RESUMO Objetivo: Avaliar as doenças autoinflamatórias (DAI) de acordo com sexo e idade no momento do diagnóstico e a resposta terapêutica em uma grande população. Métodos: Este é um estudo observacional transversal de um registro latino-americano que usou um sistema de dados coletados entre 2015 e 2018. Quaisquer achados alterados ao longo do acompanhamento foram registrados. Os formulários foram traduzidos para os idiomas português e espanhol, incluindo características demográficas, clínicas, laboratoriais, genéticas e de tratamento. Resultados: Incluímos 152 pacientes, sendo 51,3% do sexo masculino e 75% da raça branca. A média de idade de início da doença foi de 2,1 anos (0–15,6 anos) e a média de idade de diagnóstico 6,9 anos (0–21,9 anos); 111 (73%) eram crianças (0–9 anos) e 41 (27%) adolescentes/adultos jovens (10–21 anos). A síndrome de febre periódica, estomatite aftosa, faringite e adenite (PFAPA) ocorreu em 46/152 (30%), osteomielite não bacteriana crônica (CNO) em 32/152 (21%) e febre familiar do Mediterrâneo (FMF) em 24/152 (15,7%). A PFAPA foi significativamente maior em crianças pequenas (38,7 vs. 7,3%, p<0,001), e a CNO, em adolescentes/adultos jovens (13,5 vs. 41,5%, p<0,001). A frequência do sexo feminino foi significativamente maior na CNO (28,4 vs. 14,1%, p=0,031) e menor na FMF (8,1 vs. 23,1%, p=0,011). Os medicamentos mais utilizados foram glicocorticoides, anti-inflamatórios não esteroidais (AINE) e colchicina. O tratamento com glicocorticoides e colchicina foi usado em todas as DAI com resposta boa a moderada. No entanto, as síndromes periódicas associadas à criopirina (CAPS) pareciam não responder aos glicocorticoides. AINE e metotrexato foram os principais medicamentos utilizados no tratamento da CNO. Conclusões: Diferenças de pacientes com DAI foram observadas na população latino-americana em pacientes agrupados por sexo e idade ao diagnóstico da doença. Objetivo (DAI Métodos latinoamericano latino americano 201 2018 registrados espanhol demográficas clínicas laboratoriais Resultados 15 513 51 3 51,3 75 branca 21 2 1 2, 0–15,6 0156 0 6 (0–15, 69 9 6, 0–21,9 0219 (0–21, 11 73% 73 (73% 0–9 09 (0– 4 27% 27 (27% adolescentesadultos adolescentes adultos 10–21 1021 10 (10–2 anos. . periódica aftosa (PFAPA 46152 46 46/15 30%, 30 30% , (30%) (CNO 32152 32 32/15 21% (21% (FMF 24152 24 24/15 15,7%. 157 15,7% 7 (15,7%) 38,7 387 38 (38, vs 7,3% p<0,001, p0001 p p<0,001 001 p<0,001) 13,5 135 13 5 (13, 415 41,5% p<0,001. 28,4 284 28 (28, 141 14 14,1% p=0,031 p0031 031 8,1 81 8 (8, 231 23 23,1% p=0,011. p0011 p=0,011 011 p=0,011) antiinflamatórios anti inflamatórios (AINE moderada entanto CAPS (CAPS Conclusões latinoamericana americana 20 51, 0–15, 015 (0–15 0–21, 021 (0–21 (73 0– (0 (27 10–2 102 (10– 4615 46/1 (30% 3215 32/1 (21 2415 24/1 15,7 (15,7% 38, (38 7,3 p000 p<0,00 00 13, (13 41,5 28, (28 14,1 p=0,03 p003 03 8, (8 23,1 p001 p=0,01 01 0–15 (0–1 0–21 02 (0–2 (7 ( (2 10– (10 461 46/ (30 321 32/ 241 24/ 15, (15,7 (3 7, p00 p<0,0 (1 41, 14, p=0,0 23, 0–1 0–2 (15, p0 p<0, p=0, (15 p<0 p=0 p< p=

ABSTRACT Objective: To evaluate autoinflammatory diseases (AID) according to age at diagnosis and sex, and response to therapy in a large population. Methods: This is a cross-sectional observational study of a Latin American registry using a designed web system for data storage, collected between 2015 and 2018. Any altered findings during follow-up were recorded. The forms were translated into Portuguese and Spanish, including demographic, clinical, laboratory, genetic and treatment characteristics. Results: We included 152 patients, 51.3% male and 75% Caucasian. The median age at disease onset was 2.1 years (0–15.6 years) and median age at diagnosis 6.9 years (0–21.9 years); 111 (73%) were children (0–9 years old), and 41 (27%) were adolescents and young adults (AYA) (10–21 years old). Periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA) occurred in 46/152 (30%), chronic non-bacterial osteomyelitis (CNO) in 32/152 (21%), and familial Mediterranean fever (FMF) in 24/152 (15.7%). PFAPA was significantly higher in young children than in AYA (38.7% vs. 7.3%, p<0.001), while CNO were lower (13.5% vs. 41.5%, p<0.001). The frequency of females was significantly higher in CNO (28.4% vs. 14.1%, p=0.031) and lower in FMF (8.1% vs. 23.1%, p=0.011). The most used drugs were glucocorticoids, non-steroidal anti-inflammatory drugs (NSAID), and colchicine. Glucocorticoids and colchicine treatment were used in all AID with good to moderate response. However, cryopyrin-associated periodic syndromes (CAPS) seemed unresponsive to glucocorticoids. NSAIDs and methotrexate were the main medications used to treat CNO. Conclusions: Differences among AID patients were observed in the LA population regarding sex and age at disease diagnosis. Objective (AID Methods crosssectional cross sectional storage 201 2018 followup follow up recorded Spanish demographic clinical laboratory characteristics Results 15 513 51 3 51.3 75 Caucasian 21 2 1 2. 0–15.6 0156 0 6 (0–15. 69 9 6. 0–21.9 0219 (0–21. 11 73% 73 (73% 0–9 09 (0– old, old , old) 4 27% 27 (27% (AYA 10–21 1021 10 (10–2 old. . stomatitis pharyngitis (PFAPA 46152 46 46/15 30%, 30 30% (30%) nonbacterial non bacterial (CNO 32152 32 32/15 21%, 21% (21%) (FMF 24152 24 24/15 15.7%. 157 15.7% 7 (15.7%) 38.7% 387 38 (38.7 vs 7.3% p<0.001, p0001 p p<0.001 001 p<0.001) 13.5% 135 13 5 (13.5 415 41.5% p<0.001. 28.4% 284 28 (28.4 141 14 14.1% p=0.031 p0031 031 8.1% 81 8 (8.1 231 23 23.1% p=0.011. p0011 p=0.011 011 p=0.011) glucocorticoids nonsteroidal steroidal antiinflammatory anti inflammatory NSAID, NSAID (NSAID) However cryopyrinassociated cryopyrin associated CAPS (CAPS Conclusions 20 51. 0–15. 015 (0–15 0–21. 021 (0–21 (73 0– (0 (27 10–2 102 (10– 4615 46/1 (30% 3215 32/1 (21% 2415 24/1 15.7 (15.7% 38.7 (38. 7.3 p000 p<0.00 00 13.5 (13. 41.5 28.4 (28. 14.1 p=0.03 p003 03 8.1 (8. 23.1 p001 p=0.01 01 (NSAID 0–15 (0–1 0–21 02 (0–2 (7 ( (2 10– (10 461 46/ (30 321 32/ (21 241 24/ 15. (15.7 38. (38 7. p00 p<0.0 13. (13 41. 28. (28 14. p=0.0 8. (8 23. 0–1 0–2 (1 (3 (15. p0 p<0. p=0. (15 p<0 p=0 p< p=

Abstract Background Juvenile idiopathic arthritis (JIA) comprises a whole spectrum of chronic arthritis starting before 16 years of age. The study aims to explore the clinical and demographic descriptors, treatment, and disease progression of enthesitis-related arthritis (ERA) in comparison with juvenile-onset spondyloarthritis (SpA). Methods Cross-sectional analysis of consecutive patients in two dedicated clinics, with a single visit and retrospective case-notes review. Arthritis, enthesitis and sacroiliitis were evaluated by scoring disease activity and damage. Continuous variables were reported by median, interquartile range; categorical variables were reported by the frequency comparison of the two groups. Results Thirty-three cases were included, being 23 (69.7%) with ERA. The median age at diagnosis was 12.5 y (SpA) vs. 9 y (ERA) (p < 0.01); the time from symptom onset to diagnosis was 5.5 y (SpA) vs. 1.5 y (ERA) (p < 0.03). In both groups, the predominant presentation was a single joint or < 5 lower limb joints and asymmetric involvement, with a high frequency of enthesitis. There was a higher frequency of mid-tarsal and ankle synovitis in the ERA group and hip involvement in those with SpA. The comparison of the frequency of spine symptoms at presentation, 30% SpA vs. 21.7% ERA (p = 0.7), was not significant, and radiographic progression to spinal involvement occurred in 43.5% of ERA patients. The median time for spinal progression and age at onset was 2.2 and 12 y for ERA, and 4 and 16.5 y for SpA, respectively. Activity and damage scores were not significantly different between the groups. Treatment comparison resulted in 91.3% of ERA and 100% SpA being treated, predominantly with NSAIDs in both groups, followed by DMARDs and biologics, with a higher frequency of biologics in SpA. Conclusion The main differences were the late diagnoses of SpA, and the hip and spine involvement, with higher frequency of biologic treatment in juvenile-onset SpA compared to ERA. JIA (JIA 1 descriptors enthesitisrelated related (ERA juvenileonset juvenile . Crosssectional Cross sectional clinics casenotes case notes review Arthritis range groups Thirtythree Thirty three included 2 69.7% 697 69 7 (69.7% 125 12. (SpA vs p 0.01 001 0 01 0.01) 55 5. 15 1. 0.03. 003 0.03 03 0.03) midtarsal mid tarsal 30 217 21 21.7 0.7, 07 0.7 , 0.7) significant 435 43 43.5 22 2. 165 16. respectively 913 91 3 91.3 100 treated 69.7 6 (69.7 0.0 00 21. 0. 43. 91. 10 69. (69. (69 (6 (

Resumo Objetivo Avaliar as taxas de expulsão e continuação do dispositivo intrauterino (DIU) de cobre inserido no pós-parto imediato em um hospital universitário brasileiro. Materiais e Métodos Neste estudo de corte transversal, foram incluídas parturientes submetidas à inserção de DIU de cobre no pós-parto imediato entre março de 2018 e dezembro de 2019. Foram coletados dados clínicos e da ultrassonografia (US) transvaginal realizada após seis semanas. As taxas de expulsão e de continuação foram avaliadas após seis meses por meio de dados do prontuário ou por contato telefônico. O resultado principal foi a proporção de DIUs expelidos em seis meses. Para análise estatística, utilizaram-se o teste t de Student, a distribuição de Poisson, e o teste do Qui quadrado. Resultados Houve 3,728 nascimentos no período, e foram inseridos 352 DIUs, em uma taxa de 9,4%. Com 6 semanas, o DIU estava bem posicionado em 65,1% dos casos, em 10,8%, houve expulsão parcial, e, em 8,5%, fora totalmente expelido. Aos 6 meses de pós-parto, foram obtidas informações de 234 mulheres, 74,4% das quais usavam DIU, com uma taxa de expulsão geral de 25,6%. A taxa de expulsão foi maior após o parto vaginal do que após cesariana (68,4% versus 31,6%, respectivamente; p = 0,031). Não houve diferenças quanto à idade, paridade, idade gestacional, índice de massa corpórea final, e peso do recém-nascido. Conclusão Apesar da baixa taxa de inserção e alta taxa de expulsão, a taxa de continuação em longo prazo da contracepção intrauterina com DIU de cobre foi elevada, o que indica que se trata de intervenção útil para prevenir gestações indesejadas em curto intervalo de tempo. (DIU pósparto pós brasileiro transversal 201 2019 US (US semanas telefônico estatística utilizaramse utilizaram Student Poisson quadrado 3728 3 728 3,72 período 35 94 9 4 9,4% 651 65 1 65,1 casos 108 10 8 10,8% parcial 85 5 8,5% expelido pósparto, parto, 23 mulheres 744 74 74,4 256 25 25,6% 68,4% 684 68 (68,4 316 31 31,6% respectivamente 0,031. 0031 0,031 . 0 031 0,031) paridade gestacional final recémnascido. recémnascido recém nascido. nascido recém-nascido elevada tempo 20 372 72 3,7 9,4 65, 10,8 8,5 2 7 74, 25,6 68,4 (68, 31,6 003 0,03 03 37 3, 9, 10, 8, 25, 68, (68 31, 00 0,0 (6 0, (

Abstract Objective To evaluate the expulsion and continuation rates of the copper intrauterine device (IUD) inserted in the immediate postpartum period in a Brazilian public university hospital. Materials and Methods In the present cohort study, we included women who received immediate postpartum IUD at vaginal delivery or cesarean s March 2018 to December 2019. Clinical data and the findings of transvaginal ultrasound (US) scans performed 6-weeks postpartum were collected. The expulsion and continuation rates were assessed 6-months postpartum using data from the electronic medical records or by telephone contact. The primary outcome was the proportion of IUDs expelled at 6 months. For the statistical analysis, we used the Student t-test, the Poisson distribution, and the Chi-squared test. Results There were 3,728 births in the period, and 352 IUD insertions were performed, totaling a rate of 9.4%. At 6 weeks postpartum, the IUD was properly positioned in 65.1% of the cases, in 10.8% there was partial expulsion, and in 8.5% it had been completely expelled. At 6 months postpartum, information was obtained from 234 women, 74.4% of whom used IUD, with an overall expulsion rate of 25.6%. The expulsion rate was higher after vaginal delivery when compared with cesarean section (68.4% versus 31.6% respectively; p = 0.031). There were no differences in terms of age, parity, gestational age, final body mass index, and newborn weight. Conclusion Despite the low insertion rate of copper IUDs in the postpartum period and a higher expulsion rate, the rate of long-term continuation of intrauterine contraception was high, indicating that it is a useful intervention to prevent unwanted pregnancies and to reduce short-interval birth. (IUD hospital study 201 2019 US (US 6weeks collected 6months contact analysis ttest, ttest t test, test t-test distribution Chisquared Chi squared 3728 3 728 3,72 35 94 9 4 9.4% 651 65 1 65.1 cases 108 10 8 10.8 85 5 8.5 23 744 74 74.4 256 25 25.6% 68.4% 684 68 (68.4 316 31 31.6 respectively 0.031. 0031 0.031 . 0 031 0.031) age parity index weight longterm long term high shortinterval short interval birth 20 372 72 3,7 9.4 65. 10. 8. 2 7 74. 25.6 68.4 (68. 31. 003 0.03 03 37 3, 9. 25. 68. (68 00 0.0 (6 0. (

Abstract Background: Rheumatic diseases are associated with an increase in overall risks of tuberculosis (TB). The aim of this study was to evaluate the frequency of TB and the frequency of latent TB infection (LTBI), in clinical practice, for juvenile idiopathic arthritis (JIA) patients from high and low risk of TB incidence endemic countries. Methods: This is an international, multicenter, cross-sectional, observational study of data collection from Brazil and Registry of Portugal at REUMA.PT. The inclusion criteria were patients with Juvenile Idiopathic Arthritis (JIA) with age ≤ 18 years who underwent screening for Mycobacterium tuberculosis infection [tuberculin skin test (TST) and/or interferon gamma release assay (IGRA)]. Chest X-rays and history of exposure to TB were also assessed. Results: 292 JIA patients were included; mean age 14.3 years, mean disease duration 7.5 years, 194 patients (66.4%) performed only TST, 14 (4.8%) only IGRA and 84 (28.8%) both. The frequency of LTBI (10.6%) and TB was similar between the two countries. The reasons for TB screening were different; in Brazil it was performed more often at JIA onset while in Portugal it was performed when starting Disease Modified Anti-Rheumatic Drugs (DMARD) treatment (p < 0.001). Isoniazid therapy was prescribed in 40 (13.7%) patients (31 with LTBI and 9 with epidemiologic risks and/or due to contact with sick people). Only three patients (1%) developed active TB. Conclusion: We found nearly 10% of patients with LTBI, a small percentage of patients with treatment due to epide-miologic risks and only 1% with active TB. Distinct reasons and screening methods for LTBI were observed between the two countries.

Aim Although bulk fill composites have been widely used as restorative material, there is no consensus regarding the best clinical protocol in terms of composite technique and adhesive system. Therefore, this clinical trial evaluated the clinical performance of bulk fill composites for class I restorations under different protocols. Methods A randomized clinical trial including 155 class I restorations was conducted using different adhesive systems: conventional technique (phosphoric acid + conventional three-step adhesive system) (Group 1, 2 and 3); or self-etching adhesive system (Groups 4, 5 and 6). Control groups 1 and 4 were restored with conventional composite; groups 2 and 5 with low viscosity bulk fill and conventional composite as occlusal coverage; groups 3 and 6 with high viscosity bulk fill. The FDI criteria was used for clinical evaluation at baseline and after 6 months. Results All groups showed good clinical performance. At baseline, the adhesive system did not affect postoperative hypersensitivity. After 6 months, group 5 showed a significant reduction in color and translucency; group 6 a reduction in terms of anatomical form and for postoperative sensitivity and an improvement in patient satisfaction (p<0.05). Considering the same restorative technique, the use of the self-etching adhesive system showed a significant decrease in color and translucency (p<0.05). Conclusion All groups showed favorable clinical performance, and promising results were found for the conventional adhesive system and high viscosity bulk fill protocol.

Em diversos países, a atenção primária às gestantes é conduzida por enfermeiras obstetras e/ou obstetrizes. No Sistema Suplementar de Saúde no Brasil, a cobertura da assistência pré-natal é obrigatória e realizada por médicos obstetras. O objetivo deste estudo é conduzir análise de custo-efetividade, comparando desfechos clínicos e custos associados à incorporação do pré-natal por enfermeiras obstetras e obstetrizes no âmbito do Sistema de Saúde Suplementar, sob a perspectiva da operadora de planos de saúde como fonte pagadora. Foi construída uma árvore de decisão, baseada nos dados de metanálise da Colaboração Cochrane que mostrou redução do risco de parto prematuro no grupo de gestantes de risco habitual acompanhado por enfermeiras obstetras e obstetrizes. Foram considerados apenas os custos médicos diretos cobertos pelas operadoras de planos de saúde para a realização de consultas e exames essenciais, conforme protocolo do Ministério da Saúde vigente. Assumiu-se custo unitário de consulta com cada profissional como iguais e aplicou-se um aumento do custo global com exames pré-natais associado ao acompanhamento médico, conforme dado obtido na literatura. Estimou-se a razão de custo-efetividade incremental de -R$ 10.038,43 (economia de R$ 10.038,43) por parto prematuro evitado. Esse resultado mostrou-se consistente nas análises de sensibilidade, com economias associadas à substituição variando de -R$ 2.544,60 até -R$ 31.807,46 por parto prematuro evitado. Como conclusão, observou-se que o cuidado pré-natal por enfermeiras obstetras e obstetrizes é superior ao prestado por médicos obstetras para o desfecho prevenção de parto prematuro, resultando ainda em economia de recursos.

In several countries, primary care for pregnant women is performed by obstetric nurses and/or midwives. In Brazil’s Supplementary Health System (private health insurance and out-of-pocket care), coverage of prenatal care is mandatory and is performed by medical obstetricians. The objective of this study is to conduct a cost-effectiveness analysis, comparing clinical outcomes and costs associated with the incorporation of prenatal care by obstetric nurses and midwives in the Supplementary Health System, from the perspective of the operator of health plans as the payment source. A decision tree was built, based on data from a Cochrane Collaboration meta-analysis that showed a reduction in the risk of premature birth in the group of normal-risk pregnant women accompanied by obstetric nurses and midwives. The analysis only considered the direct medical costs covered by health plan operators for essential appointments and tests, according to the prevailing Ministry of Health protocol. The study assumed equal unit costs of consultations by medical professionals and applied an increase in the overall cost of prenatal tests associated with medical follow-up, based on data from the literature. Incremental cost-effective ratio was estimated at -BRL 10,038.43 (savings of BRL 10,038.43) per premature birth avoided. This result was consistent with the sensitivity analyses, with savings associated with the substitution ranging from -BRL 2,544.60 to -BRL 31,807.46 per premature death avoided. In conclusion, prenatal care provided by obstetric nurses and midwives was superior to that provided by medical obstetricians for the prevention of premature birth, besides resulting in cost savings.

En diversos países, la atención primaria a las gestantes se realiza con enfermeras obstetras y/o parteras. En el Sistema Suplementario de Salud en Brasil, la cobertura de la asistencia prenatal es obligatoria y la realizan médicos obstetras. El objetivo de este estudio es realizar un análisis de costo-efectividad, comparando resultados clínicos y costes asociados a la incorporación en el período prenatal de enfermeras obstetras y parteras, en el ámbito del Sistema de Salud Suplementaria, desde la perspectiva de una operadora de planes de salud como fuente pagadora. Se construyó un árbol de decisión, basado en datos de metaanálisis de la Colaboración Cochrane, que mostró una reducción del riesgo de parto prematuro en el grupo de gestantes de riesgo habitual, con un seguimiento de enfermeras obstetras y parteras. Se consideraron solo los costes médicos directos, cubiertos por las operadoras de planes de salud para la realización de consultas y exámenes esenciales, conforme el protocolo vigente del Ministerio de Salud. Se asumió el coste unitario de consulta con cada profesional como iguales, y se aplicó un aumento del coste global con exámenes prenatales asociado al seguimiento médico, conforme los datos obtenidos en la literatura. Se estimó la razón de costo-efectividad incremental de -BRL 10.038,43 (economía de BRL 10.038,43) por parto prematuro evitado. Este resultado se mostró consistente en los análisis de sensibilidad, con ahorros asociados a la sustitución, variando de -BRL 2.544,60 hasta -BRL 31.807,46 por parto prematuro evitado. Como conclusión, se observó que el cuidado prenatal por parte de enfermeras obstetras y parteras es superior al prestado por médicos obstetras para el desenlace de prevención de parto prematuro, resultando incluso en un ahorro de recursos.

Em diversos países, a atenção primária às gestantes é conduzida por enfermeiras obstetras e/ou obstetrizes. No Sistema Suplementar de Saúde no Brasil, a cobertura da assistência pré-natal é obrigatória e realizada por médicos obstetras. O objetivo deste estudo é conduzir análise de custo-efetividade, comparando desfechos clínicos e custos associados à incorporação do pré-natal por enfermeiras obstetras e obstetrizes no âmbito do Sistema de Saúde Suplementar, sob a perspectiva da operadora de planos de saúde como fonte pagadora. Foi construída uma árvore de decisão, baseada nos dados de metanálise da Colaboração Cochrane que mostrou redução do risco de parto prematuro no grupo de gestantes de risco habitual acompanhado por enfermeiras obstetras e obstetrizes. Foram considerados apenas os custos médicos diretos cobertos pelas operadoras de planos de saúde para a realização de consultas e exames essenciais, conforme protocolo do Ministério da Saúde vigente. Assumiu-se custo unitário de consulta com cada profissional como iguais e aplicou-se um aumento do custo global com exames pré-natais associado ao acompanhamento médico, conforme dado obtido na literatura. Estimou-se a razão de custo-efetividade incremental de -R$ 10.038,43 (economia de R$ 10.038,43) por parto prematuro evitado. Esse resultado mostrou-se consistente nas análises de sensibilidade, com economias associadas à substituição variando de -R$ 2.544,60 até -R$ 31.807,46 por parto prematuro evitado. Como conclusão, observou-se que o cuidado pré-natal por enfermeiras obstetras e obstetrizes é superior ao prestado por médicos obstetras para o desfecho prevenção de parto prematuro, resultando ainda em economia de recursos.

In several countries, primary care for pregnant women is performed by obstetric nurses and/or midwives. In Brazil’s Supplementary Health System (private health insurance and out-of-pocket care), coverage of prenatal care is mandatory and is performed by medical obstetricians. The objective of this study is to conduct a cost-effectiveness analysis, comparing clinical outcomes and costs associated with the incorporation of prenatal care by obstetric nurses and midwives in the Supplementary Health System, from the perspective of the operator of health plans as the payment source. A decision tree was built, based on data from a Cochrane Collaboration meta-analysis that showed a reduction in the risk of premature birth in the group of normal-risk pregnant women accompanied by obstetric nurses and midwives. The analysis only considered the direct medical costs covered by health plan operators for essential appointments and tests, according to the prevailing Ministry of Health protocol. The study assumed equal unit costs of consultations by medical professionals and applied an increase in the overall cost of prenatal tests associated with medical follow-up, based on data from the literature. Incremental cost-effective ratio was estimated at -BRL 10,038.43 (savings of BRL 10,038.43) per premature birth avoided. This result was consistent with the sensitivity analyses, with savings associated with the substitution ranging from -BRL 2,544.60 to -BRL 31,807.46 per premature death avoided. In conclusion, prenatal care provided by obstetric nurses and midwives was superior to that provided by medical obstetricians for the prevention of premature birth, besides resulting in cost savings.

En diversos países, la atención primaria a las gestantes se realiza con enfermeras obstetras y/o parteras. En el Sistema Suplementario de Salud en Brasil, la cobertura de la asistencia prenatal es obligatoria y la realizan médicos obstetras. El objetivo de este estudio es realizar un análisis de costo-efectividad, comparando resultados clínicos y costes asociados a la incorporación en el período prenatal de enfermeras obstetras y parteras, en el ámbito del Sistema de Salud Suplementaria, desde la perspectiva de una operadora de planes de salud como fuente pagadora. Se construyó un árbol de decisión, basado en datos de metaanálisis de la Colaboración Cochrane, que mostró una reducción del riesgo de parto prematuro en el grupo de gestantes de riesgo habitual, con un seguimiento de enfermeras obstetras y parteras. Se consideraron solo los costes médicos directos, cubiertos por las operadoras de planes de salud para la realización de consultas y exámenes esenciales, conforme el protocolo vigente del Ministerio de Salud. Se asumió el coste unitario de consulta con cada profesional como iguales, y se aplicó un aumento del coste global con exámenes prenatales asociado al seguimiento médico, conforme los datos obtenidos en la literatura. Se estimó la razón de costo-efectividad incremental de -BRL 10.038,43 (economía de BRL 10.038,43) por parto prematuro evitado. Este resultado se mostró consistente en los análisis de sensibilidad, con ahorros asociados a la sustitución, variando de -BRL 2.544,60 hasta -BRL 31.807,46 por parto prematuro evitado. Como conclusión, se observó que el cuidado prenatal por parte de enfermeras obstetras y parteras es superior al prestado por médicos obstetras para el desenlace de prevención de parto prematuro, resultando incluso en un ahorro de recursos.

Nosso objetivo foi revisar sistematicamente dados sobre o risco de infecções sexualmente transmissíveis (ISTs) e vaginose bacteriana em mulheres lésbicas e sugerir estratégias para melhorar prevenção, diagnóstico e tratamento. Uma estratégia de busca para lésbica, ISTs e vaginose bacteriana foi aplicada às bases PubMed, LILACS e BDENF. De 387 referências únicas identificadas, 22 preenchiam os critérios de inclusão (estudos seccionais relatando a prevalência de 8 ISTs/vaginose bacteriana e histórico de ISTs). A infecção mais frequentemente relatada foi vaginose bacteriana e nenhum estudo relatou dados sobre hepatite B. Uma ampla gama de prevalências foi observada para a maioria das infecções. Em termos de fatores de risco, o número de parceiras sexuais, ser ou ter sido fumante, histórico de sexo forçado e estigma sexual parecem aumentar o risco de ISTs e vaginose bacteriana. Os resultados desta revisão são discutidos à luz de diretrizes que abordam diretamente a saúde da comunidade LGBT e também de estudos relevantes que investigaram tanto práticas de sexo seguro quanto a complexa relação entre pessoas LGBT e profissionais de saúde. Um conjunto de recomendações para melhorar o cuidado preventivo para mulheres lésbicas é proposto. É razoável afirmar que pouco se sabe sobre a dimensão da transmissão de ISTs e vaginose bacteriana em atividades sexuais entre mulheres ou sobre os fatores de risco para ISTs e vaginose bacteriana em mulheres lésbicas. De fato, a qualidade dos estudos foi, de forma geral, baixa ou muito baixa, com incerteza significativa quanto a seus resultados. Contudo, consideramos que o conhecimento disponível indica alguns caminhos a serem seguidos por profissionais de saúde e na elaboração de políticas públicas para melhorar ações em direção a uma melhor saúde sexual de mulheres lésbicas.

Our aim was to systematically review data about the risk of sexually transmitted infections (STI) and bacterial vaginosis among lesbian women and to suggest strategies to improve prevention, diagnosis and treatment. A search strategy for lesbian, STI and bacterial vaginosis was applied to PubMed, LILACS and BDENF databases. Of 387 unique references retrieved, 22 fulfilled the inclusion criteria (cross-sectional studies reporting prevalence for 8 STIs/bacterial vaginosis and history of a STI). The most frequent infection reported was bacterial vaginosis, and none study reported data on hepatitis B. A wide range of prevalence was observed for most infections. In terms of risk factors, the number of sexual partners, the past or current smoking, a history of forced sex and sexual stigma seem to increase the risk of STI and bacterial vaginosis. The findings of this review are discussed considering guidelines directly addressing the LGBT community’s health and relevant studies investigating both safe sexual practices and the intricate relationship between LGBT people and their care providers. A set of recommendations to improve preventive care for lesbian women is proposed. Affirming that little is known about the extent of STIs and bacterial vaginosis transmission in female-to-female sexual activities or about the risk factors for STI and bacterial vaginosis among lesbian women is reasonable. In fact, the overall quality of the studies was low or very low with significant uncertainty around their findings. However, we consider that the available knowledge indicates some paths to be followed by care providers and policy decision-makers to improve their actions towards better sexual health of lesbian women.

Nuestro objetivo fue realizar una revisión sistemática de los datos sobre infecciones de trasmisión sexual (ITS) y riesgo de vaginosis bacteriana entre mujeres lesbianas y sugerir estrategias para mejorar su prevención, diagnóstico y tratamiento. La estrategia de búsqueda de lesbiana, ITS y vaginosis bacteriana se realizó en las bases de datos de PubMed, LILACS y BDENF. De 387 referencias únicas recogidas, 22 reunían criterios de inclusión (estudios transversales informaron sobre la prevalencia de 8 ITS/vaginosis bacteriana e historial de una ITS). La infección más frecuente fue vaginosis bacteriana y ningún estudio proporcionó datos sobre hepatitis B. se observó un amplio rango de prevalencia para la mayoría de las infecciones. En términos de factores de riesgo, el número de parejas sexuales, ser fumador o exfumador, un pasado de abusos sexuales, así como el estigma sexual parece que incrementaron el riesgo de ITS y vaginosis bacteriana. Los hallazgos del presente estudio se discuten a la luz de las directrices de salud directamente dirigidas a la comunidad LGBT, y también a estudios relevantes que investigaban tanto las prácticas sexuales seguras, como la relación intricada entre individuos del colectivo LGBT y sus proveedores de salud. Se propone un conjunto de recomendaciones para mejorar el cuidado preventivo de mujeres lesbianas. Es razonable afirmar que se conoce poco sobre el grado de transmisión de las ITS y vaginosis bacteriana en las relaciones sexuales entre mujeres o sobre los factores de riesgo para las ITS y vaginosis bacteriana entre mujeres lesbianas. De hecho, la calidad general de los estudios fue baja o muy baja con una incertidumbre significativa sobre sus resultados. No obstante, consideramos que el conocimiento disponible indica algunos caminos que pueden recorrer proveedores de salud y tomadores decisiones para mejorar sus acciones orientadas a una mejor salud sexual de las mujeres lesbianas.

Nosso objetivo foi revisar sistematicamente dados sobre o risco de infecções sexualmente transmissíveis (ISTs) e vaginose bacteriana em mulheres lésbicas e sugerir estratégias para melhorar prevenção, diagnóstico e tratamento. Uma estratégia de busca para lésbica, ISTs e vaginose bacteriana foi aplicada às bases PubMed, LILACS e BDENF. De 387 referências únicas identificadas, 22 preenchiam os critérios de inclusão (estudos seccionais relatando a prevalência de 8 ISTs/vaginose bacteriana e histórico de ISTs). A infecção mais frequentemente relatada foi vaginose bacteriana e nenhum estudo relatou dados sobre hepatite B. Uma ampla gama de prevalências foi observada para a maioria das infecções. Em termos de fatores de risco, o número de parceiras sexuais, ser ou ter sido fumante, histórico de sexo forçado e estigma sexual parecem aumentar o risco de ISTs e vaginose bacteriana. Os resultados desta revisão são discutidos à luz de diretrizes que abordam diretamente a saúde da comunidade LGBT e também de estudos relevantes que investigaram tanto práticas de sexo seguro quanto a complexa relação entre pessoas LGBT e profissionais de saúde. Um conjunto de recomendações para melhorar o cuidado preventivo para mulheres lésbicas é proposto. É razoável afirmar que pouco se sabe sobre a dimensão da transmissão de ISTs e vaginose bacteriana em atividades sexuais entre mulheres ou sobre os fatores de risco para ISTs e vaginose bacteriana em mulheres lésbicas. De fato, a qualidade dos estudos foi, de forma geral, baixa ou muito baixa, com incerteza significativa quanto a seus resultados. Contudo, consideramos que o conhecimento disponível indica alguns caminhos a serem seguidos por profissionais de saúde e na elaboração de políticas públicas para melhorar ações em direção a uma melhor saúde sexual de mulheres lésbicas.

Our aim was to systematically review data about the risk of sexually transmitted infections (STI) and bacterial vaginosis among lesbian women and to suggest strategies to improve prevention, diagnosis and treatment. A search strategy for lesbian, STI and bacterial vaginosis was applied to PubMed, LILACS and BDENF databases. Of 387 unique references retrieved, 22 fulfilled the inclusion criteria (cross-sectional studies reporting prevalence for 8 STIs/bacterial vaginosis and history of a STI). The most frequent infection reported was bacterial vaginosis, and none study reported data on hepatitis B. A wide range of prevalence was observed for most infections. In terms of risk factors, the number of sexual partners, the past or current smoking, a history of forced sex and sexual stigma seem to increase the risk of STI and bacterial vaginosis. The findings of this review are discussed considering guidelines directly addressing the LGBT community’s health and relevant studies investigating both safe sexual practices and the intricate relationship between LGBT people and their care providers. A set of recommendations to improve preventive care for lesbian women is proposed. Affirming that little is known about the extent of STIs and bacterial vaginosis transmission in female-to-female sexual activities or about the risk factors for STI and bacterial vaginosis among lesbian women is reasonable. In fact, the overall quality of the studies was low or very low with significant uncertainty around their findings. However, we consider that the available knowledge indicates some paths to be followed by care providers and policy decision-makers to improve their actions towards better sexual health of lesbian women.

Nuestro objetivo fue realizar una revisión sistemática de los datos sobre infecciones de trasmisión sexual (ITS) y riesgo de vaginosis bacteriana entre mujeres lesbianas y sugerir estrategias para mejorar su prevención, diagnóstico y tratamiento. La estrategia de búsqueda de lesbiana, ITS y vaginosis bacteriana se realizó en las bases de datos de PubMed, LILACS y BDENF. De 387 referencias únicas recogidas, 22 reunían criterios de inclusión (estudios transversales informaron sobre la prevalencia de 8 ITS/vaginosis bacteriana e historial de una ITS). La infección más frecuente fue vaginosis bacteriana y ningún estudio proporcionó datos sobre hepatitis B. se observó un amplio rango de prevalencia para la mayoría de las infecciones. En términos de factores de riesgo, el número de parejas sexuales, ser fumador o exfumador, un pasado de abusos sexuales, así como el estigma sexual parece que incrementaron el riesgo de ITS y vaginosis bacteriana. Los hallazgos del presente estudio se discuten a la luz de las directrices de salud directamente dirigidas a la comunidad LGBT, y también a estudios relevantes que investigaban tanto las prácticas sexuales seguras, como la relación intricada entre individuos del colectivo LGBT y sus proveedores de salud. Se propone un conjunto de recomendaciones para mejorar el cuidado preventivo de mujeres lesbianas. Es razonable afirmar que se conoce poco sobre el grado de transmisión de las ITS y vaginosis bacteriana en las relaciones sexuales entre mujeres o sobre los factores de riesgo para las ITS y vaginosis bacteriana entre mujeres lesbianas. De hecho, la calidad general de los estudios fue baja o muy baja con una incertidumbre significativa sobre sus resultados. No obstante, consideramos que el conocimiento disponible indica algunos caminos que pueden recorrer proveedores de salud y tomadores decisiones para mejorar sus acciones orientadas a una mejor salud sexual de las mujeres lesbianas.

Abstract Objective: To evaluate prevalence, clinical manifestations, laboratory abnormalities, treatment and outcome in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients with and without panniculitis. Methods: Panniculitis was diagnosed due to painful subcutaneous nodules and/or plaques in deep dermis/subcutaneous tissues and lobular/mixed panniculitis with lymphocytic lobular inflammatory infiltrate in skin biopsy. Statistical analysis was performed using Bonferroni correction(p < 0.004). Results: Panniculitis was observed in 6/847(0.7%) cSLE. Painful subcutaneous erythematosus and indurated nodules were observed in 6/6 panniculitis patients and painful subcutaneous plaques in 4/6. Generalized distribution was evidenced in 3/6 and localized in upper limbs in 2/6 and face in 1/6. Cutaneous hyperpigmentation and/or cutaneous atrophy occurred in 5/6. Histopathology features showed lobular panniculitis without vasculitis in 5/6(one of them had concomitant obliterative vasculopathy due to antiphospholipid syndrome) and panniculitis with vasculitis in 1/6. Comparison between cSLE with panniculitis and 60 cSLE without panniculitis with same disease duration [2.75(0-11.4) vs. 2.83(0-11.8) years,p = 0.297], showed higher frequencies of constitutional involvement (67% vs. 10%,p = 0.003) and leukopenia (67% vs. 7%,p = 0.002). Cutaneous atrophy and hyperpigmentation occurred in 83% of patients. Conclusions: Panniculitis is a rare skin manifestation of cSLE occurring in the first three years of disease with considerable sequelae. The majority of patients have concomitant mild lupus manifestations.

Abstract Objective: To evaluate autoimmune hepatitis (AIH) in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients. Methods: This retrospective multicenter study included 847 patients with cSLE, performed in 10 Pediatric Rheumatology services of São Paulo state, Brazil. AIH was defined according to the International Autoimmune Hepatitis Group criteria (IAHGC). The statistical analysis was performed using the Bonferroni's correction (p < 0.0033). Results: AIH in cSLE patients confirmed by biopsy was observed in 7/847 (0.8%) and all were diagnosed during adolescence. The majority occurred before or at cSLE diagnosis [5/7 (71%)]. Antinuclear antibodies were a universal finding, 43% had concomitantly anti-smooth muscle antibodies and all were seronegative for anti-liver kidney microsomal antibodies. All patients with follow-up ≥18 months (4/7) had complete response to therapy according to IAHGC. None had severe hepatic manifestations such as hepatic failure, portal hypertension and cirrhosis at presentation or follow-up. Further comparison of 7 cSLE patients with AIH and 28 without this complication with same disease duration [0 (0–8.5) vs. 0.12 (0–8.5) years, p = 0.06] revealed that the frequency of hepatomegaly was significantly higher in cSLE patients in the former group (71% vs. 11%, p = 0.003) with a similar median SLEDAI-2 K score [6 (0–26) vs. 7 (0–41), p = 0.755]. No differences were evidenced regarding constitutional involvement, splenomegaly, serositis, musculoskeletal, neuropsychiatric and renal involvements, and treatments in cSLE patients with and without AIH (p > 0.0033). Conclusions: Overlap of AIH and cSLE was rarely observed in this large multicenter study and hepatomegaly was the distinctive clinical feature of these patients. AIH occurred during adolescence, mainly at the first years of lupus and it was associated with mild liver manifestations.

Abstract Objective: To evaluate prevalence, clinical manifestations, laboratory abnormalities and treatment in a multicenter cohort study including 847 childhood-onset systemic lupus erythematosus (cSLE) patients with and without diffuse alveolar hemorrhage (DAH), as well as concomitant parameters of severity. Methods: DAH was defined as the presence of at least three respiratory symptoms/signs associated with diffuse interstitial/alveolar infiltrates on chest x-ray or high-resolution computer tomography and sudden drop in hemoglobin levels. Statistical analysis was performed using Bonferroni correction (p < 0.0022). Results: DAH was observed in 19/847 (2.2%) cSLE patients. Cough/dyspnea/tachycardia/hypoxemia occurred in all cSLE patients with DAH. Concomitant parameters of severity observed were: mechanical ventilation in 14/19 (74%), hemoptysis 12/19 (63%), macrophage activation syndrome 2/19 (10%) and death 9/19 (47%). Further analysis of cSLE patients at DAH diagnosis compared to 76 cSLE control patients without DAH with same disease duration [3 (1–151) vs. 4 (1–151) months, p = 0.335], showed higher frequencies of constitutional involvement (74% vs. 10%, p < 0.0001), serositis (63% vs. 6%, p < 0.0001) and sepsis (53% vs. 9%, p < 0.0001) in the DAH group. The median of disease activity score(SLEDAI-2 K) was significantly higher in cSLE patients with DAH [18 (5–40) vs. 6 (0–44), p < 0.0001]. The frequencies of thrombocytopenia (53% vs. 12%, p < 0.0001), intravenous methylprednisolone (95% vs. 16%, p < 0.0001) and intravenous cyclophosphamide (47% vs. 8%, p < 0.0001) were also significantly higher in DAH patients. Conclusions: This was the first study to demonstrate that DAH, although not a disease activity score descriptor, occurred in the context of significant moderate/severe cSLE flare. Importantly, we identified that this condition was associated with serious disease flare complicated by sepsis with high mortality rate.

Resumo Objetivos: Avaliar a vasculite digital (VD) clínica como uma manifestação inicial do lúpus eritematoso sistêmico de início na infância (LESi) em uma grande população. Métodos: Estudo transversal multicêntrico que incluiu 852 pacientes com LESi (critérios do ACR), acompanhados em dez centros de reumatologia pediátrica do Estado de São Paulo. Resultados: Observou-se VD em 25/852 (3%) pacientes com LESi. Diagnosticaram-se hemorragia periungueal em 12 (48%), infarto periungueal em sete (28%), úlcera de ponta de dígito em quatro (16%), nódulos dolorosos em um (4%) e gangrena em um (4%). Um desfecho ruim, com reabsorção digital, ocorreu em cinco (20%) pacientes. A comparação entre pacientes com e sem VD revelou maior frequência de erupção malar (80% vs. 53%, p = 0,008), erupção discoide (16% vs. 4%, p = 0,017), fotossensibilidade (76% vs. 45% p = 0,002) e outras vasculites cutâneas (80% vs. 19%, p < 0,0001), enquanto a frequência de características constitucionais totais (32% vs. 61%, p = 0,003), febre (32% vs. 56% p = 0,020) e hepatomegalia (4% vs. 23%, p = 0,026) foram menores nesses pacientes. A frequência do gênero feminino, o envolvimento grave de múltiplos órgãos, perfil de autoanticorpos e baixo complemento foram semelhantes nos dois grupos (p > 0,05). A mediana no Sledai-2 K, exclusive o descritor de VD, foi significativamente menor nos pacientes com VD em comparação com aqueles sem essa manifestação [10 (0 a 28) vs. 14 (0 a 58), p = 0,004]. Não foram observadas vasculite visceral nem morte nessa coorte de pacientes com LESi. A frequência de uso de ciclofosfamida (0% vs. 18%, p = 0,014) foi significativamente menor no grupo VD. Conclusão: Este grande estudo multicêntrico identificou a VD clínica como uma rara manifestação inicial do LESi ativo, associada a doença multissistêmica leve, apesar da ocorrência de reabsorção digital em alguns desses pacientes.

Abstract Objectives: To assess clinical digital vasculitis (DV) as an initial manifestation of childhood-onset systemic lupus erythematosus (cSLE) within a large population. Methods: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria) followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. Results: DV was observed in 25/852 (3%) cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%), periungual infarction in 7 (28%), tip finger ulceration in 4 (16%), painful nodules in 1 (4%) and gangrene in 1 (4%). A poor outcome, with digital resorption, occurred in 5 (20%). Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p = 0.008), discoid rash (16% vs. 4%, p = 0.017), photosensitivity (76% vs. 45%, p = 0.002) and other cutaneous vasculitides (80% vs. 19%, p < 0.0001), whereas the frequency of overall constitutional features (32% vs. 61%, p = 0.003), fever (32% vs. 56%, p = 0.020) and hepatomegaly (4% vs. 23%, p = 0.026) were lower in these patients. Frequency of female gender, severe multi-organ involvement, autoantibodies profile and low complement were alike in both groups (p > 0.05). SLEDAI-2K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10 (0-28) vs. 14 (0-58), p = 0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p = 0.014) was significantly lower in the DV group. Conclusion: Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients.

Resumo Objetivo: Estabelecer diretrizes baseadas em evidências científicas para manejo da síndrome de febre periódica, estomatite aftosa, faringite e adenite (PFAPA). Descrição do método de coleta de evidência: A Diretriz foi elaborada a partir de cinco questões clínicas que foram estruturadas por meio do Pico (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultados: Foram recuperados e avaliados pelo título e resumo 806 trabalhos e selecionados 32 artigos, para sustentar as recomendações. Recomendações: 1. O diagnóstico da PFAPA é clínico e de exclusão, deve a suspeita ser considerada em crianças que apresentam episódios febris de origem indeterminada recorrentes e periódicos ou amidalites de repetição, intercalados com períodos assintomáticos, sobretudo em crianças em bom estado geral e com desenvolvimento pondero-estatural mantido; 2. Os achados laboratoriais são inespecíficos. Não existem alterações patognomônicas nos exames complementares; 3. A evidência que sustenta a indicação do tratamento cirúrgico (tonsilectomia com ou sem adenoidectomia) é baseada em dois ensaios clínicos randomizados não cegos que incluíram pequeno número de pacientes; 4. O uso de prednisona no início do quadro febril em pacientes com PFAPA mostrou ser eficaz. Melhores evidências ainda são necessárias para apoiar seu uso na PFAPA; 5. Apesar de os resultados obtidos de estudos com inibidores de IL-1ß serem promissores, esses são limitados a poucos relatos de casos.

Abstract Objective: To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. Description of the evidence collection method: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. Results: 806 articles were retrieved and evaluated by title and abstract; from these, 32 articles were selected to support the recommendations. Recommendations: 1. PFAPA is a diagnosis of exclusion established on clinical grounds, and one must suspect of this problem in children with recurrent and periodic febrile episodes of unknown origin, or with recurrent tonsillitis interspersed with asymptomatic periods, especially in children in good general condition and with preservation of weight and height development. 2. Laboratory findings are nonspecific. Additional tests do not reveal pathognomonic changes. 3. The evidence supporting an indication for surgical treatment (tonsillectomy with or without adenoidectomy), is based on two non-blinded randomized clinical trials with small numbers of patients. 4. The use of prednisone at the onset of fever in patients with PFAPA proved to be an effective strategy. There is still need for more qualified evidence to support its use in patients with PFAPA. 5. Despite promising results obtained in studies with IL-1β inhibitors, such studies are limited to a few case reports.