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In 2012, a European initiative called Single Hub and Access point for paediatric 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 localised scleroderma (JLS) is a rare disease within the group of paediatric rheumatic diseases (PRD) and can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physicians’ experience. This study aims to provide recommendations for assessment and treatment of JLS. Recommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was formed, mainly from Europe, and consisted of 15 experienced paediatric rheumatologists and two young fellows. Recommendations derived from a validated systematic literature review were evaluated by an online survey and subsequently discussed at two consensus meetings using a nominal group technique. Recommendations were accepted if ≥80% agreement was reached. In total, 1 overarching principle, 10 recommendations on assessment and 6 recommendations on therapy were accepted with ≥80% agreement among experts. Topics covered include assessment of skin and extracutaneous involvement and suggested treatment pathways. The SHARE initiative aims to identify best practices for treatment of patients suffering from PRDs. Within this remit, recommendations for the assessment and treatment of JLS have been formulated by an evidence-informed consensus process to produce a standard of care for patients with JLS throughout Europe.

Near-infrared spectroscopic (NIR) technique was used, for the first time, to predict volatile phenols content, namely guaiacol, 4-methyl-guaiacol, eugenol, syringol, 4-methyl-syringol and 4-allyl-syringol, of aged wine spirits (AWS). This study aimed to develop calibration models for the volatile phenol’s quantification in AWS, by NIR, faster and without sample preparation. Partial least square regression (PLS-R) models were developed with NIR spectra in the near-IR region (12,500–4000 cm−1) and those obtained from GC-FID quantification after liquid-liquid extraction. In the PLS-R developed method, cross-validation with 50% of the samples along a validation test set with 50% of the remaining samples. The final calibration was performed with 100% of the data. PLS-R models with a good accuracy were obtained for guaiacol (r2 = 96.34; RPD = 5.23), 4-methyl-guaiacol (r2 = 96.1; RPD = 5.07), eugenol (r2 = 96.06; RPD = 5.04), syringol (r2 = 97.32; RPD = 6.11), 4-methyl-syringol (r2 = 95.79; RPD = 4.88) and 4-allyl-syringol (r2 = 95.97; RPD = 4.98). These results reveal that NIR is a valuable technique for the quality control of wine spirits and to predict the volatile phenols content, which contributes to the sensory quality of the spirit beverages.

Background To date there are no specific classification criteria for childhood-onset systemic lupus erythematosus (cSLE). This study aims to compare the performance among the American College of Rheumatology (ACR) 1997, the Systemic Lupus International Collaborating Clinics criteria (SLICC) and the new European League Against Rheumatism (EULAR)/ACR criteria, in a cSLE cohort. Methods We conducted a medical chart review study of cSLE cases and controls with defined rheumatic diseases, both ANA positive, to establish each ACR1997, SLICC and EULAR/ACR criterion fulfilled, at first visit and 1-year-follow-up. Results Study population included 122 cSLE cases and 89 controls. At first visit, SLICC criteria had higher sensitivity than ACR 1997 (89.3% versus 70.5%, p <  0.001), but similar specificity (80.9% versus 83.2%, p = 0.791), however performance was not statistically different at 1-year-follow-up. SLICC better scored in specificity compared to EULAR/ACR score ≥ 10 at first visit (80.9% versus 67.4%, p = 0.008) and at 1-year (76.4% versus 58.4%, p = 0.001), although sensitivities were similar. EULAR/ACR criteria score ≥ 10 exhibited higher sensitivity than ACR 1997 (87.7% versus 70.5%, p <  0.001) at first visit, but comparable at 1-year, whereas specificity was lower at first visit (67.4% versus 83.2%, p = 0.004) and 1-year (58.4% versus 76.4%, p = 0.002). A EULAR/ACR score ≥ 13 against a score ≥ 10, resulted in higher specificity, positive predictive value, and cut-off point accuracy. Compared to SLICC, a EULAR/ACR score ≥ 13 resulted in lower sensitivity at first visit (76.2% versus 89.3%, p <  0.001) and 1-year (91% versus 97.5%, p = 0.008), but similar specificities at both assessments. When compared to ACR 1997, a EULAR/ACR total score ≥ 13, resulted in no differences in sensitivity and specificity at both observation periods. Conclusions In this cSLE population, SLICC criteria better scored at first visit and 1-year-follow-up. The adoption of a EULAR/ACR total score ≥ 13 in this study, against the initially proposed ≥10 score, was most appropriate to classify cSLE. Further studies are necessary to address if SLICC criteria might allow fulfillment of cSLE classification earlier in disease course and may be more inclusive of cSLE subjects for clinical studies.

Different ageing technology of wine spirits (WSs) has been investigated, but little has been published on the chemical evolution of aged WS during storage in bottle. The purpose of this study was to examine how 12 months of storage in bottle affected the evolution of antioxidant activity (DPPH, FRAP and ABTS assays), total phenolic index (TPI) and low molecular weight (LMW) compounds content of the WSs aged through alternative technology using three micro-oxygenation levels (MOX) and nitrogen control (N). Results revealed the ability of phenolic compounds from aged WSs to scavenge free radicals during storage in bottle. Among the in vitro antioxidant-activity methods, FRAP assay was the more effective to differentiate WSs according to the ageing technology. Concerning the overall influence of storage in bottle on antioxidant activity, and TPI and LMW compounds content, the higher results were obtained for the MOX modalities (O15, O30 and O60), which showed a similar evolution. In summary, this study provides innovative information, demonstrating that the differences between the aged WSs imparted throughout the ageing process (resulting from different MOX levels) were mostly retained, and only slight modifications during storage in bottle were found.

In two placebo-controlled trials, canakinumab, an anti-interleukin-1β monoclonal antibody, achieved a response, prevented flares, and allowed glucocorticoid tapering in patients with systemic juvenile idiopathic arthritis. Infection was more common with canakinumab than with placebo. Systemic juvenile idiopathic arthritis (JIA), the most severe JIA subtype, is characterized by chronic arthritis; intermittently high, spiking temperatures; maculopapular rash; hepatosplenomegaly; lymphadenopathy; serositis; and a marked increase in the level of acute-phase reactants. 1 – 3 Complications of systemic JIA include growth impairment, osteoporosis, and the potentially lethal macrophage activation syndrome. 4 – 6 Until recently, systemic JIA was considered a therapeutic orphan, since the principal effective treatment was glucocorticoids, with their known toxicity and long-term growth and bone sequelae. 7 Other therapeutic options include nonsteroidal antiinflammatory drugs (NSAIDs), methotrexate, and biologic agents. Both interleukin-6 8 – 10 and, more recently, interleukin-1 11 – 14 have been found . . .

Background Surgical glue has been used in several body tissues, including perineal repair, and can benefit women. Objectives To evaluate the effectiveness of n-butyl-2-cyanoacrylate surgical glue compared to the polyglactin 910 suture in repairing first- and second-degree perineal tears and episiotomy in vaginal births. Design A parallel randomised controlled open trial. Setting Birth centre in Itapecerica da Serra, São Paulo, Brazil. Participants and methods The participants were 140 postpartum women allocated into four groups: two experimental groups repaired with surgical glue ( n  = 35 women with a first-degree tear; n  = 35 women with a second-degree tear or episiotomy); two control groups sutured with thread ( n  = 35 women with a first-degree tear; n  = 35 women with a second-degree tear or episiotomy). The outcomes were perineal pain and the healing process. Data collection was conducted in six stages: (1) up to 2 h after perineal repair; (2) from 12 to 24 h postpartum; (3) from 36 to 48 h; (4) from 10 to 20 days; (5) from 50 to 70 days; and (6) from 6 to 8 months. ANOVA, Student's t, Monte Carlo, x-square and Wald tests were used for the statistical analysis. Results One hundred forty women participated in the first three stages, 110 in stage 4, 122 in stage 5, and 54 in stage 6. The women treated with surgical glue had less perineal pain ( p  ≤ 0.001). There was no difference in the healing process, but the CG obtained a better result in the coaptation item ( p  ≤ 0.001). Conclusions Perineal repair with surgical glue has low pain intensity and results in a healing process similar to suture threads. Trial registration Brazilian Registry of Clinical Trials (UTN code: U1111-1184-2507; RBR-2q5wy8o); date of registration 01/25/2018; www.ensaiosclinicos.gov.br/rg/RBR-2q5wy8/

Wine spirits are typically aged in wooden barrels. Recently, alternative ageing technologies, such as those using wood fragments in wine spirits stored in stainless steel tanks, have been investigated. However, a significant lack of information regarding the potential evolution of these beverages after bottling still remains. This study assessed the 12-month evolution of aroma in bottled wine spirits aged with chestnut wood using different technologies, including fragment application with several micro-oxygenation strategies and barrels (traditional). Chemical analysis using GC-FID and GC–MS methods, along with sensory analysis, was conducted on all sampled aged wine spirits. Significant changes in volatile compounds were detected over time, including volatile phenols, acids, and esters. Multivariate data analysis distinguished traditional and alternative aged samples, with slight sample discrimination based on bottle storage. Regarding the sensory results, a significant effect of the time in bottle in several sensory attributes was found, while the ageing technologies mainly affected the gustatory attributes. The tasters were also asked to rate the overall quality of the samples, which seems to be favoured by the time in the bottle. This initial assessment of the impact of 1 year of glass bottle storage on the volatile and sensory composition of aged wine spirits highlights that this stage must be considered as an additional technological factor in their production process. However, the differences induced by the wood ageing technologies applied remained evident after 1 year of glass bottle storage.

Objective: to identify the prevalence of alcohol consumption patterns among Primary Health Care users and the association between risky, harmful, and probable dependence consumption patterns with sociodemographic, clinical, and behavioral characteristics. Method: cross-sectional study conducted with a sample of 2,178 participants who responded to the Alcohol Use Disorders Identification Test in primary health care services in the city of São Paulo. Descriptive and multiple inferential statistical analysis was performed using a hierarchical Poisson regression model with a significance level of 5%. Results: of the total number of participants, 18.9% met the criteria for harmful alcohol consumption. Determinants for risky and harmful alcohol consumption were male gender (PR = 1.89; 95% CI 1.57–2.28; p < 0.001), black race/color (PR = 1.65; 95% CI 1.30 - 2.10; p < 0.001), and income ≥ 10 minimum wages (PR = 1.78; 95% CI 1.07 - 2.99; p = 0.028). For probable dependence, lower educational level (PR = 13.70; 95% CI 1.56–117.63; p = 0.017) and reporting a diagnosis of depression (PR = 2.72; 95% CI 1.37–5.41; p = 0.005) were determinants. Conclusion: the prevalence of alcohol use disorders among users of primary health care services in São Paulo was higher than that observed in previous studies, suggesting that this segment of the population seeks health care at these services, highlighting the potential for screening and early diagnosis of the population. Objetivo: identificar a prevalência dos padrões de consumo de álcool entre usuários da Atenção Primária à Saúde e a associação entre os padrões de consumo de risco, nocivo e provável dependência, com características sociodemográficas, clínicas e comportamentais. Método: estudo transversal, realizado com uma amostra de 2178 participantes que responderam o Alcohol Use Disorders Identification Test, em serviços de Atenção Primária à Saúde do município de São Paulo. Realizou-se análise estatística descritiva e inferencial múltipla com modelo hierarquizado de regressão de Poisson com nível de significância de 5%. Resultados: do total de participantes, 18,9% preencheram critérios para consumo prejudicial de álcool. Foram determinantes para o consumo de risco e nocivo de álcool o sexo masculino (RP = 1,89; IC95% 1,57 - 2,28; p < 0,001), raça/cor preta (RP = 1,65; IC95% 1,30 - 2,10; p < 0,001) e renda ≥ 10 salários-mínimos (RP = 1,78; IC95% 1,07 – 2,99; p = 0,028). Para a provável dependência foram determinantes menor nível de escolaridade (RP = 13,70; IC95% 1,56 – 117,63; p = 0,017) e referir ter diagnóstico de depressão (RP = 2,72; IC95% 1,37 – 5,41; p = 0,005). Conclusão: a prevalência de transtornos por consumo de álcool entre usuários dos serviços de atenção primária à saúde de São Paulo mostrou-se maior do que observada em estudos prévios, sugerindo que essa parcela da população busca atendimento de saúde nesses serviços, destacando a potencialidade na triagem e diagnóstico precoce da população. Objetivo: identificar la prevalencia de los patrones de consumo de alcohol entre usuarios de la Atención Primaria de Salud y la asociación entre los patrones de consumo de riesgo, nocivo y probable dependencia con características sociodemográficas, clínicas y conductuales. Método: estudio transversal, realizado con una muestra de 2178 participantes que respondieron al Alcohol Use Disorders Identification Test, en servicios de Atención Primaria de Salud del municipio de São Paulo. Se realizó un análisis estadístico descriptivo e inferencial múltiple con modelo jerarquizado de regresión de Poisson con un nivel de significancia del 5%. Resultados: del total de participantes, el 18,9% cumplió criterios para consumo perjudicial de alcohol. Fueron determinantes para el consumo de riesgo y nocivo de alcohol el sexo masculino (RP = 1,89; IC95% 1,57 - 2,28; p < 0,001), raza/color negra (RP = 1,65; IC95% 1,30 - 2,10; p < 0,001) e ingresos ≥ 10 salarios mínimos (RP = 1,78; IC95% 1,07 – 2,99; p = 0,028). Para la probable dependencia fueron determinantes un menor nivel educativo (RP = 13,70; IC95% 1,56 – 117,63; p = 0,017) y referir diagnóstico de depresión (RP = 2,72; IC95% 1,37 – 5,41; p = 0,005). Conclusión: la prevalencia de trastornos por consumo de alcohol entre usuarios de los servicios de atención primaria de salud de São Paulo fue superior a la observada en estudios previos, lo que sugiere que esta parte de la población busca atención en estos servicios, destacando el potencial para el cribado y diagnóstico precoz de la población.

Few studies have investigated the influence on physicochemical and phytochemical compositions during storage in the bottle of wine spirits (WSs) aged using alternative ageing technology (AAT) compared to traditional ageing technology (TAT). The aim of this study was to evaluate the effect of the bottle storage over one and four years on the evolution of chromatic characteristics (CIELab method) and physicochemical characteristics (alcoholic strength, acidity, and total dry extract), total phenolic index (TPI), low molecular weight compound contents (HPLC-DAD technique), in vitro antioxidant activities (DPPH, FRAP, and ABTS assays), and phenolic characterisation (HPLC-DAD-ESI-MS/MS technique) of WSs aged with chestnut wood using TAT (barrels, B) and AAT (micro-oxygenation levels (MOX): O15, O30, and O60; and control (N)). The results showed that after four years of storage in the bottle, the O60 modality resulted in smaller changes in physicochemical characteristics, higher preservation of phenolic content, and greater evolution of chromatic characteristics, ensuring its overall quality compared to other modalities. Antioxidant activity decreased similarly in both technologies, such as phenolic acid content, in particular, gallic acid content. According to the findings of this study, alternative ageing technology might be the best alternative for wine spirit quality and ageing process sustainability.

Most data for treatment of dermatomyositis and juvenile dermatomyositis are from anecdotal, non-randomised case series. We aimed to compare, in a randomised trial, the efficacy and safety of prednisone alone with that of prednisone plus either methotrexate or ciclosporin in children with new-onset juvenile dermatomyositis. We did a randomised trial at 54 centres in 22 countries. We enrolled patients aged 18 years or younger with new-onset juvenile dermatomyositis who had received no previous treatment and did not have cutaneous or gastrointestinal ulceration. We randomly allocated 139 patients via a computer-based system to prednisone alone or in combination with either ciclosporin or methotrexate. We did not mask patients or investigators to treatment assignments. Our primary outcomes were the proportion of patients achieving a juvenile dermatomyositis PRINTO 20 level of improvement (20% improvement in three of six core set variables at 6 months), time to clinical remission, and time to treatment failure. We compared the three treatment groups with the Kruskal-Wallis test and Friedman's test, and we analysed survival with Kaplan-Meier curves and the log-rank test. Analysis was by intention to treat. Here, we present results after at least 2 years of treatment (induction and maintenance phases). This trial is registered with ClinicalTrials.gov, number NCT00323960. Between May 31, 2006, and Nov 12, 2010, 47 patients were randomly assigned prednisone alone, 46 were allocated prednisone plus ciclosporin, and 46 were randomised prednisone plus methotrexate. Median duration of follow-up was 35·5 months. At month 6, 24 (51%) of 47 patients assigned prednisone, 32 (70%) of 46 allocated prednisone plus ciclosporin, and 33 (72%) of 46 administered prednisone plus methotrexate achieved a juvenile dermatomyositis PRINTO 20 improvement (p=0·0228). Median time to clinical remission was 41·9 months in patients assigned prednisone plus methotrexate but was not observable in the other two treatment groups (2·45 fold [95% CI 1·2–5·0] increase with prednisone plus methotrexate; p=0·012). Median time to treatment failure was 16·7 months in patients allocated prednisone, 53·3 months in those assigned prednisone plus ciclosporin, but was not observable in patients randomised to prednisone plus methotrexate (1·95 fold [95% CI 1·20–3·15] increase with prednisone; p=0·009). Median time to prednisone discontinuation was 35·8 months with prednisone alone compared with 29·4–29·7 months in the combination groups (p=0·002). A significantly greater proportion of patients assigned prednisone plus ciclosporin had adverse events, affecting the skin and subcutaneous tissues, gastrointestinal system, and general disorders. Infections and infestations were significantly increased in patients assigned prednisone plus ciclosporin and prednisone plus methotrexate. No patients died during the study. Combined treatment with prednisone and either ciclosporin or methotrexate was more effective than prednisone alone. The safety profile and steroid-sparing effect favoured the combination of prednisone plus methotrexate. Italian Agency of Drug Evaluation, Istituto Giannina Gaslini (Genoa, Italy), Myositis Association (USA).