (1.96 [1.17–3.3]) remained significant at the 5% level, while TNFA1P3/20 and PTPN2 were no longer significant. Nevertheless, none of associations found was significant after the Bonferroni correction was applied. Conclusion. Our study does not confirm the association between a panel of selected SNP and poor prognosis in Portuguese patients with JIA. PMID:26504858

  18. What are the immunological consequences of long-term use of biological therapies for juvenile idiopathic arthritis?

    Science.gov (United States)

    Swart, Joost F; de Roock, Sytze; Wulffraat, Nico M

    2013-01-01

    This review summarizes the immunological consequences of biological therapies used in juvenile idiopathic arthritis (JIA). For every frequently used biological agent the characteristics are clearly specified (molecular target, isotype, registered indication for JIA, route of administration, half-life, contraindication, very common side effects, expected time of response and average cost in the first year). The emphasis of this review is on the immunological side effects that have been encountered for every separate agent in JIA populations. For each agent these adverse events have been calculated as incidence per 100 patient-years for the following categories: serious infections, tuberculosis, malignancies, response to vaccination, new-onset autoimmune diseases and development of anti-drug antibodies. There are large differences in side effects between various agents and there is a clear need for an international and standardized collection of post-marketing surveillance data of biologicals in the vulnerable group of JIA patients. Such an international pharmacovigilance database, called Pharmachild, has now been started.

  19. HLA-B27 predicts a more extended disease with increasing age at onset in boys with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Berntson, Lillemor; Damgård, Michael; Andersson-Gäre, Boel

    2008-01-01

    OBJECTIVE: Juvenile idiopathic arthritis (JIA) is a heterogeneous condition with very few clinical and laboratory signs that can help predict the course and severity of the disease in the individual patient. The cell-surface antigen HLA-B27 is well known to be associated with spondyloarthropathies...... to a population-based study as possible. METHODS: We studied an incidence-based cohort of 305 patients collected prospectively in 3 Nordic countries (Sweden, Norway, Denmark). Clinical and serological data of the first 3 years of the disease were collected. RESULTS: HLA-B27 was found to be positive in 25.......5% of the patients, and we found a higher proportion of HLA-B27-positive boys with older age at disease onset (p=0.034). Regression analysis showed a correlation of 0.7 in the HLA-B27-positive boys, pointing to a higher risk of more joint involvement with older age at disease onset. By Fisher's exact test...

  20. Fever as an Initial Manifestation of Enthesitis-Related Arthritis Subtype of Juvenile Idiopathic Arthritis: Retrospective Study

    Science.gov (United States)

    Guo, Ruru; Cao, Lanfang; Kong, Xianming; Liu, Xuesong; Xue, Haiyan; Shen, Lijuan; Li, Xiaoli

    2015-01-01

    Objective We wished to determine the prevalence of fever as one of the first symptoms of the enthesitis-related arthritis (ERA) subtype of juvenile idiopathic arthritis. Also, we wished to ascertain if ERA patients with fever at disease onset differed from those without fever. Methods Consecutive cases of ERA were diagnosed and followed in a retrospective observational study from 1998 to 2013. Information about clinical/laboratory data, medications, magnetic resonance imaging (MRI), and disease activity during the study period was also recorded. Results A total of 146 consecutive ERA patients were assessed. Among them, 52 patients (35.6%) had fever as one of the first symptoms at disease onset. Compared with ERA patients without fever at disease onset, patients with fever had significantly more painful joints (3.5 vs. 2.8), more swollen joints (1.1 vs. 0.8), and more enthesitis (1.0 vs. 0.4) (pfever had significantly higher mean values of erythrocyte sedimentation rate, C-reactive protein, platelet count, and child health assessment questionnaire (CHAQ) scores (40.8 vs. 26.4 mm/h; 20.7 vs. 9.7 mg/dL; 353.2×109/L vs. 275.6×109/L; 1.0 vs. 0.8, respectively; all pfever. Conclusions Fever was a frequent manifestation of ERA. ERA patients with fever had more active disease at disease onset and poorer outcomes than ERA patients without fever. PMID:26030261

  1. Synovial and inflammatory diseases in childhood: role of new imaging modalities in the assessment of patients with juvenile idiopathic arthritis

    International Nuclear Information System (INIS)

    Damasio, Maria Beatrice; Malattia, Clara; Martini, Alberto; Toma, Paolo

    2010-01-01

    Juvenile idiopathic arthritis (JIA) represents a group of heterogeneous diseases characterized by a chronic inflammatory process primarily targeting the synovial membrane. A persistent synovitis is associated with an increased risk of osteocartilaginous damage. With the advent of effective structure-modifying treatment for JIA, it may be possible to significantly reduce or even completely prevent structural damage and associated functional disability. The trend towards early suppression of inflammation, in order to prevent erosive disease, shifts the emphasis away from conventional radiographic detectable structural damage to the slightest traces of early joint damage, and drives the need for alternative imaging techniques more sensitive in detecting early signs of disease activity and damage. In this regard MRI and US are playing an increasing role in the evaluation of arthritic joints. This article will review the key aspects of the current status and recent important advances of imaging techniques available to investigate the child with rheumatic disease, briefly discussing conventional radiography, and particularly focusing on MRI and US. In this era of advancing imaging technology, knowledge of the relative values of available imaging techniques is necessary to optimize the management of children with JIA. (orig.)

  2. Effect of Adalimumab on Refractory Arthritis in Juvenile Idiopathic Inflammatory Myopathy with Anti-MDA5 Autoantibody

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    Takako Miyamae

    2018-01-01

    Full Text Available A 10-year-old girl manifested persistent fever, skin rash, leg pain, fatigue, and joint pain. Based on muscle weakness, elevated muscle-derived enzymes, magnetic resonance imaging, and skin biopsy results, the diagnosis was juvenile idiopathic inflammatory myopathies (JIIM. Chest CT was normal; the anti-melanoma differentiation-associated protein-5 (anti-MDA5 autoantibody was positive. Initial manifestations subsided after prednisolone (PSL and methotrexate treatment. After the PSL dosage was decreased, the patient presented with metacarpophalangeal (MCP joint pain and swelling in both index fingers, synovial fluid, and signals on power Doppler ultrasound. The arthritis was refractory to cyclosporine and tacrolimus. Radiography showed progressive MCP joint space narrowing and joint erosion. Adalimumab was initiated 14 months after disease onset. There was a mildly increased matrix metalloproteinase-3 (MMP3 level, an erythrocyte sedimentation ratio (ESR, and a normal CRP level. Adalimumab resulted in decreased MCP joint pain and swelling. PSL was discontinued 10 months after adalimumab initiation; after 9 more months of adalimumab, there were no significant ultrasonography findings. MMP3 and ESR levels normalized during treatment. Radiography after 2 years of adalimumab showed further progressive MCP joint space narrowing restricting dorsiflexion. This report clarified that anti-MDA5-positive JIIM joint manifestations were due to active synovitis and that adalimumab is required for severe cases. Further experience is needed to determine the pathology, severity, and prognosis of this type of arthritis.

  3. Quantitative MR characterization of disease activity in the knee in children with juvenile idiopathic arthritis: a longitudinal pilot study

    International Nuclear Information System (INIS)

    Workie, Dagnachew W.; Graham, T.B.; Laor, Tal; Racadio, Judy M.; Rajagopal, Akila; O'Brien, Kendall J.; Bommer, Wendy A.; Shire, Norah J.; Dardzinski, Bernard J.

    2007-01-01

    The development of a quantifiable and noninvasive method of monitoring disease activity and response to therapy is vital for arthritis management. The purpose of this study was to investigate the utility of quantitative dynamic contrast-enhanced MRI (DCE-MRI) based on pharmacokinetic (PK) modeling to evaluate disease activity in the knee and correlate the results with the clinical assessment in children with juvenile idiopathic arthritis (JIA). A group of 17 children with JIA underwent longitudinal clinical and laboratory assessment and DCE-MRI of the knee at enrollment, 3 months, and 12 months. A PK model was employed using MRI signal enhancement data to give three parameters, K trans ' (min -1 ), k ep (min -1 ), and V p ' and to calculate synovial volume. The PK parameters, synovial volumes, and clinical and laboratory assessments in most children were significantly decreased (P < 0.05) at 12 months when compared to the enrollment values. There was excellent correlation between the PK and synovial volume and the clinical and laboratory assessments. Differences in MR and clinical parameter values in individual subjects illustrate persistent synovitis when in clinical remission. A decrease in PK parameter values obtained from DCE-MRI in children with JIA likely reflects diminution of disease activity. This technique may be used as an objective follow-up measure of therapeutic efficacy in patients with JIA. MR imaging can detect persistent synovitis in patients considered to be in clinical remission. (orig.)

  4. MRI of the wrist in juvenile idiopathic arthritis: proposal of a paediatric synovitis score by a consensus of an international working group. Results of a multicentre reliability study

    International Nuclear Information System (INIS)

    Damasio, Maria Beatrice; Mattiuz, Chiara; Magnano, GianMichele; Malattia, Clara; Martini, Alberto; Tanturri de Horatio, Laura; Barbuti, Domenico; Toma, Paolo; Pistorio, Angela; Bracaglia, Claudia; Boavida, Peter; Ording, Lil Sophie Mueller; Juhan, Karen Lambot; Rosendahl, Karen

    2012-01-01

    MRI is a sensitive tool for the evaluation of synovitis in juvenile idiopathic arthritis (JIA). The purpose of this study was to introduce a novel MRI-based score for synovitis in children and to examine its inter- and intraobserver variability in a multi-centre study. Wrist MRI was performed in 76 children with JIA. On postcontrast 3-D spoiled gradient-echo and fat-suppressed T2-weighted spin-echo images, joint recesses were scored for the degree of synovial enhancement, effusion and overall inflammation independently by two paediatric radiologists. Total-enhancement and inflammation-synovitis scores were calculated. Interobserver agreement was poor to moderate for enhancement and inflammation in all recesses, except in the radioulnar and radiocarpal joints. Intraobserver agreement was good to excellent. For enhancement and inflammation scores, mean differences (95 % CI) between observers were -1.18 (-4.79 to 2.42) and -2.11 (-6.06 to 1.83). Intraobserver variability (reader 1) was 0 (-1.65 to 1.65) and 0.02 (-1.39 to 1.44). Intraobserver agreement was good. Except for the radioulnar and radiocarpal joints, interobserver agreement was not acceptable. Therefore, the proposed scoring system requires further refinement. (orig.)

  5. Prevalence of juvenile idiopathic arthritis in children aged 6 to 12 years in Embu das Artes, state of Sao Paulo, Brazil.

    Science.gov (United States)

    Yamashita, Edson; Terreri, Maria Teresa R A; Hilário, Maria Odete E; Len, Claudio A

    2013-01-01

    The aim of the study was to study the prevalence of juvenile idiopathic arthritis (JIA) in school children in the city of Embu das Artes in São Paulo State. 2880 school children from seven public schools, aged between 6 and 12 years, were evaluated (clinical findings) by a pediatric rheumatologist. A board certified Pediatric Rheumatologist evaluated the subjects with suspected inflammatory arthropathy. Children with higher suspicion were referred to a specialized service. One hundred and forty-one children have presented abnormalities on examination of musculoskeletal system, with isolated pain on palpation the most common finding in the first evaluation (60.9%), with improvement in almost all cases in the second examination. Most of the abnormalities were related to recent injuries or congenital malformations. Six children have clinical findings suggestive of chronic arthropathy and were referred to a specialized pediatric rheumatology clinic. Of these, a 12 year-old girl fulfilled the criteria for JIA. The other diagnoses were aseptic necrosis of the hip (P = 1) of and post-trauma synovitis (P = 4). The prevalence of JIA in children aged between 6 and 12 years was 1/2.880 (or 0.34/1.000).

  6. Validation of the Brazilian version of the Pediatric Outcomes Data Collection Instrument: a cross-sectional evaluation in children and adolescents with juvenile idiopathic arthritis.

    Science.gov (United States)

    do Monte, Felipe Alves; Ferreira, Moacir Novaes Lima; Petribu, Kátia Cristina Lima; Almeida, Nair Cristina; Gomes, José Benjamim; Mariano, Maria Helena; Mesquita, Zelina Barbosa; Simões, Diego Montarroyos; Rodrigues, André Furtado de Ayalla; Souza, Mariana Alves Nogueira

    2013-10-30

    There is a lack of health-related quality of life (HRQOL) questionnaires to evaluate pediatric musculoskeletal diseases in Brazil. The Pediatric Outcome Data Collection Instrument (PODCI) is widely used elsewhere for pediatric patients with musculoskeletal disorders, but it has not been fully validated in Brazil. Validation of the PODCI in the Brazilian Portuguese language is important to improve the assessment of pediatric patients with musculoskeletal diseases and to compare Brazilian study results with results from the international literature. This study aimed to analyze the test-re-test reliability and the convergent validity indicators for the quality of life scores obtained by application of the PODCI to children and adolescents with juvenile idiopathic arthritis (JIA). The PODCI underwent translation, transcultural adaptation, and field testing. Fifty-seven children and adolescents with JIA were administered the PODCI questionnaire. The Child Health Questionnaire - Parent Form 28 (CHQ PF-28) was used as the gold standard. Pain scales were employed, clinical examinations were performed, and laboratory inflammatory activity tests were conducted. The three versions of the PODCI exhibited good internal consistency (Cronbach's alpha coefficient > 0.70), good reproducibility (p  0.40 (p validated in patients with JIA in Brazil. This questionnaire was found to be valid, precise, and reliable. It can be successfully applied in research conducted by healthcare professionals who work with children and adolescents with musculoskeletal system disorders.

  7. Elevated tissue transglutaminase antibodies in juvenile idiopathic arthritis children: Relation to neutrophil-to-lymphocyte ratio and disease activity

    Directory of Open Access Journals (Sweden)

    Rasha E. Gheith

    2017-10-01

    Full Text Available Background: Subclinical gut inflammation is described in juvenile idiopathic arthritis (JIA, so has joint involvement been related to celiac disease (CD. The well-known involvement of tissue transglutaminase (tTG in the pathogenesis of CD stimulated progress in the field of autoimmune diseases. Aim of the work: To screen JIA children for tTG antibodies and to detect its relation to the neutrophil-lymphocyte ratio (NLR and disease activity. Patients and methods: The study included 44 JIA children with 44 matched controls. All subjects had no GIT symptoms suggestive of CD. Disease activity was assessed using the juvenile arthritis disease activity score in 27 joints (JADAS-27. The tTG antibodies (IgA and IgG were assessed. Results: The patients mean age was 12.5 ± 2.8 years and disease duration 5.01 ± 2.9 years; Female:Male 3.4:1. The mean JADAS-27 score was 12.6 ± 2.04. tTG antibodies were positive in 43.2% of the patients compared to 18.2% control (p = 0.01. Antibodies positivity was comparable according to gender and subtypes. The NLR in JIA children (1.62 ± 0.58 was significantly higher than in control (1.3 ± 0.5 (p = 0.006. Those with positive tTG antibodies had a significantly reduced body mass index (p = 0.02 and increased NLR (p = 0.02 compared to those with negative tTG. Only NLR and JADAS-27 would significantly predict antibodies positivity (p = 0.037 and p = 0.04, respectively. Conclusion: Increased tTG antibodies are frequent in JIA children raising the possibility of an associated subclinical CD. Markedly reduced BMI and increased NLR could forecast the presence of these antibodies. In addition to the JADAS-27, the NLR is a simple test that could predict this association and could be a useful biomarker.

  8. Methotrexate intolerance in oral and subcutaneous administration in patients with juvenile idiopathic arthritis: a cross-sectional, observational study.

    Science.gov (United States)

    van Dijkhuizen, E H Pieter; Pouw, Juliëtte N; Scheuern, Andrea; Hügle, Boris; Hardt, Sven; Ganser, Gerd; Kümmerle-Deschner, Jasmin Beate; Horneff, Gerd; Holzinger, Dirk; Bulatović Ćalasan, Maja; Wulffraat, Nico M

    2016-01-01

    Methotrexate (MTX) is the cornerstone disease-modifying anti-rheumatic drug (DMARD) in juvenile idiopathic arthritis (JIA). In Dutch patients, MTX intolerance occurred frequently and was associated with subcutaneous (SC) administration. The aim of this study was to assess the prevalence of MTX intolerance and its association with the route of administration in a German cohort of JIA patients. A cross-sectional study of JIA patients on MTX was performed. Primary outcome was MTX intolerance, which was determined using the validated Methotrexate Intolerance Severity Score (MISS) questionnaire. The prevalence of gastrointestinal adverse effects and MTX intolerance was compared between patients on MTX SC and MTX administered orally (PO). Of 179 JIA patients on MTX, 73 (40.8%) were intolerant. The odds of MTX intolerance were higher in patients using MTX exclusively SC compared to exclusively PO (adjusted odds ratio 3.37 [95% confidence interval 1.19-10.0]). There was strong evidence that the former experienced more behavioural complaints (76.1% vs. 47.4%, p=0.001) and weak evidence that they experienced more abdominal pain after MTX intake (43.5% vs. 27.4%, p=0.056). The prevalence of MTX intolerance was high and exclusively SC administration of MTX was associated with MTX intolerance and behavioural adverse effects. The prevalence of gastrointestinal adverse effects was at least as high as in patients on MTX PO. The frequently held assumption that SC causes fewer side effects than PO seems unwarranted. Definite answers about the differences between SC and PO administration with respect to safety and efficacy should be obtained by randomised trials.

  9. Successful treatment of methotrexate intolerance in juvenile idiopathic arthritis using eye movement desensitization and reprocessing - treatment protocol and preliminary results.

    Science.gov (United States)

    Höfel, Lea; Eppler, Bruno; Storf, Magdalena; Schnöbel-Müller, Elizabeth; Haas, Johannes-Peter; Hügle, Boris

    2018-02-13

    Methotrexate (MTX), commonly used in juvenile idiopathic arthritis (JIA), frequently has to be discontinued due to intolerance with anticipatory and associative gastrointestinal adverse effects. Eye Movement Desensitization and Reprocessing (EMDR) is a psychological method where dysfunctional experiences and memories are reprocessed by recall combined with bilateral eye movements. The objective of this study was to assess efficacy of EMDR for treatment of MTX intolerance in JIA patients. We performed an open prospective study on consecutive JIA patients with MTX intolerance. Intolerance was determined using the Methotrexate Intolerance Severity Score (MISS) questionnaire prior to treatment, directly after treatment and after four months. Health-related quality of life was determined using the PedsQL prior to and four months after treatment. Patients were treated according to an institutional EMDR protocol with 8 sessions over two weeks. Changes in MISS and PedsQL were analyzed using non-parametric statistics. Eighteen patients with MTX intolerance (median MISS at inclusion 16.5, IQR = 11.75-20.25) were included. Directly after treatment, MTX intolerance symptoms were significantly improved (median MISS 1 (IQR = 0-2). After four months, median MISS score was at 6.5 (IQR = 2.75-12.25, p = 0.001), with 9/18 patients showing MISS scores ≥6. Median PedsQL after 4 months improved significantly from 77.6% to 85.3% (p = 0.008). MTX intolerance in children with JIA was effectively treated using an EMDR protocol, with lasting effect over a period of 4 months. EMDR treatment can potentially increase quality of life of affected patients and enable continued MTX treatment.

  10. Dynamic contrast-enhanced magnetic resonance imaging can play a role in predicting flare in juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Nusman, Charlotte M., E-mail: c.m.nusman@amc.uva.nl [Department of Radiology, Academic Medical Center Meibergdreef 9, 1105 AZ, Amsterdam (Netherlands); Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Diseases, Emma Children’s Hospital, Academic Medical Center Meibergdreef 9, 1105 AZ, Amsterdam (Netherlands); Hemke, Robert; Lavini, Cristina [Department of Radiology, Academic Medical Center Meibergdreef 9, 1105 AZ, Amsterdam (Netherlands); Schonenberg-Meinema, Dieneke [Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Diseases, Emma Children’s Hospital, Academic Medical Center Meibergdreef 9, 1105 AZ, Amsterdam (Netherlands); Rossum, Marion A.J. van [Department of Pediatric Rheumatology, Reade Institute location Jan van Breemen, Doctor Jan van Breemenstraat 2, 1056 AB Amsterdam (Netherlands); Department of Pediatrics, Emma Children’s Hospital, Academic Medical Center Meibergdreef 9, 1105 AZ, Amsterdam (Netherlands); Dolman, Koert M. [Department of Pediatric Rheumatology, Reade Institute location Jan van Breemen, Doctor Jan van Breemenstraat 2, 1056 AB Amsterdam (Netherlands); Department of Pediatrics, Sint Lucas Andreas Hospital, Jan Tooropstraat 164, 1061 AE, Amsterdam (Netherlands); Berg, J. Merlijn van den [Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Diseases, Emma Children’s Hospital, Academic Medical Center Meibergdreef 9, 1105 AZ, Amsterdam (Netherlands); Maas, Mario [Department of Radiology, Academic Medical Center Meibergdreef 9, 1105 AZ, Amsterdam (Netherlands); Kuijpers, Taco W. [Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Diseases, Emma Children’s Hospital, Academic Medical Center Meibergdreef 9, 1105 AZ, Amsterdam (Netherlands)

    2017-03-15

    Highlights: • Subclinical synovitis is present in 40% of the inactive JIA patients. • One third of the inactive JIA patients flared during 2-year clinical follow-up. • DCE-MRI can play a role in predicting clinical flare in JIA patients. - Abstract: Purpose: The study was performed to determine whether conventional and dynamic contrast-enhanced (DCE) magnetic resonance imaging (MRI) parameters of a previously affected target joint in patients with clinically inactive juvenile idiopathic arthritis (JIA) have prognostic meaning for a flare of joint inflammation during follow-up. Material and methods: Thirty-two JIA patients with clinically inactive disease at the time of MRI of the knee were prospectively included. DCE-MRI provided both descriptive measures and time-intensity-curve shapes, representing functional properties of the synovium. Conventional MRI outcome measures included validated scores for synovial hypertrophy, bone marrow edema, cartilage lesions and bone erosions. During a 2-year period the patients were monitored by their pediatric rheumatologist and clinical flares were registered. Results: MRI analysis revealed synovial hypertrophy in 13 (39.4%) of the clinically inactive patients. Twelve patients (37.5%) had at least one flare during 2-year clinical follow-up. Persistently inactive and flaring patients differed significantly in the maximum enhancement of the synovium on the DCE-MRI (p < 0.05), whereas no difference was found between these two groups in any of the baseline scores of conventional MRI. Conclusions: Our prospective clinical follow-up study indicates that the assessment of ‘maximum enhancement’ upon DCE-MRI may be able to predict a clinical flare within 2 years in inactive JIA patients.

  11. Alteration of fecal microbiota profiles in juvenile idiopathic arthritis. Associations with HLA-B27 allele and disease status.

    Directory of Open Access Journals (Sweden)

    Monica Di Paola

    2016-10-01

    Full Text Available Alteration of gut microbiota is involved in several chronic inflammatory and autoimmune diseases, including rheumatoid arthritis, and gut microbial pro-arthritogenic profiles have been hypothesized. Intestinal inflammation may be involved in spondyloarthropathies and in a subset of patients affected by Juvenile Idiopathic Arthritis (JIA, the most common chronic rheumatic disease of childhood. We compared the fecal microbiota composition of JIA patients with healthy subjects (HS, evaluating differences in microbial profiles between sub-categories of JIA, such as enthesitis-related arthritis (JIA-ERA, in which inflammation of entheses occurs, and polyarticular JIA, non-enthesitis related arthritis (JIA-nERA. Through taxon-level analysis, we discovered alteration of fecal microbiota components that could be involved in subclinical gut inflammation, and promotion of joint inflammation. We observed abundance in Ruminococcaceae in both JIA categories, reduction in Clostridiaceae and Peptostreptococcaceae in JIA-ERA, and increase in Veillonellaceae in JIA-nERA, respectively compared with HS. Among the more relevant genera, we found an increase in Clostridium cluster XIVb, involved in colitis and arthritis, in JIA-ERA patients compared with HS, and a trend of decrease in Faecalibacterium, known for anti-inflammatory properties, in JIA-nERA compared with JIA-ERA and HS. Differential abundant taxa identified JIA patients for the HLA-B27 allele, including Bilophila, Clostridium cluster XIVb, Oscillibacter and Parvimonas. Prediction analysis of metabolic functions showed that JIA-ERA metagenome was differentially enriched in bacterial functions related to cell motility and chemotaxis, suggesting selection of potential virulence traits. We also discovered differential microbial profiles and intra-group variability among active disease and remission, suggesting instability of microbial ecosystem in autoimmune diseases with respect to healthy status. Similarly

  12. Internet Program for Physical Activity and Exercise Capacity in Children With Juvenile Idiopathic Arthritis: A Multicenter Randomized Controlled Trial.

    Science.gov (United States)

    Armbrust, Wineke; Bos, G J F Joyce; Wulffraat, Nico M; van Brussel, Marco; Cappon, Jeannette; Dijkstra, Pieter U; Geertzen, Jan H B; Legger, G Elizabeth; van Rossum, Marion A J; Sauer, Pieter J J; Lelieveld, Otto T H M

    2017-07-01

    To determine the effects of Rheumates@Work, an internet-based program supplemented with 4 group sessions, aimed at improving physical activity, exercise capacity, health-related quality of life (HRQoL), and participation in children with juvenile idiopathic arthritis. Patients were recruited from 3 pediatric rheumatology centers in The Netherlands for an observer-blinded, randomized controlled multicenter trial. Physical activity level, time spent in rest, light, and moderate-to-vigorous physical activity (MVPA) were recorded in a diary and with an accelerometer, before intervention, after intervention, and at followup after 3 and 12 months (intervention group only). Exercise capacity was assessed using the Bruce treadmill protocol, HRQoL was assessed with the Pediatric Quality of Life Inventory generic core scale, and participation in school and in physical education classes were assessed by questionnaire. The intervention group consisted of 28 children, and there were 21 children in the control group. MVPA , exercise capacity, and participating in school and physical education classes improved significantly in the intervention group. HRQoL improved in the control group. No significant differences were found between groups. The effect of Rheumates@Work on physical activity and exercise capacity lasted during the 12 months of followup. Improvements in physical activity were significantly better for the cohort starting in winter compared to the summer cohort. Rheumates@Work had a positive, albeit small, effect on physical activity, exercise capacity, and participation in school and physical education class in the intervention group. Improvements lasted for 12 months. Participants who started in winter showed the most improvement. Rheumates@Work had no effect on HRQoL. © 2016, American College of Rheumatology.

  13. Recent trends in medication usage for the treatment of juvenile idiopathic arthritis and the influence of tumor necrosis factor inhibitors.

    Science.gov (United States)

    Mannion, Melissa L; Xie, Fenglong; Curtis, Jeffrey R; Beukelman, Timothy

    2014-10-01

    Using administrative data from a large commercial US health insurer, we investigated temporal trends in medication use among children diagnosed with juvenile idiopathic arthritis (JIA). Children with ≥ 1 physician diagnosis code for JIA in the calendar years 2005 through 2012 were included. Use of tumor necrosis factor inhibitors (TNFi), methotrexate (MTX), nonsteroidal antiinflammatory drugs (NSAID), and oral glucocorticoids (GC) was determined. Temporal changes in medication usage were evaluated with the Cochran-Armitage test for trend. We used paired t-tests to evaluate the use of NSAID and GC in the 6 months before and after new TNFi use. We identified 4261 unique individuals with JIA. The proportion of patients receiving TNFi increased from 8.7% in 2005 to 22.4% in 2012 (p < 0.0001). MTX use increased from 18.4% to 23.2% (p = 0.02). NSAID use decreased from 49% to 40% (p = 0.02). GC use was relatively unchanged. Following new TNFi use, the mean number of NSAID prescriptions (among prevalent users) decreased from 2.8 to 2.0 (p < 0.0001), and the mean daily GC dose (among prevalent users) decreased from 7.3 mg/day to 3.9 mg/day (p < 0.0001). Many new TNFi users (57%) had not used MTX in the previous 6 months, and only 37% had any concurrent MTX use in the 6 months following new TNFi use. TNFi use in the treatment of JIA increased 2- to 3-fold over the last 8 years. New TNFi use was associated with decreased NSAID and GC use. TNFi may be replacing, rather than complementing, MTX in the treatment of many patients.

  14. Th1-Induced CD106 Expression Mediates Leukocytes Adhesion on Synovial Fibroblasts from Juvenile Idiopathic Arthritis Patients.

    Science.gov (United States)

    Maggi, Laura; Margheri, Francesca; Luciani, Cristina; Capone, Manuela; Rossi, Maria Caterina; Chillà, Anastasia; Santarlasci, Veronica; Mazzoni, Alessio; Cimaz, Rolando; Liotta, Francesco; Maggi, Enrico; Cosmi, Lorenzo; Del Rosso, Mario; Annunziato, Francesco

    2016-01-01

    This study tested the hypothesis that subsets of human T helper cells can orchestrate leukocyte adhesion to synovial fibroblasts (SFbs), thus regulating the retention of leukocytes in the joints of juvenile idiopathic arthritis (JIA) patients. Several cell types, such as monocytes/macrophages, granulocytes, T and B lymphocytes, SFbs and osteoclasts participate in joint tissue damage JIA. Among T cells, an enrichment of classic and non-classic Th1 subsets, has been found in JIA synovial fluid (SF), compared to peripheral blood (PB). Moreover, it has been shown that IL-12 in the SF of inflamed joints mediates the shift of Th17 lymphocytes towards the non-classic Th1 subset. Culture supernatants of Th17, classic and non-classic Th1 clones, have been tested for their ability to stimulate proliferation, and to induce expression of adhesion molecules on SFbs, obtained from healthy donors. Culture supernatants of both classic and non-classic Th1, but not of Th17, clones, were able to induce CD106 (VCAM-1) up-regulation on SFbs. This effect, mediated by tumor necrosis factor (TNF)-α, was crucial for the adhesion of circulating leukocytes on SFbs. Finally, we found that SFbs derived from SF of JIA patients expressed higher levels of CD106 than those from healthy donors, resembling the phenotype of SFbs activated in vitro with Th1-clones supernatants. On the basis of these findings, we conclude that classic and non-classic Th1 cells induce CD106 expression on SFbs through TNF-α, an effect that could play a role in leukocytes retention in inflamed joints.

  15. Assessment of the outcome of ophthalmological screening for uveitis in a cohort of Swedish children with juvenile idiopathic arthritis.

    Science.gov (United States)

    Papadopoulou, Maria; Zetterberg, Madeleine; Oskarsdottir, Solveig; Andersson Grönlund, Marita

    2017-11-01

    To describe clinical features, risk factors and complications in a cohort of Swedish children with juvenile idiopathic arthritis (JIA) screened for uveitis between 2002 and 2011. Medical records of 299 children with JIA (93 male, 206 female; median age 5.0 years at diagnosis) were retrospectively scrutinized focusing on subtype of JIA, onset of arthritis/uveitis, presence of antinuclear antibodies (ANA) and ophthalmological status. Uveitis was found in 32 (11%) children, 78% bilaterally affected. The median age of arthritis onset in children who developed uveitis was 2.5 years (range 1-10) versus 5.0 years (range 1-15) in those who did not. Sex ratio was 3.5:1 (girl:boy). The most prevalent JIA subtype was oligoarthritis (75%). All but one child with uveitis was found to be ANA (+). The median interval between diagnosis of arthritis and uveitis was 12 months. Only one child developed uveitis between the fourth and fifth years after arthritis onset. Ocular complications were recorded in 45.6% (26/57 affected eyes) at last follow-up. On univariate analysis, both young age at arthritis onset and ANA positivity were possible predictors for developing uveitis, but on multivariate analysis, the latter was the most important predictor (HR 16.25, 95%; CI 2.19-120.44; p = 0.006, Cox regression analysis). Almost all of the children developing JIA-associated uveitis did so within 4 years after arthritis onset, a fact that accentuates the importance of early initiation of ophthalmological screening and more frequent regular follow-ups during the first 4 years. The most important predictor for developing uveitis was ANA positivity. © 2017 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.

  16. Consensus-based recommendations for the management of uveitis associated with juvenile idiopathic arthritis: the SHARE initiative.

    Science.gov (United States)

    Constantin, Tamas; Foeldvari, Ivan; Anton, Jordi; de Boer, Joke; Czitrom-Guillaume, Severine; Edelsten, Clive; Gepstein, Raz; Heiligenhaus, Arnd; Pilkington, Clarissa A; Simonini, Gabriele; Uziel, Yosef; Vastert, Sebastian J; Wulffraat, Nico M; Haasnoot, Anne-Mieke; Walscheid, Karoline; Pálinkás, Annamária; Pattani, Reshma; Györgyi, Zoltán; Kozma, Richárd; Boom, Victor; Ponyi, Andrea; Ravelli, Angelo; Ramanan, Athimalaipet V

    2018-03-28

    In 2012, a European initiative called S ingle Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children and uveitis is possibly its most devastating extra-articular manifestation. Evidence-based guidelines are sparse and management is mostly based on physicians' experience. Consequently, treatment practices differ widely, within and between nations. To provide recommendations for the diagnosis and treatment of JIA-associated uveitis. Recommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was constituted, consisting of nine experienced paediatric rheumatologists and three experts in ophthalmology from Europe. Recommendations derived from a validated systematic literature review were evaluated by an Expert Committee and subsequently discussed at two consensus meetings using nominal group techniques. Recommendations were accepted if >80% agreement was reached (including all three ophthalmologists). In total, 22 recommendations were accepted (with >80% agreement among experts): 3 on diagnosis, 5 on disease activity measurements, 12 on treatment and 2 on future recommendations. The SHARE initiative aims to identify best practices for treatment of patients suffering from JIA-associated uveitis. Within this remit, recommendations for the diagnosis and treatment of JIA-associated uveitis have been formulated by an evidence-informed consensus process to suggest a standard of care for JIA-associated uveitis patients throughout Europe. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  17. HLA genotyping as first-line screening tool for coeliac disease in children with juvenile idiopathic arthritis.

    Science.gov (United States)

    Skrabl-Baumgartner, Andrea; Christine Hauer, Almuthe; Erwa, Wolfgang; Jahnel, Jörg

    2017-07-01

    Coeliac disease (CD) and juvenile idiopathic arthritis (JIA) often coexist. This association warrants assessment for CD in patients with JIA. We evaluated the clinical relevance and cost-effectiveness of human leucocyte antigen (HLA) genotyping in first-line screening for development of CD in children with JIA. 95 patients with JIA were screened for CD using CD-specific antibodies. In case of positivity, a small intestinal biopsy was performed to confirm diagnosis. In addition, HLA genotyping was performed. 110 age-matched and sex-matched Caucasian children from the same geographical area served as controls. CD was diagnosed in 4 of 95 patients with JIA (4.2%), a rate significantly higher compared with controls (pHLA-DQ2.5 genotype: one was homozygote, the remainder heterozygote. Twenty-two patients are, judging by their HLA genotypes, at risk of developing CD and require repeated serological screening. None of the 69 patients without HLA-DQ2/DQ8 genotypes had CD-specific antibodies. Screening with HLA genotyping becomes cheaper than screening without after the second determination. In our cohort of patients with JIA, lack of HLA-DQ2/DQ8 genotypes identified a majority not at risk of CD in whom repeated serological testing is unnecessary. Genotyping is nowadays the most efficient and cost-effective way to screen for CD risk in JIA. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  18. Early detection of temporomandibular joint arthritis in children with juvenile idiopathic arthritis - the role of contrast-enhanced MRI

    Energy Technology Data Exchange (ETDEWEB)

    Kalle, Thekla von; Stuber, Tina; Winkler, Peter [Olgahospital Klinikum Stuttgart, Pediatric Radiology, Radiologisches Institut, Stuttgart (Germany); Maier, Jan; Hospach, Toni [Olgahospital Klinikum Stuttgart, Pediatric Rheumatology, Stuttgart (Germany)

    2015-03-01

    Early treatment of temporomandibular joint (TMJ) arthritis is crucial in children with juvenile idiopathic arthritis (JIA) to prevent permanent functional impairment. As involvement of TMJs is often asymptomatic, contrast-enhanced MRI is regarded as the most sensitive noninvasive diagnostic tool. To evaluate the degree of contrast enhancement in TMJs of children and adolescents with JIA in comparison to normal controls from a previous study. Dynamic contrast-enhanced MRI of 50 children and adolescents with JIA (6.3 to 18 years of age; mean: 12 years) were retrospectively analysed. We assessed morphological abnormalities and postcontrast time-intensity curves of the soft joint tissue and the mandibular condyle. Ratios were calculated to quantify postcontrast signal intensities (SI) in relation to precontrast SI at initial (1 min postcontrast) and maximum (6 min postcontrast) increase. Time-intensity curves followed similar biphasic patterns in normal and pathological joints. In joints with morphological signs of arthritis, mean SI ratios were on average higher than in normal joints of the reference group, but ranges of values widely overlapped. Arthritis: mean initial increase of SI 62% (±2 S.D. 18-105%), mean maximum SI 106% higher than precontrast (±2 S.D. 46-166%). Normal: mean initial increase of SI 49% (±2 S.D. 14- 85%), mean maximum of SI 73% (±2 S.D. 23-123%). Given this considerable overlap of results in dynamic contrast-enhanced MRI, the degree of contrast enhancement alone did not allow differentiation between TMJs with and without signs of inflammation. Thickening of the soft joint tissue seems to remain the earliest sign to reliably indicate TMJ arthritis. (orig.)

  19. Patterns of radiographic damage to cervical spine in polyarticular juvenile idiopathic arthritis patients presenting to tertiary care hospital in pakistan

    International Nuclear Information System (INIS)

    Khyzer, E.; Aftab, T.

    2015-01-01

    Objective: To see the radiographic cervical spine damage in polyarticular juvenile idiopathic arthritis (PJIA) coming to a tertiary care hospital in Islamabad, Pakistan. Study Design: Cross-sectional descriptive study. Place and Duration of Study: The study was conducted in department of Rheumatology at Pakistan Institute of Medical Sciences from Jun 2013 to Dec 2013. Subjects and Methods: A total of 50 patients of PJIA coming to Rheumatology Outpatient Department were recruited in the study after informed consent. Radiographs of cervical spine were performed for each patient in antero-posterior, lateral with flexion and extension and open-mouth views. Radiographs were reviewed for the following eatures: loss of cervical lordosis, odontoid process erosion, anterior atlantoaxial subluxation, C1-C2 arthritis, atlantoaxial impaction, inflammation of disc, apophyseal joint arthritis, anterior ankylosis, apophyseal joint ankylosis, anterior and posterior subaxial subluxation and growth disturbances. Data was analysed using SPSS version 18. Results: Out of the total 50 patients, 28 (56%) were females while 22 (44%) were males. The mean duration of pJIA was 5.54 +- 3.28 years. Radiological cervical spine involvement was seen in 52% patients. The most common structural lesions were anterior atlantoaxial subluxation (30%), C1-C2 arthritis (22%) erosion of the odontoid process (18%), and apophyseal joint arthritis (16%). Loss of cervical lordosis was found in 7(14%) patients. There was no growth disturbances observed in vertebra. Conclusion: Cervical spine involvement is common in patients of PJIA. It is mostly asymptomatic, so routine cervical spine radiographs in all patients suffering from PJIA is recommended. (author)

  20. The Pediatric Outcomes Data Collection Instrument for a Polish sample with juvenile idiopathic arthritis: psychometric properties of proxy version.

    Science.gov (United States)

    Misterska, Ewa; Adamczak, Karolina; Kaminiarczyk-Pyzałka, Dominika; Adamczyk, Katarzyna; Głowacki, Jakub; Niedziela, Marek; Glowacki, Maciej

    2017-12-01

    The aims of the study were to investigate the psychometric properties of the Pediatric Outcomes Data Collection Instrument (PODCI) for parents of children aged 2-10 years, in a Polish sample of juvenile idiopathic arthritis and to compare the results from children with chronic arthritis to the results from a normative sample. The sample consisted of the parents of 29 (72.5%) girls and 11 (27.5%) boys. The ages of the children ranged from 2 to 10 years (mean 6.50, standard deviation [SD] 2.63). Disease course was classified as oligoarticular (n = 26, 65%) and polyarticular (n = 14, 35%). Parents who took part in the intrarater reliability study completed the Polish version of PODCI twice. The PODCI consists of the following subscales: Upper Extremity and Physical Function, Transfer and Mobility Tasks, Sports/Physical Functioning, Pain/Comfort, and Happiness. A Global Functioning Scale consists of the mean of the 'mean of items' values for the first four scales. The mean standarized Global Functioning Scale was 81.79 (SD 13.45), whereas the mean normative Global Functioning Scale score equaled 33.65 (SD 18.19). Cronbach's alpha value of the Global Functioning Scale was excellent and equaled 0.81. The effect size concerning the Global Functioning Scale equaled 0.123. Test-retest reliability equaled 0.98, whereas intrarater reliability (intraclass correlation coefficient) equaled 0.99. The translation and cross-cultural adaptation of the PODCI questionnaire provides a Polish equivalent to assess physical function in children with chronic arthritis. © 2015 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  1. Juvenile Arthritis

    Science.gov (United States)

    Juvenile arthritis (JA) is arthritis that happens in children. It causes joint swelling, pain, stiffness, and loss of motion. It can affect any joint, but ... of JA that children get is juvenile idiopathic arthritis. There are several other forms of arthritis affecting ...

  2. Aspectos da sexualidade e gravidez em adolescentes com artrite idiopática juvenil (AIJ Sexuality aspects and pregnancy of adolescents with juvenile idiopathic arthritis (JIA

    Directory of Open Access Journals (Sweden)

    Clovis Artur Almeida Silva

    2005-06-01

    Full Text Available O presente trabalho tem como objetivo descrever aspectos da sexualidade, gravidez e pós-parto de três adolescentes com artrite idiopática juvenil (AIJ. No período entre 1983 e 2004, 4.638 pacientes foram acompanhados na Unidade de Reumatologia Pediátrica do Departamento de Pediatria da FMUSP, entre os quais 537 (11,5% apresentaram o diagnóstico de AIJ (critérios do ILAR. Entre os pacientes com AIJ, três engravidaram durante o seguimento ambulatorial. A idade da primeira atividade sexual variou de 16 a 18 anos. A paciente 1 apresentou uma gestação gemelar a termo, permanecendo em atividade da doença durante toda a gravidez, em uso de 15mg/dia de prednisona. As pacientes 2 e 3 encontravam-se em remissão da doença, sem uso de medicamentos, apresentando gestação a termo sem intercorrências. A paciente 2, porém, evoluiu com recidiva da doença um ano após o parto. Todos os recém-nascidos foram adequados para idade gestacional e evoluíram adequadamente no período neonatal. Apenas a paciente 1 necessitou de prednisona, naproxeno e cloroquina na amamentação. O aumento da gravidez na adolescência é uma realidade nos serviços de reumatologia pediátrica, o que impõe novos debates sobre os aspectos da sexualidade e contracepção nessa população.The objective of the present study is to describe the sexuality aspects, pregnancy and postpartum in three adolescents with juvenile idiopathic arthritis (JIA. From 1983 to 2004, 4,638 patients were followed at the Pediatric Rheumatology Unit of the Pediatric Department of FMUSP, of which 537 (11.5% were diagnosed with JIA (ILAR criteria and three of these patients became pregnant during the follow-up period. The age at their first sexual intercourse ranged from 16 to 18 years old. Patient 1 presented a twin pregnancy, with active disease throughout pregnancy, and was on 15 mg of prednisone per day. Patients 2 and 3 were at disease remission, with no drug treatment, and had full

  3. Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Jens Ivar Brox

    2014-07-01

    Full Text Available Idiopathic scoliosis (IS is a lifetime condition and is defined as a structural, lateral rotated curvature of the spine of >10° on standing coronal plane radiographs. It should be distinguished from other causes of scoliosis. It can be classified as infantile, juvenile, and adolescent according to age. As a rule of thumb, about 80% of all curves are idiopathic, right convex thoracic, and present in otherwise healthy girls at the beginning of puberty. A family member most commonly detects scoliosis. The structural asymmetry of the spine is best observed by asking the patient to bend forward. IS is often seen in more than one member of a family, but the aetiology remains unknown. Multiple genes are likely to be involved with incomplete penetrance and variable expressivity. Early detection by screening allows for monitoring curve progression and timely initiation of bracing, but school screening is controversial and practises vary worldwide. Most patients have minor scoliosis and treatment is generally not recommended for patients with curves 45°. Scoliosis surgery was not successful until the introduction of Harrington’s instrumentation in the 1960s. Modern instrumentation has evolved from the Cotrel-Dubousset system in the 1980s, and a variety of methods are available today. Although scoliosis may be a burden, long-term studies suggest that a good quality of life is maintained in most patients.

  4. Fine-mapping the MHC locus in juvenile idiopathic arthritis (JIA) reveals genetic heterogeneity corresponding to distinct adult inflammatory arthritic diseases.

    Science.gov (United States)

    Hinks, A; Bowes, J; Cobb, J; Ainsworth, H C; Marion, M C; Comeau, M E; Sudman, M; Han, B; Becker, M L; Bohnsack, J F; de Bakker, P I W; Haas, J P; Hazen, M; Lovell, D J; Nigrovic, P A; Nordal, E; Punnaro, M; Rosenberg, A M; Rygg, M; Smith, S L; Wise, C A; Videm, V; Wedderburn, L R; Yarwood, A; Yeung, R S M; Prahalad, S; Langefeld, C D; Raychaudhuri, S; Thompson, S D; Thomson, W

    2017-04-01

    Juvenile idiopathic arthritis (JIA) is a heterogeneous group of diseases, comprising seven categories. Genetic data could potentially be used to help redefine JIA categories and improve the current classification system. The human leucocyte antigen (HLA) region is strongly associated with JIA. Fine-mapping of the region was performed to look for similarities and differences in HLA associations between the JIA categories and define correspondences with adult inflammatory arthritides. Dense genotype data from the HLA region, from the Immunochip array for 5043 JIA cases and 14 390 controls, were used to impute single-nucleotide polymorphisms, HLA classical alleles and amino acids. Bivariate analysis was performed to investigate genetic correlation between the JIA categories. Conditional analysis was used to identify additional effects within the region. Comparison of the findings with those in adult inflammatory arthritic diseases was performed. We identified category-specific associations and have demonstrated for the first time that rheumatoid factor (RF)-negative polyarticular JIA and oligoarticular JIA are genetically similar in their HLA associations. We also observe that each JIA category potentially has an adult counterpart. The RF-positive polyarthritis association at HLA-DRB1 amino acid at position 13 mirrors the association in adult seropositive rheumatoid arthritis (RA). Interestingly, the combined oligoarthritis and RF-negative polyarthritis dataset shares the same association with adult seronegative RA. The findings suggest the value of using genetic data in helping to classify the categories of this heterogeneous disease. Mapping JIA categories to adult counterparts could enable shared knowledge of disease pathogenesis and aetiology and facilitate transition from paediatric to adult services. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  5. Feasibility of diffusion-weighted magnetic resonance imaging in patients with juvenile idiopathic arthritis on 1.0-T open-bore MRI

    Energy Technology Data Exchange (ETDEWEB)

    Barendregt, Anouk M.; Nusman, Charlotte M. [University of Amsterdam, Department of Radiology, Academic Medical Center, Amsterdam (Netherlands); University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Hemke, Robert; Lavini, Cristina; Maas, Mario [University of Amsterdam, Department of Radiology, Academic Medical Center, Amsterdam (Netherlands); Amiras, Dimitri [Imperial College Healthcare NHS Trust, Radiology Department, St. Mary' s Hospital, Paddington, London (United Kingdom); Kuijpers, Taco W. [University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands)

    2015-12-15

    To evaluate the feasibility of non-invasive diffusion-weighted imaging (DWI) of the knee of children with juvenile idiopathic arthritis (JIA) and, further, to analyze the apparent diffusion coefficient (ADC) levels to distinguish synovium from effusion. Standard magnetic resonance imaging of the knee including post-contrast imaging was obtained in eight patients (mean age, 12 years 8 months, five females) using an open-bore magnetic resonance imaging system (1.0 T). In addition, axially acquired echo-planar DWI datasets (b-values 0, 50, and 600) were prospectively obtained and the diffusion images were post-processed into ADC{sub 50-600} maps. Two independent observers selected a region of interest (ROI) for both synovium and effusion using aligned post-contrast images as landmarks. Mann-Whitney U test was performed to compare ADC synovium and ADC effusion. DWI was successfully obtained in all patients. When data of both observers was combined, ADC synovium was lower than ADC effusion in the ROI in seven out of eight patients (median, 1.92 x 10{sup -3} mm{sup 2}/s vs. 2.40 x 10{sup -3} mm{sup 2}/s, p = 0.006, respectively). Similar results were obtained when the two observers were analyzed separately (observer 1: p = 0.006, observer 2: p = 0.04). In this pilot study, on a patient-friendly 1.0-T open-bore MRI, we demonstrated that DWI may potentially be a feasible non-invasive imaging technique in children with JIA. We could differentiate synovium from effusion in seven out of eight patients based on the ADC of synovium and effusion. However, to select synovium and effusion on DWI, post-contrast images were still a necessity. (orig.)

  6. Health-related quality of life in children with juvenile idiopathic arthritis - child's and parent's point of view.

    Science.gov (United States)

    Mańczak, Małgorzata; Rutkowska-Sak, Lidia; Raciborski, Filip

    2016-01-01

    To assess the quality of life (QoL) of children suffering from juvenile idiopathic arthritis (JIA) in Poland, to compare QoL of children with JIA and healthy children, and to compare children's and parents' assessments of QoL. The KIDSCREEN-52 questionnaire (children's and parents' version) was used to assess the quality of life. The QoL in JIA patients and healthy peers from European and Polish reference groups was compared by the t -test. The Bland-Altman method was used to evaluate child and parent assessment agreement. Eighty-nine questionnaires were obtained from children (median age: 14 years; 62% female; JIA history longer than 1 year) and 84 questionnaires from parents. The QoL of JIA patients was lower than in healthy peers from the European reference group in terms of physical well-being ( p < 0.001), psychological well-being ( p = 0.011), autonomy ( p < 0.001) and social support and peers ( p < 0.001). The QoL of JIA patients compared with the QoL of children from the Polish reference group was lower only in terms of physical well-being ( p < 0.001), whereas it was higher in terms of moods and emotions ( p = 0.023), parent relations and home life ( p = 0.005) and financial resources ( p < 0.001). In most terms the assessment performed by the parent was lower than the child's. The most significant differences were observed for physical well-being ( p < 0.001), psychological well-being ( p = 0.016), and self-perception ( p = 0.013). The present study is the first assessment of QoL of JIA children in Poland. In our study the quality of life in JIA children was lower than in healthy peers. Discrepancies between the assessment of the child's QoL performed by the child and the parent were found. Both assessments should be taken into account in clinical practice as well as in research studies.

  7. The comparisons between thermography and ultrasonography with physical examination for wrist joint assessment in juvenile idiopathic arthritis.

    Science.gov (United States)

    Lerkvaleekul, Butsabong; Jaovisidha, Suphaneewan; Sungkarat, Witaya; Chitrapazt, Niyata; Fuangfa, Praman; Ruangchaijatuporn, Thumanoon; Vilaiyuk, Soamarat

    2017-05-01

    This study aimed to assess infrared thermography (IRT) and ultrasonography (US) for detecting wrist arthritis in juvenile idiopathic arthritis (JIA) patients. Although IRT could help us in detecting joint inflammation, IRT studies in JIA patients with wrist arthritis are still limited. Currently, no validated US criteria exist for detecting arthritis, and the most useful parameters between gray-scale ultrasound (GSUS) or power Doppler ultrasound (PDUS) remain unclear. Forty-six JIA patients were included in this study. Detecting wrist arthritis at varying degrees using IRT and US were compared with physical examination. Sixteen patients had previous wrist arthritis that is currently inactive and 30 still had wrist arthritis. The median ages (IQR) were 7.7 (4.3) and 10.2 (4.8) years, respectively. Fifteen healthy participants were included, with a median age (IQR) of 9.2 (2.0) years. Using IRT, mean temperature (T mean ) and maximum temperature (T max ) at skin surface in the region of interest (ROI) in the arthritis group were higher than in the inactive group and the healthy controls with p  <  0.05. When patients with arthritis were subgroup analyzed by disease severity based on physical examination, the moderate to severe arthritis had T mean and T max higher than the mild arthritis group with statistical significance. The heat distribution index (HDI), two standard deviations of all pixel temperature values in the ROI, in the moderate to severe arthritis group was higher than in the healthy controls (p  =  0.027). The receiver operating characteristic analysis in arthritis detection revealed diagnostic sensitivity of 85.7% and 71.4% and specificity of 80.0% and 93.3% at cut-off points of T mean   ⩾  31.0 °C and T max   ⩾  32.3 °C, respectively. For US, GSUS and PDUS are useful in detecting arthritis, providing high sensitivity (83.3%) and specificity (81.3%). Our study demonstrated that both IRT and US were applicable tools

  8. Education and employment in patients with juvenile idiopathic arthritis - a standardized comparison to the German general population.

    Science.gov (United States)

    Schlichtiger, Jenny; Haas, Johannes-Peter; Barth, Swaantje; Bisdorff, Betty; Hager, Lisa; Michels, Hartmut; Hügle, Boris; Radon, Katja

    2017-05-22

    Although several studies show that JIA-patients have significantly lower employment rates than the general population, the research on educational and occupational attainments in patients with juvenile idiopathic arthritis (JIA) remain conflicting most likely due to small sample sizes. Therefore, aim of this study is to compare the educational achievements and employment status of 3698 JIA-patients with the German general population (GGP). "SEPIA" was a large cross-sectional study on the current status of a historic cohort of JIA-patients treated in a single center between 1952 and 2010. For the analyses of education and employment a sub-cohort was extracted, including only adult cases with a confirmed diagnosis of JIA (N = 2696). Participants were asked to fill out a standardized written questionnaire on education and employment. Outcome measures (education/unemployment) were directly standardized to the GGP using data obtained from the National Educational Panel Study 2013 (N = 11,728) and the German Unemployment Statistics 2012 of the Federal Statistical Office (N = 42,791,000). After age- and sex-standardization, 3% (95% Confidence Interval 1.9 to 4.1%) more of the JIA-patients (26%) than of the GGP (23%) had only reached primary education. In contrast, parents of JIA-patients had similar levels of education as parents in the GGP. With a standardized difference of 0.2% (95% CI: 0.16 to 0.19%), the unemployment rate in JIA-patients was slightly, but not significantly higher than in the GGP. Stratifying for disease duration and the current treatment status, differences were confirmed for persons diagnosed before 2001, whilst for patients diagnosed after 2000, differences were found only in JIA-patients with ongoing disease. Medium and high educational achievements did not differ statistically significant between JIA patients and the GPP. Educational achievements in German JIA-patients are significantly lower than in the GGP. Furthermore we were able to

  9. Evaluation of immune response to hepatitis A vaccination and vaccine safety in juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Muferet Erguven

    2011-05-01

    Conclusion: Hepatitis A vaccine was safe in patients with JIA, and response to vaccine did not differ between healthy children and patients with JIA except for children with active systemic JIA receiving anti-tumor necrosis factor alpha drugs.

  10. Prevalência de anticorpos contra peptídeos cíclicos citrulinados na artrite idiopática juvenil The prevalence of anti-cyclic citrullinated peptide antibodies in juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Sandra H. Machado

    2005-12-01

    Full Text Available OBJETIVOS: Avaliar a presença de anticorpos contra peptídeos cíclicos citrulinados em uma coorte de pacientes com artrite idiopática juvenil. MÉTODOS: A presença de anticorpos contra peptídeos cíclicos citrulinados foi avaliada por ensaio imunoenzimático (ELISA no soro de pacientes com artrite idiopática juvenil com idade inferior a 18 anos, acompanhados no ambulatório de reumatologia pediátrica do Hospital de Clínicas de Porto Alegre, com tempo de diagnóstico de doença de, no mínimo, 6 meses. Também foi estudada a presença do fator reumatóide IgM e do fator antinuclear em células Hep-2 RESULTADOS: Foram analisadas amostras séricas de 45 pacientes com artrite idiopática juvenil. A presença de títulos elevados de anticorpos contra peptídeos cíclicos citrulinados foi encontrada somente no soro de uma criança (2%, a qual apresentava quadro de poliartrite com fator reumatóide reagente. CONCLUSÕES: O anticorpo contra peptídeos cíclicos citrulinados pode ser detectado em crianças com artrite idiopática juvenil, mas em freqüência muito inferior aos adultos com artrite reumatóide. Torna-se importante avaliar se anticorpos contra peptídeos cíclicos citrulinados podem identificar os pacientes com artrite idiopática juvenil com potencial de evolução para artrite reumatóide do adulto.OBJECTIVES: To assess the presence of anti-cyclic citrullinated peptide antibodies in a cohort of patients with juvenile idiopathic arthritis. METHODS: Anti-cyclic citrullinated peptide antibodies was tested for with an enzyme linked immunoabsorbent assay (ELISA in serum samples of patients from the Hospital de Clínicas de Porto Alegre, all less than 18 years old and with previous diagnosis for at least 6 months. IgMRF (rheumatoid factor and antinuclear antibodies in Hep-2 cells were also assayed. RESULTS: Serum samples were analyzed from 45 patients. The presence of high levels of anti-cyclic citrullinated peptide antibodies was found

  11. Update on research and clinical translation on specific clinical areas: From bench to bedside: How insight in immune pathogenesis can lead to precision medicine of severe juvenile idiopathic arthritis.

    Science.gov (United States)

    Vastert, Sebastiaan; Prakken, Berent

    2014-04-01

    Despite the enormous progress in the treatment of juvenile idiopathic arthritis (JIA), innovations based on true bench-to-bedside research, performed in JIA patients, are still scarce. This chapter describes novel developments in which clinical innovations go hand in hand with basic discoveries. For the purpose of this review, we will mainly focus on developments in severe forms of JIA, most notably systemic JIA and polyarticular JIA. However, also in less severe forms of JIA, such as oligoarticular JIA, better insight will help to improve diagnosis and treatment. Facilitating the transition from bench to bedside will prove crucial for addressing the major challenges in JIA management. If successful, it will set new standards for a safe, targeted and personalized therapeutic approach for children with JIA. Copyright © 2014 Elsevier Ltd. All rights reserved.

  12. Juvenile systemic lupus erythematosus in Nigeria.

    Science.gov (United States)

    Adelowo, O O; Olaosebikan, B H; Animashaun, B A; Akintayo, R O

    2017-03-01

    Juvenile systemic lupus erythematosus (JSLE) is a complex multisystemic autoimmune disorder of unknown cause. It accounts for about one in five cases of SLE. The tendency for SLE to run a fulminant course when it starts in childhood has made JSLE a potentially more severe disease than adult SLE. Reports of JSLE from sub-Saharan Africa are scanty in spite of the increasing reports of adult SLE. We conducted a 4-year retrospective study of JSLE cases seen at the Lagos State University Teaching Hospital between January 2010 and December 2014. Out of the 12 patients studied, eight were girls and four were boys. All patients had positive antinuclear antibody and extractable nuclear antibody tests. Anti-dsDNA antibody was positive in 10 patients. Eight patients had renal disease while four patients had neuropsychiatric manifestations. Haematological abnormalities and constitutional symptoms were present in all patients. Patients were treated with pulse methylprednisolone, oral prednisolone, hydroxychloroquine and azathioprine. Three patients also received rituximab. In conclusion, JSLE exists in Nigeria and exhibits clinical and immunological characteristics similar to its pattern in other parts of the world. It is, however, diagnosed late and is possibly being underdiagnosed as there is no paediatric rheumatologist in the country.

  13. Adolescent Idiopathic Scoliosis

    Science.gov (United States)

    ... Radiation Exposure in Scoliosis Kyphosis Adolescent Back Pain Spondylolysis For Adolescents For Adults Common Questions & Glossary Resources ... Radiation Exposure in Scoliosis Kyphosis Adolescent Back Pain Spondylolysis For Adolescents For Adults Juvenile Idiopathic Scoliosis Diagnosed ...

  14. Design and acceptance of Rheumates@Work, a combined internet-based and in person instruction model, an interactive, educational, and cognitive behavioral program for children with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Armbrust, Wineke; Bos, Joyce J. F. J.; Cappon, Jeannette; van Rossum, Marion A. J. J.; Sauer, Pieter J. J.; Wulffraat, Nico; van Wijnen, Veera K.; Lelieveld, Otto T. H. M.

    2015-01-01

    Background: Juvenile idiopathic arthritis (JIA) is a chronic rheumatic disease. Patients suffer daily discomforts such as pain, fatigue, stiffness, and mood disturbances. Their exercise capacity is decreased to a variable degree and physical activity levels may be impaired. To prevent long-term

  15. Design and acceptance of Rheumates@Work, a combined internet-based and in person instruction model, an interactive, educational, and cognitive behavioral program for children with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Armbrust, Wineke; Bos, Joyce J. F. J.; Cappon, Jeannette; van Rossum, Marion A. J. J.; Sauer, Pieter J. J.; Wulffraat, Nico; van Wijnen, Veera K.; Lelieveld, Otto T. H. M.

    2015-01-01

    BACKGROUND: Juvenile idiopathic arthritis (JIA) is a chronic rheumatic disease. Patients suffer daily discomforts such as pain, fatigue, stiffness, and mood disturbances. Their exercise capacity is decreased to a variable degree and physical activity levels may be impaired. To prevent long-term

  16. Juvenile Idiopathic Arthritis

    Science.gov (United States)

    ... physical therapist about sports restrictions. Some, especially impact sports, can be hazardous to weakened joints and bones. And make sure your child eats a balanced diet that includes plenty of calcium to promote bone ...

  17. American juvenile justice system: history in the making.

    Science.gov (United States)

    Meng, Aaron; Segal, Roland; Boden, Eric

    2013-01-01

    The original theory behind separating juvenile offenders from adult offenders was to provide care and direction for youngsters instead of isolation and punishment. This idea took hold in the 19th century and became mainstream by the early 20th century. In the 1950s and 1960s, public concern grew because of a perceived lack of effectiveness and lack of rights. The Supreme Court made a series of rulings solidifying juvenile rights including the right to receive notice of charges, the right to have an attorney and the right to have charges proven beyond a reasonable doubt. In the 1980s, the public view was that the juvenile court system was too lenient and that juvenile crimes were on the rise. In the 1990s, many states passed punitive laws, including mandatory sentencing and blanket transfers to adult courts for certain crimes. As a result, the pendulum is now swinging back toward the middle from rehabilitation toward punishment.

  18. A study of school adjustment, self-concept, self-esteem, general wellbeing and parent child relationship in Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Yadav, Anita; Yadav, T P

    2013-03-01

    To assess school adjustment, self-concept, self-esteem, general wellbeing and parent-child relationship in children with Juvenile Idiopathic Arthritis (JIA)and to study the correlation of these parameters with chronicity of disease, number of active joints, laboratory parameters of disease activity and JIA subtypes. A total of 64 children (32 cases and 32 controls) were recruited for analysis. Self report questionnaires which included PGI General Wellbeing Measure, Adjustment Inventory for School Students, Parent Child Relationship Scale, Self Esteem Inventory and Self Concept Questionnaires were used to assess all the enrolled subjects. Cases had significantly lower general physical well being (p self-esteem, self-concept, adjustment in school, general wellbeing and evokes disturbed parent-child relationship.

  19. No association between vitamin D levels around time of birth and later risk of developing oligo- and polyarticular juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Thorsen, S.; Pipper, C.; Alberdi-Saugstrup, M.

    2017-01-01

    Objectives: Basic and epidemiological studies on rheumatic autoimmune diseases have suggested an association between vitamin D levels around time of birth and disease risk. The literature on vitamin D and juvenile idiopathic arthritis (JIA) is scarce. We hypothesized that low levels of 25......-hydroxyvitamin D [25(OH)D] around time of birth would be associated with increased risk of oligo- or polyarticular JIA. Method: We conducted a case–cohort study of validated cases diagnosed with oligo- and polyarticular JIA (1993–2012) and controls matched on date of birth. Cases and controls were born...... in the period 1983–2010. Cases were diagnosed using international criteria. The concentration of 25(OH)D was assessed from neonatal dried blood spot (DBS) samples using high-sensitivity liquid chromatography tandem mass spectrometry (LC-MS/MS). Odds ratios (ORs) were calculated using conditional logistic...

  20. EULAR-PReS points to consider for the use of imaging in the diagnosis and management of juvenile idiopathic arthritis in clinical practice

    DEFF Research Database (Denmark)

    Colebatch-Bourn, A N; Edwards, C J; Collado, P

    2015-01-01

    and patients from nine countries. Eleven questions on imaging in JIA were generated using a process of discussion and consensus. Research evidence was searched systematically for each question using MEDLINE, EMBASE and Cochrane CENTRAL. Imaging modalities included were conventional radiography, ultrasound, MRI...... the search process, from which 204 studies were included in the systematic review. Nine points to consider were produced, taking into account the heterogeneity of JIA, the lack of normative data and consequent difficulty identifying pathology. These encompassed the role of imaging in making a diagnosis......To develop evidence based points to consider the use of imaging in the diagnosis and management of juvenile idiopathic arthritis (JIA) in clinical practice. The task force comprised a group of paediatric rheumatologists, rheumatologists experienced in imaging, radiologists, methodologists...

  1. Health-related quality of life among Swedish children with Juvenile Idiopathic Arthritis: parent-child discrepancies, gender differences and comparison with a European cohort.

    Science.gov (United States)

    Lundberg, Veronica; Eriksson, Catharina

    2017-04-12

    This study investigates gender differences in self-reports and between parent and child reports in Health-related Quality of Life (HRQOL), measured with disease-specific and generic instruments for chronic disease. Comparison of HRQOL results in this Juvenile Idiopathic Arthritis (JIA) sample to a European cohort of children with JIA and one of children with other health conditions are also made. Fifty-three children with juvenile idiopathic arthritis (JIA), aged 8-18 years, and their parents completed the condition-specific DISABKIDS for JIA, and the DISABKIDS generic instrument for chronic conditions (DCGM-37) in a cross-sectional study. European reference data were used for comparison of child and parental reports. Child self-reports in DCGM-37 and DISABKIDS for JIA showed no gender differences. Parental and child reports of the child's HRQOL differed only in DCGM-37; this was among girls who scored their independence (p = 0.03), physical limitation (p = 0.01), social exclusion (p = 0.03), emotions (p children with JIA reported more physical limitation compared to samples of European children with JIA (p = 0.01), European children with chronic conditions (p children reported more problem with understanding compared to the European JIA sample (p = 0.03). Swedish parents perceived their children's independence significantly lower than did the European parents of JIA children (p children with chronic conditions (p = 0.03). The Swedish parents also perceived their children to have significantly lower social inclusion (p children with chronic conditions. Parent-child differences in assessment of quality of life depend on the HRQOL instrument used, especially among girls. In comparison to European cohorts, our sample of children with JIA experienced more physical limitations and less understanding.

  2. Abnormal Vasomotor System Function in Idiopathic Generalized Epileptic Patients

    Directory of Open Access Journals (Sweden)

    Mehdi Maghbooli

    2014-12-01

    Full Text Available OBJECTIVE: Autonomic dysfunction is widely recognized in both partial and generalized epilepsies. The aim of this study was to evaluate the vasomotor response in patients with generalized idiopathic epilepsy by the clinical autonomic function tests. METHODS: 124 consecutive subjects including 62 idiopathic generalized epileptic patients diagnosed for more than 3 months receiving monotherapy and 62 matched for sex and age healthy controls were assessed in this case-control study. The evaluation of the vasomotor system was made using a questionnaire and autonomic function tests including Cold pressor, Valsalva maneuver, mental arithmetic and hand-grip tests. RESULTS: Abnormal score of the Cold pressor test was seen in 59.7%, Valsalva maneuver in 64.5% and 33.9% in mental arithmetic test of epileptic patients. These results were different significantly in comparison to control group. If abnormal hand grip test was defined as an increase less than 11 mmHg in diastolic pressure, there was found no significant difference between two groups of case and control, also a few people in control group was reported normal. While defining abnormal test was interpreted by increase in only one parameter or none of systolic blood pressure, diastolic blood pressure or heart rate, 43.5% in case group versus 14.5% in controls had abnormal results with significantly difference. CONCLUSION: Vasomotor dysfunction evaluated by cardiovascular tests was present commonly in the patients with generalized epilepsy. Further confirmation requires detailed reviewing of central and peripheral limbs to the breakdown of the system to be felt.

  3. Disease severity staging system for idiopathic pulmonary fibrosis in Japan.

    Science.gov (United States)

    Kondoh, Yasuhiro; Taniguchi, Hiroyuki; Kataoka, Kensuke; Furukawa, Taiki; Ando, Masahiko; Murotani, Kenta; Mishima, Michiaki; Inoue, Yoshikazu; Ogura, Takashi; Bando, Masashi; Hagiwara, Koichi; Suda, Takafumi; Chiba, Hirofumi; Takahashi, Hiroki; Sugiyama, Yukihiko; Homma, Sakae

    2017-11-01

    In Japan, the classification of disease severity of idiopathic pulmonary fibrosis (IPF) (J-system) has been used in making decisions on medical care subsidies. The present J-system consists of arterial partial pressure of oxygen (PaO 2 ) and exercise desaturation in stages of I-IV. It provides a good prognostic classification in stages III and IV, but not in stages I and II. Therefore, we propose a revised system to improve discriminative ability in stages I and II. We compared the revised J-system with the present J-system using Cox proportional hazards model to predict mortality rate. We also evaluated the recently proposed GAP (Gender, Age and Physiology) system in comparison to both J-systems. Two-hundred and fifteen IPF patients were studied retrospectively. A univariate model showed that the present and revised J-systems and a modified GAP system were all significant prognostic factors. The C-statistic for discriminating prognosis was higher in the revised J-system than the modified GAP system and the present J-system (0.677, 0.652 and 0.659, respectively). The C-statistics of these models produced from the 10 000 bootstrap samples were similar to those of the original models, suggesting good internal validation (0.665 (95% CI: 0.621-0.705), 0.645 (0.600-0.686) and 0.659 (0.616-0.700), respectively). Multivariate analysis revealed that the revised J-system (P = 0.0038) and the modified GAP system (P = 0.0029) were independent prognostic factors. The revised J-system can provide a better mortality prediction than the present one. Both the revised J-system and the modified GAP system are independent and valuable tools for prognostication and clinical management for IPF. © 2017 Asian Pacific Society of Respirology.

  4. PHARMACOECONOMIC ASPECTS OF TREATMENT WITH THE INHIBITORS OF TUMOR NECROSIS FACTOR OF THE CHRONIC UVEITIS REFRACTORY TO THE BASIC THERAPY (INCLUDING AN ASSOCIATED WITH JUVENILE IDIOPATHIC ARTHRITIS

    Directory of Open Access Journals (Sweden)

    A.V. Rudakova

    2011-01-01

    Full Text Available Therapy of chronic uveitis refractory to the basic treatment, in juvenile idiopathic arthritis (JIA is a very complex problem in pediatrics. Substantial progress in this area resulted after the implementation in practice of inhibitors of tumor necrosis factor (TNF, as the most effective in such clinical situation drugs adalimumab and infliximab are considered (although infliximab was not officially approved in JIA. Objective. To estimate the cost effectiveness of TNF inhibitors — adalimumab, and infliximab in chronic uveitis, refractory to the basic therapy (including associated with juvenile rheumatoid arthritis. Methods. A modeling on the basis of a comparative prospective cohort clinical study was carried out. The analysis was performed by the method «cost–effectiveness» from a position of health and social accounting perspective. Results. It was shown that the frequency and time of remission did not differ when treatment with infliximab (5 mg/kg at 0–2–6 weeks and further once in 6–8 weeks and adalimumab (24 mg/m2 once in 2 weeks. Adalimumab provides a long-term maintenance of remission (no recurrence in 60% of patients within 40 months of observation, whereas 1 year after the treatment with infliximab the frequency of exacerbations was returned to that observed before therapy. The proportion of patients without relapse in the treatment with infliximab for 40 months was 18.8%. Similar results were obtained in a subset of patients with chronic uveitis associated with JIA (with follow-up of 20 months of in a group of infliximab number patients without relapse was 11.1%, with adalimumab therapy — 63.6%. In the general population of patients with refractory chronic uveitis the factor «cost–effectiveness» calculated for a patient with the maintenance of remission for 3 years with adalimumab therapy was in 2,1–2,8 times less than in the treatment with infliximab. In chronic uveitis associated with JIA, the coefficient of

  5. Development of a national audit tool for juvenile idiopathic arthritis: a BSPAR project funded by the Health Care Quality Improvement Partnership.

    Science.gov (United States)

    McErlane, Flora; Foster, Helen E; Armitt, Gillian; Bailey, Kathryn; Cobb, Joanna; Davidson, Joyce E; Douglas, Sharon; Fell, Andrew; Friswell, Mark; Pilkington, Clarissa; Strike, Helen; Smith, Nicola; Thomson, Wendy; Cleary, Gavin

    2018-01-01

    Timely access to holistic multidisciplinary care is the core principle underpinning management of juvenile idiopathic arthritis (JIA). Data collected in national clinical audit programmes fundamentally aim to improve health outcomes of disease, ensuring clinical care is equitable, safe and patient-centred. The aim of this study was to develop a tool for national audit of JIA in the UK. A staged and consultative methodology was used across a broad group of relevant stakeholders to develop a national audit tool, with reference to pre-existing standards of care for JIA. The tool comprises key service delivery quality measures assessed against two aspects of impact, namely disease-related outcome measures and patient/carer reported outcome and experience measures. Eleven service-related quality measures were identified, including those that map to current standards for commissioning of JIA clinical services in the UK. The three-variable Juvenile Arthritis Disease Activity Score and presence/absence of sacro-iliitis in patients with enthesitis-related arthritis were identified as the primary disease-related outcome measures, with presence/absence of uveitis a secondary outcome. Novel patient/carer reported outcomes and patient/carer reported experience measures were developed and face validity confirmed by relevant patient/carer groups. A tool for national audit of JIA has been developed with the aim of benchmarking current clinical practice and setting future standards and targets for improvement. Staged implementation of this national audit tool should facilitate investigation of variability in levels of care and drive quality improvement. This will require engagement from patients and carers, clinical teams and commissioners of JIA services. © The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology.

  6. Pixel-by-pixel analysis of DCE-MRI curve shape patterns in knees of active and inactive juvenile idiopathic arthritis patients

    International Nuclear Information System (INIS)

    Hemke, Robert; Lavini, Cristina; Maas, Mario; Nusman, Charlotte M.; Berg, J.M. van den; Schonenberg-Meinema, Dieneke; Kuijpers, Taco W.; Dolman, Koert M.; Rossum, Marion A.J. van

    2014-01-01

    To compare DCE-MRI parameters and the relative number of time-intensity curve (TIC) shapes as derived from pixel-by-pixel DCE-MRI TIC shape analysis between knees of clinically active and inactive juvenile idiopathic arthritis (JIA) patients. DCE-MRI data sets were prospectively obtained. Patients were classified into two clinical groups: active disease (n = 43) and inactive disease (n = 34). Parametric maps, showing seven different TIC shape types, were created per slice. Statistical measures of different TIC shapes, maximal enhancement (ME), maximal initial slope (MIS), initial area under the curve (iAUC), time-to-peak (TTP), enhancing volume (EV), volume transfer constant (K trans ), extravascular space fractional volume (V e ) and reverse volume transfer constant (k ep ) of each voxel were calculated in a three-dimensional volume-of-interest of the synovial membrane. Imaging findings from 77 JIA patients were analysed. Significantly higher numbers of TIC shape 4 (P = 0.008), median ME (P = 0.015), MIS (P = 0.001) and iAUC (P = 0.002) were observed in clinically active compared with inactive patients. TIC shape 5 showed higher presence in the clinically inactive patients (P = 0.036). The pixel-by-pixel DCE-MRI TIC shape analysis method proved capable of differentiating clinically active from inactive JIA patients by the difference in the number of TIC shapes, as well as the descriptive parameters ME, MIS and iAUC. (orig.)

  7. Assessment of the Therapeutic Effect of Total Glucosides of Peony for Juvenile Idiopathic Arthritis: A Systematic Review and Meta-Analysis

    Directory of Open Access Journals (Sweden)

    Yongsong Cai

    2016-01-01

    Full Text Available Juvenile idiopathic arthritis (JIA is the most common rheumatic disease in children; some clinical trials have reported the effects of total glucosides of peony (TGP in the treatment of JIA. However, no systematic review has yet been conducted. In this study, we assessed the efficacy and safety in patients with JIA enrolled in randomized controlled trials (RCTs of TGP. We extracted data for studies searched from 8 electronic databases that were searched and also evaluated the methodological quality of the included studies. We assessed the following outcome measures: overall response rate, pain, tender joint count (TJC, swollen joint count (SJC, duration of morning stiffness (DMS, grip strength (GS, rheumatoid factor (RF, erythrocyte sedimentation rate (ESR, C-reactive protein (CRP, and adverse effects (AEs in short term (4–8 weeks, intermediate term (9–26 weeks, and long term (>26 weeks. The final analysis showed that TGP acted as a unique nonbiologic disease-modifying antirheumatic drug (nonbiologic DMARD, and its therapeutic effects were safe and efficacious for the treatment of JIA with few AEs. However, more high-quality RCTs are needed to confirm these therapeutic effects.

  8. Assessment of the Therapeutic Effect of Total Glucosides of Peony for Juvenile Idiopathic Arthritis: A Systematic Review and Meta-Analysis

    Science.gov (United States)

    Cai, Yongsong; Yuan, Qiling; Xu, Ke; Zhu, Jialin; Li, Yuanbo; Wu, Xiaoqing; Yang, Le

    2016-01-01

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children; some clinical trials have reported the effects of total glucosides of peony (TGP) in the treatment of JIA. However, no systematic review has yet been conducted. In this study, we assessed the efficacy and safety in patients with JIA enrolled in randomized controlled trials (RCTs) of TGP. We extracted data for studies searched from 8 electronic databases that were searched and also evaluated the methodological quality of the included studies. We assessed the following outcome measures: overall response rate, pain, tender joint count (TJC), swollen joint count (SJC), duration of morning stiffness (DMS), grip strength (GS), rheumatoid factor (RF), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and adverse effects (AEs) in short term (4–8 weeks), intermediate term (9–26 weeks), and long term (>26 weeks). The final analysis showed that TGP acted as a unique nonbiologic disease-modifying antirheumatic drug (nonbiologic DMARD), and its therapeutic effects were safe and efficacious for the treatment of JIA with few AEs. However, more high-quality RCTs are needed to confirm these therapeutic effects. PMID:27525026

  9. Ultrasonography and color Doppler in juvenile idiopathic arthritis: diagnosis and follow-up of ultrasound-guided steroid injection in the wrist region. A descriptive interventional study

    Directory of Open Access Journals (Sweden)

    Laurell Louise

    2012-04-01

    Full Text Available Abstract Background The wrist region is one of the most complex joints of the human body. It is prone to deformity and functional impairment in juvenile idiopathic arthritis (JIA, and is difficult to examine clinically. The aim of this study was to evaluate the role of ultrasonography (US with Doppler in diagnosis of synovitis, guidance of steroid injections, and follow-up examinations of the wrist in JIA. Methods In 11 patients (median age 12.5 years, range 2-16, 15 wrists with clinically active arthritis were assessed clinically by US and color Doppler (Logiq 9, GE, 16-4 MHz linear transducer prior to and 1 and 4 weeks after US-guided steroid injection. Results US detected synovitis in the radio-carpal joints, the midcarpal joints, and the tendon sheaths in 87%, 53% and 33% of the wrists, respectively. Multiple compartments were involved in 67%. US-guidance allowed accurate placement of steroid in all 21 injected compartments, with a low rate of subcutaneous atrophy. Synovial hypertrophy was normalized in 86% of the wrists, hyperemia in 91%, and clinically active arthritis in 80%. Conclusions US enabled detection of synovial inflammation in compartments that are difficult to evaluate clinically and exact guidance of injections, and it was valuable for follow-up examinations. Normalization of synovitis was achieved in most cases, which supports the notion that US is an important tool in management of wrist involvement in JIA.

  10. Clinical Orofacial Examination in Juvenile Idiopathic Arthritis: International Consensus-based Recommendations for Monitoring Patients in Clinical Practice and Research Studies.

    Science.gov (United States)

    Stoustrup, Peter; Twilt, Marinka; Spiegel, Lynn; Kristensen, Kasper Dahl; Koos, Bernd; Pedersen, Thomas Klit; Küseler, Annelise; Cron, Randy Q; Abramowicz, Shelly; Verna, Carlalberta; Peltomäki, Timo; Alstergren, Per; Petty, Ross; Ringold, Sarah; Nørholt, Sven Erik; Saurenmann, Rotraud K; Herlin, Troels

    2017-03-01

    To develop international consensus-based recommendations for the orofacial examination of patients with juvenile idiopathic arthritis (JIA), for use in clinical practice and research. Using a sequential phased approach, a multidisciplinary task force developed and evaluated a set of recommendations for the orofacial examination of patients with JIA. Phase 1: A Delphi survey was conducted among 40 expert physicians and dentists with the aim of identifying and ranking the importance of items for inclusion. Phase 2: The task force developed consensus about the domains and items to be included in the recommendations. Phase 3: A systematic literature review was performed to assess the evidence supporting the consensus-based recommendations. Phase 4: An independent group of orofacial and JIA experts were invited to assess the content validity of the task force's recommendations. Five recommendations were developed to assess the following 5 domains: medical history, orofacial symptoms, muscle and temporomandibular joint function, orofacial function, and dentofacial growth. After application of data search criteria, 56 articles were included in the systematic review. The level of evidence for the 5 recommendations was derived primarily from descriptive studies, such as cross-sectional and case-control studies. Five recommendations are proposed for the orofacial examination of patients with JIA to improve the clinical practice and aid standardized data collection for future studies. The task force has formulated a future research program based on the proposed recommendations.

  11. Idiopathic inflammatory-demyelinating diseases of the central nervous system

    Energy Technology Data Exchange (ETDEWEB)

    Rovira Canellas, A. [Vall d' Hebron University Hospital, Magnetic Resonance Unit (I.D.I.), Department of Radiology, Barcelona (Spain); Rovira Gols, A. [Parc Tauli University Institute - UAB, UDIAT, Diagnostic Centre, Sabadell (Spain); Rio Izquierdo, J.; Tintore Subirana, M.; Montalban Gairin, X. [Vall d' Hebron University Hospital, Neuroimmunology Unit, Department of Neurology, Barcelona (Spain)

    2007-05-15

    Idiopathic inflammatory-demyelinating diseases (IIDDs) include a broad spectrum of central nervous system disorders that can usually be differentiated on the basis of clinical, imaging, laboratory and pathological findings. However, there can be a considerable overlap between at least some of these disorders, leading to misdiagnoses or diagnostic uncertainty. The relapsing-remitting and secondary progressive forms of multiple sclerosis (MS) are the most common IIDDs. Other MS phenotypes include those with a progressive course from onset (primary progressive and progressive relapsing) or with a benign course continuing for years after onset (benign MS). Uncommon forms of IIDDs can be classified clinically into: (1) fulminant or acute IIDDs, such as the Marburg variant of MS, Balo's concentric sclerosis, Schilder's disease, and acute disseminated encephalomyelitis; (2) monosymptomatic IIDDs, such as those involving the spinal cord (transverse myelitis), optic nerve (optic neuritis) or brainstem and cerebellum; and (3) IIDDs with a restricted topographical distribution, including Devic's neuromyelitis optica, recurrent optic neuritis and relapsing transverse myelitis. Other forms of IIDD, which are classified clinically and radiologically as pseudotumoral, can have different forms of presentation and clinical courses. Although some of these uncommon IIDDs are variants of MS, others probably correspond to different entities. MR imaging of the brain and spine is the imaging technique of choice for diagnosing these disorders, and together with the clinical and laboratory findings can accurately classify them. Precise classification of these disorders may have relevant prognostic and treatment implications, and might be helpful in distinguishing them from tumoral or infectious lesions, avoiding unnecessary aggressive diagnostic or therapeutic procedures. (orig.)

  12. Idiopathic inflammatory-demyelinating diseases of the central nervous system

    International Nuclear Information System (INIS)

    Rovira Canellas, A.; Rovira Gols, A.; Rio Izquierdo, J.; Tintore Subirana, M.; Montalban Gairin, X.

    2007-01-01

    Idiopathic inflammatory-demyelinating diseases (IIDDs) include a broad spectrum of central nervous system disorders that can usually be differentiated on the basis of clinical, imaging, laboratory and pathological findings. However, there can be a considerable overlap between at least some of these disorders, leading to misdiagnoses or diagnostic uncertainty. The relapsing-remitting and secondary progressive forms of multiple sclerosis (MS) are the most common IIDDs. Other MS phenotypes include those with a progressive course from onset (primary progressive and progressive relapsing) or with a benign course continuing for years after onset (benign MS). Uncommon forms of IIDDs can be classified clinically into: (1) fulminant or acute IIDDs, such as the Marburg variant of MS, Balo's concentric sclerosis, Schilder's disease, and acute disseminated encephalomyelitis; (2) monosymptomatic IIDDs, such as those involving the spinal cord (transverse myelitis), optic nerve (optic neuritis) or brainstem and cerebellum; and (3) IIDDs with a restricted topographical distribution, including Devic's neuromyelitis optica, recurrent optic neuritis and relapsing transverse myelitis. Other forms of IIDD, which are classified clinically and radiologically as pseudotumoral, can have different forms of presentation and clinical courses. Although some of these uncommon IIDDs are variants of MS, others probably correspond to different entities. MR imaging of the brain and spine is the imaging technique of choice for diagnosing these disorders, and together with the clinical and laboratory findings can accurately classify them. Precise classification of these disorders may have relevant prognostic and treatment implications, and might be helpful in distinguishing them from tumoral or infectious lesions, avoiding unnecessary aggressive diagnostic or therapeutic procedures. (orig.)

  13. Nymphal RNAi: systemic RNAi mediated gene knockdown in juvenile grasshopper

    Directory of Open Access Journals (Sweden)

    Dong Ying

    2005-10-01

    Full Text Available Abstract Background Grasshopper serves as important model system in neuroscience, development and evolution. Representatives of this primitive insect group are also highly relevant targets of pest control efforts. Unfortunately, the lack of genetics or gene specific molecular manipulation imposes major limitations to the study of grasshopper biology. Results We investigated whether juvenile instars of the grasshopper species Schistocerca americana are conducive to gene silencing via the systemic RNAi pathway. Injection of dsRNA corresponding to the eye colour gene vermilion into first instar nymphs triggered suppression of ommochrome formation in the eye lasting through two instars equivalent to 10–14 days in absolute time. QRT-PCR analysis revealed a two fold decrease of target transcript levels in affected animals. Control injections of EGFP dsRNA did not result in detectable phenotypic changes. RT-PCR and in situ hybridization detected ubiquitous expression of the grasshopper homolog of the dsRNA channel protein gene sid-1 in embryos, nymphs and adults. Conclusion Our results demonstrate that systemic dsRNA application elicits specific and long-term gene silencing in juvenile grasshopper instars. The conservation of systemic RNAi in the grasshopper suggests that this pathway can be exploited for gene specific manipulation of juvenile and adult instars in a wide range of primitive insects.

  14. Comparison of magnetic resonance imaging signs and clinical findings in follow-up examinations in children and juveniles with temporomandibular joint involvement in juvenile idiopathic arthritis

    International Nuclear Information System (INIS)

    Mussler, A.; Schroeder, R.J.; Allozy, B.; Landau, H.; Kallinich, T.; Trauzeddel, R.

    2010-01-01

    The aim of this study was to analyze the extent to which pathological findings of temporomandibular joint (TMJ) in magnetic resonance imaging (MRI) follow-up examinations are correlated with clinical symptoms in patients with TMJ involvement in juvenile ideopathic arthritis (JIA) over time. Data from 34 patients with TMJ involvement in JIA was retrospectively examined. Shortly after two clinical examinations, the first MRI and the follow-up MRI were performed. The MRI examinations took place with 1.5 T MRI. In both MRI examinations alterations on the condyle (MRI1: 88 %, MRT2: 91 %) and contrast enhancement (MRT1: 76 %, MRT2 65 %) were found most frequently. TMJ pain (65 %) and lower mouth opening capacity (65 %) were the number one finding in the first clinical examination. A statistically significant correlation was found between the alterations on the condyle and TMJ pain (p = 0.025) and between the alterations on the condyle and lower mouth opening capacity (p = 0.019). By comparing the results of the first MRI with the results of the follow-up MRI, we identified a trend towards a progression of TMJ arthritis, while the clinical follow-up showed an improvement in most patients. We found a discrepancy between the progressive or stable trends of pathological findings in follow-up MRI and the decrease in clinical symptoms over time. Therefore, follow-up examination by MRI shows important information for correct evaluation about the stage of TMJ arthritis and about the need for treatment. Consequently, follow-up examination by MRI is an appropriate addition to clinical examination in the therapeutic concept. (orig.)

  15. Police restorative approach in the juvenile justice system

    Directory of Open Access Journals (Sweden)

    Stefanovska Vesna

    2010-01-01

    Full Text Available Restorative justice is a new, different response to crime, response that offers and tries to establish justice again. Not going into aims, impact and basic principles of restorative justice, as well as into substance of different restorative practices, in this article we will concentrate on restorative interventions that lead to avoidance of the formal justice system. Concretely, we will analyze the role of the police in applying restorative interventions in the juvenile justice system. Particular emphasis will be put on the meaning and the aim of diversion procedures towards juvenile offenders that have committed minor offences and more serious ones for which they come in conflict with the law. In the foreign expert literature the concept of restorative policing is recognized (restorative approach in police conduct, as an attempt to introduce a new reform in performing police affairs. This subject should be approached very carefully and fundamentally, if we want consistent implementation of the new tendency and practices in the juvenile justice systems in accordance with the international standards.

  16. Just Learning: The Imperative to Transform Juvenile Justice Systems into Effective Educational Systems. A Study of Juvenile Justice Schools in the South and the Nation. Special Summary

    Science.gov (United States)

    Southern Education Foundation, 2014

    2014-01-01

    This brief summarizes the findings of the larger study, "Just Learning: The Imperative to Transform Juvenile Justice Systems into Effective Educational Systems. A Study of Juvenile Justice Schools in the South and the Nation." With awareness growing that schools are disciplining and suspending minority students at alarming rates, the…

  17. Low rate of surgery in juvenile idiopathic scoliosis treated with a complete and tailored conservative approach: end-growth results from a retrospective cohort.

    Science.gov (United States)

    Fusco, Claudia; Donzelli, Sabrina; Lusini, Monia; Salvatore, Minnella; Zaina, Fabio; Negrini, Stefano

    2014-01-01

    The main distinctive aspect of Juvenile Idiopathic Scoliosis (JIS) with respect to Adolescent Idiopathic Scoliosis (AIS) is the high risk of severe deformity and surgery. Approximately 70% of curves in patients with JIS progress and ultimately require surgery. There are presently very few studies with long-term follow-up of JIS and even fewer looking specifically at bracing Purpose To verify the effectiveness of a complete conservative treatment, including bracing and exercises, for JIS. Retrospective cohort observational study nested in a clinical prospective database of consecutive outpatients. Patient Sample Inclusion criteria: JIS, no previous treatment, all consecutive radiographies available from treatment start to end of growth (Risser sign 3). We found 30 patients, 27 females, 10 JIS type 1; mean age at first diagnosis was 7.8 +/-1.5 and mean treatment lasted 5.8 years. Cobb degrees 24.4+/-10 degrees, with 7 cases >30 degrees, and 2 > 45degrees. Outcome Measures Physiological measures. Radiographic and clinical data. Treatment (exercises alone, or elastic-rigid-highly rigid braces plus exercises) was tailored and continuously changed according to Cobb degrees, individual preferences, anthropometric characteristics, pubertal spurt, remaining growth, rotation, hump, lumbar curve take-off, and imbalance. The SOSORT Guidelines for patients' management have been followed. Funding and Conflict of Interest: no. 33.3% (95% Confidence Interval 16.4-50.2%) of patients worsened over the years. At the end of growth, 6.6% (0-15.5%) had surgical deformities (>45degrees). We observed a good correction in the first years of treatment until pubertal growth spurt, when progression was usually noted and treatment changed increasing corrective forces (hours or rigidity of bracing). 23 cases were followed up until they had two consecutive radiographies showing Risser sign 5 and showed stability. Conservative treatment initiated already in childhood may favorably change the

  18. Early prediction for over two years efficacy of the first biologic agent for polyarticular juvenile idiopathic arthritis: A multi-institutional study in Japan.

    Science.gov (United States)

    Kubota, Tomohiro; Takei, Syuji; Shimizu, Masaki; Yasumura, Junko; Nakagishi, Yasuo; Kizawa, Toshitaka; Yashiro, Masato; Wakiguchi, Hiroyuki; Yamasaki, Yuichi; Kawano, Yoshifumi

    2018-01-02

    To estimate target of treatment for long-term efficacy of the first biologic agent used to treat polyarticular juvenile idiopathic arthritis (pJIA). A retrospective cohort of patients with pJIA treated at six medical institutions in Japan between 1 March 2005 and 31 October 2014 was identified. The patients were divided by 2-year treatment periods with the first biologic agent into continuous treatment group and switching group. Three markers were examined: matrix metalloproteinase-3 (MMP-3), erythrocyte sedimentation rate (ESR), and disease activity score (DAS) 28-ESR. Thirty-two pJIA patients (8 boys, 24 girls) from 43 recruited patients were included in this study. The treatment periods with the first biologic agent in continuous treatment group (24 patients, 75%) was 40 months (median, range 24-119) and switching group (8 patients; 25%) was 9.5 months (median, 6-18). Markers [odds ratio (95% confidence interval)] at 3 months were MMP-3 [1.02 (0.99-1.05), p = .219], ESR [1.00 (0.78-1.30), p = .998], and DAS28-ESR [13.9 (2.08-409.82), p = .035]. The cut-off point for DAS28-ESR at 3 months to distinguish the two groups was 2.49 (sensitivity, 87.5%; specificity, 87.5%). DAS28-ESR of 2.49 at 3 months after initiating the first biologic agent can be a target of sustained treatment in pJIA patients.

  19. Ultrasonography and color Doppler in juvenile idiopathic arthritis: diagnosis and follow-up of ultrasound-guided steroid injection in the ankle region. A descriptive interventional study

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    Boesen Mikael

    2011-01-01

    Full Text Available Abstract Background The ankle region is frequently involved in juvenile idiopathic arthritis (JIA but difficult to examine clinically due to its anatomical complexity. The aim of the study was to evaluate the role of ultrasonography (US of the ankle and midfoot (ankle region in JIA. Doppler-US detected synovial hypertrophy, effusion and hyperemia and US was used for guidance of steroid injection and to assess treatment efficacy. Methods Forty swollen ankles regions were studied in 30 patients (median age 6.5 years, range 1-16 years with JIA. All patients were assessed clinically, by US (synovial hypertrophy, effusion and by color Doppler (synovial hyperemia before and 4 weeks after US-guided steroid injection. Results US detected 121 compartments with active disease (joints, tendon sheaths and 1 ganglion cyst. Multiple compartments were involved in 80% of the ankle regions. The talo-crural joint, posterior subtalar joint, midfoot joints and tendon sheaths were affected in 78%, 65%, 30% and 55% respectively. Fifty active tendon sheaths were detected, and multiple tendons were involved in 12 of the ankles. US-guidance allowed accurate placement of the corticosteroid in all 85 injected compartments, with a low rate of subcutaneous atrophy (4,7%. Normalization or regression of synovial hypertrophy was obtained in 89%, and normalization of synovial hyperemia in 89%. Clinical resolution of active arthritis was noted in 72% of the ankles. Conclusions US enabled exact anatomical location of synovial inflammation in the ankle region of JIA patients. The talo-crural joint was not always involved. Disease was frequently found in compartments difficult to evaluate clinically. US enabled exact guidance of steroid injections, gave a low rate of subcutaneous atrophy and was proved valuable for follow-up examinations. Normalization or regression of synovial hypertrophy and hyperemia was achieved in most cases, which supports the notion that US is an important

  20. Evidence for Updating the Core Domain Set of Outcome Measures for Juvenile Idiopathic Arthritis: Report from a Special Interest Group at OMERACT 2016.

    Science.gov (United States)

    Morgan, Esi M; Riebschleger, Meredith P; Horonjeff, Jennifer; Consolaro, Alessandro; Munro, Jane E; Thornhill, Susan; Beukelman, Timothy; Brunner, Hermine I; Creek, Emily L; Harris, Julia G; Horton, Daniel B; Lovell, Daniel J; Mannion, Melissa L; Olson, Judyann C; Rahimi, Homaira; Gallo, Maria Chiara; Calandra, Serena; Ravelli, Angelo; Ringold, Sarah; Shenoi, Susan; Stinson, Jennifer; Toupin-April, Karine; Strand, Vibeke; Bingham, Clifton O

    2017-12-01

    The current Juvenile Idiopathic Arthritis (JIA) Core Set was developed in 1997 to identify the outcome measures to be used in JIA clinical trials using statistical and consensus-based techniques, but without patient involvement. The importance of patient/parent input into the research process has increasingly been recognized over the years. An Outcome Measures in Rheumatology (OMERACT) JIA Core Set Working Group was formed to determine whether the outcome domains of the current core set are relevant to those involved or whether the core set domains should be revised. Twenty-four people from the United States, Canada, Australia, and Europe, including patient partners, formed the working group. Guided by the OMERACT Filter 2.0 process, we performed (1) a systematic literature review of outcome domains, (2) a Web-based survey (142 patients, 343 parents), (3) an idea-generation study (120 parents), (4) 4 online discussion boards (24 patients, 20 parents), and (5) a Special Interest Group (SIG) activity at the OMERACT 13 (2016) meeting. A MEDLINE search of outcome domains used in studies of JIA yielded 5956 citations, of which 729 citations underwent full-text review, and identified additional domains to those included in the current JIA Core Set. Qualitative studies on the effect of JIA identified multiple additional domains, including pain and participation. Twenty-one participants in the SIG achieved consensus on the need to revise the entire JIA Core Set. The results of qualitative studies and literature review support the need to expand the JIA Core Set, considering, among other things, additional patient/parent-centered outcomes, clinical data, and imaging data.

  1. Idiopathic Thrombocytopenic Purpura as Initial Manifestation of Systemic Lupus Erythematosus: A Case Report

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    Fatih Bağcıer

    2016-08-01

    Full Text Available Systemic lupus erythematosus (SLE is a chronic autoimmune disease that can affect almost any organ system. Its presentation and course are highly variable. The classic presentation of SLE in a woman of childbearing age is fever, arthritis, and malar rash triad. However, patients may present rare types of manifestations such as idiopathic thrombocytopenic purpura.

  2. EFFECTIVENESS OF INFLIXIMAB IN PATIENT WITH SYSTEMIC TYPE OF JUVENILE RHEUMATOID ARTHRITIS

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    E.S. Zholobova

    2011-01-01

    Full Text Available The article presents the case report of a child with juvenile rheumatoid arthritis, systemic type. The efficacy of infliximab (Remicade in treatment of systemic types of juvenile rheumatoid arthritis (JRA is analyzed. Treatment of JRA with high doses of prednisolone is inacceptable due to the risk of severe adverse events.Key words: juvenile rheumatoid arthritis, systemic type, infliximab, prednisolone, treatment.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2011; 10 (2: 201–204

  3. Idiopathic thrombosis.

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    Tsuda, Hiroko

    2017-01-01

    Idiopathic thrombosis involves a group of inherited thrombophilia predisposed to severe thrombosis of early onset and associated with an adverse outcome due to recurrence, and therefore, requires long-term anticoagulation therapy. The causative factors of a predisposition to thrombosis include immobility, dehydration, infection, surgery, injury, cancer, pregnancy, and estrogen use. The inherited deficiencies of antithrombin (AT), protein C (PC), and protein S (PS) are specified as "Specific Pediatric Chronic Diseases." However, medical expense assistance for patients terminates when they reach the age of 20 years. On April 1 st 2017, "Idiopathic Thrombosis due to Inherited Thrombophilia," consisting of inherited AT, PC, and PS deficiencies, was specified as an "Intractable Disease," and aid for medical expenses became available. Accordingly, progress in the research and practice of idiopathic thrombosis is expected in future to improve the medical care system and to construct a database via clinical surveys.

  4. Experience of the Tocilizumab Application in Systemic Onset Juvenile Arthritis

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    A. V. Krasnopol’skaja

    2015-01-01

    Full Text Available The article provides information on the unfavourable course of systemic onset juvenile arthritis, resistant to immunosuppressive therapy with methotrexate in combination with cyclosporine, and pulse therapy with methylprednisolone and methotrexate. We describe the successful use of genetically engineered biological drug tocilizumab in the patient with systemic onset juvenile arthritis. After the first injection, pain was already significantly reduced; after the second, fever was relieved and non-steroid anti-inflammatory drugs were cancelled; after the third, lymphadenopathy and splenomegaly disappeared and the child’s functional activity improved significantly. After 12 months of treatment, an inactive phase of the disease was achieved, the joints’ kinetics (with the exception of the right hip were almost entirely restored and the patient’s quality of life had significantly improved. At the same time, metabolic disorders and changes in the cardiovascular system were reversed. This example demonstrated the high effectiveness of interleukin-6 antagonist tocilizumab in systemic arthritis, which allowed arresting joint affection as well as extra-articular manifestations of the disease, providing normal puberty, the restoration of growth and sexual development.

  5. Is hydrotherapy cost-effective? A randomised controlled trial of combined hydrotherapy programmes compared with physiotherapy land techniques in children with juvenile idiopathic arthritis.

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    Epps, H; Ginnelly, L; Utley, M; Southwood, T; Gallivan, S; Sculpher, M; Woo, P

    2005-10-01

    To compare the effects of combined hydrotherapy and land-based physiotherapy (combined) with land-based physiotherapy only (land) on cost, health-related quality of life (HRQoL) and outcome of disease in children with juvenile idiopathic arthritis (JIA). Also to determine the cost-effectiveness of combined hydrotherapy and land-based physiotherapy in JIA. A multicentre randomised controlled, partially blinded trial was designed with 100 patients in a control arm receiving land-based physiotherapy only (land group) and 100 patients in an intervention arm receiving a combination of hydrotherapy and land-based physiotherapy (combined group). Three tertiary centres in the UK. Patients aged 4-19 years diagnosed more than 3 months with idiopathic arthritides, onset before their 16th birthday, stable on medication with at least one active joint. Patients in the combined and land groups received 16 1-hour treatment sessions over 2 weeks followed by local physiotherapy attendances for 2 months. Disease improvement defined as a decrease of > or =30% in any three of six core set variables without there being a 30% increase in more than one of the remaining three variables was used as the primary outcome measure and assessed at 2 months following completion of intervention. Health services resource use (in- and outpatient care, GP visits, drugs, interventions, and investigations) and productivity costs (parents' time away from paid work) were collected at 6 months follow-up. HRQoL was measured at baseline and 2 and 6 months following intervention using the EQ-5D, and quality-adjusted life-years (QALYs) were calculated. Secondary outcome measures at 2 and 6 months included cardiovascular fitness, pain, isometric muscle strength and patient satisfaction. Seventy-eight patients were recruited into the trial and received treatment. Two months after intervention 47% patients in the combined group and 61% patients in the land group had improved disease with 11 and 5% with worsened

  6. Can Seeding in the Clinic Reach a Wide Audience? A Proof of Concept Study on Spreading a Health Message About Juvenile Idiopathic Arthritis Using a Shareable Online Video.

    Science.gov (United States)

    Fay, Michaela; Rapley, Tim; Foster, Helen; Pain, Clare; Gerrand, Craig

    2016-02-22

    Shareable online video offers the potential for spreading a health message across online and real world social networks. Seeding a message in a clinical setting may be advantageous. To investigate the potential of an online video to spread a health message about juvenile idiopathic arthritis (JIA) when delivered or seeded in a clinical setting and investigate factors that influence sharing behavior. Multimethod proof of concept study. Concepts for two different styles of video were developed using focus groups and interviews and reviewed by an online market research panel. We compared dissemination of the two videos from two specialist pediatric rheumatology clinics in NHS Hospitals. Participants were 15 patients, family members, and clinical staff with knowledge of JIA at concept stage; 300 market research panel members in development stage; and 38 patients and their parents or guardians in the seeding stage. Newly diagnosed patients with JIA and/or parents or guardians were invited to view and share an online video with a health message about JIA across real-life and electronic social networks. Main outcome measures were viewing statistics, sharing behavior and patterns, and participant feedback. Of 38 patients and/or their parents or guardians given links, 26 visited the video webpage and shared the link, 2 visited and did not share, and 10 did not visit. Most links were viewed and shared within a few days. A total of 3314 pageviews were recorded with a mean of 89.6 pageviews per link (range 0-1245). Links were accessed from 26 countries, with most viewers in the United Kingdom (82.5%). Mothers were the most active group of sharers. Distribution of a video link in a clinical setting may be an effective way to spread a health message. Parents or guardians of children with JIA are more likely to share a link than young people. Dissemination depends on a small number of active sharers, the content of the video, and the willingness of participants to share health

  7. Socioeconomic costs and health-related quality of life in juvenile idiopathic arthritis: a cost-of-illness study in the United Kingdom.

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    Angelis, Aris; Kanavos, Panos; López-Bastida, Julio; Linertová, Renata; Serrano-Aguilar, Pedro

    2016-08-02

    Juvenile idiopathic arthritis (JIA) refers to a number of rare chronic inflammatory diseases. Although JIA imposes a significant societal burden, limited data are available on the cost of JIA. The study's objective is to quantify the socioeconomic burden of JIA patients in the United Kingdom (UK), along with their health-related quality of life (HRQoL). A bottom-up, cross-sectional, cost-of-illness analysis of 23 patients was carried out. To collect data on demographic characteristics, health resource utilization, informal care, productivity losses and HRQoL, questionnaires were administered to and completed by patients or their caregivers. The EuroQol five dimensions (EQ-5D) instrument was used to measure HRQoL. This study found that the average annual cost for a JIA patient was €31,546, with direct health care costs equalling €14,509 (46.0 % of total costs), direct non-health care costs amounting to €8,323 (26.4 %) and productivity losses being €8,715 (27.6 %). This was calculated using unit costs for 2012. The largest expenditures on average were accounted for by early retirement (27.0 %), followed by informal care (24.1 %), medications (21.1 %), outpatient and primary health care visits (13.2 %) and diagnostic tests (7.9 %). Important differences existed between JIA patients in need of caregiver assistance and those with no need (€39,469 vs. €25,452 respectively). Among adult JIA patients, mean EQ-5D index scores and visual analogue scale (VAS) scores were found to be 0.26 and 49.00 respectively; the same scores among caregivers were 0.66 and 67.14 respectively. JIA poses a significant cost burden on the UK society. Over half of the total average costs (54 %) are related to non-health care and productivity losses. HRQoL of JIA patients is considerably worse than the UK general population.

  8. Efficacy and Safety of Immunization With Pneumococcal Polysaccharide Vaccine in Children With Juvenile Idiopathic Arthritis: Preliminary Results of a Prospective Open-Label Study

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    Ekaterina I. Alexeeva

    2017-01-01

    Full Text Available Juvenile idiopathic arthritis (JIA is one of the most frequent and most disabling rheumatic diseases in children. Children with JIA receiving immunosuppressive and genetically engineered biologic drugs belong to the high-risk group for the development of bacterial and viral infections, including those administered by preventive vaccines.Objective: Our aim was to evaluate the efficacy and safety of 13-valent pneumococcal polysaccharide vaccine (PPV in children with JIA.Methods. In a prospective open-label comparative study, the efficacy of vaccination was determined by the level of specific anti-pneumococcal antibodies (anti-SPPIgG to Streptococcus pneumonia in the blood serum in patients with JIA. The safety of vaccination was assessed by determining a high-sensitivity C-reactive protein and S-100 protein as well as by the number of adverse events, by recording the number of infections of the upper respiratory tract and pneumonias, by the number of joints with active arthritis. Vaccination with 13-valent PPV was performed subcutaneously with one dose of 0.5 ml during therapy of the main disease with methotrexate or etanercept or 3 weeks before the appointment of methotrexate or etanercept. Patients were followed up for 1 year.Results. The study included 42 children with JIA: 21 with JIA in the active phase of the disease, 21 in remission of the disease. As a result of vaccination, the level of anti-pneumococcal antibodies (antiSPPIgG increased in the group of children with JIA in the active phase from 26.1 (14.3; 52.1 to 73.0 (52.5; 156.0 mg/l (p = 0.001, with JIA in remission — from 27.4 (18.2; 59.1 to 54.6 (35.3; 96.0 mg/l (p = 0.029. The concentration of the predictor of S-100 protein high activity after vaccination was not increased (p = 0.192. JIA aggravation episodes were not fixed in any patient. Serious adverse events were not observed during the trial.Conclusion. The vaccination of children with JIA with 13-valent PPV is highly

  9. Feasibility of a Website and a Hospital-Based Online Portal for Young Adults With Juvenile Idiopathic Arthritis: Views and Experiences of Patients.

    Science.gov (United States)

    Ammerlaan, Judy Jw; Scholtus, Lieske W; Drossaert, Constance Hc; van Os-Medendorp, Harmieke; Prakken, Berent; Kruize, Aike A; Bijlsma, Johannes Jw

    2015-08-14

    To improve knowledge and to encourage active involvement of young adults with juvenile idiopathic arthritis (JIA), an informative website with written and video information and an online portal with access to the personal medical record, self-monitoring, and e-consult functionalities were developed. Before implementing these applications in daily practice, it is important to gain insight into their feasibility in terms of ease of use, perceived usefulness and intention to use. The aim of this study was to evaluate and to examine the feasibility of the website and the online portal for young adults with JIA. A qualitative, feasibility study was conducted among the first users: 13 young adults with JIA. After provided access to the website and online portal, patients were interviewed on perceived usefulness, ease of use, and intention to (re)use the applications. Participants in the study considered the website and online portal as useful and easy-to-use. New medical information and feedback would motivate them to revisit the applications again. On the website, videos showing other young adults, telling how they handle their condition, were found as the most useful. On the portal, access to their medical records was most appreciated: it made the young JIA patients feel in control and it helped them monitor symptoms and disease activity. e-consults were thought to facilitate communication with physicians. The young adults considered both the website and the online portal as feasible, but they also had valuable suggestions to improve accessibility and use. Based on these findings, a news and event section was added on the website and a direct link was made to a discussion board and social media. To provide and support health information, the website is actively used in daily care. Considering the online portal, the use of self-monitoring tools and e-consult can be stimulated if there is direct linkage to treatment and feedback from the multidisciplinary team

  10. Mental Health and the Juvenile Justice System: Issues Related to Treatment and Rehabilitation

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    Hovey, Katrina A.; Zolkoski, Staci M.; Bullock, Lyndal M.

    2017-01-01

    Children and youth with mental health issues and learning difficulties are common in the juvenile justice system and finding ways to effectively rehabilitate, treat, and educate them is complicated, yet imperative. In this article, we examine the prevalence rates of mental health disorders in youth involved in the juvenile justice system, discuss…

  11. Alterações da articulação temporomandibular e suas repercussões orofaciais em pacientes portadores de artrite idiopática juvenil Temporomandibular joint alterations and their orofacial complications in patients with juvenile idiopathic arthritis

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    Renata Teixeira de Carvalho

    2012-12-01

    Full Text Available Os pacientes com artrite idiopática juvenil (AIJ podem apresentar alterações no metabolismo ósseo e no crescimento esqueletal, além de danos na articulação temporomandibular (ATM, o que pode gerar alterações extraorais e/ou intraorais, acarretando distúrbios craniofaciais. Nosso objetivo é promover uma revisão da literatura sobre as alterações orofaciais em pacientes com AIJ. Dentre os distúrbios orofaciais em pacientes com AIJ, as mudanças no crescimento mandibular, geradas por disfunções na região da ATM, parecem de grande prevalência nesses pacientes. As alterações mais encontradas são: retrognatismo, micrognatia, mordida aberta anterior, apinhamento dentário, assimetria facial e limitação de abertura de boca. Assim, o reumatologista torna-se uma peça-chave na detecção precoce dessas disfunções, ajudando no encaminhamento do paciente ao dentista. O diagnóstico, por sua vez, deve ser realizado pelo ortodontista, utilizando exames clínico e de imagem, o que possibilita tratamento precoce e prognóstico favorável. Distúrbios na ATM devem ser tratados de forma multidisciplinar, incluindo tratamento farmacológico para o controle da dor e tratamento odontológico por meio de aparelhagem funcional e/ou ortodôntica, fisioterapêutico e por vezes fonoaudiológico. Concluímos que dentre os distúrbios orofaciais em pacientes com AIJ, as mudanças no crescimento mandibular geradas por disfunções na região da ATM parecem de grande prevalência. Tais disfunções podem promover, principalmente, mordida aberta, retrusão mandibular, micrognatia, apinhamento dentário e assimetria facial. O reumatologista pode detectar tais alterações precocemente, encaminhando o paciente de imediato a uma equipe que deve ser, preferencialmente, multidisciplinar, composta por ortodontista, fisioterapeuta e fonoaudiólogo, visando reduzir futuras complicações oclusais e de crescimento mandibular.Patients with juvenile idiopathic

  12. Juvenile technologies as a system of organisation of social partnership of the state and society

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    Portnyagina E.V.

    2016-01-01

    Full Text Available The article presents the analysis of different approaches to the definition of "juvenile technologies", presented both in scientific publications and in the activities of institutions engaged in the work with minors, including those who have found themselves in difficult situations and/or in conflict with the law. The analysis of the organization of interdepartmental cooperation between governmental bodies of Omsk Region and social institutions, as well as educational organizations on the implementation of juvenile technologies in Omsk region has been conducted. The study allowed posing the problem of inconsistencies in the understanding of juvenile technologies, which does not allow establishing an effective juvenile system. The authors propose an alternative vision of the concepts. It is proposed to increase the efficiency of the use of juvenile technologies at the expense of the organization of continuous monitoring of the implementation of juvenile technologies in the region, where pedagogical, psychological, legal, financial valuation criteria would be presented.

  13. SNPs in the FOXP3 gene region show no association with Juvenile Idiopathic Arthritis in a UK Caucasian population

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    Eastell, T.; Hinks, A.; Thomson, W.

    2007-01-01

    Objective A region on the short arm of the X-chromosome, Xp11, has previously been linked to childhood-onset polyarthritis. Mapping to the linked region is FOXP3, a transcription factor that regulates regulatory T cell (Treg) development and function. The objective of this study was to determine whether single nucleotide polymorphisms (SNPs) in the FOXP3 gene region contribute to JIA susceptibility. Method Nine FOXP3 SNPs were genotyped in 761 JIA cases and 402 controls using the Sequenom® MassARRAY® system. Association was measured using either χ2 or Fisher's exact test at the allelic and genotypic level. Furthermore, cases and controls were stratified by gender and association measured for each stratum. Results None of the SNPs showed an association with JIA. Similarly, the lack of association was also evident in both the female and male cohorts. Conclusion Although FOXP3 presents itself as a good candidate for contributing to JIA susceptibility, this study, which was powered to detect associations with genotypic relative risk >2 in the female cohort, has failed to find an association between SNPs in the FOXP3 gene region and JIA. PMID:17526924

  14. Factors associated with choice of biologic among children with Juvenile Idiopathic Arthritis: results from two UK paediatric biologic registers.

    Science.gov (United States)

    Kearsley-Fleet, Lianne; Davies, Rebecca; Baildam, Eileen; Beresford, Michael W; Foster, Helen E; Southwood, Taunton R; Thomson, Wendy; Hyrich, Kimme L

    2016-09-01

    The objectives of this study were to describe patients starting first-line biologics for JIA, to describe characteristics over time among patients starting etanercept, and to describe patterns of second biologic prescribing. The British Society for Paediatric and Adolescent Rheumatology Etanercept Cohort Study, and the Biologics for Children with Rheumatic Diseases study are ongoing prospective observational cohorts, collecting data on patients starting biologic therapy for JIA. Patients registered from 1 January 2010 starting their first biologic were compared between therapies. Patients starting etanercept before 2010 were included to analyse changes in etanercept prescribing. The pathway of patients starting a second biologic was recorded in all patients. To 26 August 2014, 931 patients were recruited starting a first-line biologic (142 Biologics for Children with Rheumatic Diseases; 789 British Society for Paediatric and Adolescent Rheumatology Etanercept Cohort Study). From 2010, patients with systemic JIA (sJIA) were almost exclusively prescribed anakinra or tocilizumab. Choice between anti-TNF therapies was largely driven by history of chronic anterior uveitis (CAU). When investigating trends in patients starting etanercept over time, disease duration at etanercept start, patients with sJIA, a history of CAU, and those who received concomitant oral corticosteroids decreased over time. Patients who started a second biologic from 1 January 2010 showed a similar stratification. Although etanercept remains the most common biologic prescribed for JIA, there has been a clear shift towards the use of alternative biologics, largely driven by disease subtype and history of CAU. This channelling of children towards specific therapies should be considered carefully in future studies and in clinical guidelines and ongoing research. © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology.

  15. Baby with neonatal systemic juvenile xanthogranuloma born within a cross-cousin marriage

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    Hikmet Tekin Nacaroglu

    2015-12-01

    Full Text Available Juvenile xanthogranuloma is a non-Langerhans cell histiocytosis seen most commonly in childhood and adolescence. Extracutaneous involvement is rare. We report an interesting and extremely rare case of systemic (skin, lung, spleen, and colon “juvenile xanthogranuloma” in the neonatal period. Our case was the first ever reported case born to a cross-cousin marriage.

  16. Clinical balance tests, proprioceptive system and adolescent idiopathic scoliosis.

    Science.gov (United States)

    Le Berre, Morgane; Guyot, Marc-Alexandre; Agnani, Olivier; Bourdeauducq, Isabelle; Versyp, Marie-Christine; Donze, Cécile; Thévenon, André; Catanzariti, Jean-Francois

    2017-06-01

    Adolescent idiopathic scoliosis (AIS) is a three-dimensional deformity of the spinal column of unknown etiology. Multiple factors could be involved, including neurosensory pathways and, potentially, an elective disorder of dynamic proprioception. The purpose of this study was to determine whether routine balance tests could be used to demonstrate an elective alteration of dynamic proprioception in AIS. This was a multicentre case-control study based on prospectively collected clinical data, in three hospitals pediatric, with spine consultation, from January 2013 through April 2015. From an original population of 547 adolescents, inclusion and non-inclusion criteria indentified 114 adolescents with right thoracic AIS (mean age 14.5 ± 1.9 years, Cobb angle 35.7 ± 15.3°) and 81 matched adolescents without scoliosis (mean age 14.1 ± 1.9 years). Participants performed three routine clinical balance tests to assess the static and dynamic proprioception: the Fukuda-Utenberger stepping test (angle of rotation in degrees and distance of displacement in cm) to assess dynamic balance; the sharpened Romberg test and the unipedal stance test (eyes closed) to assess static balance. There was no significant difference between AIS subjects and controls for the static tests, but there was a significant difference for the dynamic test for both measures: distance of displacement (p < 0.01) and angle of rotation (p < 0.0001). This result confirms our initial these: the dynamic proprioception is altered electively in AIS. These findings confirm recent AIS studies. Our results might be related to immature central integration of dynamic proprioceptive input leading to a poorly adapted motor response, particularly for postural control of the, in AIS. These balance tests can be performed in routine practice. Their validity as a biomarker for screening and monitoring purposes should be assessed.

  17. Fluorescence optical imaging and musculoskeletal ultrasonography in juvenile idiopathic polyarticular disease before and during antirheumatic treatment - a multicenter non-interventional diagnostic evaluation.

    Science.gov (United States)

    Klein, Ariane; Just, Georg Werner; Werner, Stephanie Gabriele; Oommen, Prasad T; Minden, Kirsten; Becker, Ingrid; Langer, Hans-Eckhard; Klee, Dirk; Horneff, Gerd

    2017-06-30

    Valid detection of inflamed joints is essential for correct classification, therapeutic decisions, prognosis and assessment of treatment efficacy in juvenile idiopathic arthritis (JIA). Fluorescence optical imaging (FOI) enables visualization of inflammation in arthritis of finger and hand joints and might be used for monitoring. A 24-week observational study in polyarticular JIA patients newly starting treatment with methotrexate or an approved biologic was performed in three centers. Patients were evaluated clinically, by gray-scale ultrasonography (GSUS), power-Doppler ultrasonography (PDUS) and FOI at baseline, week 12 and week 24. Of 37 patients enrolled, 24 patients started methotrexate and 13 patients a biologic for the first time (etanercept n = 11, adalimumab and tocilizumab n = 1 each). Mean JADAS 10 decreased significantly from 17.7 at baseline to 12.2 and 7.2 at week 12 and 24 respectively. PedACR 30/50/70/100 response rates at week 24 were 85%/73%/50%/27%. The total number of clinically active joints in hand and fingers at baseline/week 12/week 24 was 262 (23.6%)/162 (16.4%)/162 (9.0%). By GSUS, at baseline/week 12/week 24, 192 (19.4%)/135 (16.1%)/83 (11.5%) joints showed effusions and 186 (18.8%)/107 (12.7%)/69 (9.6%) showed synovial thickening, and by PDUS 68 (6.9%)/15 (1.8%)/36 (5%) joints showed hyperperfusion. Any sign of arthritis was detected by US in a total of 243 joints (24.5%) at baseline, 161 joints (19.2%) at week 12 and 123 joints (17%) at week 24. By FOI at baseline/week 12/week 24, 430 (38.7%)/280 (29.2%)/215 (27.6%) showed a signal enhancement in at least one phase. Summarizing all three points of time, the highest numbers of signals were detected by FOI with 32% of joints, especially in phase 2, while by US 20.7% and by clinical examination 17.5% of joints were active. A high number of joints (21.1%) had FOI signals but were inactive by clinical examination. A total 20.1% of joints with signals in FOI did not show effusion

  18. Bone mineral density in juvenile systemic lupus erythematosus

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    Castro T.C.M.

    2002-01-01

    Full Text Available We evaluated spine bone mineral density (BMD in Brazilian children with juvenile systemic lupus erythematosus (JSLE in order to detect potential predictors of reduction in bone mass. A cross-sectional study of BMD at the lumbar spine level (L2-L4 was conducted on 16 female JSLE patients aged 6-17 years. Thirty-two age-matched healthy girls were used as control. BMD at the lumbar spine was measured by dual-energy X-ray absorptiometry. Weight, height and pubertal Tanner stage were determined in patients and controls. Disease duration, mean daily steroid doses, mean cumulative steroid doses and JSLE activity measured by the systemic lupus erythematosus disease activity index (SLEDAI were determined for all JSLE patients based on their medical charts. All parameters were used as potential determinant factors for bone loss. Lumbar BMD tended to be lower in the JSLE patients, however, this difference was not statistically significant (P = 0.10. No significant correlation was observed in JSLE girls between BMD and age, height, Tanner stage, disease duration, corticosteroid use or disease activity. We found a weak correlation between BMD and weight (r = 0.672. In the JSLE group we found no significant parameters to correlate with reduced bone mass. Disease activity and mean cumulative steroid doses were not related to BMD values. We did not observe reduced bone mass in female JSLE.

  19. Idiopathic systemic granulomatous pathology causing sudden death due to myocarditis: A rare case report

    Directory of Open Access Journals (Sweden)

    Harpal Singh

    2015-01-01

    Full Text Available Idiopathic granulomatous myocarditis is extremely rare, particularly since the introduction of drugs effective against tuberculosis (TB, viruses, fungi and the effective treatment of sarcoidosis. Here is a case of a 65-year-old female prisoner having history of sudden collapse and ultimately death. Autopsy findings of various viscera on histopathological examination show granulomatous pathology, that is, in spleen, liver and in the left ventricular wall of heart. Ziehl-Neelsen staining of the sections show the absence of acid fast bacilli, negative for fungal staining as most of the granulomas are noncaseating type with presence of giant cells having no asteroid body and Schuamann body, real-time polymerase chain reaction for TB is negative. Idiopathic giant cell myocarditis is a disease of relatively young adults, that is, between 3 rd and 4 th decade of life. So, this case is strongly considered to be a case of sudden death due to myocarditis as a result of idiopathic systemic granulomatous pathology, a rare case in in literature.

  20. EXPERIENCE OF TREATMENT WITH RITUXIMAB IN PATIENT WITH SYSTEMIC TYPE OF JUVENILE RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    K.B. Isaeva

    2009-01-01

    Full Text Available The article gives a case report on patient with severe systemic type of juvenile arthritis, refractory to treatment with classical immuno suppressants and blocker of tumor necrotizing factor Successive application of biological agent — rituximab was described. In 9 weeks, extra articular symptoms of disease and acute inflammatory lesions in joints were stopped, and functional activity of child increased. Presented case report demonstrates high effectiveness of rituximab: patient with severe systemic type of juvenile arthritis has clinical and laboratory remission of disease during 12 months.Key words: children, juvenile rheumatoid arthritis, rituximab.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2009;8(3:132-138

  1. Juvenile Salmon Acoustic Telemetry System Transmitter Downsize Assessment

    Energy Technology Data Exchange (ETDEWEB)

    Carlson, Thomas J.; Myjak, Mitchell J.

    2010-04-30

    At the request of the U.S. Army Corps of Engineers, Portland District, researchers from Pacific Northwest National Laboratory investigated the use of an application-specific integrated circuit (ASIC) to reduce the weight and volume of Juvenile Salmon Acoustic Telemetry System (JSATS) transmitters while retaining current functionality. Review of the design of current JSATS transmitters identified components that could be replaced by an ASIC while retaining the function of the current transmitter and offering opportunities to extend function if desired. ASIC design alternatives were identified that could meet transmitter weight and volume targets of 200 mg and 100 mm3. If alternatives to the cylindrical batteries used in current JSATS transmitters can be identified, it could be possible to implant ASIC-based JSATS transmitters by injection rather than surgery. Using criteria for the size of fish suitable for surgical implantation of current JSATS transmitters, it was concluded that fish as small as 70 mm in length could be implanted with an ASIC-based transmitter, particularly if implantation by injection became feasible.

  2. Transportation of juvenile tambaqui (Colossoma macropomum in a closed system

    Directory of Open Access Journals (Sweden)

    L. C. Gomes

    Full Text Available The objective of this study was to investigate the effect of density, duration and the use of additives to the water during the transportation of juvenile tambaqui (Colossoma macropomum and use of this data to establish a safe transportation protocol for the species. The tested products and dosages were: salt (1000, 2000 and 3000 mg/L, gypsum (100, 300 and 500 mg/L and benzocaine (10, 20 and 30 mg/L. Fish were transported in closed systems (plastic bag at different densities and time periods of up to 24 h. Fish survival (FS and water quality parameters were monitored immediately after transportation. The remaining fish were kept in floating cages in order to evaluate mortality which occurred up to 96 h after transportation (S96. The best fish density, additives dosages and time period of the transportation was estimated with a general linear model. The effect of the condition factor on FS and S96 was also evaluated. As expected, FS and S96 were significantly related to time and density. FS but not S96, were also were significantly related to treatment. FS with gypsum treatment was not different from controls and FS with table salt and benzocaine treatments were significantly reduced. The condition factor was not related to either FS or S96. FS was inversely correlated with carbon dioxide concentration. It was concluded that the additives did not improve fish transportation survival. Linear models were developed to predict the best transportation densities as a function of time.

  3. Transportation of juvenile tambaqui (Colossoma macropomum) in a closed system.

    Science.gov (United States)

    Gomes, L C; Araujo-Lima, C A R M; Chippari-Gomes, A R; Roubach, R

    2006-05-01

    The objective of this study was to investigate the effect of density, duration and the use of additives to the water during the transportation of juvenile tambaqui (Colossoma macropomum) and use of this data to establish a safe transportation protocol for the species. The tested products and dosages were: salt (1000, 2000 and 3000 mg/L), gypsum (100, 300 and 500 mg/L) and benzocaine (10, 20 and 30 mg/L). Fish were transported in closed systems (plastic bag) at different densities and time periods of up to 24 h. Fish survival (FS) and water quality parameters were monitored immediately after transportation. The remaining fish were kept in floating cages in order to evaluate mortality which occurred up to 96 h after transportation (S96). The best fish density, additives dosages and time period of the transportation was estimated with a general linear model. The effect of the condition factor on FS and S96 was also evaluated. As expected, FS and S96 were significantly related to time and density. FS but not S96, were also were significantly related to treatment. FS with gypsum treatment was not different from controls and FS with table salt and benzocaine treatments were significantly reduced. The condition factor was not related to either FS or S96. FS was inversely correlated with carbon dioxide concentration. It was concluded that the additives did not improve fish transportation survival. Linear models were developed to predict the best transportation densities as a function of time.

  4. A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis

    DEFF Research Database (Denmark)

    Ingegnoli, Francesca; Boracchi, Patrizia; Gualtierotti, Roberta

    2015-01-01

    OBJECTIVE: Qualitative capillaroscopy patterns in juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database. METHODS: Data collected between June 2004 and April 2013 were examined with focus on capillar......OBJECTIVE: Qualitative capillaroscopy patterns in juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database. METHODS: Data collected between June 2004 and April 2013 were examined with focus....... Juvenile-onset SSc showed scleroderma pattern more frequently than adult-onset SSc (93.3% and 88%). The OR was 2.44 and 95% CI 0.57-10.41. An active scleroderma pattern was present in 58% of juvenile- and 61% of adult-onset SSc. The OR was 0.91 and 95% CI 0.28-2.93. The late scleroderma pattern was present...... in 61% of juvenile- and 55.5% of adult-onset SSc. The OR was 1.06 and 95% CI 0.34-3.56. CONCLUSION: This is the first exploratory study on the comparison of capillaroscopy between juvenile- and adult-onset SSc in adulthood. Juvenile-onset SSc had an increase prevalence of scleroderma pattern...

  5. A Critical Appraisal of the Juvenile Justice System under Cameroon's 2005 Criminal Procedure Code: Emerging Challenges

    Directory of Open Access Journals (Sweden)

    S Tabe

    2012-03-01

    Full Text Available The objective of this article is to examine the changes introduced by the 2005 Cameroonian Criminal Procedure Code on matters of juvenile justice, considering that before this Code, juvenile justice in Cameroon was governed by extra-national laws. In undertaking this analysis, the article highlights the evolution of the administration of juvenile justice 50 years after independence of Cameroon. It also points out the various difficulties and shortcomings in the treatment of juvenile offenders in Cameroon since the enactment of the new Criminal Procedure Code. The article reveals that the 2005 Code is an amalgamation of all hitherto existing laws in the country that pertained to juvenile justice, and that despite the considerable amount of criticism it has received, the Code is clearly an improvement of the system of juvenile justice in Cameroon, since it represents a balance of the due process rights of young people, the protection of society and the special needs of young offenders. This is so because the drafters of the Code took a broad view of the old laws on juvenile justice. Also a wide range of groups were consulted, including criminal justice professionals, children’s service organisations, victims, parents, young offenders, educators, advocacy groups and social-policy analysts. However, to address the challenges that beset the juvenile justice system of Cameroon, the strategy of the government should be focussed on three areas: the prevention of youth crime, the provision of meaningful consequences for the actions of young people, and the rehabilitation and reintegration of young offenders. Cameroonian law should seek educative solutions rather than to impose prison sentences or other repressive measures on young offenders. Special courts to deal with young offenders should be established outside the regular penal system and should be provided with resources that are adequate for and appropriate to fostering their understanding of

  6. Systemic Besnoitiosis in a Juvenile Roe Deer (Capreolus capreolus).

    Science.gov (United States)

    Arnal, M C; Gutiérrez-Expósito, D; Martínez-Durán, D; Regidor-Cerrillo, J; Revilla, M; Fernández de Luco, D; Jiménez-Meléndez, A; Ortega-Mora, L M; Álvarez-García, G

    2017-10-01

    Herein, we report the first incidence of systemic besnoitiosis in a male juvenile roe deer Capreolus capreolus. The animal was found dead in an area where bovine besnoitiosis is endemic and showed cachexia and multiple skin erosions in the metacarpal and metatarsal areas. Moreover, round and elevated white structures suggestive of Besnoitia spp. tissue cysts were also present. Twenty-eight tissue samples from different anatomical locations were collected for microscopic lesion and parasite detection through histopathology and PCR. Immunohistochemistry was performed to confirm Besnoitia-positive reaction in the tissue cysts. In addition, the identity of Besnoitia spp. in PCR-positive tissue samples was also investigated using microsatellite (MS) markers, and the comparison of protein disulphide isomerase gene sequences (BbPDI) of B. besnoiti and B. tarandi isolated from cattle and reindeer, respectively. Besnoitia cysts were detected in the skin (several parts), respiratory and upper digestive tracts, eyes, kidney, liver, testicle, cardiac muscle and lymphoid tissue. Remarkably, the presence of tissue cysts in the brain confirmed the capacity of Besnoitia spp. to form tissue cysts in the central nervous system (CNS). Finally, the Besnoitia species detected showed the same MS genotype as B. besnoiti, and BbPDI sequences from roe deer and two B. besnoiti isolates were genetically identical throughout multiple sequence alignment. Thus, for the first time, there is evidence that roe deer might act as an intermediate host of B. besnoiti. Further molecular analyses and parasite isolations are needed to corroborate these findings. © 2016 Blackwell Verlag GmbH.

  7. [The role of dysfunction of the autonomic nervous system in idiopathic overactive urinary bladder in women].

    Science.gov (United States)

    Borisov, V V; Akarachkova, E S; Shvarkov, S B; Grigorashvili, I I; Romikh, V V

    2012-01-01

    A complex urological and neurological examination of female patients with idiopathic overactive bladder (IOAB) has detected clinical signs and neurophysiological correlates of vegetative dysfunction in the form of disturbed sympatho-parasympathic interrelations, failure of segmental and compensatory enhancement of suprasegmental mechanisms of regulation of lower urinary tract activity. The treatment with selective inhibitors of serotonin re-entry reduced severity ofpsychovegetative syndrome and symptoms of the lower urinary tract. This confirms an essential role of the autonomic nervous system in pathogenetic mechanisms of IOAB development and gives grounds for recommending selective inhibitors of serotonin re-entry for combined treatment of IOAB patients.

  8. Treating Juvenile Crime.

    Science.gov (United States)

    Gelber, Seymour

    1983-01-01

    Although juvenile crime rates have not changed significantly in the last five years, the juvenile courts' ability to handle crime has deteriorated. To treat the problem of juvenile crime effectively requires intervention at the earliest sign of delinquency and an assessment of the juvenile courts and school system. (AM)

  9. Children within the Juvenile Justice System in Nigeria ...

    African Journals Online (AJOL)

    Background: Many children in Nigeria face a life of poverty, family instability, inadequate educational opportunities and poor physical and mental health which hinder their ability to develop into healthy adults, live an improved quality of life or fulfil their life aspirations. These factors have also been associated with juvenile ...

  10. A Critical Appraisal of the Juvenile Justice System under ...

    African Journals Online (AJOL)

    This is so because the drafters of the Code took a broad view of the old laws on juvenile justice. Also a wide range of groups were consulted, including criminal justice professionals, children's service organisations, victims, parents, young offenders, educators, advocacy groups and social-policy analysts. However, to ...

  11. Gastrointestinal system manifestations in juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Sönmez, Hafize Emine; Karhan, Asuman Nur; Batu, Ezgi Deniz; Bilginer, Yelda; Gümüş, Ersin; Demir, Hülya; Yüce, Aysel; Özen, Seza

    2017-07-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease which may involve gastrointestinal system (GIS). The aim of this study was to present GIS manifestations of pediatric SLE patients. The medical files of 69 children with SLE followed between January 2011 and January 2016 were reviewed. All fulfilled the Systemic Lupus International Collaborating Clinics criteria. All patients (≤18 years of age) with GIS manifestations were included. GIS manifestations were observed in 19 (27.5%) out of 69 SLE patients and present at the time of SLE diagnosis in 13 (68.4%). The GIS manifestations due to SLE were autoimmune hepatitis (AIH) (n = 8) and lupus enteritis (n = 1). Manifestations associated with SLE were hepatomegaly and hypertransaminasemia due to macrophage activation syndrome (MAS) (n = 3) and hepatic steatosis (n = 1). GIS manifestations as a result of the adverse events of drugs were as follows: toxic hepatitis (n = 3; associated with methotrexate and nonsteroidal anti-inflammatory drugs in one, methotrexate in another, and azathioprine in another patient), azathioprine-induced cholestatic hepatitis (n = 1), and gastritis associated with corticosteroid (n = 1). In one patient, acute appendicitis occurred as a coincidence. In this study, one of every five pediatric SLE patients had GIS-related manifestations. GIS involvement may occur as an initial manifestation of the disease.

  12. Being (almost invisible: Victims of crime in the Italian juvenile criminal justice system

    Directory of Open Access Journals (Sweden)

    Vezzadini Susanna

    2014-01-01

    Full Text Available From 2008 to 2013 the author has been a Special Judge in the Juvenile Criminal Court of the Emilia Romagna Region. From that privileged perspective, it was possible to observe the dynamics of how victims of underage offenders were considered before the law, no differences if they are adults or minors, too. The reflections presented will first consider EU and UN provision on victims of crime; then, the normative framework supporting the Italian criminal juvenile justice system will be considered by an examining of the difficulties victims meet in that peculiar context. The implementation of juvenile criminal law shows the paradox victims of crime have to cope with. The Juvenile Criminal Court in Bologna recently started to promote a wide use of restorative justice measures as an attempt to correct the unfair consequences in the application of law, with judicial discretion interpreted as an instrument to favour victims’ harm recognition and to protect their dignity as persons.

  13. Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome.

    Science.gov (United States)

    Moreira, D C; Ng, C J; Quinones, R; Liang, X; Chung, D W; Di Paola, J

    2016-12-01

    Essentials Idiopathic systemic capillary leak syndrome (SCLS) is characterized by episodes of vascular leakage. We present the case of a patient with SCLS who developed microangiopathic hemolytic anemia (MAHA). We propose that this anemia is the result of ADAMTS-13 loss in the third-space fluid. This suggests that MAHA can occur in patients with significant extravasation of proteins. Idiopathic systemic capillary leak syndrome (SCLS) is a rare process characterized by acute and recurrent episodes of vascular leakage with severe hypotension, hypoalbuminemia, hemoconcentration and edema. Anemia and thrombocytopenia are not part of this syndrome, but here we present the case of a pediatric patient with a clinical presentation consistent with SCLS who subsequently developed microangiopathic hemolytic anemia at a time when she had significant fluid loss and anasarca. Based on serial ADAMTS-13 levels, we propose that the anemia in this patient developed as a result of ADAMTS-13 loss in the third-space fluid, a novel mechanism for acquired microangiopathic hemolytic anemia. © 2016 International Society on Thrombosis and Haemostasis.

  14. Association of neopterin as a marker of immune system activation and juvenile rheumatoid arthritis activity

    Directory of Open Access Journals (Sweden)

    Mones M. Abu Shady

    2015-08-01

    Full Text Available OBJECTIVE: To evaluate neopterin plasma concentrations in patients with active juvenile idiopathic arthritis (JIA and correlate them with disease activity.METHODS: Sixty patients diagnosed as active JIA, as well as another 60 apparently healthy age- and gender-matched children as controls, were recruited from the Pediatrics Allergy and Immunology Clinic, Ain Shams University. Disease activity was assessed by the Juvenile Arthritis Disease Activity Score 27 (JADAS-27. Laboratory investigations were performed for all patients, including determination of hemoglobin concentration (Hgb, erythrocyte sedimentation rate (ESR, and C-reactive protein. Serum concentrations of tumor necrosis factor-alpha (TNF-a, interleukin-6 (IL-6, monocyte chemoattractant protein-1 (MCP-1, and neopterin were measured.RESULTS: Significant differences were found between JIA patients and controls with regard to the mean levels of Hgb, ESR, TNF-a, IL-6, and MCP-1 (p 0.05. Multiple linear regression analysis showed that JADAS- 27 and ESR were the main variables associated with serum neopterin in JIA patients (p < 0.05.CONCLUSION: The elevation of plasma neopterin concentrations in early JIA patients may indicate stimulation of immune response. Serum neopterin can be used as a sensitive marker for assaying background inflammation and disease activity score in JIA patients.

  15. Association of neopterin as a marker of immune system activation and juvenile rheumatoid arthritis activity.

    Science.gov (United States)

    Shady, Mones M Abu; Fathy, Hanan A; Ali, Alaa; Youness, Eman R; Fathy, Gihan A

    2015-01-01

    To evaluate neopterin plasma concentrations in patients with active juvenile idiopathic arthritis (JIA) and correlate them with disease activity. Sixty patients diagnosed as active JIA, as well as another 60 apparently healthy age- and gender-matched children as controls, were recruited from the Pediatrics Allergy and Immunology Clinic, Ain Shams University. Disease activity was assessed by the Juvenile Arthritis Disease Activity Score 27 (JADAS-27). Laboratory investigations were performed for all patients, including determination of hemoglobin concentration (Hgb), erythrocyte sedimentation rate (ESR), and C-reactive protein. Serum concentrations of tumor necrosis factor-alpha (TNF-α), interleukin-6 (IL-6), monocyte chemoattractant protein-1 (MCP-1), and neopterin were measured. Significant differences were found between JIA patients and controls with regard to the mean levels of Hgb, ESR, TNF-α, IL-6, and MCP-1 (p0.05). Multiple linear regression analysis showed that JADAS- 27 and ESR were the main variables associated with serum neopterin in JIA patients (p<0.05). The elevation of plasma neopterin concentrations in early JIA patients may indicate stimulation of immune response. Serum neopterin can be used as a sensitive marker for assaying background inflammation and disease activity score in JIA patients. Copyright © 2015 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

  16. RESEARCHES REGARDING THE GROWTH OF HYPOPTHALMICHTHYS MOLITRIX AND CTENOPHARYNGODON IDELLA JUVENILES IN AN INTENSIVE SYSTEM

    Directory of Open Access Journals (Sweden)

    ALINA RODICA ANI

    2008-10-01

    Full Text Available The intensive net cage system is based on the superior capitalization of theaccumulation lakes or other different basins through the use of different types anddimensions, stationary or floating net cages, depending on categories like age,species and destination. This study is about the use of net cage intensive system typein the growth of juveniles Ctenopharyngodon idella and Hypopthalmichthys molitrixspecies. From the experimental point of view, the objectives were represented by theconstruction of a net cage mini-system and the surveillance of the biologicalmaterial inside this system. The experiment was unfolded on the length of 38 days atthe Mǎrtinesti Fish Farm from Cluj County. A net cage system designed andconceived by the research collective was populated with 4000 juveniles fish fromeach species. At the beginning of this experiment the juveniles fish had a mediumweight of 2 grams/sample. At the end of this experiment the fish had a mediumweight of 9 grams.

  17. Genetics Home Reference: juvenile Paget disease

    Science.gov (United States)

    ... J. A mutation in the gene TNFRSF11B encoding osteoprotegerin causes an idiopathic hyperphosphatasia phenotype. Hum Mol Genet. ... PM, Jones JL, Podgornik MN, McAlister WH, Mumm S. Osteoprotegerin deficiency and juvenile Paget's disease. N Engl J ...

  18. Juvenile Justice System in Italy. Research and interventions / Sistema de Justicia Juvenil en Italia: investigaciones e intervenciones

    Directory of Open Access Journals (Sweden)

    Patrizia Meringolo

    2012-12-01

    Full Text Available This paper talks about the juvenile justice system in Italy. The author describes the interventions done with minors, boys and girls aged from 14 until 18 years, who have committed offenses of the civil or penal code, by the New Code of Criminal Procedure for Minors (1988. The Procedures have had some positive psychological aspects, aimed to avoid detention,thanks to alternative measures and strategies for inclusion, including also the minors living in the South, that are often involved in mafia-crimes. Nonetheless there are more negative psychological issues, because alternative punishments are not often applied to minors that lack social networks, particularly to foreign ones. Three examples of participatory researcheswill be shown, promoted by the Municipality of Florence, Department of Psychology and Third Sector Associations, aimed to promote psychological and social inclusion of minors (particularly those coming from abroad, with the commitment of active citizenship organizations, with an evaluation oftheir strengths and weaknesses.

  19. Suicidal Ideation and Behavior in Youth in the Juvenile Justice System: A Review of the Literature.

    Science.gov (United States)

    Stokes, Marquita L; McCoy, Kathleen P; Abram, Karen M; Byck, Gayle R; Teplin, Linda A

    2015-07-01

    Suicide is prevalent among youth, especially those involved in the juvenile justice system. Although many studies have examined suicidal ideation and behavior in delinquent youth, prevalence rates vary widely. This article reviews studies of suicidal ideation and behavior in youth in the juvenile justice system, focusing on the point of contact: incarceration status and stage of judicial processing. Suicidal ideation and behavior are prevalent and increase with greater involvement in the juvenile justice system. Depression, sexual abuse, and trauma were the most commonly identified predictors of suicidal ideation and behavior. Prevalence rates of suicidal ideation and behavior vary by gender and race/ethnicity, indicating the need for gender-specific and culturally relevant interventions. © The Author(s) 2015.

  20. Delinquent-Victim Youth-Adapting a Trauma-Informed Approach for the Juvenile Justice System.

    Science.gov (United States)

    Rapp, Lisa

    2016-01-01

    The connection between victimization and later delinquency is well established and most youth involved with the juvenile justice system have at least one if not multiple victimizations in their history. Poly-victimized youth or those presenting with complex trauma require specialized assessment and services to prevent deleterious emotional, physical, and social life consequences. Empirical studies have provided information which can guide practitioners work with these youth and families, yet many of the policies and practices of the juvenile justice system are counter to this model. Many youth-serving organizations are beginning to review their operations to better match a trauma-informed approach and in this article the author will highlight how a trauma-informed care model could be utilized to adapt the juvenile justice system.

  1. Idiopathic anaphylaxis.

    Science.gov (United States)

    Fenny, Nana; Grammer, Leslie C

    2015-05-01

    Idiopathic anaphylaxis is a diagnosis of exclusion after other causes have been thoroughly evaluated and excluded. The pathogenesis of idiopathic anaphylaxis remains uncertain, although increased numbers of activated lymphocytes and circulating histamine-releasing factors have been implicated. Signs and symptoms of patients diagnosed with idiopathic anaphylaxis are indistinguishable from the manifestations of other forms of anaphylaxis. Treatment regimens are implemented based on the frequency and severity of patient symptoms and generally include the use of epinephrine autoinjectors, antihistamines, and steroids. The prognosis of idiopathic anaphylaxis is generally favorable with well-established treatment regimens and effective patient education. Copyright © 2015 Elsevier Inc. All rights reserved.

  2. Skeletal manifestations of juvenile hypothyroidism and the impact of treatment on skeletal system

    Directory of Open Access Journals (Sweden)

    Manish Gutch

    2013-01-01

    Full Text Available Thyroid hormone mediates growth and development of the skeleton through its direct effects and through its permissive effects on growth hormone. The effect of hypothyroidism on bone is well described in congenital hypothyroidism, but the impact of thyroid hormone deficiency on a growing skeleton, as it happens with juvenile hypothyroidism, is less defined. In addition, the extent to which the skeletal defects of juvenile hypothyroidism revert on the replacement of thyroid hormone is not known. A study was undertaken in 29 juvenile autoimmune hypothyroid patients to study the skeletal manifestations of juvenile hypothyroidism and the impact of treatment of hypothyroidism on the skeletal system of juvenile patients. Hypothyroidism has a profound impact on the skeletal system and delayed bone age, dwarfism, and thickened bands at the metaphyseal ends being the most common findings. Post treatment, skeletal findings like delayed bone age and dwarfism improved significantly, but there were no significant changes in enlargement of sella, presence of wormian bones, epihyseal dysgenesis, vertebral changes and thickened band at the metaphyseal ends. With the treatment of hypothyroidism, there is an exuberant advancement of bone age, the catch up of bone age being approximately double of the chronological age advancement.

  3. Malignant Hemispheric Cerebral Infarction Associated with Idiopathic Systemic Capillary Leak Syndrome

    Directory of Open Access Journals (Sweden)

    Kei Miyata

    2013-10-01

    Full Text Available Idiopathic systemic capillary leak syndrome (ISCLS is a rare condition that is characterized by unexplained episodic capillary hyperpermeability due to a shift of fluid and protein from the intravascular to the interstitial space. This results in diffuse general swelling, fetal hypovolemic shock, hypoalbuminemia, and hemoconcentration. Although ISCLS rarely induces cerebral infarction, we experienced a patient who deteriorated and was comatose as a result of massive cerebral infarction associated with ISCLS. In this case, severe hypotensive shock, general edema, hemiparesis, and aphasia appeared after serious antecedent gastrointestinal symptoms. Progressive life-threatening ischemic cerebral edema required decompressive hemicraniectomy. The patient experienced another episode of severe hypotension and limb edema that resulted in multiple extremity compartment syndrome. Treatment entailed forearm and calf fasciotomies. Cerebral edema in the ischemic brain progresses rapidly in patients suffering from ISCLS. Strict control of fluid volume resuscitation and aggressive diuretic therapy may be needed during the post-leak phase of fluid remobilization.

  4. Superior oblique tendon (Brown’s syndrome as the presenting finding in childhood onset HLA-B27-related enthesitis and juvenile idiopathic oligoarticular arthritis

    Directory of Open Access Journals (Sweden)

    C. Pham

    2014-11-01

    Full Text Available We report two patients who presented with Brown’s syndrome. The first is a 7-year-old boy who at the time of his diagnosis was also found to have enthesitis and HLA-B27 positivity. The second patient was diagnosed with bilateral Brown’s syndrome at 13 months of age. At age 7 she developed a persistent oligoarticular arthritis and unilateral anterior iritis consistent with the oligoarticular Juvenile Idiopatic Arthritis (JIA phenotype. These cases highlight ophthalmologic findings and diagnostic considerations with respect to Brown’s syndrome and associated childhood onset rheumatologic disease.

  5. Youth with Disabilities in the Corrections System: Prevalence Rates and Identification Issues. Monograph Series on Education, Disability and Juvenile Justice.

    Science.gov (United States)

    Rutherford, Robert B., Jr.; Bullis, Michael; Anderson, Cindy Wheeler; Griller-Clark, Heather M.

    This monograph, one of a series on youth with disabilities and the juvenile justice system, reviews current data on disabilities requiring special education and related supports. Statistics on the prevalence of juvenile crime are followed by statistics on the prevalence of special education disabilities in the system, specifically specific…

  6. School Personnel Perceptions of Youth with Disabilities Returning to High School from the Juvenile Justice System

    Science.gov (United States)

    Sinclair, James S.; Unruh, Deanne K.; Griller Clark, Heather; Waintrup, Miriam G.

    2017-01-01

    Little is known about the perceptions of teachers of their students returning from the juvenile justice system, which can influence student relationships and student engagement, both of which are critical to reduce recidivism rates. This study utilized an online survey to examine the perceptions of a convenience sample of 283 school personnel…

  7. Special Education Advocacy in the Juvenile Justice System: Perspectives from Probation Officers

    Science.gov (United States)

    Burke, Meghan M.; Dalmage, Heather

    2016-01-01

    Although students with disabilities are over-represented in the juvenile justice system and frequently receive poor educational services, few studies have examined strategies to increase compliance with student needs and individualized education programs. In this study, we conducted interviews with eight probation officers in the Advocacy Unit of…

  8. Cluster-Derived Groupings of the Behavior Assessment System for Children among Male Juvenile Offenders

    Science.gov (United States)

    Scarborough, Zane T.; Glaser, Brian A.; Calhoun, Georgia B.; Stefurak, Tres; Petrocelli, John V.

    2004-01-01

    Examined the cluster-derived typologies of males in a juvenile offender sample. Adolescent males, age 12-17, in a regional youth detention center were given the Behavior Assessment System for Children-Self Report-Adolescent (BASC-SRP-A; C. Reynolds &R. Kamphaus, 1992) and the Millon Adolescent Clinical Inventory (MACI; T. Millon, 1993).…

  9. Disease activity patterns in juvenile systemic lupus erythematosus and its relation to early aggressive treatment

    NARCIS (Netherlands)

    Otten, M. H.; Cransberg, K.; van Rossum, M. A. J.; Groothoff, J. W.; Kist-van Holthe, J. E.; ten Cate, R.; van Suijlekom-Smit, L. W. A.

    2010-01-01

    This study aimed to determine disease activity patterns in juvenile systemic lupus erythematosus (jSLE) and its relation to early treatment. All jSLE patients who visited the outpatient departments of three Dutch university hospitals for at least 6 months were included. Data were retrospectively

  10. Hormone profile in juvenile systemic lupus erythematosus with previous or current amenorrhea

    NARCIS (Netherlands)

    Silva, Clovis A.; Deen, Maria E. J.; Febronio, Marilia V.; Oliveira, Sheila K.; Terreri, Maria T.; Sacchetti, Silvana B.; Sztajnbok, Flavio R.; Marini, Roberto; Quintero, Maria V.; Bica, Blanca E.; Pereira, Rosa M.; Bonfa, Eloisa; Ferriani, Virginia P.; Robazzi, Teresa C.; Magalhaes, Claudia S.; Hilario, Maria O.

    To identify the underlying mechanism of amenorrhea in juvenile systemic lupus erythematosus (JSLE) patients, thirty-five (11.7%) JSLE patients with current or previous amenorrhea were consecutively selected among the 298 post-menarche patients followed in 12 Brazilian pediatric rheumatology centers.

  11. Near-infrared spectroscopy during exercise and recovery in children with juvenile dermatomyositis

    NARCIS (Netherlands)

    Habers, G.E.; de Knikker, R.; van Brussel, M.; Hulzebos, E.; Stegeman, D.F.; van Royen, A.

    2013-01-01

    Introduction: We hypothesized that microvascular disturbances in muscle tissue play a role in the reduced exercise capacity in juvenile dermatomyositis (JDM). Methods: Children with JDM, children with juvenile idiopathic arthritis (clinical controls), and healthy children performed a maximal

  12. The importance of trunk perception during brace treatment in moderate juvenile idiopathic scoliosis: What is the impact on self-image?

    Science.gov (United States)

    Paolucci, Teresa; Piccinini, Giulia; Iosa, Marco; Piermattei, Cristina; De Angelis, Simona; Zangrando, Federico; Saraceni, Vincenzo Maria

    2017-01-01

    The perception of body image and the deformity of the trunk in patients with adolescent idiopathic scoliosis (AIS) are a silver lining that has yet to be discussed in the relevant literature during brace rehabilitation treatment. To determine whether and how the use of the brace changes perception of the trunk in patients with AIS by the drawing test. We observed 32 subjects with AIS from our Rehabilitation outpatient clinic and divided them into the brace treatment (BG-16 subjects) and the non-brace treatment (CG-16 subjects). Trunk perception and quality of life were evaluated using the Trunk Appearance Perception Scale and Scoliosis Research Society-22 questionnaire, and the perception of one's back was measured by the drawing test. Pain was lower in BG versus CG (p= 0.095). Satisfaction with the treatment was higher in BG than in CG (p= 0.002). Self-image did not differ significantly between the groups in terms of TAPS. Drawings of the most severe cases of scoliosis were made by the group without the brace. The use of the brace corrects the function of the trunk and has a positive influence on its perception.

  13. ScolioMedIS: web-oriented information system for idiopathic scoliosis visualization and monitoring.

    Science.gov (United States)

    Devedžić, Goran; Cuković, Saša; Luković, Vanja; Milošević, Danijela; Subburaj, K; Luković, Tanja

    2012-11-01

    Adolescent idiopathic scoliosis is the most common type of abnormal curvature observed in spine and it progresses rapidly during the puberty period. The most followed clinical way of assessing the spinal deformity is subjective by measuring the characteristic angles of spinal curve from a set of radiographic images. This paper presents a web-based information system (called ScolioMedIS) based on parameterized 3D anatomical models of the spine to quantitatively assess the deformity and to minimize the amount of radiation exposure by reducing the number of radiographs required. The main components of the system are 3D parametric solid model of spine, back surfaces, relevant clinical information and scoliosis ontology. The patient-specific spine model is regenerated from the parametric model and surface data using anatomical information extracted from radiographic images. The system is designed to take inherent advantage of Web for facilitating multi-center data collection and collaborative clinical decisions. The preliminary analysis of patient data showed promising results, which involve improved documentation standard, clinical decision knowledge base record, facilitated exchange and retrieval of medical data between institutions in multi-center clinical studies, 3D visualization of spinal deformity, and permanent monitoring of treatments. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  14. Design and acceptance of Rheumates@Work, a combined internet-based and in person instruction model, an interactive, educational, and cognitive behavioral program for children with juvenile idiopathic arthritis.

    Science.gov (United States)

    Armbrust, Wineke; Bos, Joyce J F J; Cappon, Jeannette; van Rossum, Marion A J J; Sauer, Pieter J J; Wulffraat, Nico; van Wijnen, Veera K; Lelieveld, Otto T H M

    2015-07-23

    Juvenile idiopathic arthritis (JIA) is a chronic rheumatic disease. Patients suffer daily discomforts such as pain, fatigue, stiffness, and mood disturbances. Their exercise capacity is decreased to a variable degree and physical activity levels may be impaired. To prevent long-term cardiovascular risks associated with JIA and medication, it is important to encourage physical activity. To achieve this we developed Rheumates@Work (R@W), a combined internet-based and in person instruction model, an interactive, educational, and cognitive behavioral program. The aim of this study is twofold: to describe the theoretical background and design of R@W based on Pender's Health Promotion Model, and to assess its acceptance. We enrolled 8 to 13-year-old JIA patients, from 3 outpatients clinics in The Netherlands, in R@W. Inclusion criteria were a low disease activity (VAS physician anonymous questionnaire concerning f.e. time investment and perceived benefits. Costs were monitored. Of the 64 patients we enrolled, 23 boys and 41 girls, 93.8 % completed the program. Participant-initiated interaction was seen in 10.7 %, 24.7 % send a mail because of technical problems. Eighty-two percent of the participants and 99 % of the parents liked the program, and 85 % of the participants indicated that they had learnt something, or quite a lot. Development costs of the program were low. The HPM is suitable for a behavioral intervention program such as R@W. Acceptance and satisfaction of R@W were high and the costs of the program were low. ISRCTN92733069.

  15. Short and long-term immunogenicity and safety following the 23-valent polysaccharide pneumococcal vaccine in juvenile idiopathic arthritis patients under conventional DMARDs with or without anti-TNF therapy.

    Science.gov (United States)

    Aikawa, Nadia E; França, Ivan L A; Ribeiro, Ana C; Sallum, Adriana M E; Bonfa, Eloisa; Silva, Clovis A

    2015-01-29

    To assess immunogenicity and safety of the 23-valent polysaccharide pneumococcal vaccine (PPV23) in juvenile idiopathic arthritis (JIA) patients under conventional DMARDs with or without anti-TNF therapy. The influences of demographic data, disease activity and treatment on immune response and the potential deleterious effects of vaccine on disease itself were also evaluated. 17 JIA patients immediately pre-etanercept (Group 1) and 10 JIA patients on stable dose of methotrexate (Group 2) received one dose of PPV23. All patients were evaluated pre-vaccination, 2 months and 12 months post-vaccination for seven pneumoccocal serotypes. Serology was performed by enzyme immunoassay and the immunogenicity endpoints included seroprotection (SP), seroconversion (SP) and geometric mean concentration of antibodies(GMC). Clinical and laboratorial parameters of JIA were evaluated before and after vaccination. Groups 1 and 2 were comparable regarding age, gender, disease duration and other DMARDs use (p>0.05). Pre-immunization SP and GMC were alike in patients with and without anti-TNF therapy (p>0.05). The frequencies of patients achieving adequate vaccine response (seroconversion in ≥50% of all serotypes) at 2 months (53 vs. 30%, p=0.424) and 12 months (36 vs. 40%, p=1.0) were similar in JIA patients with and without anti-TNF therapy. Further comparison of patients with and without adequate response at 2 months revealed no influence of demographic, clinical and laboratorial JIA parameters (p>0.05). Serious adverse events were not observed. Anti-TNF therapy in JIA patients does not seem to have an additional deleterious effect on short/long-term PPV23 immunogenicity compared to MTX alone and no influence on disease parameters was observed with this vaccine. Copyright © 2014 Elsevier Ltd. All rights reserved.

  16. Levonorgestrel-Releasing Intrauterine System (52 mg) for Idiopathic Heavy Menstrual Bleeding: A Health Technology Assessment.

    Science.gov (United States)

    2016-01-01

    Heavy menstrual bleeding affects as many as one in three women and has negative physical, economic, and psychosocial impacts including activity limitations and reduced quality of life. The goal of treatment is to make menstruation manageable, and options include medical therapy or surgery such as endometrial ablation or hysterectomy. This review examined the evidence of effectiveness and cost-effectiveness of the 52-mg levonorgestrel-releasing intrauterine system (LNG-IUS) as a treatment alternative for idiopathic heavy menstrual bleeding. We conducted a systematic review of the clinical and economic evidence comparing LNG-IUS with usual medical therapy, endometrial ablation, or hysterectomy. Medline, EMBASE, Cochrane, and the Centres for Reviews and Dissemination were searched from inception to August 2015. The quality of the evidence was assessed according to the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) Working Group criteria. We also completed an economic evaluation to determine the cost-effectiveness and budget impact of the LNG-IUS compared with endometrial ablation and with hysterectomy. The economic evaluation was conducted from the perspective the Ontario Ministry of Health and Long-Term Care. Relevant systematic reviews (n = 18) returned from the literature search were used to identify eligible randomized controlled trials, and 16 trials were included. The LNG-IUS improved quality of life and reduced menstrual blood loss better than usual medical therapy. There was no evidence of a significant difference in these outcomes compared with the improvements offered by endometrial ablation or hysterectomy. Mild hormonal side effects were the most commonly reported. The quality of the evidence varied from very low to moderate across outcomes. Results from the economic evaluation showed the LNG-IUS was less costly (incremental saving of $372 per person) and more effective providing higher quality-adjusted life years (incremental

  17. Síndrome de ativação macrofágica em paciente com lúpus eritematoso sistêmico juvenil Macrophage activation syndrome in a patient with juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Simone Manso de Carvalho

    2008-08-01

    Full Text Available A hemofagocitose reativa ou síndrome de ativação macrofágica (SAM é uma complicação das doenças inflamatórias sistêmicas, causada por expansão de células T e macrófagos, com produção maciça de citocinas pró-inflamatórias, ocorrendo mais freqüentemente na artrite idiopática juvenil sistêmica e raramente no lúpus eritematoso sistêmico juvenil (LESJ. OBJETIVO: Relatar um caso de LESJ que evoluiu com SAM precipitada por infecção e infarto esplênico, com desfecho fatal. RELATO DE CASO: Uma menina de 7 anos, com diagnóstico de LESJ desde os 5 anos, evoluiu com artrite em atividade, alopecia intensa, citopenias, cefaléia, infecções respiratórias recorrentes e elevação intermitente de transaminases. Os anticorpos anti-DNA e anticardiolipina IgG e IgM foram identificados e a biópsia renal evidenciou glomerulonefrite lúpica de classe III. A paciente foi tratada com pulso de metilprednisolona, prednisona, azatioprina e hidroxicloroquina. Após dois anos, na vigência de pneumonia apresentou abdome agudo e convulsões, evoluindo para o choque hemorrágico fatal após esplenectomia, que evidenciou infarto esplênico e infiltração maciça por macrófagos hemofagocíticos CD163+. CONCLUSÃO: A revisão do desfecho sugere a SAM precipitada por infecção e sobreposta a atividade inflamatória do lúpus com febre persistente, citopenias, disfunção hepática, hepatomegalia e esplenomegalia, como efeitos do excesso de produção de citocinas. Os anticorpos anticardiolipina podem ter tido papel precipitante na coagulopatia, que resultou infarto esplênico e choque hemorrágico.Reactive haemophagocytosis or macrophage activation syndrome (MAS is a complication of systemic inflammatory disorders, caused by expansion of T cells and haemophagocytic macrophages, with cytokine overproduction. It has been described most often in systemic juvenile idiopathic arthritis and rarely in juvenile systemic lupus erythematosus (JSLE

  18. EFFICACY OF RECURRENT RITUXIMAB TREATMENT IN PATIENT WITH SEVERE REFRACTORY SYSTEMIC JUVENILE RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    E.I. Alexeeva

    2011-01-01

    Full Text Available The article contains clinical case description of a severe systemic juvenile rheumatoid arthritis, that was refractory to classic immunosuppressant therapy. The disease was characterized by such extraarticular manifestations as fever, lymphadenopathy,  hepatosplenomegaly, polyserositis, generalized joint involvement and high activity in lab tests. As a result of severe clinical course of the disease, patients develop bilateral aseptic bone necrosis in coxofemoral joints and coxarthrosis. Against the background of glucocorticosteroid treatment the patient has developed hormone-dependency and hormone resistance. Inclusion into the treatment of anti-CD20 monoclonal antibodies (rituximab has stopped systemic manifestations of the disease, inflammation in the joints, normalized lab activity rates. The positive therapeutic effect allowed to perform surgery due to bilateral coxarthrosis. These results show that rituximab is highly effective in children with systemic juvenile rheumatoid arthritis, that is resistant to classic immunosupressants and glucocorticoides. Key words: children, systemic juvenile rheumatoid arthritis, rituximab, recurrent treatment, prosthetics, hip joint. (Voprosy sovremennoi pediatrii — Current Pediatrics. — 2011; 10 (5: 157–163.

  19. A Research Framework for Understanding the Practical Impact of Family Involvement in the Juvenile Justice System: The Juvenile Justice Family Involvement Model.

    Science.gov (United States)

    Walker, Sarah Cusworth; Bishop, Asia S; Pullmann, Michael D; Bauer, Grace

    2015-12-01

    Family involvement is recognized as a critical element of service planning for children's mental health, welfare and education. For the juvenile justice system, however, parents' roles in this system are complex due to youths' legal rights, public safety, a process which can legally position parents as plaintiffs, and a historical legacy of blaming parents for youth indiscretions. Three recent national surveys of juvenile justice-involved parents reveal that the current paradigm elicits feelings of stress, shame and distrust among parents and is likely leading to worse outcomes for youth, families and communities. While research on the impact of family involvement in the justice system is starting to emerge, the field currently has no organizing framework to guide a research agenda, interpret outcomes or translate findings for practitioners. We propose a research framework for family involvement that is informed by a comprehensive review and content analysis of current, published arguments for family involvement in juvenile justice along with a synthesis of family involvement efforts in other child-serving systems. In this model, family involvement is presented as an ascending, ordinal concept beginning with (1) exclusion, and moving toward climates characterized by (2) information-giving, (3) information-eliciting and (4) full, decision-making partnerships. Specific examples of how courts and facilities might align with these levels are described. Further, the model makes predictions for how involvement will impact outcomes at multiple levels with applications for other child-serving systems.

  20. Processes of constitution of the uruguayan juvenile criminal system. ¿hybrids paradigms?

    OpenAIRE

    López Gallego, Laura

    2017-01-01

    In this paper, the analysis focuses on adolescence as a particularized space of social criminal control   through devices that have historically constituted the Uruguayan juvenile criminal system. Social criminal control practices make up a heterogeneous field composed of a multiplicity of vectors which show the ways in which people are tried and/or treated according to their departures from the prevailing criminal law in a particularly socio-historical context . They set concrete social prac...

  1. PROCESSES OF CONSTITUTION OF THE URUGUAYAN JUVENILE CRIMINAL SYSTEM. ¿HYBRIDS PARADIGMS?

    OpenAIRE

    López Gallego, Laura

    2017-01-01

    In this paper, the analysis focuses on adolescence as a particularized space of social criminal control   through devices that have historically constituted the Uruguayan juvenile criminal system. Social criminal control practices make up a heterogeneous field composed of a multiplicity of vectors which show the ways in which people are tried and/or treated according to their departures from the prevailing criminal law in a particularly socio-historical context . They set concrete social prac...

  2. Miliary tuberculosis: a severe opportunistic infection in juvenile systemic lupus erythematosus patients

    OpenAIRE

    Freire, Priscilla S.; Montoni, João D.; Ribeiro, Aline S.M.; Marques, Heloísa H.; Mauad, Thais; Silva, Clovis A.

    2016-01-01

    Abstract Introduction One of the main issues in juvenile systemic lupus erythematosus (JSLE) patients is infection, such as tuberculosis (TB). Of note, SLE patients are susceptible to pulmonary and extrapulmonary TB. However, to our knowledge, this contagious disease was rarely reported in pediatric lupus population, particularly diffuse or miliary TB. Therefore, from January 1983 to December 2011, 5,635 patients were followed-up at our Pediatric Rheumatology Unit and 285 (5%) of them met th...

  3. Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature

    OpenAIRE

    Chiewchengchol, Direkrit; Murphy, Ruth; Edwards, Steven W; Beresford, Michael W

    2015-01-01

    Patients diagnosed with juvenile-onset systemic lupus erythematosus (JSLE) often have skin and oral lesions as part of their presentation. These mucocutaneous lesions, as defined by the American College of Rheumatology (ACR) in 1997, include malar rash, discoid rash, photosensitivity and oral ulcers. It is therefore essential to recognize mucocutaneous lesions to accurately diagnose JSLE. The mucocutaneous lesions can be divided into those with classical histological features (LE specific) an...

  4. Oral Ulcers in Juvenile-Onset Systemic Lupus Erythematosus: A Review of the Literature

    OpenAIRE

    Rodsaward, Pongsawat; Prueksrisakul, Titipong; Deekajorndech, Tawatchai; Edwards, Steven W.; Beresford, Michael W.; Chiewchengchol, Direkrit

    2017-01-01

    Oral ulcers are the most common mucosal sign in juvenile-onset systemic lupus erythematosus (JSLE). The ulcers are one of the key clinical features; however, the terminology of oral ulcers, especially in JSLE patients, is often vague and ill-defined. In fact, there are several clinical manifestations of oral ulcers in JSLE, and some lesions occur when the disease is active, indicating that early management of the disease should be started. Oral ulcers are classified as lupus erythematosus (LE...

  5. Trauma, Delinquency, and Substance Use: Co-occurring Problems for Adolescent Girls in the Juvenile Justice System

    OpenAIRE

    Smith, Dana K.; Saldana, Lisa

    2013-01-01

    Girls in the juvenile justice system are known to have high rates of co-occurring childhood abuse, trauma, and substance abuse. Girls with this constellation of problems are at high risk for serious adverse outcomes, including problems with drug dependence and abuse. The relationship between childhood sexual abuse, childhood physical abuse, other types of childhood trauma, and rates of substance use during adolescence were examined for girls in the juvenile justice system. As expected, childh...

  6. Consenso em reumatologia pediátrica: parte I - definição dos critérios de doença inativa e remissão em artrite idiopática juvenil/artrite reumatóide juvenil Consensus in pediatric rheumatology: part I - criteria definition of inactive disease and remission in juvenile idiopathic arthritis / juvenile rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    Claudia Machado

    2005-02-01

    Full Text Available Não há critérios universalmente aceitos para a remissão clínica em artrite idiopática juvenil/artrite reumatóide juvenil (AIJ/ARJ. OBJETIVO: formar consenso sobre estes critérios. MÉTODOS: foi utilizado um inquérito pelo método Delphi para reunir os critérios vigentes e utilizados por especialistas em reumatologia pediátrica (RP no mundo todo. A análise dos resultados constituiu a base para uma Consensus Conference utilizando a nominal group technique (NGT para alcançar o consenso nas questões não resolvidas após a análise dos questionários deste inquérito. Cento e trinta RP de 34 países responderam ao inquérito e 20 RP de nove países elegeram os critérios durante dois dias, em processo de discussão estruturada, para formar consenso pela NGT. RESULTADOS: os critérios de doença inativa deveriam incluir: 1 nenhuma articulação com artrite em atividade; 2 ausência de febre, rash, serosite, esplenomegalia ou linfadenopatia generalizada atribuída à AIJ/ARJ; 3 ausência de uveíte em atividade; 4 VHS ou PCR negativas (se ambos forem testados, ambos devem ser normais; 5 a avaliação global pelo médico deve indicar o melhor escore possível, indicando doença inativa. CONCLUSÕES: de acordo com o voto de consenso, seis meses contínuos de doença inativa são necessários para se considerar um paciente em estado de remissão com medicação; 12 meses contínuos de doença inativa e sem medicação são necessários para considerar um paciente em estado de remissão sem medicação. O critério para remissão sem medicação deve prever com acurácia de 95% a probabilidade inferior a 20% de recaída em cinco anos.Validated and widely accepted criteria for clinical remission in JIA/JRA do not currently exist. OBJECTIVE: To achieve consensus in this matter. METHODS: The Delphi consensus-formation approach was used to gather the criteria in use by pediatric rheumatologists (PR worldwide. Results from the questionnaires

  7. Youth, Guns, and the Juvenile Justice System. Research Report.

    Science.gov (United States)

    Butts, Jeffrey; Coggeshall, Mark; Gouvis, Caterina; Mears, Daniel; Travis, Jeremy; Waul, Michelle; White, Ruth

    This report documents trends in youth gun violence and the response within the justice system, noting the growing variety of data resources available to investigate the effect of new gun laws on youth, communities, and public safety. The first section reviews recent trends, examining the major wave of gun violence in the United States during the…

  8. Juvenile Arrests, 2007. Juvenile Justice Bulletin

    Science.gov (United States)

    Puzzanchera, Charles

    2009-01-01

    This Bulletin summarizes 2007 juvenile crime and arrest data reported by local law enforcement agencies across the country and cited in the FBI report, "Crime in the United States 2007." The Bulletin describes the extent and nature of juvenile crime that comes to the attention of the justice system. It serves as a baseline for comparison for…

  9. Role of the immune system in the pathogenesis of idiopathic nephrotic syndrome

    NARCIS (Netherlands)

    van den Berg, José G.; Weening, Jan J.

    2004-01-01

    Idiopathic NS (nephrotic syndrome) is characterized by massive proteinuria, due to a leak in the glomerular barrier to proteins. Genetic defects that affect the function and the composition of the glomerular capillary wall, in particular of the visceral epithelial cells, have recently been

  10. Idiopathic Interstitial Pneumonias

    Science.gov (United States)

    ... Most Often Affected Percentage of Affected People Who Smoke Cigarettes Treatment Outlook Idiopathic pulmonary fibrosis More frequently, men ... HealthDay Vitamin D Linked to Lower Risk of Respiratory Infections News HealthDay Remede System Approved for Sleep Apnea News HealthDay Nasal Swab ...

  11. Oral health and the masticatory system in juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Fernandes, E G C; Savioli, C; Siqueira, J T T; Silva, C A A

    2007-01-01

    Our objectives were to evaluate the oral health and the masticatory system of 48 juvenile systemic lupus erythematosus (JSLE) patients and to compare them with 48 healthy children and adolescents. Demographic data, clinical manifestations and therapies of JSLE were reviewed. The DMFT index (DMFTI), the plaque (PI) and the gingival bleeding (GI) indices, dental relationship, facial profile, clinical dysfunction and mandibular mobility indices were evaluated. The two groups were homogeneous regarding age, gender, Brazilian social-economic class and dental decay index (P > 0.05). Of note, the medians of the PI and the GI were higher in JSLE patients than in controls (61.5 versus 38.10, P = 0.003 and 26.0 versus 15.95, P = 0.014; respectively). Likewise, a linear statistical correlation was evidenced between the JSLE duration and the GI (P = 0.017, r = 0.11), cumulative dose of prednisone and the PI (P = 0.01, r = 0.385) and cumulative dose of prednisone and the GI ( P = 0.001, r = 0.471). The clinical dysfunction and mandibular mobility indices were higher in JSLE patients versus controls (P = 0.002, P = 0.025). Moreover, the median of the mandibular mobility index was higher in JSLE patients who used at least one immunosuppressive than on those who did not use this medication (P = 0.0001). These results suggest that JSLE patients had an inadequate oral hygiene, higher incidence of gingivitis and temporomandibular joint dysfunction.

  12. Juvenile systemic lupus erythematosus onset patterns in Vietnamese children

    DEFF Research Database (Denmark)

    Dung, Nguyen Thi Ngoc; Loan, Huynh Thoai; Nielsen, Susan

    2013-01-01

    to have systemic lupus erythematosus (f/m = 4/1) were referred to the Ho Chi Minh City Children's Hospital No.1 during a 12-month period in 2009. RESULTS: The mean age at diagnosis was 12.8 years (SD = 2.5). Thirty-seven (82%) fulfilled criteria for lupus nephritis (LN). At diagnosis, impressively high...... No. 1 during a16 month period from 2008-2009. These patients had a strikingly high prevalence of Coombs positive anaemia, a high prevalence of lupus nephritis, and very high SLEDAI and ECLAM scores at the time of diagnosis. While there may be referral biases, our Vietnamese SLE patients appear...

  13. Idiopathic epileptic syndromes and cognition.

    Science.gov (United States)

    Hommet, Caroline; Sauerwein, Hannelore C; De Toffol, Bertrand; Lassonde, Maryse

    2006-01-01

    Epilepsy is frequently associated with cognitive impairments which result from various interacting factors. The present paper deals with the contribution of neuropsychology to the characterization of the type of epilepsy and the possible mechanisms underlying idiopathic epileptic syndromes. The non-lesional, so-called idiopathic epilepsies, constitute an interesting model for assessing the relationship between epileptiform EEG discharges and cognition. Among the idiopathic generalized epilepsies, disorders of social integration and personality have been frequently reported in juvenile myoclonic epilepsy (JME). Since similar disturbances are observed in frontal-lobe-lesioned patients, impairments in other frontal lobe functions (e.g. executive functions) might be expected in JME. This gives rise to speculation about the possible underlying pathophysiological mechanisms in JME. With regard to partial idiopathic epilepsies, benign childhood epilepsy with centrotemporal spikes (BCECTS) may provide a useful model for the study of the relationship between epileptiform EEG discharges in the peri-sylvian region and language functions. Furthermore, the description of mild cognitive dysfunctions in BCECTS, and their persistence into adulthood, can provide information about compensatory mechanisms and may allow for the generation of remedial strategies. Thus, 'lesional' neuropsychology has given way to 'dynamic' neuropsychology based on specific postulates. By using the cognitive profile to specify the mechanism underlying the behavioral disturbances observed in different types of epilepsy, neuropsychology may eventually contribute to a revision of the present classification of epileptic syndromes. In addition, the neuropsychological data may help predict the extent and limits of functional recovery and cerebral plasticity.

  14. Avaliação de provas de fase aguda em crianças e adolescentes com artrite idiopática juvenil e sua correlação com atividade da doença Acute phase reactants evaluation in children and adolescents with juvenile idiopathic arthritis and its correlation with disease activity

    Directory of Open Access Journals (Sweden)

    Aline Alencar M. F. Nicácio

    2009-06-01

    Full Text Available OBJETIVO:Analisar a relação entre as provas de fase aguda e a atividade clínica da artrite idiopática juvenil e avaliar a concordância entre velocidade de hemossedimentação e proteína C reativa (VHS e PCR na fase aguda da doença. MÉTODOS: Foi realizado estudo retrospectivo tipo coorte a partir da análise de prontuários de 30 crianças e adolescentes que preenchiam os critérios diagnósticos para artrite idiopática juvenil, estavam em atendimento em ambulatório de Reumatologia Pediátrica e haviam realizado as provas de fase aguda (VHS e PCR. RESULTADOS: Dos 30 pacientes, 21 (70% eram do sexo feminino e 19 (63,3% apresentavam o subtipo oligoarticular da doença. A média de idade de início dos sintomas foi 65,6 meses, a idade de diagnóstico de 85,3 e o tempo de evolução, 57,2 meses. As provas de fase aguda mostraram associação positiva com a atividade de doença. A anemia não teve relação com a atividade de doença. A concordância entre as duas provas de fase aguda foi superior a 80%. CONCLUSÕES: As provas de fase aguda mantêm relação positiva com a atividade da doença e o seu uso concomitante aumenta a especificidade.OBJECTIVE:To analyze the relationship between the acute phase reactants and the disease activity of Juvenile Idiopathic Arthritis (JIA and to evaluate the agreement between erythrocyte sedimentation rate and C-reactive protein during the acute phase of the disease. METHODS: a cohort retrospective study has been conducted based on the analysis of 30 children and adolescents who fulfilled the diagnostic criteria of JIA. All of them were in current follow-up at the pediatric rheumatology outpatient clinic and had acute phase reactants blood tests performed. RESULTS: Studied population comprised 30 patients: 21 (70% of them were females and 19 (63.3% presented oligoarticular subtype. The mean age at disease onset was 65.6 months; the age at diagnosis was 85.3 months and the follow-up had 57.2 months of

  15. A radiographic classification system in juvenile rheumatoid arthritis applied to the knee

    International Nuclear Information System (INIS)

    Dale, K.; Paus, A.C.; Laires, K.

    1994-01-01

    A new radiographic grading system for evaluation of juvenile rheumatoid arthritis (JRA) for the knee is presented. The classification is based on known arthritic criteria in childhood. Joints with erosion are given a higher score than growth disturbances alone. Signs of osteoarthrosis including joint space narrowing were excluded from the classification. The femorotibial and patello-femoral joints are assessed together. Verbal definitions are used for the classification, but, regarding the erosions, standard reference films are used. The intra- and inter-observer variations of the method were low. (P < 0.01) (orig.)

  16. Social Justice for Crossover Youth: The Intersection of the Child Welfare and Juvenile Justice Systems.

    Science.gov (United States)

    Kolivoski, Karen M; Goodkind, Sara; Shook, Jeffrey J

    2017-10-01

    Social workers are critical to promoting racial and social justice. "Crossover youth," a term used to describe youths who have contact with both the child welfare and juvenile justice systems, are an especially vulnerable but often overlooked population with whom social workers engage. A disproportionate number of crossover youth are African American. Empirical research on crossover youth is growing, but such scholarship rarely engages with a human rights and social justice perspective. African American children and youths have a distinct place within the history and current context of the child welfare and juvenile justice systems. These systems have historically excluded them or treated them differently; now, African American youths are overrepresented in each of them, and evidence suggests they are more likely to cross over. The purpose of this article is to describe the historical and current context of crossover youth, with a particular focus on African American youths, to provide the foundation for a discussion of what social workers can do to promote racial and social justice for crossover youth, including specific implications for practice and policy, as well as broader implications for human and civil rights. © 2017 National Association of Social Workers.

  17. Accountability in Teenage Dating Violence: A Comparative Examination of Adult Domestic Violence and Juvenile Justice Systems Policies

    Science.gov (United States)

    Zosky, Diane L.

    2010-01-01

    Unlike in the adult criminal justice system, where domestic violence policies hold perpetrators accountable for their violence, the juvenile justice system rarely addresses teenage dating violence. Although the adult criminal justice system has pursued policies toward intimate partner violence grounded on a "zero tolerance" ideology, the juvenile…

  18. Acute Lymphoblastic Leukaemia presenting as Juvenile Idiopathic

    African Journals Online (AJOL)

    PROF. EZECHUKWU

    2013-04-28

    Apr 28, 2013 ... malignancies that occur in children <15year of age and. Acute lymphoblastic leukaemia (ALL) accounts for about 77% of cases of childhood leukemias.1 Bone pain, particularly of the lower extremities is a common manifestation of childhood ALL. Although the initial presentation of childhood ALL is usually ...

  19. Perceived parental security profiles in African American adolescents involved in the juvenile justice system.

    Science.gov (United States)

    Andretta, James R; Ramirez, Aaron M; Barnes, Michael E; Odom, Terri; Roberson-Adams, Shelia; Woodland, Malcolm H

    2015-12-01

    Many researchers have shown the importance of parent attachment in childhood and adolescence. The present study extends the attachment literature to African Americans involved in the juvenile justice system (N = 213), and provides an initial inquiry using person-oriented methods. The average age was 16.17 years (SD = 1.44), and the sample was predominantly male (71%). Results of a confirmatory factor analysis of Inventory of Parent and Peer Attachment-Short Form (IPPA-S) scores supported a 3-factor model: (a) Communication, (b) Trust, and (c) Alienation. Model-based clustering was applied to IPPA-S scores, and results pointed to 4 perceived parental security profiles: high security, moderately high security, moderately low security, and low security. In keeping with our hypotheses, IPPA-S profiles were associated with prosocial behaviors, depression, anxiety, and oppositional defiance. Contrary to hypotheses, IPPA-S profiles were not associated with perspective taking, emotional concern, or behaviors characteristic of a conduct disorder. Results also showed that gender, age, family member with whom the participant resides, charge severity, and offense history did not have an effect on IPPA-S clustering. Implications for therapeutic jurisprudence in African Americans involved with the juvenile justice system are provided. (c) 2015 APA, all rights reserved).

  20. Initial manifestations and clinical course of systemic onset juvenile idiopathic arthritis: A ten-year retrospective study

    Directory of Open Access Journals (Sweden)

    Hu-Yuan Tsai

    2012-10-01

    Conclusion: In SoJIA, extra-articular features such as fever, rash, and lymphadenopathy are most prominent. Leukocytosis and polyarticular pattern on presentation may indicate a refractory clinical course.

  1. Successful management of acute respiratory failure in an Idiopathic Pulmonary Fibrosis patient using an extracorporeal carbon dioxide removal system.

    Science.gov (United States)

    Vianello, Andrea; Arcaro, Giovanna; Paladini, Luciana; Iovino, Silvia

    2016-08-01

    Patients with Idiopathic Pulmonary Fibrosis (IPF) requiring Invasive Mechanical Ventilation (IMV) following unsuccessful treatment with Non-Invasive Ventilation (NIV) have a high mortality rate. IMV is, moreover, an independent predictor of poor outcome during the post-transplantation period in patients on waiting lists for Lung Transplantation (LT). Here we describe the successful management of an IPF patient with acute respiratory failure (ARF) using a pump-assisted veno-venous system for extracorporeal CO2 removal (ECCO2R) (ProLUNG® system) as an alternative to endotracheal intubation (ETI) following NIV failure. Given this positive experience, further studies are warranted focusing on the ECCO2R system's tolerability, safety, and efficacy in patients with IPF and severe ARF in whom NIV alone is ineffective.

  2. Explaining Ethnic Inequality in the Juvenile Justice System, an analysis of the outcomes of Dutch Prosecutorial Decision Making

    NARCIS (Netherlands)

    Weenink, D.

    2009-01-01

    Most studies of the treatment of minorities in criminal justice systems show that ethnic minorities are punished more harshly. This paper aims to explain ethnic inequality in prosecutorial decision making in the Dutch juvenile justice system. Based on statistical analyses of 409 case files, it

  3. Power, Process, and Protection: Juveniles as Defendants in the Justice System.

    Science.gov (United States)

    Woolard, Jennifer L; Henning, Kristin; Fountain, Erika

    The juvenile court was created in 1899 in part to remedy the unfairness of trying youth in the adult criminal justice system, but its success at rectifying those problems is unclear. One concern is that the vast majority of youth who are adjudicated delinquent are adjudicated after waiving their right to trial and entering a guilty plea. Fairness and equity in the plea bargaining process are premised on the assumption that youth have the capacity to understand and elect between available options and will be given a meaningful opportunity to choose without coercion and deception. In legal terms, the Constitution will only sanction a plea when the defendant makes a knowing, voluntary, and intelligent waiver of her right to trial. In this chapter, we briefly describe the juvenile court process and explain the circumstances of a plea bargain, which constitutes both a waiver of Constitutional rights and an agreement to certain conditions. Then we evaluate the research and practice knowledge regarding the legal components of a valid waiver-that it must be knowing and voluntary. We consider how information, capacity, and circumstance contribute to a knowing waiver. Then we examine how procedural justice, paternalism, and coercion may affect a voluntary waiver. Throughout, we consider whether the people, policies, and practices meant to assess and safeguard that waiver decision fulfill their intended purpose. © 2016 Elsevier Inc. All rights reserved.

  4. Idiopathic oedema

    DEFF Research Database (Denmark)

    Courtnadge, Christina; Madsen, Flemming; Bygum, Anette

    2018-01-01

    Idiopathic oedema is a non-inflammatory interstitial oedema seen in fertile women. A hallmark is a daily increase in weight > 1.4 kg from morning until evening due to fluid retention, which shows as facial puffiness in the morning and more often abdominal increase and swollen lower extremities...... during the day. Patients may be disabled due to accompanying symptoms like headache, dizziness, fatigue, anxiety, and depression. The diagnosis is based on clinical features and exclusion of other causes of oedema. Non-pharmacological interventions and pharmacological therapies are reviewed....

  5. Estimating the Optimal Dosage of Sodium Valproate in Idiopathic Generalized Epilepsy with Adaptive Neuro-Fuzzy Inference System

    Directory of Open Access Journals (Sweden)

    Somayyeh Lotfi Noghabi

    2012-07-01

    Full Text Available Introduction: Epilepsy is a clinical syndrome in which seizures have a tendency to recur. Sodium valproate is the most effective drug in the treatment of all types of generalized seizures. Finding the optimal dosage (the lowest effective dose of sodium valproate is a real challenge to all neurologists. In this study, a new approach based on Adaptive Neuro-Fuzzy Inference System (ANFIS was presented for estimating the optimal dosage of sodium valproate in IGE (Idiopathic Generalized Epilepsy patients. Methods: 40 patients with Idiopathic Generalized Epilepsy, who were referred to the neurology department of Mashhad University of Medical Sciences between the years 2006-2011, were included in this study. The function Adaptive Neuro- Fuzzy Inference System (ANFIS constructs a Fuzzy Inference System (FIS whose membership function parameters are tuned (adjusted using either a back-propagation algorithm alone, or in combination with the least squares type of method (hybrid algorithm. In this study, we used hybrid method for adjusting the parameters. Methods: The R-square of the proposed system was %598 and the Pearson correlation coefficient was significant (P 0.05. Although the accuracy of the model was not high, it wasgood enough to be applied for treating the IGE patients with sodium valproate. Discussion: This paper presented a new application of ANFIS for estimating the optimal dosage of sodium valproate in IGE patients. Fuzzy set theory plays an important role in dealing with uncertainty when making decisions in medical applications. Collectively, it seems that ANFIS has a high capacity to be applied in medical sciences, especially neurology.

  6. Increased Fas and Bcl-2 Expression on Peripheral Blood T and B Lymphocytes from Juvenile-Onset Systemic Lupus Erythematosus, but not from Juvenile Rheumatoid Arthritis and Juvenile Dermatomyositis

    Directory of Open Access Journals (Sweden)

    Bernadete L. Liphaus

    2006-01-01

    Full Text Available Defective regulation of apoptosis may play a role in the development of autoimmune diseases. Fas and Bcl-2 proteins are involved in the control of apoptosis. The aims of this study were to determine the expression of Fas antigen and Bcl-2 protein on peripheral blood T and B lymphocytes from patients with juvenile-onset systemic lupus erythematosus (JSLE, juvenile rheumatoid arthritis (JRA and juvenile dermatomyositis (JDM. Thirty-eight patients with JSLE, 19 patients with JRA, 10 patients with JDM and 25 healthy controls entered the study. Freshly isolated peripheral blood mononuclear cells (PBMC were stained for lymphocyte markers CD3, CD4, CD8, CD19 and for Fas and Bcl-2 molecules. Expressions were measured by three-color flow cytometry. Statistical analysis was performed using Kruskal–Wallis test. Percentages of freshly isolated T lymphocytes positively stained for Fas protein from JSLE patients were significantly increased compared to healthy controls, patients with JRA and patients with JDM. Percentages of B lymphocytes positive for Fas from JSLE patients were higher than healthy controls and JRA patients. In addition, Fas expression on T cells from patients with JRA was increased compared to JDM patients. Otherwise, Fas expression on T and B cells from JRA and JDM patients were similar to healthy controls. MFI of Bcl-2 positive T lymphocytes from JSLE patients were significantly increased compared to healthy controls and JRA patients. MFI of Bcl-2 protein on B lymphocytes from JSLE patients was similar to healthy controls and patients with JRA and JDM. Bcl-2 expression did not differ between JRA and JDM patients and healthy controls. In conclusion, increased expression of Fas and Bcl-2 proteins observed in circulating T and B lymphocytes from patients with JSLE, but not from patients with JRA and JDM, suggests that abnormalities of apoptosis may be related to the pathogenesis of JSLE and probably are not a result of chronic inflammation.

  7. Expression of immune system-related genes during ontogeny in experimentally wounded common carp (Cyprinus carpio) larvae and juveniles

    DEFF Research Database (Denmark)

    Schmidt, Jacob; Nielsen, Michael Engelbrecht

    2014-01-01

    they were microscopically indistinguishable from normal tissue by day 3 post-wounding in all but the juvenile carp wounded on day 49 post-fertilization. In these juveniles the wounded area was still visible even 7days post-wounding. On the transcriptional level a very limited response was observed......We investigated the effect of full-thickness incisional wounding on expression of genes related to the immune system in larvae and juveniles of common carp (Cyprinus carpio). The wounds were inflicted by needle puncture immediately below the anterior part of the dorsal fin on days 7, 14, 28 and 49...... after fertilization. We followed the local gene expression 1, 3 and 7days after wounding by removing head and viscera before extracting RNA from the remaining part of the fish, including the wound area. In addition, we visually followed wound healing. Overall the wounds had regenerated to a point where...

  8. Angiotensin II type 1 and 2 receptors and lymphatic vessels modulate lung remodeling and fibrosis in systemic sclerosis and idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Parra, Edwin Roger; Ruppert, Aline Domingos Pinto; Capelozzi, Vera Luiza

    2014-01-01

    To validate the importance of the angiotensin II receptor isotypes and the lymphatic vessels in systemic sclerosis and idiopathic pulmonary fibrosis. We examined angiotensin II type 1 and 2 receptors and lymphatic vessels in the pulmonary tissues obtained from open lung biopsies of 30 patients with systemic sclerosis and 28 patients with idiopathic pulmonary fibrosis. Their histologic patterns included cellular and fibrotic non-specific interstitial pneumonia for systemic sclerosis and usual interstitial pneumonia for idiopathic pulmonary fibrosis. We used immunohistochemistry and histomorphometry to evaluate the number of cells in the alveolar septae and the vessels stained by these markers. Survival curves were also used. We found a significantly increased percentage of septal and vessel cells immunostained for the angiotensin type 1 and 2 receptors in the systemic sclerosis and idiopathic pulmonary fibrosis patients compared with the controls. A similar percentage of angiotensin 2 receptor positive vessel cells was observed in fibrotic non-specific interstitial pneumonia and usual interstitial pneumonia. A significantly increased percentage of lymphatic vessels was present in the usual interstitial pneumonia group compared with the non-specific interstitial pneumonia and control groups. A Cox regression analysis showed a high risk of death for the patients with usual interstitial pneumonia and a high percentage of vessel cells immunostained for the angiotensin 2 receptor in the lymphatic vessels. We concluded that angiotensin II receptor expression in the lung parenchyma can potentially control organ remodeling and fibrosis, which suggests that strategies aimed at preventing high angiotensin 2 receptor expression may be used as potential therapeutic target in patients with pulmonary systemic sclerosis and idiopathic pulmonary fibrosis.

  9. Nervous system development in lecithotrophic larval and juvenile stages of the annelid Capitella teleta.

    Science.gov (United States)

    Meyer, Néva P; Carrillo-Baltodano, Allan; Moore, Richard E; Seaver, Elaine C

    2015-01-01

    Reconstructing the evolutionary history of nervous systems requires an understanding of their architecture and development across diverse taxa. The spiralians encompass diverse body plans and organ systems, and within the spiralians, annelids exhibit a variety of morphologies, life histories, feeding modes and associated nervous systems, making them an ideal group for studying evolution of nervous systems. We describe nervous system development in the annelid Capitella teleta (Blake JA, Grassle JP, Eckelbarger KJ. Capitella teleta, a new species designation for the opportunistic and experimental Capitella sp. I, with a review of the literature for confirmed records. Zoosymposia. 2009;2:25-53) using whole-mount in situ hybridization for a synaptotagmin 1 homolog, nuclear stains, and cross-reactive antibodies against acetylated α-tubulin, 5-HT and FMRFamide. Capitella teleta is member of the Sedentaria (Struck TH, Paul C, Hill N, Hartmann S, Hosel C, Kube M, et al. Phylogenomic analyses unravel annelid evolution. Nature. 2011;471:95-8) and has an indirectly-developing, lecithotrophic larva. The nervous system of C. teleta shares many features with other annelids, including a brain and a ladder-like ventral nerve cord with five connectives, reiterated commissures, and pairs of peripheral nerves. Development of the nervous system begins with the first neurons differentiating in the brain, and follows a temporal order from central to peripheral and from anterior to posterior. Similar to other annelids, neurons with serotonin-like-immunoreactivity (5HT-LIR) and FMRFamide-like-immunoreactivity (FMRF-LIR) are found throughout the brain and ventral nerve cord. A small number of larval-specific neurons and neurites are present, but are visible only after the central nervous system begins to form. These larval neurons are not visible after metamorphosis while the rest of the nervous system is largely unchanged in juveniles. Most of the nervous system that forms during

  10. Oral Ulcers in Juvenile-Onset Systemic Lupus Erythematosus: A Review of the Literature.

    Science.gov (United States)

    Rodsaward, Pongsawat; Prueksrisakul, Titipong; Deekajorndech, Tawatchai; Edwards, Steven W; Beresford, Michael W; Chiewchengchol, Direkrit

    2017-12-01

    Oral ulcers are the most common mucosal sign in juvenile-onset systemic lupus erythematosus (JSLE). The ulcers are one of the key clinical features; however, the terminology of oral ulcers, especially in JSLE patients, is often vague and ill-defined. In fact, there are several clinical manifestations of oral ulcers in JSLE, and some lesions occur when the disease is active, indicating that early management of the disease should be started. Oral ulcers are classified as lupus erythematosus (LE) specific, where the lesional biopsy shows a unique pattern of mucosal change in LE, and LE nonspecific, where the ulcers and their histopathological findings can be found in other oral diseases. Here, the clinical manifestations, diagnosis and management of oral ulcers in JSLE patients are reviewed.

  11. NDTAC Practice Guide: Quality Education Services Are Critical for Youth Involved with the Juvenile Justice and Child Welfare Systems

    Science.gov (United States)

    Gonsoulin, Simon; Clark, Heather Griller; Rankin, Victoria E.

    2015-01-01

    This National Evaluation and Technical Assistance Center for the Education of Children and Youth Who are Neglected, Delinquent, or At-Risk (NDTAC) practice guide examines the principle that quality education services are critical for youth involved with the juvenile justice and child welfare systems. This principle asserts that, to address the…

  12. Trends in nitrogen isotope ratios of juvenile winter flounder reflect changing nitrogen inputs to Rhode Island, USA estuarine systems

    Science.gov (United States)

    Nitrogen isotope ratios (d 15N) in juvenile winter flounder, Pseudopleuronectes americanus, were used to examine changes in nitrogen inputs to several Rhode Island, USA estuarine systems. Fish were collected over two three-year periods with a ten-year interval between sampling pe...

  13. Research-Based Practices for Reintegrating Students with Emotional and Behavioral Disorders from the Juvenile Justice System

    Science.gov (United States)

    Mathur, Sarup R.; Griller Clark, Heather; LaCroix, Leslie; Short, James

    2018-01-01

    This article discusses the unique and influential role of school teachers in the rehabilitation and reintegration of students with emotional and behavioral disorders who have been involved with the juvenile justice (JJ) system. By adopting evidenced-based practices, highlighted within this article, teachers can prevent further escalation of…

  14. Idiopathic hemihypertrophy.

    Science.gov (United States)

    Mohanna, Mabrook A; Sallam, Abdul Khalig

    2014-04-01

    Idiopathic hemihypertrophy is a congenital overgrowth disorder associated with an augmented risk for embryonal tumors. We present 4 cases of hemihypertrophy in Yemeni children living in Sana`a city. They presented to the outpatient clinics in a private hospital in Sana`a city, Yemen, over a period of 3 years from January 2009 to December 2011. The first 2 cases had no complaints apart from asymmetrical size of one side of their bodies (left side hemihypertrophy). The third case presented with left side hemihypertrophy and an abdominal mass, which upon referral to a cancer center was confirmed to be Wilms` tumor. The fourth case had overgrowth of the right leg and foot with polydactyly, and a right sided abdominal mass. He was diagnosed to have an ureteropelvic junction stricture with hydronephrosis and absent left kidney.

  15. Understanding Child Maltreatment: Juvenile Delinquency. From Research to Effective Program, Practice, and Systemic Solutions.

    Science.gov (United States)

    Wiig, Janet; Widom, Cathy Spatz

    This monograph details the growing body of research showing the connection between child maltreatment and juvenile delinquency. In 2000, nearly 879,000 children were victims of child abuse and neglect. Although juvenile crime has declined recently, the level of crime committed by youth remains high. This monograph describes an array of program,…

  16. [Idiopathic autonomic neuropathy (pandysautonomia)].

    Science.gov (United States)

    Jankowicz, E; Drozdowski, W; Pogumirski, J

    2001-01-01

    On the basis of current literature, clinical and neuropathologic features of idiopathic autonomic neuropathy is presented. Idiopathic autonomic neuropathy is a disease characterized by acute or subacute onset, monophasic course over a period of several years, it is often preceded by an infection. The spectrum of autonomic changes ranges from cholinergic or adrenergic dysfunction to pandysautonomia, leading to heterogeneity of its clinical features. Possible sympathetic system abnormalities found in autonomic neuropathy are: poor pupillary response to light in darkness, orthostatic hypotension leading to syncope, hypotension without compensatory tachycardia, ejaculation disturbances and vasomotor instability. Possible parasympathetic dysfunctions are: salivation and lacrimation disturbances, absent pupillary constriction to light and near gaze, gastrointestinal tract immobility and impairment of gastrointestinal function, atonic bladder with large residual volume, erectile impotence. Pandysautonomia is thought to result from an immune mediated mechanism and responds well to plasmaferesis and intravenous immunoglobin therapy leading to gradual, sometimes not full, recovery. Moreover in this article we pay attention to the clinical value of many tests like cardiovascular or pharmacological studies in the diagnosis of pandysautonomia and in differentiation of pre- and postganglionic changes. In order to diagnose idiopathic autonomic neuropathy one has to rule out a large number of diseases with autonomic dysfunction e.g.: diabetes, malignant neoplasms, acute intermittent porphyria, Shy-Drager syndrome, Riley-Day's dysautonomia, Parkinson's disease, amyloidosis and others.

  17. Bypass system modification at Bonneville Dam on the Columbia River improved the survival of juvenile salmon

    Science.gov (United States)

    Ferguson, J.W.; Sandford, B.P.; Reagan, R.E.; Gilbreath, L.G.; Meyer, E.B.; Ledgerwood, R.D.; Adams, N.S.

    2007-01-01

    From 1987 to 1992, we evaluated a fish bypass system at Bonneville Dam Powerhouse 2 on the Columbia River. The survival of subyearling Chinook salmon Oncorhynchus tshawytscha released into the system ranged from 0.774 to 0.911 and was significantly lower than the survival of test fish released into turbines and the area immediately below the powerhouse where bypass system flow reentered the river. Yearling and subyearling Chinook salmon and yearling coho salmon O. kisutch released into the bypass system were injured or descaled. Also, levels of blood plasma cortisol and lactate were significantly higher in yearling and subyearling Chinook salmon that passed through the bypass system than in fish released directly into a net located over the bypass exit. This original system was then extensively modified using updated design criteria, and the site where juvenile fish reentered the river was relocated 2.8 km further downstream to reduce predation on bypassed fish by northern pikeminnow Ptychocheilus oregonensis. Based on studies conducted from 1999 to 2001, the new bypass system resulted in high fish survival, virtually no injuries to fish, fish passage times that were generally similar to water travel times, and mild stress responses from which fish recovered quickly. The mean estimated survival of subyearling Chinook salmon passing through the new bypass system was 0.946 in 2001, which was an usually low-flow year. Survival, physical condition, passage timing, and blood physiological indicators of stress were all useful metrics for assessing the performance of both bypass systems and are discussed. The engineering and hydraulic criteria used to design the new bypass system that resulted in improved fish passage conditions are described.

  18. Elevated serum levels of macrophage migration inhibitory factor and stem cell growth factor β in patients with idiopathic and systemic sclerosis associated pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    K. Stefanantoni

    2015-03-01

    Full Text Available Pulmonary arterial hypertension (PAH can be idiopathic or secondary to autoimmune diseases, and it represents one of the most threatening complications of systemic sclerosis (SSc. Macrophage migration inhibitory factor (MIF is a pleiotropic cytokine with proinflammatory functions that appears to be involved in the pathogenesis of hypoxia-induced PH. In SSc patients, high serum levels of MIF have been associated with the development of ulcers and PAH. Stem cell growth factor β (SCGF β is a human growth factor that, together with MIF, is involved in the pathogenesis of chronic spinal cord injury. The aim of our study was to measure serum levels of MIF in patients with idiopathic and SSc-associated PAH. We enrolled 13 patients with idiopathic PAH and 15 with SSc-associated PAH. We also selected 14 SSc patients without PAH and 12 normal healthy controls, matched for sex and age. PAH was confirmed by right hearth catheterism (mPAP>25 mmHg. MIF and SCGF β levels were measured by ELISA. We found significantly higher circulating levels of MIF and of SCGF β in patients with idiopathic PAH (P=0.03 and P=0.004 and with PAH secondary to SSc (P=0.018 and P=0.023 compared to SSc patients without PAH. Higher levels of MIF were found in those patients with an higher New York Heart Association (NYHA class (P=0.03. We can hypothesize that MIF and SCGF β are able to play a role in PAH, both idiopathic or secondary, and in the future they may be evaluated as useful biomarkers and prognostic factors for this serious vascular disease.

  19. Elevated serum levels of macrophage migration inhibitory factor and stem cell growth factor β in patients with idiopathic and systemic sclerosis associated pulmonary arterial hypertension.

    Science.gov (United States)

    Stefanantoni, K; Sciarra, I; Vasile, M; Badagliacca, R; Poscia, R; Pendolino, M; Alessandri, C; Vizza, C D; Valesini, G; Riccieri, V

    2015-03-31

    Pulmonary arterial hypertension (PAH) can be idiopathic or secondary to autoimmune diseases, and it represents one of the most threatening complications of systemic sclerosis (SSc). Macrophage migration inhibitory factor (MIF) is a pleiotropic cytokine with proinflammatory functions that appears to be involved in the pathogenesis of hypoxia-induced PH. In SSc patients, high serum levels of MIF have been associated with the development of ulcers and PAH. Stem cell growth factor β (SCGF β) is a human growth factor that, together with MIF, is involved in the pathogenesis of chronic spinal cord injury. The aim of our study was to measure serum levels of MIF in patients with idiopathic and SSc-associated PAH. We enrolled 13 patients with idiopathic PAH and 15 with SSc-associated PAH. We also selected 14 SSc patients without PAH and 12 normal healthy controls, matched for sex and age. PAH was confirmed by right hearth catheterism (mPAP>25 mmHg). MIF and SCGF β levels were measured by ELISA. We found significantly higher circulating levels of MIF and of SCGF β in patients with idiopathic PAH (P=0.03 and P=0.004) and with PAH secondary to SSc (P=0.018 and P=0.023) compared to SSc patients without PAH. Higher levels of MIF were found in those patients with an higher New York Heart Association (NYHA) class (P=0.03). We can hypothesize that MIF and SCGF β are able to play a role in PAH, both idiopathic or secondary, and in the future they may be evaluated as useful biomarkers and prognostic factors for this serious vascular disease.

  20. Association between periphyton and bioflocs systems in intensive culture of juvenile Nile tilapia

    Directory of Open Access Journals (Sweden)

    Davi de Holanda Cavalcante

    2016-05-01

    Full Text Available The present work aimed at determining the effects of the association between the periphyton-based system with the bioflocs-based system in the intensive culture of juvenile Nile tilapia (1.56 ± 0.07 g; 72 fish m-3, on variables of water quality, growth performance and effluent quality after 10 weeks. The experiment was arranged in a 2 x 2 factorial randomized block design with four treatments and five repetitions each. The factors tested were the following: ‘underwater structure’ (absence and presence and ‘adjustment of the C: N ratio of water’ (no and yes. The final fish body weight, specific growth rate and yield were higher (p < 0.05 in the C: N-adjusted tanks. The presence of submerged structures in the tanks had no significant influence on those same variables. It was concluded that the periphyton-based system is not indicated for intensive farming of Nile tilapia, in which there is a high allowance of artificial feed to fish.

  1. Idiopathic oedema and diuretics.

    Science.gov (United States)

    Dunnigan, M. G.; Denning, D. W.; Henry, J. A.; de Wolff, F. A.

    1987-01-01

    Diuretic abuse has been invoked as the cause of idiopathic oedema. In this study, eight patients with idiopathic oedema were studied. Symptoms and weight variation continued despite the proven absence of diuretics in seven of them as determined by urinary chromatograms. Idiopathic oedema cannot therefore be attributed to diuretic use alone. PMID:3671223

  2. Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature.

    Science.gov (United States)

    Chiewchengchol, Direkrit; Murphy, Ruth; Edwards, Steven W; Beresford, Michael W

    2015-01-01

    Patients diagnosed with juvenile-onset systemic lupus erythematosus (JSLE) often have skin and oral lesions as part of their presentation. These mucocutaneous lesions, as defined by the American College of Rheumatology (ACR) in 1997, include malar rash, discoid rash, photosensitivity and oral ulcers. It is therefore essential to recognize mucocutaneous lesions to accurately diagnose JSLE. The mucocutaneous lesions can be divided into those with classical histological features (LE specific) and those strongly associated with and forming part of the diagnostic spectrum, but without the classical histological changes of lupus (LE nonspecific). A malar rash is the most commonly associated LE specific dermatological presentation. This skin manifestation is an acute form and also correlates with disease activity. Subacute (polycyclic or papulosquamous lesions) and chronic (discoid lesions) forms, whilst showing classical histological changes supportive of lupus, are less commonly associated with systemic lupus and do not correlate with disease activity. The most commonly associated skin lesions without classical lupus changes are cutaneous vasculitis, oral ulcers and diffuse non-scarring alopecia. These signs frequently relate to disease activity. An understanding of cutaneous signs and symptoms of lupus in children is important to avoid delay in diagnosis. They will often improve as lupus is adequately controlled and their reappearance is often the first indicator of a disease flare.

  3. Juvenile Justice in Mexico

    Directory of Open Access Journals (Sweden)

    Martha Frías Armenta

    2014-08-01

    Full Text Available The first tribunal in Mexico was established in the central state of San Luis Potosi in 1926. The Law Regarding Social Prevention and Juvenile Delinquency for the Federal District and Mexican territories was promulgated in 1928. In 2005, Article 18 of the Mexican Constitution was modified to establish a comprehensive system (“Sistema Integral de justicia” in Spanish of justice for juveniles between 12 and 18 years old who had committed a crime punishable under criminal law. Its objective was to guarantee juveniles all the due process rights established for adults, in addition to the special ones recognized for minors. The constitutional reform also provides a framework that includes special tribunals as well as alternative justice options for juveniles. With these reforms, institutionalization of minors was to be considered an extreme measure applicable only to felonies and to juveniles older than 14. In 2006, all states within the Mexican federation enacted the “Law of justice for adolescents”. This system, at both the federal and state levels, formalizes a new global paradigm with regard to the triangular relationship between children, the State and the Law. It recognizes that children are also bearers of the inherent human rights recognized for all individuals, instead of simply objects in need of protection. However, despite formally aligning Mexican juvenile justice law with the Convention on the Rights of the Child (CRC, issues of actual substantive rights remained and new ones have appeared. For example, juveniles younger than 14 who have not committed a felony are released from institutions without any rehabilitation or treatment options, and alternative forms of justice were included without evaluating their possibilities of application or their conditions for success. In addition, the economic status of most juvenile detainees continues to be one of the most important determining factors in the administration of justice

  4. Steps towards improving the system of records, collection and monitoring of data relevant to statistics within the system of juvenile justice in the Republic of Serbia

    Directory of Open Access Journals (Sweden)

    Stevanović Ivana

    2012-01-01

    Full Text Available The paper emphasises the importance of „good statistics of juvenile justice“ as one of the basis for a clearer overview of the juvenile crime situation, in order to create unique policies at local and national levels for the suppression and prevention of this phenomenon, and to create appropriate areas for action in terms of improving the system reform. The author particularly gives a review of the „Global Indicators in Juvenile Justice“ which present the basic set of data and comparative tool that provides a basis for the assessment and evaluation of services and policies in the field of juvenile justice, and highlights the importance of compatibility of „national indicators“ with them. Particular attention in the paper has been devoted to the overview and analysis of the necessary measures to improve this field, that were prepared and delivered to the relevant ministries and institutions by the Council for Monitoring and Promoting the work of Bodies Engaged in Criminal Proceeding and Enforcement of Juvenile Criminal Sanctions Involving Juveniles - Juvenile Justice Council (hereinafter: the Council. It was pointed, first of all, at the suggestions made by the Council to the Ministry of Justice with the aim to improve the Program for automated record keeping, at the necessary changes of the Court rules, and certain amendments to Forms SK- 3 and SK- 4 of the Statistical Office of the Republic of Serbia were presented. [Projekat Ministarstva nauke Republike Srbije, br. 47011: Kriminal u Srbiji - fenomenologija, rizici i mogućnosti socijalne intervencije

  5. Syphilis mimicking idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

    2011-01-01

    Idiopathic intracranial hypertension (IIH) is a condition of yet unknown aetiology affecting predominantly obese females of childbearing age. IIH is a diagnosis of exclusion as raised cerebrospinal fluid pressure may occur secondary to numerous other medical conditions. An atypical phenotype...... or an atypical disease course should alert the physician to reevaluate a presumed IIH-diagnosis. The authors report a case of a 32-year-old non-obese male with intracranial hypertension, secondary to a syphilitic central nervous system infection, initially misdiagnosed as being idiopathic. Upon relevant...

  6. Idiopathic portal hypertension

    International Nuclear Information System (INIS)

    Han, Tae Kyun; Ryu, Dae Sik; Kim, Heung Chul; Hur, Hun; Eom, Kyeung Tae; Namkung, Sook; Park, Man Soo; Hwang, Woo Chul; Lee, Kwan Seop

    1996-01-01

    To describe the radiologic findings of idiopathic portal hypertension and to find the points of differentiation between idiopathic portal hypertension and liver cirrhosis. Four portograms in five patients who for four years had suffered from pathologically confirmed idiopathic portal hypertension were retrospectively analyzed and compared with a portogram obtained from a control subject with liver cirrhosis. Portographic finding s of idiopathic portal hypertension were paucity of medium-sized portal branches, irregular and obtuse-angled division of peripheral branches, abrupt interruption and an avascular area beneath the liver margin. A portogram of idiopathic portal hypertension may be useful in differentiation this and liver cirrhosis

  7. Common Variable Immunodeficiency Associated with Hepatosplenic T-Cell Lymphoma Mimicking Juvenile Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    A. A. Jesus

    2011-01-01

    Full Text Available Common variable immunodeficiency (CVID is a heterogeneous disorder with susceptibility to infections, autoimmune manifestations, and cancer. To our knowledge, CIVD with T-cell lymphoma mimicking juvenile systemic lupus erythematosus (JSLE was not described in the literature, and one case was reported herein. An 8-year-old female was admitted in our Pediatric Immunology Unit with a clinical history of hypogammaglobulinemia, recurrent upper respiratory infections, and pneumonias. She had a marked decrease of three serum immunoglobulin isotypes, and the diagnosis of CVID was established. At the age of 17 years, she presented with oral ulceration, nonerosive arthritis, nephritis, serositis, cytopenia, positive antiphospholipid antibodies, and positive antinuclear antibody fulfilling the American College of Rheumatology (ACR criteria for SLE. She was treated with intravenous methylprednisolone for three consecutive days, and intravenous immunoglobulin, and maintenance therapy of chloroquine, azathioprine and prednisone 40 mg/day. Two months later, she died of septic shock secondary to acute pneumonia. The necropsy showed hepatosplenic T-cell lymphoma with diffuse involvement of bone marrow, spleen, liver, and lungs. The lymphoma cells were positive for CD3 immunostaining and negative for CD20 and lysozyme. In conclusion, the association of CVID and hepatosplenic T-cell lymphoma may simulate JSLE diagnosis.

  8. LAW ENFORCEMENT PREPAREDNESS FOR THE IMPLEMENTATION OF INDONESIA’S LAW ON JUVENILE JUSTICE SYSTEM

    Directory of Open Access Journals (Sweden)

    Dani Krisnawati

    2014-03-01

    Full Text Available Paradigmatic changes stipulated in Law Number 11 of 2012 on Juvenile Justice System, including regulations concerning restorative justice and diversion require the competency and skills of the law enforcement officers. This research identifies measures that have been taken and the existing barriers in preparing for the implementation of this Law. The research demonstrates that the readiness of the investigators and child prosecutors are merely limited to the outreach of Law Number 11 of 2012, whilst knowledge of the court judges only covers the draft Law. The number of officers receiving outreach is limited and should be increased. Negative perception on the officers due to the risk of a bribery accusation is feared to hamper the implementation of diversion regulation based on a restorative justice. Perubahan paradigmatik yang termuat dalam Undang-Undang Nomor 11 Tahun 2012 tentang Sistem Peradilan Pidana Anak, termasuk diantaranya ketentuan mengenai keadilan restoratif dan diversi, memerlukan kompetensi dan keahlian aparat penegak hukum. Penelitian ini mengidentifikasi langkah yang telah dilakukan dan kendala persiapan implementasi Undang-Undang tersebut. Hasil penelitian memperlihatkan bahwa kesiapan penyidik dan penuntut umum anak masih terbatas pada partisipasi sosialisasi Undang-Undang Nomor 11 Tahun 2012, sedangkan hakim anak memiliki pengetahuan hanya pada Rancangan Undang-Undang. Jumlah aparat yang menerima sosialisasi Undang-Undangmasih terbatas dan perlu ditingkatkan jumlahnya. Pandangan negatif terhadap aparat karena bisa menimbulkan dugaan suap dikhawatirkan menjadi penghambat diterapkannya ketentuan diversi dengan pendekatan keadilan restoratif.

  9. Common variable immunodeficiency associated with hepatosplenic T-cell lymphoma mimicking juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Jesus, A A; Jacob, C M A; Silva, C A; Dorna, M; Pastorino, A C; Carneiro-Sampaio, M

    2011-01-01

    Common variable immunodeficiency (CVID) is a heterogeneous disorder with susceptibility to infections, autoimmune manifestations, and cancer. To our knowledge, CIVD with T-cell lymphoma mimicking juvenile systemic lupus erythematosus (JSLE) was not described in the literature, and one case was reported herein. An 8-year-old female was admitted in our Pediatric Immunology Unit with a clinical history of hypogammaglobulinemia, recurrent upper respiratory infections, and pneumonias. She had a marked decrease of three serum immunoglobulin isotypes, and the diagnosis of CVID was established. At the age of 17 years, she presented with oral ulceration, nonerosive arthritis, nephritis, serositis, cytopenia, positive antiphospholipid antibodies, and positive antinuclear antibody fulfilling the American College of Rheumatology (ACR) criteria for SLE. She was treated with intravenous methylprednisolone for three consecutive days, and intravenous immunoglobulin, and maintenance therapy of chloroquine, azathioprine and prednisone 40  mg/day. Two months later, she died of septic shock secondary to acute pneumonia. The necropsy showed hepatosplenic T-cell lymphoma with diffuse involvement of bone marrow, spleen, liver, and lungs. The lymphoma cells were positive for CD3 immunostaining and negative for CD20 and lysozyme. In conclusion, the association of CVID and hepatosplenic T-cell lymphoma may simulate JSLE diagnosis.

  10. Columbia River System Operation Review : Final Environmental Impact Statement, Appendix C: Anadromous Fish and Juvenile Fish Transportation.

    Energy Technology Data Exchange (ETDEWEB)

    Columbia River System Operation Review (U.S.)

    1995-11-01

    This Appendix C of the Final Environmental Impact Statement for the Columbia River System discusses impacts on andromous fish and juvenile fish transportation. The principal andromous fish in the Columbia basin include salmonid species (Chinook, coho, and sockeye salmon, and steelhead) and nonsalmoinid andromous species (sturgeon, lamprey, and shad). Major sections in this document include the following: background, scope and process; affected environment for salmon and steelhead, shaded, lamprey, sturgeon; study methods; description of alternatives: qualitative and quantitative findings.

  11. A Cabled Acoustic Telemetry System for Detecting and Tracking Juvenile Salmon: Part 1. Engineering Design and Instrumentation

    Science.gov (United States)

    Weiland, Mark A.; Deng, Z. Daniel; Seim, Tom A.; LaMarche, Brian L.; Choi, Eric Y.; Fu, Tao; Carlson, Thomas J.; Thronas, Aaron I.; Eppard, M. Brad

    2011-01-01

    In 2001 the U.S. Army Corps of Engineers, Portland District (OR, USA), started developing the Juvenile Salmon Acoustic Telemetry System, a nonproprietary sensing technology, to meet the needs for monitoring the survival of juvenile salmonids through eight large hydroelectric facilities within the Federal Columbia River Power System (FCRPS). Initial development focused on coded acoustic microtransmitters and autonomous receivers that could be deployed in open reaches of the river for detection of the juvenile salmonids implanted with microtransmitters as they passed the autonomous receiver arrays. In 2006, the Pacific Northwest National Laboratory began the development of an acoustic receiver system for deployment at hydropower facilities (cabled receiver) for detecting fish tagged with microtransmitters as well as tracking them in two or three dimensions for determining route of passage and behavior as the fish passed at the facility. The additional information on route of passage, combined with survival estimates, is used by the dam operators and managers to make structural and operational changes at the hydropower facilities to improve survival of fish as they pass the facilities through the FCRPS. PMID:22163918

  12. Migration depth and residence time of juvenile salmonids in the forebays of hydropower dams prior to passage through turbines or juvenile bypass systems: implications for turbine-passage survival.

    Science.gov (United States)

    Li, Xinya; Deng, Zhiqun D; Brown, Richard S; Fu, Tao; Martinez, Jayson J; McMichael, Geoffrey A; Skalski, John R; Townsend, Richard L; Trumbo, Bradly A; Ahmann, Martin L; Renholds, Jon F

    2015-01-01

    Little is known about the three-dimensional depth distributions in rivers of individually marked fish that are in close proximity to hydropower facilities. Knowledge of the depth distributions of fish approaching dams can be used to understand how vulnerable fish are to injuries such as barotrauma as they pass through dams. To predict the possibility of barotrauma injury caused by pressure changes during turbine passage, it is necessary to understand fish behaviour relative to acclimation depth in dam forebays as they approach turbines. A guiding study was conducted using high-resolution three-dimensional tracking results of salmonids implanted with Juvenile Salmon Acoustic Telemetry System transmitters to investigate the depth distributions of subyearling and yearling Chinook salmon (Oncorhynchus tshawytscha) and juvenile steelhead (Oncorhynchus mykiss) passing two dams on the Snake River in Washington State. Multiple approaches were evaluated to describe the depth at which fish were acclimated, and statistical analyses were performed on large data sets extracted from ∼28 000 individually tagged fish during 2012 and 2013. Our study identified patterns of depth distributions of juvenile salmonids in forebays prior to passage through turbines or juvenile bypass systems. This research indicates that the median depth at which juvenile salmonids approached turbines ranged from 2.8 to 12.2 m, with the depths varying by species/life history, year, location (which dam) and diel period (between day and night). One of the most enlightening findings was the difference in dam passage associated with the diel period. The amount of time that turbine-passed fish spent in the immediate forebay prior to entering the powerhouse was much lower during the night than during the day. This research will allow scientists to understand turbine-passage survival better and enable them to assess more accurately the effects of dam passage on juvenile salmon survival.

  13. Estimating the Optimal Dosage of Sodium Valproate in Idiopathic Generalized Epilepsy with Adaptive Neuro-Fuzzy Inference System

    Directory of Open Access Journals (Sweden)

    Amir Hooshang Mohammadpour

    2012-07-01

    Full Text Available Epilepsy is a clinical syndrome in which seizures have a tendency to recur. Sodium valproate is the most effective drug in the treatment of all types of generalized seizures. Finding the optimal dosage (The lowest effective dose of sodium valproate is a real challenge to all neurologists. In this study, a new approach based on Adaptive Neuro-Fuzzy Inference System (ANFIS was presented for estimating the optimal dosage of sodium valproate in IGE patients.   40 patients with Idiopathic Generalized Epilepsy, who were referred to the neurology department of Mashhad University of Medical Sciences between the years 2006-2011,were included in this study. The function Adaptive Neuro-Fuzzy Inference System (ANFIS constructs a Fuzzy Inference System (FIS whose membership function parameters are tuned (adjusted using either a back-propagation algorithm alone, or in combination with a least squares type of method (hybrid algorithm. In this study, we usedhybrid method for adjusting the parameters.The R-square of the proposed system was %598 and thePearson correlation coefficient was significant (P 0.05 . Although the accuracy of the model was not high, it wasgood enough to be applied for treating the IGE patients with sodium valproate. This paper presented a new application of ANFIS for estimating the optimal dosage of s odium valproate in IGE patients. Fuzzy set theory plays an important role in dealing with uncertainty when making decisions in medical applications. Collectively, it seems that ANFIS has a high capacity to be applied in medical sciences, especially neurology.

  14. Arbitrary arbitration: diverting juveniles into the justice system--a reexamination after 22 years.

    Science.gov (United States)

    Béchard, Stephanie; Ireland, Connie; Berg, Bruce; Vogel, Brenda

    2011-06-01

    This article reports on a study of a juvenile diversion program based in California. It replicates a similar 1986 study of a Florida-based juvenile diversion program. Both studies examine the appropriateness of the actual juvenile population serviced in comparison with the declared and described programs' target populations. Findings in the current research support earlier findings that the diversion program services self-serving, low-risk populations, rather than their intended "first-time nonviolent," more seriously at risk of continued delinquent behavior, target population. This finding is examined in terms of its relationship to issues of labeling and net widening, and suggests how the current study diversion program manages to create a façade that more seriously at-risk youth are being properly targeted by the program.

  15. Salivary proteomics: A new adjuvant approach to the early diagnosis of familial juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Abrão, Aline Lauria P; Falcao, Denise Pinheiro; de Amorim, Rivadávio Fernandes Batista; Bezerra, Ana Cristina B; Pombeiro, Gilson Augusto N M; Guimarães, Luciano Junqueira; Fregni, Felipe; Silva, Luciano Paulino; da Mota, Licia Maria Henrique

    2016-04-01

    Systemic lupus erythematosus (SLE) is a chronic multisystemic disease characterized by autoimmune inflammatory disturbance. Pleomorphic manifestations are present and a potentially progressive and debilitating course can be detected. SLE rarely manifests before age 5, and its onset peaks is around puberty. Although clinical manifestations, immunological alterations and treatment do not differ between juvenile and adult SLE, children tend to present with a more aggressive disease course than adults. Hence, autoimmune rheumatic diseases are the most common cause of morbidity and mortality in pediatric populations. Blood serum analysis plays an especially important role in the detection and monitoring of autoantibodies in SLE. However, since blood sampling is an uncomfortable procedure, especially in children, novel less invasive techniques and approaches are of utmost importance to evaluate pediatric subjects. In this regard, saliva samples have several advantages, such as: easy access, fast collection, painless and riskless procedure. Saliva has antimicrobial, immunomodulatory and anti-inflammatory properties, as well as several other relevant features. The whole saliva is a complex mixture of major and minor salivary gland secretion, gingival crevicular fluid, transudates plasma protein, keratinocyte products and oral microbiota. This biological fluid reflects the physiological state of the body, including the emotional condition, and endocrine, nutritional and metabolic changes. Therefore, salivary proteomics is becoming increasingly used for the early diagnosis of several diseases such as breast cancer, oral cancer, Sjögren's syndrome, diffuse systemic sclerosis, rheumatoid arthritis, among others. Considering the detection of some potential markers related to SLE in serum and urine, this study aims to conduct an initial evaluation of the possible presence of such biomarkers in saliva. Furthermore, it is expected to track down new salivary proteins that could be

  16. Detection of nitrogen deficiency QTL in juvenile wild barley introgression lines growing in a hydroponic system

    Directory of Open Access Journals (Sweden)

    Hoffmann Astrid

    2012-10-01

    Full Text Available Abstract Background In this report we studied the genetic regulation of juvenile development of wild barley introgression lines (S42ILs under two contrasting hydroponic nitrogen (N supplies. Ten shoot and root related traits were examined among 42 S42ILs and the recurrent parent ‘Scarlett’. The traits included tiller number, leaf number, plant height, leaf and root length, leaf to root length ratio, shoots and root dry weight, shoot to root weight ratio, and chlorophyll content. Our aims were (1 to test the suitability of a hydroponic system for early detection of favourable S42ILs, (2 to locate quantitative trait loci (QTL that control the examined traits, (3 to identify favourable wild barley alleles that improve trait performances in regard to N treatment and, finally, (4 to validate the identified QTL through comparison with previously reported QTL originating from the same parental cross. Results The phenotypic data were analysed in a mixed model association study to detect QTL. The post-hoc Dunnett test identified 28 S42ILs that revealed significant (P Hsp effects for tiller number, leaf number, leaf length, plant height and leaf to root ratio on the long arm of chromosome 7H. These QTL correspond to QTL for ears per plant and plant height that were previously detected in field trials conducted with the same S42ILs or with the S42 population. Conclusion Our results suggest that the QTL we identified under hydroponic N cultivation partly correspond to QTL detected in field experiments. Due to this finding, screening of plants in early developmental stages grown in a hydroponic system may be a fast and cost effective method for early QTL detection and marker-assisted allelic selection, potentially speeding up elite barley breeding programs.

  17. Detection of nitrogen deficiency QTL in juvenile wild barley introgression lines growing in a hydroponic system.

    Science.gov (United States)

    Hoffmann, Astrid; Maurer, Andreas; Pillen, Klaus

    2012-10-20

    In this report we studied the genetic regulation of juvenile development of wild barley introgression lines (S42ILs) under two contrasting hydroponic nitrogen (N) supplies. Ten shoot and root related traits were examined among 42 S42ILs and the recurrent parent 'Scarlett'. The traits included tiller number, leaf number, plant height, leaf and root length, leaf to root length ratio, shoots and root dry weight, shoot to root weight ratio, and chlorophyll content. Our aims were (1) to test the suitability of a hydroponic system for early detection of favourable S42ILs, (2) to locate quantitative trait loci (QTL) that control the examined traits, (3) to identify favourable wild barley alleles that improve trait performances in regard to N treatment and, finally, (4) to validate the identified QTL through comparison with previously reported QTL originating from the same parental cross. The phenotypic data were analysed in a mixed model association study to detect QTL. The post-hoc Dunnett test identified 28 S42ILs that revealed significant (P hydroponic N study corresponded to QTL that were also detected in field trials with adult plants of a similar S42IL set or of the original S42 population. For instance, S42IL-135, -136 and -137, revealed increasing Hsp effects for tiller number, leaf number, leaf length, plant height and leaf to root ratio on the long arm of chromosome 7H. These QTL correspond to QTL for ears per plant and plant height that were previously detected in field trials conducted with the same S42ILs or with the S42 population. Our results suggest that the QTL we identified under hydroponic N cultivation partly correspond to QTL detected in field experiments. Due to this finding, screening of plants in early developmental stages grown in a hydroponic system may be a fast and cost effective method for early QTL detection and marker-assisted allelic selection, potentially speeding up elite barley breeding programs.

  18. Increased IgE serum levels are unrelated to allergic and parasitic diseases in patients with juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Bernadete L. Liphaus

    2012-11-01

    Full Text Available OBJECTIVE: The aim of this study was to assess the IgE serum levels in juvenile systemic lupus erythematosus patients and to evaluate possible associations with clinical and laboratory features, disease activity and tissue damage. METHODS: The IgE serum concentrations in 69 consecutive juvenile systemic lupus erythematosus patients were determined by nephelometry. IgG, IgM and IgA concentrations were measured by immunoturbidimetry. All patients were negative for intestinal parasites. Statistical analysis methods included the Mann-Whitney, chi-square and Fisher's exact tests, as well as the Spearman rank correlation coefficient. RESULTS: Increased IgE concentrations above 100 IU/mL were observed in 31/69 (45% juvenile systemic lupus erythematosus patients. The mean IgE concentration was 442.0 ± 163.4 IU/ml (range 3.5-9936.0 IU/ml. Fifteen of the 69 patients had atopic disease, nine patients had severe sepsis and 56 patients presented with nephritis. The mean IgE level in 54 juvenile systemic lupus erythematosus patients without atopic manifestations was 271.6 ± 699.5 IU/ml, and only nine of the 31 (29% patients with high IgE levels had atopic disease. The IgE levels did not statistically differ with respect to the presence of atopic disease, severe sepsis, nephritis, disease activity, or tissue damage. Interestingly, IgE concentrations were inversely correlated with C4 levels (r = -0.25, p = 0.03 and with the SLICC/ACR-DI score (r = -0.34, p = 0.005. The IgE concentration was also found to be directly correlated with IgA levels (r = 0.52, p = 0.03. CONCLUSIONS: The present study demonstrated for the first time that juvenile systemic lupus erythematosus patients have increased IgE serum levels. This increase in IgE levels was not related to allergic or parasitic diseases. Our results are in line with the hypothesis that high IgE levels can be considered a marker of immune dysregulation.

  19. Juveniles in criminal procedure

    Directory of Open Access Journals (Sweden)

    Lukić Tatjana

    2012-01-01

    Full Text Available Taking into consideration the importance and role of children in modern society, as well as their position, this article has as its focus juveniles in criminal procedure. The existence of a separate juvenile justice system independent of the criminal law applicable to the adult offenders and general criminal procedure, as well as the periodic changes of the dominant approach in theory and practice reflects the ascendancy of different theoretical perspectives in the juvenile justice. In this paper, the authors scrutinize the models of responding to juvenile crime - justice and welfare model - as two models of the greatest importance in the present reaction of the society to the crimes conducted by the youngest delinquents at the beginning of the new century and millennium. Furthermore, the paper deals with a matter of international legal standards which, to a large extent, give shape to the legal framework for juvenile offenders and provide their rights and position in the criminal procedure. The authors refer to the internationally accepted documents on several levels. From the (almost universally accepted multilateral conventions on human rights, through the field of recommendations, rules and guidelines which are obeyed and enforced in practice of the juvenile justice although they are of non-binding nature, via the regional European legislative to the national provisions in a particular number of countries. On all the levels mentioned above the rights of the juveniles are regulated having in mind their possible role in the criminal procedure as a perpetrator of a criminal act, as a victim or as a witness. This paper also analyzes the criminal procedure with respect to juvenile perpetrators of the criminal acts in the Republic of Serbia and compliance of the provisions currently in vigor with the international legal standards contained in the international conventions and other internationally accepted and recognized instruments.

  20. Juvenile Prostitution.

    Science.gov (United States)

    Csapo, Marg

    1986-01-01

    Recent research and Canadian government committee reports concerning juvenile prostitution are reviewed. Proposals are made in the realms of law and social policy; and existing programs are described. (DB)

  1. Hormone profile in juvenile systemic lupus erythematosus with previous or current amenorrhea.

    Science.gov (United States)

    Silva, Clovis A; Deen, Maria E J; Febrônio, Marilia V; Oliveira, Sheila K; Terreri, Maria T; Sacchetti, Silvana B; Sztajnbok, Flavio R; Marini, Roberto; Quintero, Maria V; Bica, Blanca E; Pereira, Rosa M; Bonfá, Eloisa; Ferriani, Virginia P; Robazzi, Teresa C; Magalhães, Claudia S; Hilário, Maria O

    2011-08-01

    To identify the underlying mechanism of amenorrhea in juvenile systemic lupus erythematosus (JSLE) patients, thirty-five (11.7%) JSLE patients with current or previous amenorrhea were consecutively selected among the 298 post-menarche patients followed in 12 Brazilian pediatric rheumatology centers. Pituitary gonadotrophins [follicle-stimulating hormone (FSH) and luteinizing hormone (LH)] and estradiol were evaluated in 32/35 patients, and prolactin and total testosterone in 29/35 patients. Patient's medical records were carefully reviewed according to demographic, clinical and therapeutic findings. The mean duration of amenorrhea was 7.2 ± 3.6 months. Low FSH or LH was observed in 7/32 (22%) JSLE patients and normal FSH or LH in 25 (78%). Remarkably, low levels of FSH or LH were associated with higher frequency of current amenorrhea (57% vs. 0%, P = 0.001), higher median disease activity (SLEDAI) and damage (SLICC/ACR-DI) (18 vs. 4, P = 0.011; 2 vs. 0, P = 0.037, respectively) and higher median current dose of prednisone (60 vs. 10 mg/day, P = 0.0001) compared to normal FSH or LH JSLE patients. None of them had decreased ovarian reserve and premature ovarian failure. Six of 29 (21%) patients had high levels of prolactin, and none had current amenorrhea. No correlations were observed between levels of prolactin and SLEDAI, and levels of prolactin and SLICC/ACR-DI scores (Spearman's coefficient). We have identified that amenorrhea in JSLE is associated with high dose of corticosteroids indicated for active disease due to hypothalamic-pituitary-ovary axis suppression.

  2. Osteonecrosis of the jaw on imaging exams of patients with juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Fernandes, Elisabeth Gonzaga Canova; Guissa, Vanessa Ramos; Saviolli, Cynthia; Siqueira, José Tadeu Tesseroli; Valente, Marcelo; da Silva, Clovis Artur Almeida

    2010-01-01

    The objective of the present study was to evaluate radiographic changes of the temporomandibular joint (TMJ) in patients with juvenile systemic lupus erythematosus (JSLE) and a control group. Panoramic radiographies of the TMJ of 26 JSLE patients and 28 healthy individuals were evaluated. Multislice computed tomography (MCT) was performed on those patients who presented flattening and/or destruction of mandibular condyles. Demographic data, oral health indices, clinical manifestations, laboratory exams, and treatment were evaluated. Important radiographic changes consistent with osteonecrosis of the mandible, confirmed by MCT of the TMJ, were observed in 2/26 (8%) JSLE patients versus 0% in the control group (P = 0.22). Mild clinical dysfunction and abnormal TMJ mobility were observed in 67% and 54% of the patients, respectively. Age of onset, disease duration, and current age were similar in JSLE patients with and without severe radiographic changes of TMJ (9.3 versus 10.8 years, P = 0.77; 3.3 versus 2 years, P = 0.63; 12.6 versus 13.5 years, P = 0.74, respectively). Significant differences in gender, socioeconomical status, oral health indices, clinical manifestations, laboratory exams, and treatment were not observed between both subgroups (P 0.05). Both JSLE patients with osteonecrosis of the mandible had active chronic disease, used corticosteroids for a prolonged period, and had mild TMJ dysfunction. Antiphospholipid antibodies were not observed in those two patients, and neither one had been treated with bisphosphonate. Osteonecrosis of the mandible with mild TMJ dysfunction was observed in some of the patients, demonstrating the importance of odontological assessment during clinical follow-up.

  3. Bone mineral density in children with systemic lupus erythematosus and juvenile rheumatoid arthritis

    International Nuclear Information System (INIS)

    Kashef, S.; Saki, F.; Karamizadeh, Z.; Kashef, Mohammed Amin

    2007-01-01

    Although there is increasing interest in bone metabolism in patients with rheumatic disorders, few data exist on bone mineral density (BMD) in children with rheumatic disorders or on the association of BMD with disease-related variables. We determined BMD in Iranian children with systemic lupus erythematosus (SLE) and juvenile rheumatoid arthritis (JRA) to evaluate the relationship between disease related variables and BMD. Twenty patients (13 girls and 7 boys) with SLE (n=5) and JRA (n=5) with a mean age of 13.10+-3.29 years (range, 6-17 years), attending a pediatric rheumatology clinic and 20 healthy controls (matched for age and sex with each patient) were enrolled in a cross-sectional study between 2001 and 2003. BMD (g/cm) of the femoral neck (BMD-F) and lumbar vertebrae (BMD-L) were measured by dual energy x-ray absorptiometry (DEXA). The correlation between BMD and cumulative dose of steroids, daily dose of steroid, disease duration, disease activity, height, weight and age was investigated. BMD in the patients (BMD-F=0.72+-0.15, BMD-L=0.70+-0.19) was significantly lower than controls (BMD-F=0.95+-0.17, BMD-L=0.98+-0.20, P<0.001). The severity of decreased BMD was more prominent in lumbar vertebrae than the femoral neck (P=0.04). None of the variables were consistently related to decrease in BMD. BMD was significantly lower in patients compared with controls. It was more prominent in lumbar vertebrae (trabecular bone). Although cumulative doses of steroids and disease duration appeared to have some influence on BMD, none were independently correlated with BMD. (author)

  4. Determination of juvenile hormone titers by means of LC-MS/MS/MS and a juvenile hormone-responsive Gal4/UAS system in Aedes aegypti mosquitoes.

    Science.gov (United States)

    Zhao, Bo; Hou, Yuan; Wang, Jianjun; Kokoza, Vladimir A; Saha, Tusar T; Wang, Xue-Li; Lin, Ling; Zou, Zhen; Raikhel, Alexander S

    2016-10-01

    In anautogenous mosquitoes, juvenile hormone III (JH) plays an essential role in female post-eclosion (PE) development, preparing them for subsequent blood feeding and egg growth. We re-examined the JH titer during the reproductive cycle of female Aedes aegypti mosquitoes. Using liquid chromatography coupled with triple tandem mass spectrometry (LC-MS/MS/MS), we have shown that it reaches its peak at 48-54 h PE in the female hemolymph and at 72 h PE in whole body extracts. This method represents an effective assay for determination of JH titers. The 2.1-kb 5' promoter region of the Early Trypsin (ET) gene, which is specifically expressed in the female midgut under the control of JH during the PE phase, was utilized to genetically engineer the Ae. aegypti mosquito line with the ET-Gal4 activator. We then established the ET-GAL4>UAS-enhanced green fluorescent protein (EGFP) system in Ae. aegypti. In ET-Gal4>UAS-EGFP female mosquitoes, the intensity of the midgut-specific EGFP signal was observed to correspond to the ET gene transcript level and follow the JH titer during the PE phase. The EGFP signal and the EGFP transcript level were significantly diminished in midguts of transgenic female mosquitoes after RNA interference depletion of the JH receptor Methoprene-tolerant (Met), providing evidence of the control of ET gene expression by Met. Topical JH application caused premature enhancement of the EGFP signal and the EGFP transcript level in midguts of newly eclosed ET-Gal4>UAS-EGFP female mosquitoes, in which endogenous JH titer is still low. Hence, this novel ET-Gal4>UAS system permits JH-dependent gene overexpression in the midgut of Ae. aegypti female mosquitoes prior to a blood meal. Copyright © 2016 Elsevier Ltd. All rights reserved.

  5. Significance of cardiac sympathetic nervous system abnormality for predicting vascular events in patients with idiopathic paroxysmal atrial fibrillation

    Energy Technology Data Exchange (ETDEWEB)

    Akutsu, Yasushi; Kaneko, Kyouichi; Kodama, Yusuke; Li, Hui-Ling; Kawamura, Mitsuharu; Asano, Taku; Hamazaki, Yuji; Tanno, Kaoru; Kobayashi, Youichi [Showa University School of Medicine, Division of Cardiology, Department of Medicine, Tokyo (Japan); Suyama, Jumpei; Shinozuka, Akira; Gokan, Takehiko [Showa University School of Medicine, Department of Radiology, Tokyo (Japan)

    2010-04-15

    Neuronal system activity plays an important role for the prognosis of patients with atrial fibrillation (AF). Using {sup 123}I metaiodobenzylguanidine ({sup 123}I-MIBG) scintigraphy, we investigated whether a cardiac sympathetic nervous system (SNS) abnormality would be associated with an increased risk of vascular events in patients with paroxysmal AF. {sup 123}I-MIBG scintigraphy was performed in 69 consecutive patients (67 {+-} 13 years, 62% men) with paroxysmal AF who did not have structural heart disease. SNS integrity was assessed from the heart to mediastinum (H/M) ratio on delayed imaging. Serum concentration of C-reactive protein (CRP) was measured before {sup 123}I-MIBG study. During a mean of 4.5 {+-} 3.6 years follow-up, 19 patients had myocardial infarction, stroke or heart failure (range: 0.2-11.5 years). SNS abnormality (H/M ratio <2.7) and high CRP ({>=}0.3 mg/dl) were associated with the vascular events (58.3% in 14 of 24 patients with SNS abnormality vs 11.1% in 5 of 45 patients without SNS abnormality, p < 0.0001, 52.4% in 11 of 21 patients with high CRP vs 16.7% in 8 of 48 patients without high CRP, p < 0.0001). After adjustment for potential confounding variables such as age, left atrial dimension and left ventricular function, SNS abnormality was an independent predictor of vascular events with a hazard ratio of 4.1 [95% confidence interval (CI): 1.3-12.6, p = 0.014]. Further, SNS abnormality had an incremental and additive prognostic power in combination with high CRP with an adjusted hazard ratio of 4.1 (95% CI: 1.5-10.9, p = 0.006). SNS abnormality is predictive of vascular events in patients with idiopathic paroxysmal AF. (orig.)

  6. Growth of juvenile Atlantic cod Gadus morhua in land-based recirculation systems: Effects of feeding regime, photoperiod and diet

    DEFF Research Database (Denmark)

    Fülberth, Michael; Moran, Damian; Jarlbæk, Henrik

    2009-01-01

    feeding duration. In each RAS unit three diets (A, B and C) were tested, which were broadly similar in composition but from different manufacturers. Water exchange rate averaged 10–19% in the three recirculation systems, and key water quality parameters such as NH4+ and CO2 remained at low effect......The combined effect of feeding regime and photoperiod on the growth of juvenile Atlantic cod Gadus morhua in land-based recirculating aquaculture systems (RAS) was examined using three different commercial diets. Fish of 8–10 g were reared in 1 m3 tanks at an initial density of 10 kg m− 3 for 78 d...

  7. Coexisting Juvenile Dermatomyositis and Sickle Cell Disease: Maintaining a High Degree of Suspicion.

    Science.gov (United States)

    Fraint, Ellen; Leffler, Maureen; Schultz, Corinna L

    2017-07-01

    Juvenile dermatomyositis is an idiopathic inflammatory myopathy of childhood not previously described in a patient with sickle cell disease. We present a case of an 11-year-old girl with sickle cell disease who was diagnosed subsequently with juvenile dermatomyositis, and highlight the diagnostic and therapeutic challenges of these concurrent chronic diseases. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. RESTORATIVE JUSTICE DALAM SISTEM PERADILAN PIDANA ANAK / Restorative Justice In Juvenile Justice System

    Directory of Open Access Journals (Sweden)

    Randy Pradityo

    2016-11-01

    Full Text Available Anak sebagai generasi penerus bangsa sudah selayaknya mendapatkan perhatian khusus. Hal tersebut bertujuan dalam rangka pembinaan anak untuk mewujudkan sumber daya manusia yang berkualitas. Maka dari itu, diperlukan pula sarana dan prasarana hukum yang mengantisipasi segala permasalahan yang timbul. Sarana hukum ini bertujuan untuk mengantisipasi stigma atau cap jahat yang ditimbulkan ketika anak berhadapan dengan hukum, sekaligus memulihkan dan memasyarakatkan kembali anak tersebut. Salah satu solusinya adalah dengan mengalihkan atau menempatkan pelaku tindak pidana anak keluar dari sistem peradilan pidana serta memberikan alternatif bagi penyelesaian dengan pendekatan keadilan demi kepentingan terbaik bagi anak, yang kemudian dikenal dengan pendekatan restorative justice. Restorative justice yang merupakan implementasi konsep dari diversi telah dirumuskan dalam sistem peradilan pidana anak, namun sistem yang baik haruslah diiringi dengan suatu sikap yang dijiwai kehendak untuk memandang dan berkeyakinan bahwa dunia ini selalu menjadi lebih baik. Selain itu, hendaknya prinsip the best interest of the children selalu diutamakan ketika menangani anak yang berhadapan dengan hukum.   Children as the nation's next generation is already deserve special attention. It aims in order to develop the child to realize the quality of human resources. Therefore, it is also necessary legal infrastructure to anticipate any problems that arise. The legal means to anticipate stigma or stamp evil inflicted when the child against the law, as well as restoring and re-socialize the child. One solution is to divert or placing the offender children out of the criminal justice system as well as providing an alternative to the settlement with justice approach in the best interests of the child, who was then known as restorative justice approach. Restorative justice which is the implementation of the concept of diversion has been formulated in the juvenile justice system

  9. Restorative Discipline as an Alternative to Retributive Discipline within the Juvenile Court System: An Analysis of the Metro County Juvenile Court Community Restorative Board

    Science.gov (United States)

    Banjoko, Ajamu A.

    2009-01-01

    Giroux (2003) indicated that the prison industry has become a major economic industry with many states spending more money on prison reforms than on educational reforms. Juvenile delinquent behavior should be punished but fair treatment and equal rights for all human beings under the rule of law is paramount to punishment. Casella (2001) indicated…

  10. Primary idiopathic systemic amyloidosis – rare classical cases with fatal outcome

    Directory of Open Access Journals (Sweden)

    Shivakumar Patil

    2016-07-01

    Full Text Available Primary systemic amyloidosis is a rare condition. We report two cases of primary systemic amyloidosis. Both the cases were without any hematological abnormality. Cutaneous features were the predominant presenting symptoms in these patients. The patients presented with typical waxy lesions on face and macroglossia. Diagnosis was confirmed by histopathology with haematoxylin and eosin staining and Congo red staining

  11. Herpes zoster em pacientes com lúpus eritematoso sistêmico juvenil Herpes zoster in patients with juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Paula da Silva Neves

    2007-04-01

    Full Text Available Infecção pelo vírus varicela zoster (VVZ em pacientes com lúpus eritematoso sistêmico juvenil (LESJ tem sido pouco descrita. Durante um período de 12 anos, ocorreram 195 internações em 77 pacientes com LESJ e estas foram acompanhadas pela Unidade de Reumatologia Pediátrica do Instituto da Criança do Hospital das Clínicas da Universidade de São Paulo. Onze pacientes (14%, dez do sexo feminino, apresentaram 14 internações (7,1% pelo VVZ. Nesses pacientes, a média de idade foi de 16 anos e 5 meses e a média do tempo de duração do LESJ até a primeira infecção devido ao VVZ foi de 4 anos. Todos os episódios das infecções estavam associados com atividade da doença, que se apresentaram como lesões vesicobolhosas seguindo trajeto nervoso. As regiões do tórax e membros foram mais comumente afetadas. Todos haviam utilizado prednisona e quatro usaram ciclofosfamida EV. Todos receberam aciclovir EV por 7 a 10 dias. Nenhum paciente apresentou neuralgia pós-herpética, infecção bacteriana secundária ou evoluiu para óbito. Entretanto, uma paciente em uso de aciclovir apresentou amaurose aguda por vasculite necrosante retiniana bilateral associado ao VVZ, necessitando de duas aplicações de ganciclovir intravítreo e gamaglobulina EV (2 g/kg/dose, com recuperação parcial da acuidade visual. Assim sendo, infecção por VVZ em pacientes com LESJ foi infreqüente, habitualmente associada à atividade da doença e à corticoterapia. Essa infecção foi controlada com aciclovir, e os pacientes raramente apresentaram complicações.Varicella zoster virus (VZV infection in patients with juvenile systemic lupus erythematosus (JSLE has been rarely described. 195 hospitalizations of 77 JSLE patients occurred in a period of 12 years and were followed at the Pediatric Rheumatology Unity of the Instituto da Criança - Hospital das Clínicas - Universidade de São Paulo. Eleven patients (14%, 10 female, had 14 hospitalizations (7.1% due to

  12. CLINICAL SIGNIFICANCE OF ANTIPHOSPHOLIPID ANTIBODIES AND GENE MUTATIONS IN HEMOSTASIS OF CHILDREN WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND JUVENILE DERMATOMYOSITIS

    Directory of Open Access Journals (Sweden)

    O.A. Solntseva

    2006-01-01

    Full Text Available Thrombophilia in children with diffuse connective tissue disorders as systemic lupus erythematosus (SLE and juvenile dermatomyositis (JDM could arise from various causes including peripherial blood circulation of antiphospholipid antibodies (APH and genetic mutations in the system of hemostasis. Thrombosis is a serious and prognostically unfavorable complication that has negative impact on the underlying disease course. The study included 96 children, 65 of them had diagnosed SLE and the other 31 had JDM. The Elisa method was used to detect antiphospholipid antibodies, coagulation method was used to detect lupus anticoagulant (LAC and antibodies to cardiolipins (anticl, ?2:glycoprotein 1 (anti ? 2 gp 1 and prothrombin (APT. The PCR method (DNA diagnostics was used to detect DNA mutations as factor resistance to of activated protein c (Leiden 5,10 methylen tetrahydrofolate reductase (MTHFR gene polymorphism. The incidence of APL antibodies was registered in 61.5% patients with SLE and in 32.2% of patients with JDM. Ac ligg, anti ?2 gp 1 Igg were clinically significant in thrombotic events in patients with SLE and JDM, and so was LAC in patients with SLE. The prevalence of the hemostasis system mutations is concordant with reported data. Conclusion thrombophilia is frequently associated with APH antibodies or combination of APH antibodies with genetic abnormalities. Sole genetic mutations are salient in patients with JDM.Key words: thrombophilia, systemic lupus erythematosus, juvenile dermatomyositis, antiphospholipid antibodies, lupus anticoagulant, leiden, prothrombin, methylentet rahydrofolate reductase.

  13. A critical examination of "being Black" in the juvenile justice system.

    Science.gov (United States)

    Peck, Jennifer H; Jennings, Wesley G

    2016-06-01

    The current study examined the role of race in juvenile court outcomes across 3 decision-making stages. This analysis was conducted with a random sample of all delinquent referrals in a Northeast state from January 2000 through December 2010 (N = 68,188). In addition to traditional logistic regression analysis, a propensity score matching (PSM) approach was utilized to create comparable samples of Black and White youth and provide a more rigorous methodological test of the relationship between race and juvenile court processing. Results indicated that even after the use of PSM techniques, race was still found to influence the likelihood of intake (OR = 1.54; 95% C.I. = 1.48-1.62, p social control. They also reaffirm the noticeable role that selection bias can play in the research surrounding race differences in juvenile court outcomes, and highlight the importance of utilizing a more stringent statistical model to control for selection bias. (PsycINFO Database Record (c) 2016 APA, all rights reserved).

  14. Idiopathic pulmonary hemosiderosis

    OpenAIRE

    Sunilkumar, B M; Sathishkumar, K M; Somashekhar, A R; Maiya, P P

    2010-01-01

    Recurrent or chronic pulmonary hemorrhage is rare in children. Idiopathic pulmonary hemosiderosis (IPH) manifests as hemoptysis, diffuse parenchymal infiltrates on chest radiographs and microcytic hypochromic anemia. The hemoptysis present may be mistaken for more common diseases, delaying the diagnosis and further management. Idiopathic pulmonary hemosiderosis is a disorder of unknown etiology. Treatment of IPH includes immunosuppressive drugs along with supportive measures.

  15. Effects of dietary oxidized fish oil supplementation on oxidative stress and antioxidant defense system in juvenile rainbow trout (Oncorhynchus mykiss).

    Science.gov (United States)

    Fontagné-Dicharry, Stéphanie; Larroquet, Laurence; Dias, Karine; Cluzeaud, Marianne; Heraud, Cécile; Corlay, Dominique

    2018-03-01

    The objective of the study was to characterize the response of the antioxidant defense system against dietary prooxidant conditions in rainbow trout juveniles. Fish (initial mean weight: 62 ± 1 g) were fed three fishmeal and plant-derived protein-based diets supplemented with 15% fresh fish oil (CTL diet), 15% fresh fish oil from tuna by-products (BYP diet) or 15% autooxidized fish oil (OX diet) over a 12-week growth trial at 17.5 ± 0.5 °C. No significant differences in growth performance were recorded between dietary groups. Muscle lipid content was reduced and n-6 PUFA levels were increased in rainbow trout fed diets BYP and OX compared to CTL. After 12 weeks of feeding, the level of lipid peroxidation products in muscle was not affected whereas the 8-isoprostane content in liver was increased in fish fed diet OX as well as plasma total and oxidized glutathione contents. The hepatic and muscle contents for α-tocopherol were decreased in fish fed BYP and OX. Hepatic antioxidant enzyme activities and mRNA levels were not affected after 12 weeks of feeding, except for catalase and glutathione peroxidase 1b2 mRNA levels that were decreased in trout fed diet OX. Fish fed diet OX and BYP displayed also reduced cytosolic Nrf2 and both cytosolic and nuclear NF-κB protein levels in liver. The present work indicates that feeding rainbow trout juveniles with fresh fish oil from by-products or moderately oxidized lipid appears not to be detrimental to the growth performance of fish. The mechanisms beyond the control of the antioxidant defense system by moderately oxidized lipid require further investigations in rainbow trout juveniles. Copyright © 2017 Elsevier Ltd. All rights reserved.

  16. The influence of intrauterine exposure to immunosuppressive treatment on changes in the immune system in juvenile Wistar rats.

    Science.gov (United States)

    Kabat-Koperska, Joanna; Kolasa-Wołosiuk, Agnieszka; Wojciuk, Bartosz; Wojciechowska-Koszko, Iwona; Roszkowska, Paulina; Krasnodębska-Szponder, Barbara; Paczkowska, Edyta; Safranow, Krzysztof; Gołembiewska, Edyta; Machaliński, Bogusław; Ciechanowski, Kazimierz

    2016-01-01

    In our study, we assessed the impact of immunosuppressive drug combinations on changes in the immune system of juvenile Wistar rats exposed to these drugs during pregnancy. We primarily concentrated on changes in two organs of the immune system - the thymus and the spleen. The study was conducted on 40 (32+8) female Wistar rats administered full and half dose of drugs, respectively, subjected to regimens commonly used in therapy of human kidney transplant recipients ([1] cyclosporine A, mycophenolate mofetil, and prednisone; [2] tacrolimus, mycophenolate mofetil, and prednisone; [3] cyclosporine A, everolimus, and prednisone). The animals received drugs by oral gavage 2 weeks before pregnancy and during 3 weeks of pregnancy. There were no statistically significant differences in the weight of the thymus and spleen, but changes were found in the results of blood hematology, cytometry from the spleen, and a histologic examination of the examined immune organs of juvenile Wistar rats. In the cytokine assay, changes in the level of interleukine 17 (IL-17) after increasing amounts of concanavaline A were dose-dependent; the increase of IL-17 was blocked after administration of higher doses of immunosuppressive drugs. However, after a reduction of doses, its increase resumed. Qualitative, quantitative, and morphological changes in the immune system of infant rats born to pharmacologically immunosuppressed females were observed. Thymus structure, spleen composition, and splenocyte IL-17 production were mostly affected in a drug regimen-dependent manner.

  17. Validation of the systemic lupus erythematosus responder index for use in juvenile-onset systemic lupus erythematosus.

    Science.gov (United States)

    Mina, Rina; Klein-Gitelman, Marisa S; Nelson, Shannen; Eberhard, B Anne; Higgins, Gloria; Singer, Nora G; Onel, Karen; Tucker, Lori; O'Neil, Kathleen M; Punaro, Marilynn; Levy, Deborah M; Haines, Kathleen; Martini, Alberto; Ruperto, Nicolino; Lovell, Daniel; Brunner, Hermine I

    2014-02-01

    This study tested the concurrent validity of the systemic lupus erythematosus responder index (SRI) in assessing improvement in juvenile-onset systemic lupus erythematosus (jSLE). The SRI considers changes in the SELENA-SLEDAI, BILAG and a 3-cm visual analogue scale of physician-rated disease activity (PGA) to determine patient improvement. Using prospectively collected data from 760 unique follow-up visit intervals of 274 jSLE patients, we assessed the sensitivity and specificity of the SRI using these external standards: physician-rated improvement (MD-change), patient/parent-rated major improvement of wellbeing (patient-change) and decrease in prescribed systemic corticosteroids (steroid-change). Modifications of the SRI that considered different thresholds for the SELENA-SLEDAI, BILAG and 10-cm PGA were explored and agreement with the American College of Rheumatology/PRINTO provisional criteria for improvement of jSLE (PCI) was examined. The sensitivity/specificity in capturing major improvement by the MD-change were 78%/76% for the SRI and 83%/78% for the PCI, respectively. There was fair agreement between the SRI and PCI (kappa=0.35, 95% CI 0.02 to 0.73) in capturing major improvement by the MD-change. Select modified versions of the SRI had improved accuracy overall. All improvement criteria tested had lower sensitivity when considering patient-change and steroid-change as external standards compared to MD-change. The SRI and its modified versions based on meaningful changes in jSLE have high specificity but at most modest sensitivity for capturing jSLE improvement. When used as an endpoint of clinical trials in jSLE, the SRI will provide a conservative estimate regarding the efficacy of the therapeutic agent under investigation.

  18. Surgical Resection of Idiopathic Distal Anterior Choroidal Artery Aneurysm Assisted by Frameless Stereotactic System Guidance: Case Report and Literature Review.

    Science.gov (United States)

    Ye, Feng; Tao, Chuanyuan; Xiong, Shenghua; You, Chao

    2017-01-01

    Idiopathic distal anterior choroidal artery (AChoA) aneurysms are particularly rare. Their diagnosis and treatment are difficult because of the particular characteristic of pathological anatomy. Our case mainly presented with intraventricular hemorrhage. His idiopathic aneurysm was located in the trigone of the undilated ventricle. Computed tomographic angiography demonstrated both the precise aneurysm and parent artery even when there was no dilated parent artery for the first time. It is also the first time this tiny aneurysm was resected assisted by frameless stereotactic guidance during operation after absorption of intraventricular blood. Computed tomographic angiography and frameless stereotactic guidance benefit the diagnosis and treatment of this kind of aneurysm. Sacrificing plexual AChoA did not cause any neurologic deficit.

  19. Retinoschisis (Juvenile)

    Science.gov (United States)

    ... of central and peripheral (side) vision due to degeneration of the retina. What are the symptoms? Juvenile retinoschisis, also known ... compounds that can preserve vision. Future stem cell treatments might also ... dorzolamide may improve retinal health and restore some vision in people with ...

  20. Juvenile angiofibromer

    DEFF Research Database (Denmark)

    Thuesen, Anne Daugaard; Jakobsen, John; Nepper-Rasmussen, Jørgen

    2005-01-01

    Juvenile angiofibroma is a rare, benign, rich vascular tumor, and approximately one new case is diagnosed in Denmark each year. It sits in the foramen sphenopalatinum and occurs in boys from 14 to 25 years of age. The most frequent initial symptoms are nasal obstruction and epistaxis. Through...

  1. Lifetime exposure to traumatic events among adolescents in contact with the Nigerian juvenile justice systems compared with a comparison group of secondary school students.

    Science.gov (United States)

    Atilola, Olayinka; Omigbodun, Olayinka; Bella-Awusah, Tolulope

    2014-05-01

    There are some knowledge gaps in what is known about pre-contact exposure to traumatic events among adolescents within the juvenile justice system. Data often focus on psychological sequelae without describing the traumatic events. In addition, there are few data from sub-Saharan Africa where juvenile justice inmates are often minor offenders and may themselves have been victims of abuse and neglect. To present detailed data on the lifetime prevalence rate and pattern of traumatic events among a cohort of adolescents in juvenile justice custody in Nigeria and to compare inmates who are 'offenders' with those who are 'victims'. Inmates of a borstal and a remand home comprised the study group and age- and gender-matched adolescents from two government schools were the secondary comparison group. The trauma-checklist of the Current and Lifetime Version of the Kiddies Schedule for Affective Disorders and Schizophrenia was used as a guide in assessing traumatic events. Of a total of 408 adolescents, 204 were recruited from the two juvenile justice institutions and 204 from secondary schools. Ninety per cent of participants were male and the mean (SD) age was 15·9 (2·8) years. The prevalence rate of lifetime exposure to traumatic events among the juvenile justice offenders was 88·7% compared with 48·5% of the comparison group (P = 0·001). The most commonly reported specific lifetime traumatic event was physical abuse (52·8%). The institutionalised adolescents were significantly more likely to report lifetime exposure to almost all the traumatic events assessed. Apart from the perpetrators of violent crime, there was no statistically significant difference in the prevalence and pattern of lifetime exposure to traumatic events between the offenders and the victims. This study provides further evidence that exposure to traumatic events is a fact of life for inmates of juvenile institutions, irrespective of whether they are offenders or victims. The implications

  2. Syphilis mimicking idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

    2011-01-01

    Idiopathic intracranial hypertension (IIH) is a condition of yet unknown aetiology affecting predominantly obese females of childbearing age. IIH is a diagnosis of exclusion as raised cerebrospinal fluid pressure may occur secondary to numerous other medical conditions. An atypical phenotype...... or an atypical disease course should alert the physician to reevaluate a presumed IIH-diagnosis. The authors report a case of a 32-year-old non-obese male with intracranial hypertension, secondary to a syphilitic central nervous system infection, initially misdiagnosed as being idiopathic. Upon relevant...... antibiotic treatment, signs and symptoms of elevated intracranial pressure resolved completely. Syphilis is a rare, but very important, differential diagnosis that in this case was clinically indistinguishable from IIH....

  3. Clinical, laboratory and neuroimage findings in juvenile systemic lupus erythematosus presenting involvement of the nervous system Achados clínicos, laboratoriais e de imagem no lupus eritematoso sistêmico juvenil com comprometimento do sistema nervoso

    Directory of Open Access Journals (Sweden)

    Mônica Jaques Spinosa

    2007-06-01

    Full Text Available OBJECTIVE: To characterize neurological involvement in juvenile systemic lupus erythe-matosus. METHOD: The charts of all patients with the diagnosis of systemic lupus erythematosus before the age of 16 years, followed at the Rheumatology Unit of Pequeno Príncipe Hospital, from January 1992 to January 2006, were retrospectively reviewed, highlighting neuropsychiatric aspects. RESULTS: Forty-seven patients were included. Neuropsychiatric syndromes were found 29 (61.7%: seizures (17 / 36.2%, intractable headache (7 / 14.9%, mood disorders (5 / 10.6%, cerebrovascular disease (4 / 8.5%, acute confusional state (3 / 6.4%, aseptic meningitis (3 / 6.4%, psychosis (3 / 6.4%, chorea (3 / 6.4%, Guillain-Barré syndrome (2 / 4.3% and cranial neuropathy (1 / 2.1%. Morbidity indexes (SLEDAI and SLICC were higher among patients with neuropsychiatric manifestations (pOBJETIVO: Caracterizar o comprometimento neurológico no lupus eritematoso sistêmico juvenil. MÉTODO: Os prontuários dos pacientes com o diagnóstico de lupus eritematoso sistêmico antes dos 16 anos de idade, em acompanhamento na Unidade de Reumatologia do Hospital Pequeno Príncipe, de janeiro de 1992 a janeiro de 2006, foram revisados retrospectivamente enfatizando aspectos neuropsiquiátricos. RESULTADOS: Quarenta e sete pacientes foram incluídos. Síndromes neuropsiquiátricas foram encontradas em 29 (61,7%: crises convulsivas (17 / 36,2%, cefaléia intratável (7 / 14,9%, distúrbios do humor (5 / 10,6%, doença cerebrovascular (4 / 8,5%, estado confusional agudo (3 / 6,4%, meningite asséptica (3 / 6,4%, psicose (3 / 6,4%, coréia (3 / 6,4%, síndrome de Guillain-Barré (2 / 4,3% e neuropatia craniana (1 / 2,1%. Índices de morbidade (SEDAI e SLICC foram maiores em pacientes com manifestações neuropsiquiátricas (p<0,05. CONCLUSÃO: Síndromes neuropsiquiátricas são um achado freqüente que acrescenta morbidade significativa ao lupus eritematoso sistêmico juvenil.

  4. Adolescent Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Safak Ekinci

    2014-06-01

    Full Text Available Scoliosis is called idiopathic when no other underlying disease can be identified. The etiology of adolescent idiopathic scoliosis (AIS is still unknown despite many years of research effort. Theories on AIS's etiology have included mechanical, hormonal, metabolic, neuromuscular, growth, and genetic abnormalities. Skeletally immature patients with adolescent idiopathic scoliosis are at risk of curve progression. The adolescent onset of severe idiopathic scoliosis has traditionally been evaluated using standing posteroanterior radiographs of the full spine to assess lateral curvature with the Cobb method. Scoliosis in children of school age and above primarily occurs in girls. The therapeutic goal in children is to prevent progression. In children, scoliosis of 20 and deg; or more should be treated with a brace, and scoliosis of 45 and deg; or more with surgery. [Arch Clin Exp Surg 2014; 3(3.000: 174-182

  5. Understanding idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Markey, Keira A; Mollan, Susan P; Jensen, Rigmor H

    2016-01-01

    Idiopathic intracranial hypertension is a disorder characterised by raised intracranial pressure that predominantly affects young, obese women. Pathogenesis has not been fully elucidated, but several causal factors have been proposed. Symptoms can include headaches, visual loss, pulsatile tinnitus...

  6. Efficacy of systemic and intratympanic corticosteroid combination therapy versus intratympanic or systemic therapy in patients with idiopathic sudden sensorineural hearing loss: a randomized controlled trial.

    Science.gov (United States)

    Ashtiani, Mohammadtaghi Khorsandi; Firouzi, Farzad; Bastaninejad, Shahin; Dabiri, Sasan; Nasirmohtaram, Sevil; Saeedi, Niloufar; Ghazavi, Hossein; Sahebi, Leyla

    2018-01-01

    The present study was conducted to compare the rates of recovery from idiopathic sudden deafness after the treatment with oral and intratympanic corticosteroids in both mono and combination therapies. Triple-blind randomized clinical trial. Tertiary referral hospital. A total of 112 patients who were admitted to the ENT emergency department randomly divided into three groups: an oral corticosteroid plus intratympanic placebo (systemic corticosteroid monotherapy group); an intratympanic corticosteroid plus oral placebo group (IT monotherapy group); and a combination therapy group (IT plus systemic combination group). All patients were treated additionally with antiviral and proton pomp inhibitor. An audiometry was performed once before beginning the therapies and again at the end of the therapy. Of the total of 112 patients, 32 received intratympanic (IT) corticosteroids, 45 were receiving systemic corticosteroids, and 35 were receiving a combination of the two. A total of 74 patients (66.1%) responded positively [response to treatment was calculated as gain of at least 10 dB in 10 dB in average threshold or with the minimum improvement of 15% in speech discrimination scores (SDS)] to corticosteroid therapy. No significant differences were observed between the three groups (IT, systemic group, and combination therapy group) in their overall response to treatment (p = 0.5). Patients who suffered from concomitant tinnitus and dizziness responded less positively to the treatment (p family history of SSNHL seems to be negative prognostic factors in the response to treatment (p hearing loss (p = 0.9). This study did not find any difference in the rate of hearing improvement between systemic, intratympanic, and combined corticosteroid therapy for sudden hearing loss. 1b.

  7. High disease activity: an independent factor for reduced immunogenicity of the pandemic influenza a vaccine in patients with juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Campos, Lucia M A; Silva, Clovis A; Aikawa, Nadia E; Jesus, Adriana A; Moraes, Julio C B; Miraglia, Joao; Ishida, Maria A; Bueno, Cleonice; Pereira, Rosa M R; Bonfa, Eloisa

    2013-07-01

    Recent findings demonstrated a reduced immunogenicity of the influenza A H1N1/2009 vaccine in juvenile rheumatic diseases. However, a point of concern is whether the vaccine could induce disease flares. The aim of this study was to assess the disease safety of and the possible influence of disease parameters and therapy on nonadjuvant influenza A H1N1 vaccine response of juvenile systemic lupus erythematosus (SLE) patients. One hundred eighteen juvenile SLE patients and 102 healthy controls of a comparable age were vaccinated. Seroprotection rate, seroconversion rate, and factor increase in geometric mean titer (GMT) were calculated and effective immune response was defined by the Food and Drug Administration and the European Committee for Proprietary Medicinal Products vaccine immunologic standards. Disease parameters, treatment, and adverse events were evaluated. Age was comparable in juvenile SLE patients and controls (mean ± SD 16.0 ± 3.5 versus 15.9 ± 4.5 years; P = 0.26). Three weeks after immunization, seroprotection rate (73.7% versus 95.1%; P vaccine adverse events were observed. The present study demonstrated adequate disease safety and is the first to discriminate that high disease activity impairs influenza A H1N1/2009 vaccine antibody production in juvenile SLE, in spite of an overall immune response within recommended levels. Copyright © 2013 by the American College of Rheumatology.

  8. Juvenile systemic lupus erythematosus in a adolescent with acquired immunodeficiency syndrome

    OpenAIRE

    Sacilotto, Nathália de Carvalho; Yamashiro, Cintia Yukimi; Nishimoto, Teresa Maria Isaac

    2010-01-01

    O lúpus eritematoso sistêmico juvenil (LESJ) é uma doença inflamatória crônica, multissistêmica e autoimune. Algumas manifestações clínicas dessa condição são semelhantes às encontradas na síndrome da imunodeficiência adquirida (SIDA). A coexistência da SIDA com o LESJ é rara, especialmente na população pediátrica, sendo descritos na literatura pesquisada apenas cinco casos de pacientes com infecção congênita por HIV que desenvolveram essa enfermidade reumatológica, tendo como manifestação in...

  9. Thrombotic thrombocytopenic purpura at presentation of juvenile systemic lupus erythematosus patients

    OpenAIRE

    Campos, Lucia M. A.; Spadoni, Maria Silvia; Michelin, Cintia M.; Jesus, Adriana A.; Carneiro, Jorge D. A.; Silva, Clovis Artur Almeida da

    2013-01-01

    Púrpura trombocitopênica trombótica (PTT) é uma alteração hematológica rara e com risco de morte, caracterizada por trombocitopenia, anemia hemolítica microangiopática e alterações neurológicas e/ou renais. A PTT foi descrita em raros pacientes com lúpus eritematoso sistêmico juvenil (LESJ) e, até onde se sabe, a prevalência dessa manifestação em uma população de lúpus pediátrico ainda não foi estudada. Assim, entre janeiro de 1983 e dezembro de 2010, revisamos os prontuários de 5.508 pacient...

  10. Juvenile rheumatoid arthritis

    Science.gov (United States)

    ... joints. This form of JIA may turn into rheumatoid arthritis. It may involve 5 or more large and ... no known prevention for JIA. Alternative Names Juvenile rheumatoid arthritis (JRA); Juvenile chronic polyarthritis; Still disease; Juvenile spondyloarthritis ...

  11. A Pediatric Case of Systemic Lupus Erythematosus Developed 10 Years after Cord Blood Transplantation for Juvenile Myelomonocytic Leukemia

    Directory of Open Access Journals (Sweden)

    Masayuki Nagasawa

    2012-01-01

    Full Text Available Allogeneic hematopoietic stem cell transplantation (allo-HSCT is a most powerful immunotherapy for hematological malignancies. However, the impact of immunological disturbances as a result of allo-HSCT is not understood well. We experienced an 11-year-old boy who presented with systemic lupus erythemathosus (SLE 10 years after unrelated cord blood transplantation of male origin for juvenile myelomonocytic leukemia (JMML with monosomy 7. Bone marrow examination showed complete remission without monosomy 7. Genetic analysis of peripheral blood revealed mixed chimera with recipient cells consisting of <5% of T cells, 50–60% of B cells, 60–75% of NK cells, 70–80% of macrophages, and 50–60% of granulocytes. Significance of persistent mixed chimera as a cause of SLE is discussed.

  12. EVALUATION OF THICKNESS OF INTIMA-MEDIA COMPLEX OF COMMON CAROTID ARTERIES IN CHILDREN WITH JUVENILE ARTHRITIS AND SYSTEMIC LUPUS ERYTHEMATOSUS

    Directory of Open Access Journals (Sweden)

    A.B. Sugak

    2010-01-01

    Full Text Available Rheumatic diseases in adults are associated with accelerated atherosclerosis, and its early signs can be stated by the thickening of intima-media complex of common carotid arteries (CCA. This symptom is detected during ultrasound examination in 49% of children with systemic lupus erythematosus, in 24% of patients with juvenile rheumatoid arthritis and in 13% of children with juvenile spondylarthritis. Besides, 36% of children with systemic lupus erythematosus and 17% — with systemic type of juvenile rheumatoid arthritis had structure changes of CCA wall. A dependence of these disorders on cholesterol and glucose levels in blood serum, overweight and Cushing syndrome, age, duration and activity of a disease, levels of ESR, C-reactive protein and white blood cells was not showed. Authors detected a correlation between the thickness of intima-media complex of CCA and hemostasis parameters.Key words: children, juvenile arthritis, systemic lupus erythematosus, intima-media complex, ultrasound diagnostics.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2010;9(2:64-69

  13. Idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Noble Paul W

    2008-03-01

    Full Text Available Abstract Idiopathic pulmonary fibrosis (IPF is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000 than in women (13.2/100,000. The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock. IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP. The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis, forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational exposures. IPF is typically progressive and leads to significant

  14. Iatrogenic effect of juvenile justice.

    Science.gov (United States)

    Gatti, Uberto; Tremblay, Richard E; Vitaro, Frank

    2009-08-01

    The present study uses data from a community sample of 779 low-SES boys to investigate whether intervention by the juvenile justice system is determined, at least in part, by particular individual, familial and social conditions, and whether intervention by the juvenile courts during adolescence increases involvement in adult crime. The study considers self-reported crime in childhood and adolescence, and introduces individual, familial and social variables into its analysis. The results show that youths who are poor, impulsive, poorly supervised by their parents, and exposed to deviant friends are more likely, for the same degree of antisocial behavior, to undergo intervention by the Juvenile Court, and that this intervention greatly increases the likelihood of involvement with the penal system in adulthood. The results also show that the various measures recommended by the Juvenile Court exert a differential criminogenic effect; those that involve placement have the most negative impact.

  15. Dermatomiositis juvenil

    OpenAIRE

    Goldaracena, Pablo; Pérez, Federico

    2008-01-01

    La dermatomiositis juvenil (DMJ) es una enfermedad multi sistémica de etiología desconocida, caracterizada por una vasculitis que ocasiona una inflamación no supurativa del músculo estriado y lesiones cutáneas distintivas. La cobertura de los criterios de Bohan y Peter establece el diagnóstico: exantema patognomónico junto a debilidad muscular proximal simétrica, elevación sérica de enzimas musculares, s...

  16. Effects of juvenile host density and food availability on adult immune response, parasite resistance and virulence in a Daphnia-parasite system.

    Directory of Open Access Journals (Sweden)

    Corine N Schoebel

    Full Text Available Host density can increase infection rates and reduce host fitness as increasing population density enhances the risk of becoming infected either through increased encounter rate or because host condition may decline. Conceivably, potential hosts could take high host density as a cue to up-regulate their defence systems. However, as host density usually covaries with food availability, it is difficult to examine the importance of host density in isolation. Thus, we performed two full-factorial experiments that varied juvenile densities of Daphnia magna (a freshwater crustacean and food availability independently. We also included a simulated high-density treatment, where juvenile experimental animals were kept in filtered media that previously maintained Daphnia at high-density. Upon reaching adulthood, we exposed the Daphnia to their sterilizing bacterial parasite, Pasteuria ramosa, and examined how the juvenile treatments influenced the likelihood and severity of infection (Experiment I and host immune investment (Experiment II. Neither juvenile density nor food treatments affected the likelihood of infection; however, well-fed hosts that were well-fed as juveniles produced more offspring prior to sterilization than their less well-fed counterparts. By contrast, parasite growth was independent of host juvenile resources or host density. Parasite-exposed hosts had a greater number of circulating haemocytes than controls (i.e., there was a cellular immune response, but the magnitude of immune response was not mediated by food availability or host density. These results suggest that density dependent effects on disease arise primarily through correlated changes in food availability: low food could limit parasitism and potentially curtail epidemics by reducing both the host's and parasite's reproduction as both depend on the same food.

  17. A multicenter study analyzing the relationship of a standardized radiographic scoring system of adolescent idiopathic scoliosis and the Scoliosis Research Society outcomes instrument.

    Science.gov (United States)

    Wilson, Philip L; Newton, Peter O; Wenger, Dennis R; Haher, Thomas; Merola, Andrew; Lenke, Larry; Lowe, Thomas; Clements, David; Betz, Randy

    2002-09-15

    A multicenter study examining the association between radiographic and outcomes measures in adolescent idiopathic scoliosis. To evaluate the association between an objective radiographic scoring system and patient quality of life measures as determined by the Scoliosis Research Society outcomes instrument. Although surgical correction of scoliosis has been reported to be positively correlated with patient outcomes, studies to date have been unable to demonstrate an association between radiographic measures of deformity and outcomes measures in patients with adolescent idiopathic scoliosis. A standardized radiographic deformity scoring system and the Scoliosis Research Society outcome tool were used prospectively in seven scoliosis centers to collect data on patients with adolescent idiopathic scoliosis. A total of 354 data points for 265 patients consisting of those with nonoperative or preoperative curves >or=10 degrees, as well as those with surgically treated curves, were analyzed. Correlation analysis was performed to identify significant relationships between any of the radiographic measures, the Harms Study Group radiographic deformity scores (total, sagittal, coronal), and the seven Scoliosis Research Society outcome domains (Total Pain, General Self-Image, General Function, Activity, Postoperative Self-Image, Postoperative Function, and Satisfaction) as well as Scoliosis Research Society outcomes instrument total scores. Radiographic measures that were identified as significantly correlated with Scoliosis Research Society outcome scores were then entered into a stepwise regression analysis. The coronal measures of thoracic curve and lumbar curve magnitude were found to be significantly correlated with the Total Pain, General Self-Image, and total Scoliosis Research Society scores (P Society domain and total scores. No radiographic measures taken after surgery were significantly correlated with the postoperative domains of the Scoliosis Research Society

  18. Atypical idiopathic inflammatory demyelinating lesions

    DEFF Research Database (Denmark)

    Wallner-Blazek, Mirja; Rovira, Alex; Fillipp, Massimo

    2013-01-01

    Atypical lesions of a presumably idiopathic inflammatory demyelinating origin present quite variably and may pose diagnostic problems. The subsequent clinical course is also uncertain. We, therefore, wanted to clarify if atypical idiopathic inflammatory demyelinating lesions (AIIDLs) can be class...

  19. Hypoxia tolerance and antioxidant defense system of juvenile jumbo squids in oxygen minimum zones

    Science.gov (United States)

    Trübenbach, Katja; Teixeira, Tatiana; Diniz, Mário; Rosa, Rui

    2013-10-01

    Jumbo squid (Dosidicus gigas) is a large oceanic squid endemic off the Eastern Tropical Pacific that undertakes diel vertical migrations into mesopelagic oxygen minimum zones. One of the expected physiological effects of such migration is the generation of reactive oxygen species (ROS) at the surface, promoted by the transition between hypoxia and reoxygenation states. The aim of this study was to investigate the energy expenditure rates and the antioxidant stress strategies of juvenile D. gigas under normoxia and hypoxia, namely by quantifying oxygen consumption rates, antioxidant enzyme activities [including superoxide dismutase (SOD), catalase (CAT) and glutathione-S-transferase (GST)], heat shock protein expression (Hsp70/Hsc70), and lipid peroxidation [malondialdehyde (MDA) levels]. A high significant decrease (68%) in squid's metabolic rates was observed during hypoxia (p0.05), with the latter indicating no enhancement of lipid peroxidation (i.e. cellular damage) at the warmer and normoxic surface waters. The understanding of such physiological strategies that are linked to oxygen deprivation and reoxygenation phases may provide valuable information about how this species is quickly responding to the impacts of environmental stressors coupled with global climate change.

  20. Fighting Juvenile Gun Violence. Juvenile Justice Bulletin.

    Science.gov (United States)

    Sheppard, David; Grant, Heath; Rowe, Wendy; Jacobs, Nancy

    This bulletin describes the Office of Juvenile Justice and Delinquency Prevention's efforts to fight juvenile gun violence. The Office awarded four community demonstration grants to implement "Partnerships To Reduce Juvenile Gun Violence." Partnership goals include increasing the effectiveness of existing strategies by enhancing and…

  1. Responses of earthworms (Lumbricus rubellus) to copper and cadmium as determined by measurement of juvenile traits in a specifically designed test system.

    Science.gov (United States)

    Spurgeon, D J; Svendsen, C; Kille, P; Morgan, A J; Weeks, J M

    2004-01-01

    In this study, the effects of two metals, copper and cadmium, on the growth and development of juvenile Lumbricus rubellus were measured in a toxicity test in which individuals were grown in isolation. This design had a number of advantages over traditional test systems for earthworms. Importantly, the test is specifically designed to measure two juvenile traits (survival over and length of the juvenile period) that have been shown to have a high sensitivity for determining population growth rate. The test system also maximizes replication, while allowing time-series-based monitoring of individual growth. For both metals, significant exposure-dependent effects on survival, growth, development time, and (less certainly) maturation weight were observed. Comparisons of the relative toxicity of the two metals indicated different concentration-response relationships. For copper, hormesis was found at low levels, while only at the highest soil concentration tested (10.07 micromol g(-1)) were (severe) toxic effects present. For cadmium, hormesis was also evident at the lowest concentration tested; however, at soil levels above this, a graded concentration-dependent toxic effect was apparent. These differences in the exposure response patterns can be (tentatively) explained in terms of the mechanisms for handling copper (an essential metal for earthworms) and cadmium (a putative nonessential element). The applicability of the test for routine measurement of chemical effects on ecologically relevant juvenile traits is also outlined and future developments are discussed.

  2. Evaluation of dietary soybean meal as fish meal replacer for juvenile whiteleg shrimp, Litopenaeus vannamei reared in biofloc system

    Directory of Open Access Journals (Sweden)

    Hyeonho Yun

    2017-01-01

    Full Text Available Abstract Different levels of dietary soybean meal (SBM as a fish meal (FM replacer, with and without amino acid supplementation, for whiteleg shrimp, Litopenaeus vannamei reared in the biofloc system was examined in eight weeks of feeding trial. Eight experimental diets consisted of a basal diet with 0% FM replacement by SBM provided in clear sea water without biofloc system (S0SW, four diets replacing FM at 0% (S0, 33% (S33, 67% (S67 and 100% (S100 by SBM, and three diets replacing FM at 33% (S33A, 67% (S67A and 100% (S100A by SBM supplemented with amino acids (methionine and lysine in the seawater biofloc system. Results of water quality analyses showed significantly lower total suspended solids and nitrate for S0SW group than all other treatments. Diets S0 and S33A resulted in higher weight gain and specific growth rate among all groups, with no significant differences with S33 group. In addition, whole-body protein and amino acid compositions of shrimp fed S0SW were lower than most biofloc groups. Haemolymph parameters showed significant differences in total protein, cholesterol and triglyceride between groups S0 and S0SW. Also, superoxide dismutase activity showed a decreasing trend with increasing replacement level. In conclusion, based on these results, SBM could replace up to 33% of FM with or without amino acid supplementation in juvenile whiteleg shrimp diets reared in the biofloc system.

  3. Cultivable intestinal microbiota of yellowtail juveniles (Seriola lalandi in an aquaculture system

    Directory of Open Access Journals (Sweden)

    Eduardo Aguilera

    2013-07-01

    Full Text Available The yellowtail (Seriola lalandi has been farmed for many years and is becoming a promising aquaculture species. Knowledge of the intestinal microbiota of this species is very limited. Thus, the aim of this study is to describe the bacterial populations associated with the intestinal tract of Seriola lalandi reared in Chile. The microbiota composition was analyzed at two growth stages distinguished by weight and parameters such as Specific Growth Rate (SGR and Feed Conversion Ratio (FCR. Juveniles (mean initial weight 7.33 ± 0.30 g and pre-adults (81.7 ± 19.0 g were fed with commercial diet for 33 and 50 days, respectively. The first intestinal samples were collected at the end of Trial 1 from specimens weighing approximately 50 g while the second samples were obtained at the end of Trial 2 from specimens weighing approximately 370 g. The microbiota composition was examined using conventional isolation in Tryptic Soy Agar (TSA followed by 16S rRNA sequencing and identification. In total, 16 genera were identified. Pseudomonas, Vibrio and Staphylococcus were the predominant genera in fish at the 50 g stage, whereas Microbacterium and Francisella were the predominant genera in the 370 g stage. The microbiota composition showed different assemblages, depending on host size, with Bacillus and Vibrio being the only genera that were shared. Knowledge of the intestinal microbiota of Seriola lalandi is the first step in the exploration of microbiota management and the development of probiotics, as well as in the identification of the bacterial populations in healthy fish under cultured conditions.

  4. Acute exacerbation of idiopathic pulmonary fibrosis triggered by Aspergillus empyema

    Directory of Open Access Journals (Sweden)

    Atsushi Suzuki

    Full Text Available Acute exacerbation (AE is a severe and life-threatening complication of idiopathic pulmonary fibrosis (IPF. In 2016, the definition and diagnostic criteria for AE-IPF were updated by an international working group. The new definition includes any acute, clinically significant respiratory deterioration (both idiopathic and triggered events characterized by evidence of new widespread alveolar abnormality in patients with IPF. There are no currently proven beneficial management strategies for idiopathic and triggered AE-IPF. This is the first report describing AE-IPF triggered by Aspergillus empyema, which was improved by a combination of corticosteroid, systemic antifungal therapy, local antifungal therapy, and additional pharmacological therapies. Future research may reveal optimal strategies for both idiopathic and triggered AE-IPF. Keywords: Idiopathic pulmonary fibrosis, Acute exacerbation, AE-IPF, Triggered AE, Aspergillus infection

  5. The influence of intrauterine exposure to immunosuppressive treatment on changes in the immune system in juvenile Wistar rats

    Directory of Open Access Journals (Sweden)

    Kabat-Koperska J

    2016-07-01

    Full Text Available Joanna Kabat-Koperska,1 Agnieszka Kolasa-Wołosiuk,2 Bartosz Wojciuk,3 Iwona Wojciechowska-Koszko,3 Paulina Roszkowska,3 Barbara Krasnodębska-Szponder,3 Edyta Paczkowska,4 Krzysztof Safranow,5 Edyta Gołembiewska,1 Bogusław Machaliński,4 Kazimierz Ciechanowski1 1Department of Nephrology, Transplantology and Internal Medicine, 2Department of Histology and Embryology, 3Department of Microbiology and Immunological Diagnostics, 4Department of General Pathology, 5Department of Biochemistry and Medical Chemistry, Pomeranian Medical University, Szczecin, Poland Background: In our study, we assessed the impact of immunosuppressive drug combinations on changes in the immune system of juvenile Wistar rats exposed to these drugs during pregnancy. We primarily concentrated on changes in two organs of the immune system – the thymus and the spleen. Methods: The study was conducted on 40 (32+8 female Wistar rats administered full and half dose of drugs, respectively, subjected to regimens commonly used in therapy of human kidney transplant recipients ([1] cyclosporine A, mycophenolate mofetil, and prednisone; [2] tacrolimus, mycophenolate mofetil, and prednisone; [3] cyclosporine A, everolimus, and prednisone. The animals received drugs by oral gavage 2 weeks before pregnancy and during 3 weeks of pregnancy. Results: There were no statistically significant differences in the weight of the thymus and spleen, but changes were found in the results of blood hematology, cytometry from the spleen, and a histologic examination of the examined immune organs of juvenile Wistar rats. In the cytokine assay, changes in the level of interleukine 17 (IL-17 after increasing amounts of concanavaline A were dose-dependent; the increase of IL-17 was blocked after administration of higher doses of immunosuppressive drugs. However, after a reduction of doses, its increase resumed. Conclusion: Qualitative, quantitative, and morphological changes in the immune system of infant

  6. Optimization of Concurrent Deployments of the Juvenile Salmon Acoustic Telemetry System and Other Hydroacoustic Equipment at John Day Dam

    Energy Technology Data Exchange (ETDEWEB)

    Ploskey, Gene R.; Hughes, James S.; Khan, Fenton; Kim, Jina; Lamarche, Brian L.; Johnson, Gary E.; Choi, Eric Y.; Faber, Derrek M.; Wilberding, Matthew C.; Deng, Zhiqun; Weiland, Mark A.; Zimmerman, Shon A.; Fischer, Eric S.; Cushing, Aaron W.

    2008-09-01

    The purpose of this report is to document the results of the acoustic optimization study conducted at John Day Dam during January and February 2008. The goal of the study was to optimize performance of the Juvenile Salmon Acoustic Telemetry System (JSATS) by determining deployment and data acquisition methods to minimize electrical and acoustic interference from various other acoustic sampling devices. Thereby, this would allow concurrent sampling by active and passive acoustic methods during the formal evaluations of the prototype surface flow outlets at the dam during spring and summer outmigration seasons for juvenile salmonids. The objectives for the optimization study at John Day Dam were to: 1. Design and test prototypes and provide a total needs list of pipes and trolleys to deploy JSATS hydrophones on the forebay face of the powerhouse and spillway. 2. Assess the effect on mean percentage decoded of JSATS transmissions from tags arrayed in the forebay and detected on the hydrophones by comparing: turbine unit OFF vs. ON; spill bay OPEN vs. CLOSED; dual frequency identification sonar (DIDSON) and acoustic Doppler current profiler (ADCP) both OFF vs. ON at a spill bay; and, fixed-aspect hydroacoustic system OFF vs. ON at a turbine unit and a spill bay. 3. Determine the relationship between fixed-aspect hydroacoustic transmit level and mean percentage of JSATS transmissions decoded. The general approach was to use hydrophones to listen for transmissions from JSATS tags deployed in vertical arrays in a series perpendicular to the face of the dam. We used acoustic telemetry equipment manufactured by Technologic and Sonic Concepts. In addition, we assessed old and new JSATS signal detectors and decoders and two different types of hydrophone baffling. The optimization study consisted of a suite of off/on tests. The primary response variable was mean percentage of tag transmissions decoded. We found that there was no appreciable adverse effect on mean percentage

  7. Juvenile systemic lupus erythematosus in Portugal: clinical and immunological patterns of disease expression in a cohort of 56 patients.

    Science.gov (United States)

    Cabral, Marta; Escobar, Carlos; Conde, Marta; Ramos, Margarida; Melo Gomes, José A

    2013-01-01

    To define the pattern of disease expression and to gain better understanding in patients with juvenile onset systemic lupus erythematosus (SLE) in Portugal. The features of unselected patients with systemic lupus erythematosus who had disease onset before the age of 18 years were retrospectively analysed in three Portuguese centres with Pediatric Rheumatology Clinic over a 24-year period (1987-2011). Demographic, clinical and laboratory manifestations, therapy and outcome were assessed. A cohort of 56 patients with a mean age at disease onset of 12.6 ± 4.04 years (mean ± 1SD) (range, 1.0-17.0 years) and a mean period of follow-up of 5.5 ± 5.4 years. Forty six (82.1%) patients were female. The most common disease manifestations were musculoskeletal (87.5%), mucocutaneous (80.3%) and haematological abnormalities (75%). Lupus nephritis was diagnosed in 46.4% of patients and consisted of glomerular nephritis in all cases. Neuropsychiatric manifestations occurred in 21.4% but severe central nervous system complications were uncommon, as brain infarcts and organic brain syndrome in 4 (7.1%) patients. Antinuclear antibodies and anti-double stranded DNA were positive in most patients in (98.2% and 71.4% respectively), as well as low C3 and/or C4 were observed frequently (85.7%). Generally, most patients had a good response to therapy as demonstrated by a significant decreasing of SLEDAI score from disease presentation to the last evaluation. The SLEDAI at diagnosis, the maximum SLEDAI and the incidence of complications were significantly higher in patients with neurolupus and/or lupus nephritis. Therapy included oral steroids (87.5%), hydroxychloroquine (85.7%), azathioprine (55.4%), IV cyclophosphamide (28.6%) along with other drugs. Six (10.7%) patients were treated with rituximab. Long-term remission was achieved in 32%, disease was active in 68%, adverse reactions to therapy occurred in 53.6% and complications/severe manifestations in 23.2%. Two patients died, being

  8. Trends in nitrogen isotope ratios of juvenile winter flounder reflect changing nitrogen inputs to Rhode Island, USA estuarine systems.

    Science.gov (United States)

    Pruell, Richard J; Taplin, Bryan K; Miller, Kenneth M

    2017-05-15

    Nitrogen isotope ratios (δ 15 N) in juvenile winter flounder, Pseudopleuronectes americanus, were used to examine changes in nitrogen inputs to several Rhode Island, USA estuarine systems. Fish were collected over two three-year periods with a ten-year interval between sampling periods (2002-2004 and 2012-2014). During that interval numerous changes to nutrient management practices were initiated in the watersheds of these estuarine systems including the upgrade of several major wastewater treatment facilities that discharge to Narragansett Bay, which significantly reduced nitrogen inputs. Following these reductions, the δ 15 N values of flounder in several of the systems decreased as expected; however, isotope ratios in fish from upper Narragansett Bay significantly increased. We believe that low δ 15 N values measured in 2002-2004 were related to concentration-dependent fractionation at this location. Increased δ 15 N values measured between 2012 and 2014 may indicate reduced fractionation or that changes in wastewater treatment processes altered the nitrogen isotopic ratios of the effluents. Published by Elsevier Ltd.

  9. Development of a new multiple sampling trawl with autonomous opening/closing net control system for sampling juvenile pelagic fish

    Science.gov (United States)

    Oozeki, Yoshioki; Hu, Fuxiang; Tomatsu, Chiaki; Kubota, Hiroshi

    2012-03-01

    A new multiple layer sampling trawl with an autonomous net opening/closing control system was developed to sample pelagic juvenile fish quantitatively. The new trawl system, based on the Matsuda-Oozeki-Hu Trawl (MOHT), has a rigid-frame 3.3 m high and 2.35 m wide and five nets of 11.0 m length with a rectangular mouth of 2.22 m×1.81 m (4 m2 mouth area; large-scale prototype). A cambered V-shape depressor is hung below the frame and two bridles are attached at the midpoint of the side frames. A net-release controller is used, which not only controls the net release mechanism but also records the net depth, temperature and flow rate during net towing. The controller sends stored command signals to the net release mechanism as depth settings and/or time settings and does not require any commands from the surface through a conducting cable or by acoustic signals. Two other models were constructed before the construction of the large-scale prototype, which are a small-scale prototype (2 m2 mouth area) for testing the net release mechanism and a 1/4-scale model of the large-scale prototype for flume tank tests. Flume tank tests with the 1/4-scale model showed that the frame leaned forward at a tilt angle from 5 to 15 degrees at towing speeds from 0.8 to 1.4 m s-1. Opened nets closed smoothly and sequentially nets were completely opened when the trigger was released by the command. Net depth rarely changed even during changes in towing speed. Sea trials both by the small-scale and the large-scale prototype demonstrated the same towing characteristics expected from the flume tank tests. The newly developed multiple layer opening/closing MOHT (MOC-MOHT) is considered to be suitable for quantitative layer sampling of juvenile fish.

  10. Diffuse idiopathic skeletal hyperostosis in ancient clergymen.

    NARCIS (Netherlands)

    Verlaan, J.J.; Oner, F.C.; Maat, G.J.

    2007-01-01

    Diffuse idiopathic skeletal hyperostosis (DISH) is a common but often unrecognized systemic disorder observed mainly in the elderly. DISH is diagnosed when the anterior longitudinal ligament of the spine is ossified on at least four contiguous spinal levels or when multiple peripheral enthesopathies

  11. Diffuse idiopathic skeletal hyperostosis in ancient clergymen

    NARCIS (Netherlands)

    Verlaan, J.J.; Oner, F.C.; Maat, G.R.J.

    2007-01-01

    Diffuse idiopathic skeletal hyperostosis (DISH) is a common but often unrecognized systemic disorder observed mainly in the elderly. DISH is diagnosed when the anterior longitudinal ligament of the spine is ossified on at least four contiguous spinal levels or when multiple peripheral

  12. THE STUDY OF FEATURES OF GUILT OF JUVENILE OFFENDERS IN THE CONTEXT OF JUVENILE JUSTICE

    Directory of Open Access Journals (Sweden)

    Natalija Vladimirovna Galkina

    2015-08-01

    Full Text Available The article is devoted to the results of empirical studies of the experiences of guilt of juvenile offenders in the context of juvenile justice where a minor appears as the subject of legal relations. Restorative approach of juvenile justice is based on an admission of guilt to the victim. In connection with it, the research of features of the guilt of minors who have committed an offence and the conditions for the development of the subjectivity will enhance understanding of the possibilities of restorative juvenile justice system in the prevention of juvenile delinquency.Thus, the results of empirical research presented in the article are important for determining of the psychological bases of realization of rehabilitation programs in the context of juvenile justice. In particular, the results are important for the organization and conduct of psychological work to overcome the psychological barriers in the behavior of juveniles having inherently maladaptive guilt and destructive psychological defense mechanisms.

  13. Chronic idiopathic constipation

    African Journals Online (AJOL)

    2009-05-12

    May 12, 2009 ... idiopathic constipation are categorised into three groups: • patients with normal colon transit and evacuation. • patients with evacuatory disorders. • patients with slow colon transit. A colon transit study is the best way to differentiate these categories of patients. Twenty radio-opaque pellets are swallowed.

  14. Idiopathic epiretinal membrane

    NARCIS (Netherlands)

    Bu, Shao-Chong; Kuijer, Roelof; Li, Xiao-Rong; Hooymans, Johanna M M; Los, Leonoor I

    2014-01-01

    Background: Idiopathic epiretinal membrane (iERM) is a fibrocellular membrane that proliferates on the inner surface of the retina at the macular area. Membrane contraction is an important sight-threatening event and is due to fibrotic remodeling. Methods: Analysis of the current literature

  15. Idiopathic inflammatory myopathies

    NARCIS (Netherlands)

    van der Kooi, A. J.; de Visser, M.

    2014-01-01

    Idiopathic inflammatory myopathies (IIMs), except for sporadic inclusion body myositis (sIBM), present with subacute symmetrical weakness of the limb girdle muscles, an elevated serum creatine kinase activity, and inflammatory cells in the muscle biopsy (necrotizing autoimmune myopathy being an

  16. Idiopathic Retroperitoneal Hematoma

    African Journals Online (AJOL)

    In the early stages it typically presents as generalized abdominal pain, nausea, anorexia. The symptoms and signs of hypovolemic shock normally present late. As management of idiopathic retroperitoneal hematoma is usually conservative, early diagnosis can save the patient an unnecessary exploration. This will improve.

  17. Idiopathic scrotal calcinosis

    DEFF Research Database (Denmark)

    Tareen, Ali; Ibrahim, Rami Mossad

    2018-01-01

    INTRODUCTION: Idiopathic scrotal calcinosis is a rare benign condition which presents with asymptomatic multiple nodules on the scrotal skin. PRESENTATION OF CASE: Our patient, a 64-year-old Indian male with Fitzpatrick skin type 4, presented with multiple nodules, which were completely surgicall...

  18. Idiopathic granulomatous orbital inflammation

    NARCIS (Netherlands)

    Mombaerts, I.; Schlingemann, R. O.; Goldschmeding, R.; Koornneef, L.

    1996-01-01

    PURPOSE: Granulomatous orbital inflammation may occur as an isolated condition of unknown origin. These idiopathic granulomatous lesions are believed to belong to the orbital pseudotumor group by some authors, whereas others consider them sarcoidosis limited to the orbit. The aim of this study is to

  19. Idiopathic ophthalmodynia and idiopathic rhinalgia: two topographic facial pain syndromes.

    Science.gov (United States)

    Pareja, Juan A; Cuadrado, María L; Porta-Etessam, Jesús; Fernández-de-las-Peñas, César; Gili, Pablo; Caminero, Ana B; Cebrián, José L

    2010-09-01

    To describe 2 topographic facial pain conditions with the pain clearly localized in the eye (idiopathic ophthalmodynia) or in the nose (idiopathic rhinalgia), and to propose their distinction from persistent idiopathic facial pain. Persistent idiopathic facial pain, burning mouth syndrome, atypical odontalgia, and facial arthromyalgia are idiopathic facial pain syndromes that have been separated according to topographical criteria. Still, some other facial pain syndromes might have been veiled under the broad term of persistent idiopathic facial pain. Through a 10-year period we have studied all patients referred to our neurological clinic because of facial pain of unknown etiology that might deviate from all well-characterized facial pain syndromes. In a group of patients we have identified 2 consistent clinical pictures with pain precisely located either in the eye (n=11) or in the nose (n=7). Clinical features resembled those of other localized idiopathic facial syndromes, the key differences relying on the topographic distribution of the pain. Both idiopathic ophthalmodynia and idiopathic rhinalgia seem specific pain syndromes with a distinctive location, and may deserve a nosologic status just as other focal pain syndromes of the face. Whether all such focal syndromes are topographic variants of persistent idiopathic facial pain or independent disorders remains a controversial issue.

  20. The impact of disease and antirheumatic therapy factors on growth retardation in children suffering from juvenile rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    T.M. Bzarova

    2006-01-01

    Full Text Available THE STUDY EVALUATED THE IMPACT OF REFRACTORY JUVENILE IDIOPATHIC ARTHRITIS (JIA AND INTERFERENCE OF ANTIRHEUMATIC THERAPY ON PHYSICAL STATURE AND GROWTH AND PHYSICAL DEVELOPMENT IN CHILDREN. WE OBSERVED 133 PATIENTS WITH JIA WITH AGE FROM 4.6 TO 18 YEARS. WE ASSESSED PATIENTS' PHYSICAL STATURE (DOCUMENTING HEIGHT AND WEIGHT AND FURTHER GROWTH AND DEVELOPMENT STARING FROM BIRTH, BEFORE THE ONSET OF JIA, AT THE FIRST YEAR FROM THE ONSET OF THE DISB EASE AND LATER ON. PHYSICAL RETARDATION WAS MARKED IN ALL CHILDREN IN THE FIRST YEAR FROM THE ONSET OF JIA. SYSTEMIC JIA, WHICH IS KNOWN FOR HIGH ACTIVITY AND POLYARTICULAR OR GENERALIZED JOINT LESION, WAS LINKED TO SIGNIFICANT PHYSICAL RETARDATION IN ALL CHILDREN WITH SYSBTEMIC JIA. LONGBTERM GLUCOCORTICOID ADMINISTRATION, EVEN AT LOW DOSES, ACCENTUATED THE NEGATIVE IMPACT OF JIA ON GROWTH RESULTING IN MARKED GROWTH DELAY OR ITS' TOTAL ARREST. WE HAVE ELABORATED RISK FACTORS FOR SHORT STATURE IN CHILDREN WITH JIA, WHICH SUGGEST THERAPY GUIDELINES, PRESENTED IN THIS ARTICLE.KEY WORDS: JUVENILE IDIOPATHIC ARTHRITIS, СHILDREN.

  1. Comparison of clinical and serological differences among juvenile-, adult-, and late-onset systemic lupus erythematosus in Korean patients.

    Science.gov (United States)

    Choi, J H; Park, D J; Kang, J H; Yim, Y R; Lee, K E; Lee, J W; Wen, L; Kim, T J; Park, Y W; Lee, J K; Lee, S S

    2015-10-01

    We investigated whether systemic lupus erythematosus (SLE) patients could be distinguished based on the time of disease onset and, if so, whether the groups differed in their clinical and laboratory features in ethnically homogeneous Korean patients. We enrolled 201 SLE patients with available clinical data at the time of onset of SLE from the lupus cohort at Chonnam National University Hospital. Sociodemographic, clinical, and laboratory data, including autoantibodies, and concomitant diseases were found at the time of diagnosis of SLE by reviewing patient charts. We divided SLE patients according to age at SLE diagnosis into three groups: juvenile-onset SLE (JSLE, diagnosed at ≤ 18 years), adult-onset SLE (ASLE, diagnosed at 19-50 years), and late-onset SLE (LSLE, diagnosed at >50 years), and compared baseline demographic, clinical, and relevant laboratory findings. Of the 201 patients, 27 (14.4%), 149 (74.1%), and 25 (12.4%) were JSLE, ASLE, and LSLE patients, respectively. Fever, oral ulcers, nephritis, anemia, and thrombocytopenia were more common in JSLE patients than ASLE or LSLE patients (p manifestations according to age at disease onset. JSLE patients have more severe disease activity and more frequent renal involvement and LSLE patients have milder disease activity, more commonly accompanied by Sjögren's syndrome, at disease onset. © The Author(s) 2015.

  2. Urinary interleukin 22 binding protein as a marker of lupus nephritis in Egyptian children with juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Badr, Ahmed Mohamed Mahmoud; Farag, Yomna; Abdelshafy, Maie; Riad, Nermine Magdi

    2018-02-01

    Juvenile systemic lupus erythematosus (JSLE) is a multi-system autoimmune inflammatory disease. Generally, 60% of patients will develop lupus nephritis (LN); thus, early recognition and treatment is associated with better outcome. Interleukin 22 (IL-22) is involved in tissue inflammation and is regulated by interleukin 22 binding protein (IL-22BP). This study aimed to use IL-22BP as a non-invasive marker for disease activity in JSLE and LN. This is a cross-sectional study conducted on 82 subjects: 51 JSLE patients and 31 healthy controls of matched age and gender. Urinary IL-22BP was measured using enzyme-linked immunosorbent assay, and its level was correlated with different clinical and laboratory data in JSLE as well as Systemic Lupus Erythematous Disease Activity Index 2000 (SLEDAI-2k), renal SLEDAI-2k, and Systemic Lupus International Collaborating Clinics (SLICC) renal activity score which were used to assess overall disease and renal activity. Our results showed that urinary IL-22BP level was significantly higher in JSLE patients with mean level of 4.13 ± 1.10, as compared to controls 1.63 ± 0.61 (P value < 0.001); also, patients with active LN had urinary levels of IL-22BP (5.47 ± 1.03) higher than patients with active JSLE without LN (4.23 ± 0.72) and patients with non-active JSLE/LN (3.5 ± 0.65) with a highly significant P value < 0.001. There was a positive correlation with SLEDAI-2k, renal SLEDAI, and renal activity scores (P < 0.001). Urinary IL-22BP may be used as a non-invasive marker for assessment of disease activity in children with JSLE and LN.

  3. Decreased serotonin level during pregnancy alters morphological and functional characteristics of tonic nociceptive system in juvenile offspring of the rat

    Directory of Open Access Journals (Sweden)

    Mikhailenko Victor A

    2003-11-01

    Full Text Available Abstract Serotonin (5-HT contributes to the prenatal development of the central nervous system, acting as a morphogen in the young embryo and later as a neurotransmitter. This biologically active agent influences both morphological and biochemical differentiation of raphe neurons, which give rise to the descending serotonergic paths that regulate the processing of acutely evoked nociceptive inputs. The involvement of 5-HT in the prenatal development of tonic nociceptive system has not been studied. In the present study we evaluated the effects of a single injection (400 mg/kg, 2 ml, i.p. of the 5-HT synthesis inhibitor, para-chlorophenylalanine (pCPA, given to pregnant rats during the critical period fetal serotonin development. The functional integrity of the tonic nociceptive response was investigated in 25 day old rats using the classic formalin test. Morphological analysis of brain structures involved in formalin-induced pain and 5-HT levels in the heads of 12-day embryos were also evaluated. Embryonic levels of 5-HT were significantly lowered by the treatment. The juvenile rats from pCPA-treated females showed altered brain morphology and cell differentiation in the developing cortex, hippocampus, raphe nuclei, and substantia nigra. In the formalin test, there were significant decreases in the intensity and duration of the second phase of the formalin-induced response, characterizing persistent, tonic pain. The extent of impairments in the brain structures correlated positively with the level of decrease in the behavioral responses. The data demonstrate the involvement of 5-HT in the prenatal development of the tonic nociceptive system. The decreased tonic component of the behavioral response can be explained by lower activity of the descending excitatory serotonergic system originating in the raphe nuclei, resulting in decreased tonic pain processing organized at the level of the dorsal horn of the spinal cord.

  4. Systemic Mastocytosis Associated with Chronic Idiopathic Myelofibrosis: A Distinct Subtype of Systemic Mastocytosis-Associated Clonal Hematological Nonmast Cell Lineage Disorder Carrying the Activating Point Mutations KITD816V and JAK2V617F

    OpenAIRE

    Sotlar, Karl; Bache, Anja; Stellmacher, Florian; Bültmann, Burkhard; Valent, Peter; Horny, Hans-Peter

    2008-01-01

    In ∼20 to 30% of patients with systemic mastocytosis (SM), an associated clonal hematological nonmast cell lineage disorder (AHNMD) is diagnosed. Although SM may be considered to be closely related to the myeloproliferative disorders (MPDs), it is unknown whether JAK2V617F+ MPD may occur as AHNMD in patients with SM. We here describe five patients with SM and co-existing chronic idiopathic myelofibrosis (SM-CIMF). In five of five patients, we detected the SM-related KIT mutation D816V, and in...

  5. Understanding the role of the immune system in adolescent idiopathic scoliosis: Immunometabolic CONnections to Scoliosis (ICONS) study protocol

    Science.gov (United States)

    Samaan, M Constantine; Missiuna, Paul; Peterson, Devin; Thabane, Lehana

    2016-01-01

    Introduction Adolescent idiopathic scoliosis (AIS) affects up to 3% of children around the world. There is limited knowledge of AIS aetiopathogenesis, and this evidence is needed to develop new management strategies. Paraspinal muscle in AIS demonstrates evidence of differential fibrosis based on curve sidedness. Fibrosis is the hallmark of macrophage-driven inflammation and tissue remodelling, yet the mechanisms of fibrosis in paraspinal muscle in AIS are poorly understood. Objectives The primary objective of this study is to determine the influence of curve sidedness on paraspinal muscle inflammation. Secondary objectives include defining the mechanisms of macrophage homing to muscle, and determining muscle–macrophage crosstalk in muscle fibrosis in AIS. Methods and analysis This is a cross-sectional study conducted in a tertiary paediatric centre in Hamilton, Ontario, Canada. We will recruit boys and girls, 10–17 years of age, who are having surgery to correct AIS. We will exclude children who have an active infection or are on immunosuppressive therapies within 2 weeks of surgery, smokers and pregnant girls. Paraspinal muscle biopsies will be obtained at the start of surgery. Also, blood and urine samples will be collected from participants, who will fill questionnaires about their lifestyle. Anthropometric measures will also be collected including height, weight, waist and hip circumferences. Ethics and dissemination This study has received ethics authorisation by the institutional review board. This work will be published in peer-reviewed journals and will be presented in oral and poster formats at scientific meetings. Discussion This study will explore the mechanisms of paraspinal muscle inflammation, remodelling and fibrosis in AIS. This will help identify pathways and molecules as potential therapeutic targets to treat and prevent AIS. It may also yield markers that predict scoliosis progression and response to treatment in these children. PMID

  6. Understanding the role of the immune system in adolescent idiopathic scoliosis: Immunometabolic CONnections to Scoliosis (ICONS) study protocol.

    Science.gov (United States)

    Samaan, M Constantine; Missiuna, Paul; Peterson, Devin; Thabane, Lehana

    2016-07-08

    Adolescent idiopathic scoliosis (AIS) affects up to 3% of children around the world. There is limited knowledge of AIS aetiopathogenesis, and this evidence is needed to develop new management strategies. Paraspinal muscle in AIS demonstrates evidence of differential fibrosis based on curve sidedness. Fibrosis is the hallmark of macrophage-driven inflammation and tissue remodelling, yet the mechanisms of fibrosis in paraspinal muscle in AIS are poorly understood. The primary objective of this study is to determine the influence of curve sidedness on paraspinal muscle inflammation. Secondary objectives include defining the mechanisms of macrophage homing to muscle, and determining muscle-macrophage crosstalk in muscle fibrosis in AIS. This is a cross-sectional study conducted in a tertiary paediatric centre in Hamilton, Ontario, Canada. We will recruit boys and girls, 10-17 years of age, who are having surgery to correct AIS. We will exclude children who have an active infection or are on immunosuppressive therapies within 2 weeks of surgery, smokers and pregnant girls. Paraspinal muscle biopsies will be obtained at the start of surgery. Also, blood and urine samples will be collected from participants, who will fill questionnaires about their lifestyle. Anthropometric measures will also be collected including height, weight, waist and hip circumferences. This study has received ethics authorisation by the institutional review board. This work will be published in peer-reviewed journals and will be presented in oral and poster formats at scientific meetings. This study will explore the mechanisms of paraspinal muscle inflammation, remodelling and fibrosis in AIS. This will help identify pathways and molecules as potential therapeutic targets to treat and prevent AIS. It may also yield markers that predict scoliosis progression and response to treatment in these children. Published by the BMJ Publishing Group Limited. For permission to use (where not already

  7. Seasonal idiopathic rhinitis with local inflammatory response and specific IgE in absence of systemic response.

    Science.gov (United States)

    Rondón, C; Doña, I; López, S; Campo, P; Romero, J J; Torres, M J; Mayorga, C; Blanca, M

    2008-10-01

    Patients with idiopathic rhinitis (IR) are considered to be nonallergic because they have a negative skin prick test (SPT) and allergen specific-IgE in serum. The concept of localized mucosal allergy in the absence of atopy has recently been proposed. The immunological mechanisms involved in seasonal IR have not been sufficiently studied. We examined nasal mucosa inflammation, the presence of nasal specific-IgE and the response to nasal allergen provocation test (NAPT) in patients with seasonal IR who presented symptoms only in spring. We evaluated 32 patients with seasonal IR and 35 with persistent allergic rhinitis to pollen (PAR-P) and compared these with healthy controls and persons with PAR to house dust mite during the pollen season, as well as by NAPT out-of-season with grass and Olea europea. We measured the nasal leukocyte-lymphocyte phenotype (CD45, CD33, CD16, CD3, CD4 and CD8), eosinophil-cationic-protein, and total and specific-IgE to grass and olive pollen in serum and nasal lavage and performed NAPT. In the IR group, 62.5% had a positive NAPT (IR-PosNAPT), 20/32 to grass, with four of these having a positive NAPT to olive pollen as well. IR-PosNAPT patients showed a similar nasal leukocyte-lymphocyte profile to the PAR-P patients and different to controls. We detected nasal specific-IgE in 35% of IR-PosNAPT patients. These results support the hypothesis that a subgroup of patients with IR have seasonal symptoms with evidence of a nasal allergic immune reaction in the absence of a positive SPT or serum specific IgE.

  8. Forensic aspects of juvenile violence.

    Science.gov (United States)

    Haller, L H

    2000-10-01

    The juvenile justice system was created because it was recognized that youthful offenders needed to be managed differently from adults. They were to receive habilitation services instead of punishment. It is now more than a century since the creation of the first juvenile court. After 67 years, the US Supreme Court, in Kent v United States stated that the model was not working because juveniles in the criminal justice system received no treatment and they had no rights. Because the issue that had been appealed was the lack of rights (not lack of treatment), the Court mandated that juveniles, like adults, be given certain rights. The following year, in In re Gault, the Court expanded these rights. Subsequent Supreme Court cases have dealt with these kinds of issues--that is, whether juvenile offenders are entitled to the same rights as adults and subject to the same penalties. The Supreme Court has never heard a "right to treatment" case, which is the other part of the juvenile court system. Cases have been brought in lower courts (e.g., Nelson v. Heyne, 1972) alleging inadequate treatment services, but no national impact has resulted. Thus, in general, children in the juvenile court system do not have an enforceable right to treatment and can obtain only what services are available in their jurisdictions. The services often are woefully inadequate. Sentencing a youth to probation, with the requirement that he or she participate in counseling or mental health treatment, is meaningless if services are not available. Community-based, model programs that provide effective treatment do exist. They are, as yet, the rare exception rather than the norm and, therefore, are not available to most youthful offenders. Incarcerated juveniles, obviously, cannot avail themselves of community programs. Litigation to give these youth the same rights as adults in penal institutions is not the answer because incarcerated adults don't have a right to treatment, only a right to be free

  9. Immunology of idiopathic nephrotic syndrome.

    Science.gov (United States)

    Colucci, Manuela; Corpetti, Giorgia; Emma, Francesco; Vivarelli, Marina

    2018-04-01

    The pathogenesis of idiopathic nephrotic syndrome (INS) is as yet unknown, but several lines of evidence indicate that the immune system may play a crucial pathogenic role in non-genetic INS. The most important of these are, first, the effectiveness of therapy based on immunosuppression and, second, a vast body of data derived both from experimental models and from patient studies that implicate T cells and more recently B cells as major players in INS pathogenesis. However, recent findings also suggest a direct role of podocytes as drivers of the disease process, and the interplay between the glomerulus and the immune system is still being elucidated. In this review we provide an overview of current knowledge on the role of different components of the immune system in determining disease. Advances in our understanding of the pathogenesis of INS may help drive new, more tailored therapeutic approaches.

  10. SYSTEMIC ADMINISTRATION OF BORDETELLA PERTUSSIS ENHANCES PULMONARY SENSITIZATION TO HOUSE DUST MITE IN JUVENILE RATS

    Science.gov (United States)

    The incidence of allergies and asthma has increased significantly in the past few decades. The objectives of this study were to establish an allergy model in weanling rats to more closely reflect the developing immune system of children, and to determine whether systemic administ...

  11. Genetics Home Reference: idiopathic pulmonary fibrosis

    Science.gov (United States)

    ... Twitter Home Health Conditions Idiopathic pulmonary fibrosis Idiopathic pulmonary fibrosis Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Idiopathic pulmonary fibrosis is a chronic, progressive lung disease. This condition ...

  12. Genetics Home Reference: adolescent idiopathic scoliosis

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions Adolescent idiopathic scoliosis Adolescent idiopathic scoliosis Printable PDF Open All Close All ... Javascript to view the expand/collapse boxes. Description Adolescent idiopathic scoliosis is an abnormal curvature of the ...

  13. Clinical patterns of juvenile idiopathic arthritis: A single tertiary ...

    African Journals Online (AJOL)

    NSAIDs were used in all the patients. NSAIDS were combined with corticosteroids in 38/68 (55.9%) patients while NSAIDs, corticosteroids and methotrexate were used in 16/68 (23.5%) patients and biologics were received by 6/68 (8.8%) patients at different and varying length of time. Conclusion: This is the first study of JIA ...

  14. Juvenile idiopathic arthritis – an update on its diagnosis and ...

    African Journals Online (AJOL)

    2015-12-03

    Dec 3, 2015 ... Administration. Common side-effects Precautions. Monitoring. TNF inhibitors Etanercept. Adalimumab. Infliximab. Golimumab. Etanercept S/C once weekly. Adalimumab S/C every. 2 weeks. Infliximab IV 2 - 8-weekly. Golimumab S/C monthly. Hypersensitivity infusion reactions. For all biologics: omit biologic.

  15. Non-HLA gene polymorphisms in juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Alberdi-Saugstrup, M.; Enevold, C.; Zak, M.

    2017-01-01

    were disease remission, actively inflamed joints and joints with limitation of motion (LOM), articular or extra-articular damage, and history of uveitis. Results: Evidence of associations between genotypes and endpoints were found for STAT4, ADAD1-IL2-IL21, PTPN2, and VTCN1 (p = 0.003–0.05). STAT4_rs......7574865 TT was associated with the presence of actively inflamed joints [odds ratio (OR) 20.6, 95% confidence interval (CI) 2.2–> 100, p = 0.003] and extra-articular damage (OR 7.9, 95% CI 1–56.6, p = 0.057). ADAD1_rs17388568 AA was associated with a lower risk of having joints with LOM (OR 0.1, 95% CI 0...

  16. Plasma TNF binding capacity in patients with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Bjørnhart, Birgitte; Svenningsen, Pernille; Gudbrandsdottir, Sif

    2005-01-01

    Tumour necrosis factor (TNF)-alpha and TNF-beta, also called lymphotoxin (LT), are bound by soluble truncated TNF receptors (sTNFRI and II) that are released from cell surfaces and act as natural inhibitors of TNF-induced inflammation. We investigated the plasma levels of sTNFRI and II in parallel...

  17. Idiopathic inflammatory myopathies and the lung

    Directory of Open Access Journals (Sweden)

    Jean-Christophe Lega

    2015-06-01

    Full Text Available Idiopathic inflammatory myositis (IIM is a group of rare connective tissue diseases (CTDs characterised by muscular and extramuscular signs, in which lung involvement is a challenging issue. Interstitial lung disease (ILD is the hallmark of pulmonary involvement in IIM, and causes morbidity and mortality, resulting in an estimated excess mortality of 50% in some series. Except for inclusion body myositis, these extrapulmonary disorders are associated with the general and visceral involvement frequently found in other CTDs including fever, Raynaud's phenomenon, arthralgia, nonspecific cutaneous modifications and ILD, for which the prevalence is estimated to be up to 65%. Substantial heterogeneity exists within the spectrum of IIMs, and each condition is associated with various frequencies and subtypes of pulmonary involvement. This heterogeneity is partly related to the presence of various autoantibodies encompassing anti-synthetase, anti-MDA5 and anti-PM/Scl. ILD is present in all subsets of IIM including juvenile myositis, but is more frequent in dermatomyositis and overlap myositis. IIM can also be associated with other presentations of respiratory involvement, namely pulmonary arterial hypertension, pleural disease, infections, drug-induced toxicity, malignancy and respiratory muscle weakness. Here, we critically review the current knowledge about adult and juvenile myositis-associated lung disease with a detailed description of therapeutics for chronic and rapidly progressive ILD.

  18. Parenting and juvenile delinquency

    NARCIS (Netherlands)

    Hoeve, M.

    2008-01-01

    Juvenile delinquency is a noteworthy problem. This thesis addressed the association between parenting and juvenile delinquency by analyzing the concepts of parenting adopted in family research in relation to criminological concepts and measures of delinquent behavior. Four studies were conducted.

  19. Acute Idiopathic Scrotal Edema

    Directory of Open Access Journals (Sweden)

    Micheál Breen

    2013-01-01

    Full Text Available We report a case of acute idiopathic scrotal edema (AISE in a 4-year-old boy who presented with acute scrotal pain and erythema. The clinical features, ultrasound appearance, and natural history of this rare diagnosis are reviewed. In this report, we highlight the importance of good ultrasound technique in differentiating the etiology of the acute scrotum and demonstrate the color Doppler “Fountain Sign” that is highly suggestive of AISE.

  20. “Denervation” of autonomous nervous system in idiopathic pulmonary arterial hypertension by low-dose radiation: a case report with an unexpected outcome

    Directory of Open Access Journals (Sweden)

    Hohenforst-Schmidt W

    2014-03-01

    Full Text Available Wolfgang Hohenforst-Schmidt,1 Paul Zarogoulidis,2 Filiz Oezkan,3 Christian Mahnkopf,4 Gerhard Grabenbauer,5 Alfons Kreczy,6 Rolf Bartunek,7 Kaid Darwiche,3 Lutz Freitag,3 Qiang Li,8 Haidong Huang,8 Thomas Vogl,9 Patrick LePilvert,10 Theodora Tsiouda,11 Kosmas Tsakiridis,12 Konstantinos Zarogoulidis,2 Johannes Brachmann11II Medical Clinic, Coburg Clinic, University of Würzburg, Coburg, Germany; 2Pulmonary Department-Oncology Unit, G Papanikolaou General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece; 3Department of Interventional Pneumology, Ruhrlandklinik, West German Lung Center, University Hospital, University of Duisburg-Essen, Essen, Germany; 4II Medizinische Klinik, Klinik für Kardiologie, Angiologie, Pneumologie, Klinikum Coburg, 5Department of Radiotherapy, 6Department of Pathology, Cytology and Molecular Diagnostics, 7Institute of Diagnostic and Interventional Radiology, Coburg Clinic, University of Wüerzburg, Coburg, Germany; 8Department of Respiratory Diseases, Changhai Hospital/First Affiliated Hospital of the Second Military Medical University, Shanghai, People’s Republic of China; 9Department of Diagnostic and Interventional Radiology, Goethe University of Frankfurt, Frankfurt, Germany; 10Interventional Drug Delivery Systems and Strategies (ID2S2, Medical Cryogenics, Lakeland Court Jupiter, FL, USA; 11Internal Medicine Unit, Theagenio Cancer Hospital, Thessaloniki, 12Cardiothoracic Surgery Department, Saint Luke Private Hospital, Thessaloniki, GreeceAbstract: Vasointestinal peptide metabolism plays a key physiological role in multimodular levels of vasodilatory, smooth muscle cell proliferative, parenchymal, and inflammatory lung reactions. In animal studies, vasointestinal peptide relaxes isolated pulmonary arterial segments from several mammalian species in vitro and neutralizes the pulmonary vasoconstrictor effect of endothelin. In some animal models, it reduces pulmonary vascular resistance in vivo and

  1. Challenges and prospects of the juvenile justice administration in ...

    African Journals Online (AJOL)

    Juvenile justice administration in Nigeria is weak and has been given very little priority, despite Nigeria being signatory to the major international instruments relevant to the administration of juvenile justice. This is attributable to the history of the penal system of Nigeria, with laws guiding juvenile justice administration having ...

  2. Idiopathic Inflammatory Myopathies: An update

    Directory of Open Access Journals (Sweden)

    Bulent KURT

    2016-06-01

    Full Text Available Idiopathic inflammatory myopathies (IIM are a heterogeneous group of disease with complex clinical features. It has been sub-classified as: (1 Dermatomyositis, (2 Polymyositis, and (3 Inclusion body myositis (IBM. Nowadays, there are some studies in literature suggest necrotizing autoimmune myopathy and immune-mediated necrotizing myopathy should also be added to this group of disease. There is a debate in the diagnosis of IIMs and up until now, about 12 criteria systems have been proposed. Some of the criteria systems have been used widely such as Griggs et al.'s proposal for IBM. Clinical findings, autoantibodies, enzymes, electrophysiological, and muscle biopsy findings are diagnostic tools. Because of diseases' complexity, none of the findings are diagnostic alone. In this study, we discussed the diagnostic criteria of IMMs and described detailed morphological features. [J Interdiscipl Histopathol 2016; 4(2.000: 41-45

  3. Juvenile Arrests, 2000. Juvenile Justice Bulletin.

    Science.gov (United States)

    Snyder, Howard N.

    This bulletin examines the national and state juvenile arrest rate in 2000 using data reported annually by local law enforcement agencies nationwide to the FBI's Uniform Crime Reporting program. Results indicate that the murder rate in 2000 was the lowest since 1965; juvenile arrests for violence in 2000 were the lowest since 1988; few juveniles…

  4. Professional Development: A Capacity-Building Model for Juvenile Correctional Education Systems

    Science.gov (United States)

    Mathur, Sarup R.; Clark, Heather Griller; Schoenfeld, Naomi A.

    2009-01-01

    Youth in correctional facilities experience a broad range of educational, psychological, medical, and social needs. Professional development, a systemic process that improves the likelihood of student success by enhancing educator abilities, is a powerful way to positively affect student outcomes in correctional settings. This article offers a…

  5. Concepts Shaping Juvenile Justice

    Science.gov (United States)

    White, Rob

    2008-01-01

    Rob White's paper explores ways in which community building can be integrated into the practices of juvenile justice work. He provides a model of what can be called "restorative social justice", one that builds upon the juvenile conferencing model by attempting to fuse social justice concerns with progressive juvenile justice practices.

  6. "How I treat" autoimmune diseases: State of the art on the management of rare rheumatic diseases and ANCA-associated systemic idiopathic vasculitis.

    Science.gov (United States)

    Roccatello, Dario

    2017-10-01

    This Special Issue of Autoimmunity Reviews constitutes summaries of presentations at the 20th International Meeting on Immunopathology and Orphan Diseases, held in Torino, Italy, 25-28th January 2017. As such, these presentations represent the state of the art on the pathophysiology of autoimmune diseases as well as the most recent insights into the management of these pathologic conditions. The latter includes both the optimal use of established drugs and approaches as well as novel knowledge on the means and consequences of targeted blocking of molecules or cellular mechanisms. The 2nd Turin Congress on systemic idiopathic vasculitis concluded the works of the International Meeting on Immune Pathology and Orphan Diseases. This Satellite Congress was mainly addressed to the management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitis: advances on induction therapy and maintenance treatment. Guidelines and recommendations were critically discussed, reviewing available evidence and providing experts' insights. New intensive therapeutic approaches had been also reported. Copyright © 2017 Elsevier B.V. All rights reserved.

  7. Juvenile Residential Facility Census, 2010: Selected Findings. Juvenile Offenders and Victims: National Report Series. Bulletin NCJ 241134

    Science.gov (United States)

    Hockenberry, Sarah; Sickmund, Melissa; Sladky, Anthony

    2013-01-01

    This bulletin is part of the "Juvenile Offenders and Victims National Report Series." The "National Report" offers a comprehensive statistical overview of the problems of juvenile crime, violence, and victimization and the response of the juvenile justice system. During each interim year, the bulletins in the "National…

  8. JUVENILE-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS: CLINICAL FEATURES AND CURRENT DIAGNOSTIC APPROACHES

    Directory of Open Access Journals (Sweden)

    M. I. Kaleda

    2017-01-01

    Full Text Available Systemic lupus erythematosus (SLE is an autoimmune disease with various organs and systems involved in the pathological process, with an unpredictable course, a risk for life-threatening conditions, and an onset from childhood through adolescence in 10–20% of cases. The onset of SLE prior to adult age affects its clinical manifestations, course, and prognosis. Based on the data available in the literature, the paper analyzes the features of the onset and clinical presentations of SLE in children and adolescents and discusses the aspects of verifying its diagnosis, by taking into account the need to use early effective personalized therapy and to improve prognosis.

  9. Clinical characteristics of children with Juvenile Systemic Sclerosis: follow-up of 23 patients in a single tertiary center

    Directory of Open Access Journals (Sweden)

    Katsicas María M

    2007-05-01

    Full Text Available Abstract Background Juvenile systemic sclerosis (JSS is a multisystem connective tissue disease characterized by skin fibrosis and internal organ involvement. It has a low prevalence, even in a tertiary facility setting. The purpose of the present study is to describe and analyze the clinical and laboratory characteristics of a group of children with JSS followed in a single center. Methods Clinical charts of children with a diagnosis of JSS who were seen at a tertiary referral center between 1995 and 2005 were reviewed. Clinical features were recorded and analysed. Results Twenty-three patients who met preliminary classification criteria for JSS were included. Age at first symptom attributable to JSS was 6 (1–14 years, The first symptom attributable to JSS was Raynaud's phenomenon in 14 cases. Proximal sclerosis (23 patients, 100%, sclerodactyly (21, 91%, Raynaud's phenomenon (19, 83%, and periungual capillaropathy (17, 74% were the most consistent clinical findings during follow-up. Respiratory involvement occurred in two thirds of our patients, and it manifested as dyspnea as well as abnormal imaging and/or pulmonary function tests; pulmonary hypertension was an infrequent finding. Dysphagia was the commonest gastrointestinal symptom (9 patients, 39%. The most frequent musculoskeletal symptom was arthralgia (14 children, 6%; symmetrical arthritis was found in 8 (35% patients. Periungual capillary abnormalities were evident during physical examination in 17 children; capillaroscopy revealed abnormalities in all 19 examined patients. ANA were present in 17 (74% children: homogeneous pattern was the most frequent (8 patients, nucleolar (5 and speckled (4 were less common. Conclusion Raynaud's phenomenon heralds the beginning of the disease. Capilaroscopy is a major adjuvant in the diagnosis, since autoantibody determination may not offer sensitive and specific markers. Skin and vascular manifestations are the most common clinical features

  10. The effect of carnitine on ketogenesis in perfused livers from juvenile visceral steatosis mice with systemic carnitine deficiency.

    Science.gov (United States)

    Nakajima, T; Horiuchi, M; Yamanaka, H; Kizaki, Z; Inoue, F; Kodo, N; Kinugasa, A; Saheki, T; Sawada, T

    1997-07-01

    Juvenile visceral steatosis (JVS) mice have been reported to have systemic carnitine deficiency, and the carnitine concentration in the liver of JVS mice was markedly lower than that of controls (11.6 +/- 2.6 versus 393.5 +/- 56.4 nmol/g of wet liver). To evaluate the role of carnitine in mitochondrial beta-oxidation in liver, we examined the effects of carnitine on ketogenesis in perfused liver from control and JVS mice. In control mice, ketogenesis was increased by the infusion of 0.3 mM oleate, but not by L-carnitine. In contrast, although ketogenesis in JVS mice was not increased by the infusion of oleate, it was increased 2.5-fold by the addition of 1000 microM L-carnitine. Addition of 50, 100, and 200 microM L-carnitine increased ketogenesis in a dose-dependent manner. The infusion of 0.3 mM octanoate or butyrate increased ketogenesis in a carnitine-independent fashion in both control and JVS mice. These findings suggest that endogenous long chain fatty acids from accumulated triglycerides may be used as substrates in the presence of carnitine in JVS mice. The relationship between ketogenesis and free carnitine concentration was examined in livers from JVS mice. Ketogenesis increased as free carnitine levels increased until concentrations exceeded about 100 nmol/g of wet liver (340 microM). The free carnitine concentration required for half-maximal ketone body production in liver of JVS mice was 45 microM (13 nmol/g of wet liver), which corresponds to a K(m) value of carnitine palmitoyltransferase I. We conclude that carnitine is a rate-limiting factor for beta-oxidation in liver only when the carnitine level in liver is very low.

  11. Predictors of emotional functioning in youth after surgical correction of idiopathic scoliosis.

    Science.gov (United States)

    Zebracki, Kathy; Thawrani, Dinesh; Oswald, Timothy S; Anadio, Jennifer M; Sturm, Peter F

    2013-09-01

    Patients with idiopathic scoliosis, although otherwise healthy, often have significant concerns about their self-image and appearance. In a group of juveniles and adolescents, this can impact adjustment in school, functioning in peer groups, and general sense of well-being. There are limited data to help physicians reliably and precisely identify those who are at higher risk of poor emotional adjustment even after spine deformity correction. The purpose of this study was to determine the predictors of emotional maladjustment in juvenile and adolescent patients after surgical correction of idiopathic scoliosis. A total of 233 juveniles, mean age 11.26 ± 1.02 (range, 8 to 12) years and 909 adolescents, mean age 14.91 ± 1.61 (range, 13 to 21) years, who underwent surgical correction for idiopathic scoliosis and who were participating in a prospective longitudinal multicenter database, were enrolled in the study. Participants completed the Scoliosis Research Society-22 (SRS-22) questionnaire before surgery and 2 years postoperatively. Radiographs were used to measure Cobb angle and surface measurements were used to determine decompensation and trunk shift. Adolescents reported poorer mental health preoperatively (Pself-image (Pself-image (Padolescent group, mental health 2 years postoperatively was predicted by preoperative mental health (Pself-image 2 years postoperatively was predicted by preoperative mental health (Pself-image (PSelf-image and mental health are significantly improved after spine deformity correction in juveniles and adolescents with idiopathic scoliosis. However, consistent with normative development, adolescents are at higher risk for emotional maladjustment than juveniles. Surgical decision making in scoliosis correction should take the emotional status of the patient into consideration.

  12. Evolution of Juvenile Ankylosing Spondylitis

    Directory of Open Access Journals (Sweden)

    Ye.V. Prohorov

    2013-02-01

    Full Text Available Evolution of juvenile ankylosing spondylitis tend to follow a more frequent involvement in the pathological process of elbow and ankle joints, development of enthesiopathies, changes of intraarticular meniscal horns, forming of Baker’s cysts, cartilage flaps and systemic osteoporosis, and total value of all these signs 13 times exceeds thereof in patients with with the debut of disease in adulthood, but for juvenile ankylosing spondylitis vertebral lesion is less common. Age dimorphism of the use of certain groups of drugs and physiotherapy facilities is observed.

  13. Central Nervous System Idiopathic Inflammatory Demyelinating Disorders in South Americans: A Descriptive, Multicenter, Cross-Sectional Study.

    Science.gov (United States)

    Papais-Alvarenga, Regina Maria; Vasconcelos, Claudia Cristina Ferreira; Carra, Adriana; de Castillo, Ibis Soto; Florentin, Sara; Diaz de Bedoya, Fernando Hamuy; Mandler, Raul; de Siervi, Luiza Campanella; Pimentel, Maria Lúcia Vellutini; Alvarenga, Marina Papais; Alvarenga, Marcos Papais; Grzesiuk, Anderson Kuntz; Gama Pereira, Ana Beatriz Calmon; Gomes Neto, Antonio Pereira; Velasquez, Carolina; Soublette, Carlos; Fleitas, Cynthia Veronica; Diniz, Denise Sisteroli; Armas, Elizabeth; Batista, Elizabeth; Hernandez, Freda; Pereira, Fernanda Ferreira Chaves da Costa; Siqueira, Heloise Helena; Cabeça, Hideraldo; Sanchez, Jose; Brooks, Joseph Bruno Bidin; Gonçalves, Marcus Vinicius; Barroso, Maria Cristina Del Negro; Ravelo, Maria Elena; Castillo, Maria Carlota; Ferreira, Maria Lúcia Brito; Rocha, Maria Sheila Guimarães; Parolin, Monica Koncke Fiuza; Molina, Omaira; Marinho, Patricia Beatriz Christino; Christo, Paulo Pereira; Brant de Souza, Renata; Pessanha Neto, Silvio; Camargo, Solange Maria das Graças; Machado, Suzana Costa; Neri, Vanderson Carvalho; Fragoso, Yara Dadalti; Alvarenga, Helcio; Thuler, Luiz Claudio Santos

    2015-01-01

    The idiopathic inflammatory demyelinating disease (IIDD) spectrum has been investigated among different populations, and the results have indicated a low relative frequency of neuromyelitis optica (NMO) among multiple sclerosis (MS) cases in whites (1.2%-1.5%), increasing in Mestizos (8%) and Africans (15.4%-27.5%) living in areas of low MS prevalence. South America (SA) was colonized by Europeans from the Iberian Peninsula, and their miscegenation with natives and Africans slaves resulted in significant racial mixing. The current study analyzed the IIDD spectrum in SA after accounting for the ethnic heterogeneity of its population. A cross-sectional multicenter study was performed. Only individuals followed in 2011 with a confirmed diagnosis of IIDD using new diagnostic criteria were considered eligible. Patients' demographic, clinical and laboratory data were collected. In all, 1,917 individuals from 22 MS centers were included (73.7% female, 63.0% white, 28.0% African, 7.0% Mestizo, and 0.2% Asian). The main disease categories and their associated frequencies were MS (76.9%), NMO (11.8%), other NMO syndromes (6.5%), CIS (3.5%), ADEM (1.0%), and acute encephalopathy (0.4%). Females predominated in all main categories. The white ethnicity also predominated, except in NMO. Except in ADEM, the disease onset occurred between 20 and 39 years old, early onset in 8.2% of all cases, and late onset occurred in 8.9%. The long-term morbidity after a mean disease time of 9.28±7.7 years was characterized by mild disability in all categories except in NMO, which was scored as moderate. Disease time among those with MS was positively correlated with the expanded disability status scale (EDSS) score (r=0.374; p=<0.001). This correlation was not observed in people with NMO or those with other NMO spectrum disorders (NMOSDs). Among patients with NMO, 83.2% showed a relapsing-remitting course, and 16.8% showed a monophasic course. The NMO-IgG antibody tested using indirect

  14. Central Nervous System Idiopathic Inflammatory Demyelinating Disorders in South Americans: A Descriptive, Multicenter, Cross-Sectional Study.

    Directory of Open Access Journals (Sweden)

    Regina Maria Papais-Alvarenga

    Full Text Available The idiopathic inflammatory demyelinating disease (IIDD spectrum has been investigated among different populations, and the results have indicated a low relative frequency of neuromyelitis optica (NMO among multiple sclerosis (MS cases in whites (1.2%-1.5%, increasing in Mestizos (8% and Africans (15.4%-27.5% living in areas of low MS prevalence. South America (SA was colonized by Europeans from the Iberian Peninsula, and their miscegenation with natives and Africans slaves resulted in significant racial mixing. The current study analyzed the IIDD spectrum in SA after accounting for the ethnic heterogeneity of its population. A cross-sectional multicenter study was performed. Only individuals followed in 2011 with a confirmed diagnosis of IIDD using new diagnostic criteria were considered eligible. Patients' demographic, clinical and laboratory data were collected. In all, 1,917 individuals from 22 MS centers were included (73.7% female, 63.0% white, 28.0% African, 7.0% Mestizo, and 0.2% Asian. The main disease categories and their associated frequencies were MS (76.9%, NMO (11.8%, other NMO syndromes (6.5%, CIS (3.5%, ADEM (1.0%, and acute encephalopathy (0.4%. Females predominated in all main categories. The white ethnicity also predominated, except