Serum 25-hydroxyvitamin D and biochemical markers of bone metabolism in patients with juvenile idiopathic arthritis

Our objective was to evaluate the concentrations of serum 25-hydroxyvitamin D [25(OH)D], serum calcium, serum phosphorus, alkaline phosphatase, and parathormone (PTH) in patients with polyarticular juvenile idiopathic arthritis (JIA) and to associate them with disease duration and activity, bone mineral density and use of medications. in a cross-sectional and controlled study, 30 patients with polyarticular JIA were evaluated and compared to 30 healthy individuals matched for age and gender. Clinical status, anthropometry, laboratory markers in both patients and controls, and bone mineral density, only in the patients, were measured. of the 30 patients included in the study, 23 (76.7%) were female and 16 (53.3%) non-Caucasian; mean age was 14 years (range = 4 to 20 years). Mean disease duration was 5 years (range = 1 to 12 years). the mean concentrations of serum albumin-corrected calcium (9.04 +/- 0.41 mg/dL) and alkaline phosphatase (153.3 +/- 100.1 IU) were significantly lower in patients with JIA than in controls (P < 0.0001 and P = 0.001, respectively). No differences in 25(OH)D, PTH or serum phosphorus were observed between JIA and control subjects. Regarding 25(OH)D concentration, 8 patients (26.7%) and 5 controls (16.7%) had 25(OH)D concentrations compatible with deficiency (lower than 20 ng/mL) and 14 patients (46.7%) and 18 controls (60%) had concentrations compatible with insufficiency (20-32 ng/mL). These values were not associated with disease activity, use of medications or bone mineral density. We observed a high frequency of 25(OH)D insufficiency and deficiency in the study sample. the compromised bone metabolism emphasizes the importance of follow-up of JIA patients.Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Universidade Federal de São Paulo, Dept Pediat, Div Alergia Imunol Clin & Reumatol, Unidade Reumatol Pediat, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Pediat, Div Alergia Imunol Clin & Reumatol, Unidade Reumatol Pediat, São Paulo, BrazilFAPESP: 08/57420-3Web of Scienc

Brazilian multicenter study of 71 patients with juvenile-onset Takayasu's arteritis: clinical and angiographic features

Objective: To describe the clinical and angiographic characteristics of Takayasu's arteritis in Brazilian children and adolescents. Methods: A retrospective data collection was performed in 71 children and adolescents followed in 10 Brazilian reference centers in Pediatric Rheumatology. The evaluation was carried out in three different time points: from onset of symptoms to diagnosis, from the 6th to 12th month of diagnosis, and in the last visit. Results: Of 71 selected patients, 51 (71.8%) were girls. The mean age of onset of symptoms and of time to diagnosis was 9.2 (+/- 4.2) years and 1.2 (+/- 1.4) years, respectively. At the end of the study, 20 patients were in a state of disease activity, 39 in remission and 5 had evolved to death. The most common symptoms in baseline assessment, second evaluation, and final evaluation were, respectively: constitutional, musculoskeletal, and neurological symptoms. A decrease in peripheral pulses was the most frequent cardiovascular signal, and an increase in erythrocyte sedimentation rate was the most frequent laboratory finding in all three evaluation periods. The tuberculin test was positive in 41% of those tested. Stenosis was the most frequent angiographic lesion, abdominal artery was the most affected segment, and angiographic type IV the most frequent. Most (90%) participants were treated with glucocorticoids, 85.9% required another immunosuppressive drug, and 29.6% underwent angioplasty. Conclusion: This is the largest study on juvenile-onset Takayasu arteritis, and a high number of patients under the age of 10 years, with predominance of constitutional symptoms early in the disease, was observed. (C) 2016 Elsevier Editora Ltda. All rights reserved.Univ Fed Sao Paulo, Dept Pediat, Sect Pediat Rheumatol, Sao Paulo, SP, BrazilUniv Sao Paulo, Inst Crianca, Pediat Rheumatol Sect, Sao Paulo, SP, BrazilSanta Casa Misericordia Sao Paulo, Sao Paulo, SP, BrazilUniv Sao Paulo, Sch Med, Ribeirao Preto, SP, BrazilUniv Estado Rio de Janeiro, BR-20550011 Rio De Janeiro, RJ, BrazilUniv Fed Rio de Janeiro, Inst Pediat Martagao Gesteira, Rio De Janeiro, RJ, BrazilUniv Fed Rio de Janeiro, Rheumatol Unit, Rio De Janeiro, RJ, BrazilUniv Fed Pernambuco, Rheumatol Unit, Recife, PE, BrazilUniv Fed Bahia, Salvador, BA, BrazilHosp Pequeno Principe, Curitiba, Parana, BrazilUniv Fed Sao Paulo, Dept Pediat, Sect Pediat Rheumatol, Sao Paulo, SP, BrazilWeb of Scienc

Genetic architecture distinguishes systemic juvenile idiopathic arthritis from other forms of juvenile idiopathic arthritis: Clinical and therapeutic implications

Objectives Juvenile idiopathic arthritis (JIA) is a heterogeneous group of conditions unified by the presence of chronic childhood arthritis without an identifiable cause. Systemic JIA (sJIA) is a rare form of JIA characterised by systemic inflammation. sJIA is distinguished from other forms of JIA by unique clinical features and treatment responses that are similar to autoinflammatory diseases. However, approximately half of children with sJIA develop destructive, long-standing arthritis that appears similar to other forms of JIA. Using genomic approaches, we sought to gain novel insights into the pathophysiology of sJIA and its relationship with other forms of JIA. Methods We performed a genome-wide association study of 770 children with sJIA collected in nine countries by the International Childhood Arthritis Genetics Consortium. Single nucleotide polymorphisms were tested for association with sJIA. Weighted genetic risk scores were used to compare the genetic architecture of sJIA with other JIA subtypes. Results The major histocompatibility complex locus and a locus on chromosome 1 each showed association with sJIA exceeding the threshold for genome-wide significance, while 23 other novel loci were suggestive of association with sJIA. Using a combination of genetic and statistical approaches, we found no evidence of shared genetic architecture between sJIA and other common JIA subtypes. Conclusions The lack of shared genetic risk factors between sJIA and other JIA subtypes supports the hypothesis that sJIA is a unique disease process and argues for a different classification framework. Research to improve sJIA therapy should target its unique genetics and specific pathophysiological pathways

Quality of life and impact of the disease on primary caregivers of juvenile idiopathic arthritis patients

Objectives: To evaluate the quality of life and the disease burden on primary caregivers of patients with juvenile idiopathic arthritis (JIA). Methods: Seventy patients with JIA and their respective caregivers were enrolled in this study. Health, quality of life and psychological status were assessed by using the childhood health assessment questionnaire (CHAQ), the medical outcomes study 36-item short-form health survey (SF-36) and the psychiatric screening questionnaire (SRQ-20). Burden of disease on the caregivers was measured by the caregiver burden scale (CB Scale).Results: Most caregivers were women (91.4%), married (76.6%), mothers (92.2%) and their average age was 37.2 +/- 8.8 years. Low education and socioeconomic state were observed in most of the caregivers. Psychoemotional disorders measured by the SRQ-20 were detected in 34.3% of the caregivers. Pain and mental health were the items in the SF-36 questionnaire most affected in these individuals. Mean score of global burden measured by the CB Scale was 1.6 +/- 0.3 (disappointment and environment had the lowest scores). the CB Scale was significantly correlated with the SRQ-20 (r = 0.6), number of limited joints (r = 0.3), number of visits (r = 0.2), family income (r = -0.3) and mental health (r = -0.6), emotional aspects (r = -0.4), social aspects (r = -0.4), vitality (r = -0.5) and general health state of the SF-36 (r = -0.4). SRQ-20 was the most important determinant of CB Scale and of the components pain and mental health of the SF-36 questionnaire.Conclusions: Most of the disease burden on the caregivers depends upon emotional aspects rather than on the physical status of the patients. (C) 2007 Elsevier Masson SAS. All rights reserved.Universidade Federal de São Paulo, Div Rheumatol, BR-04023900 São Paulo, BrazilUniv São Paulo, Dept Pediat, Pediat Rheumatol Unit, São Paulo, BrazilUniversidade Federal de São Paulo, Div Rheumatol, BR-04023900 São Paulo, BrazilWeb of Scienc

Health related quality of life of children with rheumatic heart diseases: reliability of the Brazilian version of the pediatric quality of life inventory (TM) cardiac module scale

Background: This study aimed to translate the 'Pediatric Quality of Life Inventory (TM) (PedsQL (TM) 3.0) Cardiac Module' into Portuguese, adapt it to Brazilian culture, and assess its psychometric properties (validity and reproducibility), and to calculate health-related quality of life scores on the PedsQL 4.0 and PedsQL (TM) 3.0 Cardiac Module Scales for a group of patients 5 to 18 years old with rheumatic heart disease.Methods: the methods suggested by the authors of the original version of the questionnaire included 1) translation by an expert panel; 2) translation back into English and revision by the authors of the original version; 3) pilot study with seven children and parents in each of three age ranges (5 to 7, 8 to 12, and 13 to 18 years old); and 4) assessment of the measurement properties. in this stage, the PedsQL (TM) 3.0 Cardiac Module and the PedsQL 4.0 Generic Scale were applied to a sample comprising 109 children and adolescents with rheumatic heart disease and their parents or caregivers. the version for parents or caregivers was administered separately on the same day.Results: the values of Cronbach's alpha for all scales assessed in the questionnaire (heart problems and treatment [symptoms], problems with perceived physical appearance, treatment anxiety, cognitive problems, and communication problems) varied from 0.6 to 0.8, indicating good internal consistency. Correlation was found between the scores for the Cardiac Module and the Generic Scale (0.36-0.86), demonstrating convergent validity (Spearman's correlation coefficient, p < 0.01). the symptoms, problems with perceived physical appearance, and cognitive and communication problem domains were able to distinguish between groups of patients with mild and moderate/severe heart disease (Student's t-test, p < 0.05). the intraclass correlation of the interobserver reproducibility was adequate (0.76 to 0.94 among the patients [children/adolescents] and 0.76 to 0.84 among their caregivers). the correlation between the patients' scores and their parents' scores varied from 0.50 to 0.86 (Pearson's correlation coefficient, p < 0.01).Conclusions: the Brazilian version of the PedsQL (TM) 3.0 Cardiac Module was shown to be reliable. the application of this questionnaire in practice will be very useful for all professionals charged with the care of children and adolescents with heart diseases

Dyslipidemia in Pediatric Systemic Lupus Erythematosus: the Relationship with Disease Activity and Plasma Homocysteine and Cysteine Concentrations

Objective: To evaluate the presence of dyslipidemia and plasma concentrations of homocysteine (Hcy) and cysteine (Cys) in adolescents with juvenile systemic lupus erythematosus (SLE) and relate these findings to disease activity (Systemic Lupus Erythematosus Disease Activity Index, SLEDAI) and cardiovascular risk factors. Methods: A cross-sectional controlled study including 26 female adolescents with SLE and 26 healthy controls was conducted. We evaluated SLEDAI, medications, anthropometric data, dietary intake, lipid profile, proteinuria, Hcy, Cys, folic acid, vitamin B-12, and high-sensitivity C-reactive protein levels. Results: Dyslipidemia was observed in 46.2% of the patients and in 19.2% of the controls. the SLE group had a higher Cys concentration and a lower high-density lipoprotein cholesterol concentration compared with the controls. in the multivariate analysis only Hcy was significantly and independently associated with the presence of dyslipidemia in the juvenile SLE group; an increase of 1 mu mol/l in the Hcy concentration doubled the chance of dyslipidemia (OR: 2.1; 95% Cl: 1.1-4.9; p = 0.030). the Cys concentration was correlated with Hcy, total cholesterol, low-density lipoprotein cholesterol, and triglyceride concentrations. Conclusion: We observed the presence of cardiovascular risk factors in adolescents with juvenile SLE. the early identification of biochemical alterations allows the development of intervention strategies that may lower the risk of cardiovascular disease. Copyright (C) 2013 S. Karger AG, BaselUniversidade Federal de São Paulo, Dept Pediat, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Pediat, São Paulo, BrazilWeb of Scienc

The case for utilizing more strict quantitative Doppler echocardiographic criterions for diagnosis of subclinical rheumatic carditis

Aim: Our aim was to perform a comparative, quantitative and qualitative, analysis of valvar echocardiographic findings in patients with acute rheumatic fever, with or without clinical manifestations of carditis, as compared to healthy controls. Methods and results: We analyzed cross-sectional Doppler echocardiographic images of 31 patients with acute rheumatic fever diagnosed according to the Jones criterions as modified in 1992. of 31 patients, 22 presented with clinical carditis, while 9 had subclinical carditis. the patients, and a control group of 20 healthy individuals, underwent cardiac examination and echocardiographic assessment, assessing quantitative and qualitative findings of mitral and aortic valvar abnormalities. the leaflets of the mitral valve were statistically thicker in those with clinical and subclinical carditis when compared to controls (p less than 0.001). We observed a greater frequency of mitral variance, convergence of mitral flow, and aortic regurgitation for those with clinical and subclinical carditis when compared to controls (p less than 0.001, p less than 0.001 and p equal to 0.003, respectively). Patients with clinical and subclinical carditis had more quantitative and qualitative changes in the parameters than did the controls. Conclusion: Echocardiography is a sensitive method to detect valvar abnormalities in patients with acute rheumatic fever and carditis. Additionally, by using regular standardized criterions, abnormalities that lead to a diagnosis of subclinical carditis are found in those patients with acute rheumatic fever in the apparent absence of cardiac involvement.Universidade Federal de São Paulo, Dept Pediat, Div Allergy Clin Immunol & Rhumatol, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Med, Div Pediat Cardiol, Discipline Cardiol, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Pediat, Div Allergy Clin Immunol & Rhumatol, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Med, Div Pediat Cardiol, Discipline Cardiol, São Paulo, BrazilWeb of Scienc