The Brazilian Portuguese version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR)

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Brazilian Portuguese language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the three Likert assumptions, floor/ceiling effects, internal consistency, Cronbach\u2019s alpha, interscale correlations, test\u2013retest reliability, and construct validity (convergent and discriminant validity). A total of 231 JIA patients (14.7% systemic, 43.3% oligoarticular, 22.5% RF negative polyarthritis, 19.5% other categories) and 72 healthy children, were enrolled in three centres. The JAMAR components discriminated well healthy subjects from JIA patients. All JAMAR components revealed good psychometric performances. In conclusion, the Brazilian Portuguese version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research

A experiência da vida real com complicações cardiovasculares na primeira dose de fingolimode

Fingolimod is a new and efficient treatment for multiple sclerosis (MS). The drug administration requires special attention to the first dose, since cardiovascular adverse events can be observed during the initial six hours of fingolimod ingestion. The present study consisted of a review of cardiovascular data on 180 patients with MS receiving the first dose of fingolimod. The rate of bradycardia in these patients was higher than that observed in clinical trials with very strict inclusion criteria for patients. There were less than 10% of cases requiring special attention, but no fatal cases. All but one patient continued the treatment after this initial dose. This is the first report on real-life administration of fingolimod to Brazilian patients with MS, and one of the few studies with these characteristics in the world.Fingolimode é um tratamento novo e eficaz para esclerose múltipla (EM). A administração desta droga requer atenção especial para a primeira dose, uma vez que eventos adversos cardiovasculares podem ser observados nas seis horas iniciais da ingestão de fingolimode. O presente estudo consistiu de uma revisão de dados cardiovasculares de 180 pacientes com EM ao receberem a primeira dose de fingolimode. A taxa de bradicardia nestes pacientes foi maior do que aquele observada em estudos clínicos que tem critérios de inclusão muito rigorosos para seleção de pacientes. Menos de 10% dos casos necessitou de atenção especial, mas não houve casos fatais. Todos os pacientes exceto por um continuaram o tratamento após esta dose inicial. Este é o primeiro relato de dados de administração de fingolimode na vida real de pacientes brasileiros com EM, e um dos poucos trabalhos com estas características no mundo.Universidade Metropolitana de Santos Departamento de NeurologiaUniversidade Positivo Departamento de NeurologiaUniversidade Federal do Paraná Departamento de NeurologiaUniversidade Estadual de Campinas Departamento de NeurologiaUniversidade Federal de Juiz de Fora Departamento de NeurologiaHospital de Clínicas de Porto Alegre Departamento de NeurologiaPontifícia Universidade Católica Sorocaba Departamento de NeurologiaClínica Holus MedServiceHospital Beneficência Portuguesa de São Paulo Departamento de NeurologiaCentro Hospitalar Unimed Departamento de NeurologiaUniversidade Federal Fluminense Departamento de NeurologiaUniversidade Federal de São Paulo (UNIFESP) Departamento de NeurologiaHospital de Base do Distrito Federal Departamento de NeurologiaInstituto de Neurologia de Curitiba Departamento de NeurologiaUNIFESP, Depto. de NeurologiaSciEL

The Real-life Experience With Cardiovascular Complications In The First Dose Of Fingolimod For Multiple Sclerosis.

Fingolimod is a new and efficient treatment for multiple sclerosis (MS). The drug administration requires special attention to the first dose, since cardiovascular adverse events can be observed during the initial six hours of fingolimod ingestion. The present study consisted of a review of cardiovascular data on 180 patients with MS receiving the first dose of fingolimod. The rate of bradycardia in these patients was higher than that observed in clinical trials with very strict inclusion criteria for patients. There were less than 10% of cases requiring special attention, but no fatal cases. All but one patient continued the treatment after this initial dose. This is the first report on real-life administration of fingolimod to Brazilian patients with MS, and one of the few studies with these characteristics in the world.72712-

Profile of paediatric rheumatology specialists and services in the state of São Paulo

INTRODUCTION: Paediatric rheumatology (PR) is an emerging specialty, practised by a limited number of specialists. Currently, there is neither a record of the profile of rheumatology patients being treated in Brazil nor data on the training of qualified rheumatology professionals in the country. OBJECTIVE: To investigate the profile of PR specialists and services, as well as the characteristics of paediatric patients with rheumatic diseases, for estimating the current state of rheumatology in the state of São Paulo. PATIENTS AND METHODS: In 2010, the scientific department of PR of the Paediatric Society of São Paulo administered a questionnaire that was answered by 24/31 accredited specialists in PR practising in state of São Paulo and by 8/21 institutions that provide PR care. RESULTS: Most (91%) of the surveyed professionals practise in public institutions. Private clinics (28.6%) and public institutions (37.5%) reported not having access to nailfold capillaroscopy, and 50% of the private clinics reported not having access to acupuncture. The average duration of professional practise in PR was 9.4 years, and 67% of the physicians had attended postgraduate programmes. Seven (87.5%) public institutions perform teaching activities, in which new paediatric rheumatologists are trained, and five (62.5%) offer postgraduate programmes. Two-thirds of the surveyed specialists use immunosuppressants and biological agents classified as restricted use by the Health Secretariat. The disease most frequently reported was juvenile idiopathic arthritis (29.1-34.5%), followed by juvenile systemic lupus erythematosus (JSLE) (11.6-12.3%) and rheumatic fever (9.1-15.9%). The incidence of vasculitis (including Henoch-Schönlein purpura, Wegener's granulomatosis, and Takayasu's arteritis) and autoinflammatory syndromes was higher in public institutions compared to other institutions (P = 0.03, P = 0.04, P = 0.002, and P < 0.0001, respectively). Patients with JSLE had the highest mortality rate (68% of deaths), mainly due to infection. CONCLUSION: The field of PR in the state of São Paulo has a significant number of specialists with postgraduate degrees who mostly practise at teaching institutions with infrastructures appropriate for the care of high-complexity patients.INTRODUÇÃO: A reumatologia pediátrica (RP) é uma especialidade emergente, com número restrito de especialistas, e ainda não conta com uma casuística brasileira sobre o perfil dos pacientes atendidos e as informações sobre a formação de profissionais capacitados. OBJETIVO: Estudar o perfil dos especialistas e dos serviços em RP e as características dos pacientes com doenças reumáticas nessa faixa etária a fim de estimar a situação atual no estado de São Paulo (ESP). PACIENTES E MÉTODOS: No ano de 2010 o departamento científico de RP da Sociedade de Pediatria de São Paulo encaminhou um questionário respondido por 24/31 especialistas com título de especialização em RP que atuam no ESP e por 8/12 instituições com atendimento nesta especialidade. RESULTADOS: A maioria (91%) dos profissionais exerce suas atividades em instituições públicas. Clínicas privadas (28,6%) e instituições (37,5%) relataram não ter acesso ao exame de capilaroscopia e 50% das clínicas privadas não tem acesso à acupuntura. A média de tempo de prática profissional na especialidade foi de 9,4 anos, sendo 67% deles pós-graduados. Sete (87,5%) instituições públicas atuam na área de ensino, formando novos reumatologistas pediátricos. Cinco (62,5%) delas têm pós-graduação. Dois terços dos especialistas utilizam imunossupressores e agentes biológicos de uso restrito pela Secretaria da Saúde. A doença mais atendida foi artrite idiopática juvenil (29,1%-34,5%), seguida de lúpus eritematoso sistêmico juvenil (LESJ) (11,6%-12,3%) e febre reumática (9,1%-15,9%). Vasculites (púrpura de Henoch Schönlein, Wegener, Takayasu) e síndromes autoinflamatórias foram mais incidentes nas instituições públicas (P = 0,03; P = 0,04; P = 0,002 e P < 0,0001, respectivamente). O LESJ foi a doença com maior mortalidade (68% dos óbitos), principalmente por infecção. CONCLUSÃO: A RP no ESP conta com um número expressivo de especialistas pós-graduados, que atuam especialmente em instituições de ensino, com infraestrutura adequada ao atendimento de pacientes de alta complexidade.Universidade Federal de São Paulo (UNIFESP) Departamento de Pediatria Setor de Reumatologia PediátricaUniversidade de São Paulo Faculdade de Medicina Hospital das ClínicasSanta Casa de Misericórdia de São Paulo Departamento de Pediatria Setor de ReumatologiaUniversidade Estadual de Campinas Unidade de Reumatologia PediátricaUniversidade de São Paulo Faculdade de Medicina de Ribeirão Preto Departamento de Puericultura e PediatriaSanta Casa de Misericórdia de Santos Setor de Reumatologia PediátricaFaculdade de Medicina de Botucatu Hospital Estadual de Bauru Serviço de Reumatologia PediátricaFaculdade de Medicina de BotucatuUNIFESP, Depto. de Pediatria Setor de Reumatologia PediátricaSciEL

Health related quality of life measure in systemic pediatric rheumatic diseases and its translation to different languages: an international collaboration

Background: Rheumatic diseases in children are associated with significant morbidity and poor health-related quality of life (HRQOL). There is no health-related quality of life (HRQOL) scale available specifically for children with less common rheumatic diseases. These diseases share several features with systemic lupus erythematosus (SLE) such as their chronic episodic nature, multi-systemic involvement, and the need for immunosuppressive medications. HRQOL scale developed for pediatric SLE will likely be applicable to children with systemic inflammatory diseases.Findings: We adapted Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY (c)) to Simple Measure of Impact of Illness in Youngsters (SMILY (c)-Illness) and had it reviewed by pediatric rheumatologists for its appropriateness and cultural suitability. We tested SMILY (c)-Illness in patients with inflammatory rheumatic diseases and then translated it into 28 languages. Nineteen children (79% female, n= 15) and 17 parents participated. the mean age was 12 +/- 4 years, with median disease duration of 21 months (1-172 months). We translated SMILY (c)-Illness into the following 28 languages: Danish, Dutch, French (France), English (UK), German (Germany), German (Austria), German (Switzerland), Hebrew, Italian, Portuguese (Brazil), Slovene, Spanish (USA and Puerto Rico), Spanish (Spain), Spanish (Argentina), Spanish (Mexico), Spanish (Venezuela), Turkish, Afrikaans, Arabic (Saudi Arabia), Arabic (Egypt), Czech, Greek, Hindi, Hungarian, Japanese, Romanian, Serbian and Xhosa.Conclusion: SMILY (c)-Illness is a brief, easy to administer and score HRQOL scale for children with systemic rheumatic diseases. It is suitable for use across different age groups and literacy levels. SMILY (c)-Illness with its available translations may be used as useful adjuncts to clinical practice and research.Rutgers State Univ, Robert Wood Johnson Med Sch, New Brunswick, NJ 08903 USARutgers State Univ, Child Hlth Inst New Jersey, New Brunswick, NJ 08901 USAHosp Special Surg, New York, NY 10021 USAUniv Michigan, Ann Arbor, MI 48109 USARed Cross War Mem Childrens Hosp, Cape Town, South AfricaAin Shams Univ, Pediat Allergy Immunol & Rheumatol Unit, Cairo, EgyptAin Shams Univ, Pediat Rheumatol Pediat Allergy Immunol & Rheum, Cairo, EgyptKing Faisal Specialist Hosp & Res Ctr, Riyadh 11211, Saudi ArabiaCharles Univ Prague, Prague, Czech RepublicGen Univ Hosp, Prague, Czech RepublicUniv Hosp Motol, Dept Pediat, Prague, Czech RepublicAarhus Univ, Hosp Skejby, Aarhus, DenmarkRigshosp, Juliane Marie Ctr, DK-2100 Copenhagen, DenmarkUniv Med Ctr, Dept Pediat Immunol, Utrecht, NetherlandsWilhelmina Childrens Hosp, Utrecht, NetherlandsGreat Ormond St Hosp Sick Children, Children NHS Fdn Trust, Renal Unit, London, EnglandLyon Univ, Hosp Civils Lyon, Rheumatol & Dermatol Dept, Lyon, FranceMed Univ Innsbruck, A-6020 Innsbruck, AustriaPrim Univ Doz, Bregenz, AustriaHamburg Ctr Pediat & Adolescence Rheumatol, Hamburg, GermanyAsklepios Clin Sankt, Augustin, GermanyUniv Zurich, Childrens Hosp, Zurich, SwitzerlandAristotle Univ Thessaloniki, Pediat Immunol & Rheumatol Referral Ctr, GR-54006 Thessaloniki, GreeceIsrael Meir Hosp, Kefar Sava, IsraelSanjay Gandhi Postgrad Inst Med Sci, Lucknow, Uttar Pradesh, IndiaSemmelweis Univ, H-1085 Budapest, HungaryAnna Meyer Hosp, Florence, ItalyUniv Siena, Res Ctr System Autoimmune & Autoinflammatory Dis, I-53100 Siena, ItalyUniv Florence, Florence, ItalyOsped Pediat Bambino Gesu, IRCCS, Pediat Rheumatol Unit, Rome, ItalyUniv Genoa Pediat II Reumatol, Ist G Gaslini EULAR, Ctr Excellence Rheumatol, Genoa, ItalyUniv Cattolica Sacro Cuore, Inst Pediat, Rome, ItalyUniv Padua, Dept Pediat, Pediat Rheumatol Unit, Padua, ItalyYokohama City Univ, Sch Med, Yokohama, Kanagawa 232, JapanUniv Estadual Paulista, UNESP, Botucatu, SP, BrazilUniversidade Federal de São Paulo, Dept Pediat, São Paulo, BrazilUniv Estadual Campinas, Dept Med, Campinas, SP, BrazilUniv Fed Rio de Janeiro, Dept Pediat, Rio de Janeiro, BrazilUniv Estado do, Adolescent Hlth Care Unit, Div Pediat Rheumatol, Rio de Janeiro, BrazilUniv São Paulo, Fac Med, Childrens Inst, Dept Pediat,Pediat Rheumatol Unit, São Paulo, BrazilChildrens Inst, Pediat Rheumatol Unit, São Paulo, BrazilClin Pediat I, Cluj Napoca, RomaniaInst Rheumatol, Belgrade, SerbiaUniv Childrens Hosp, Univ Med Ctr Ljubljana, Ljubljana, SloveniaHead Rheumatol Hosp Pedro Elizalde, Buenos Aires, DF, ArgentinaHosp Gen Mexico City, Mexico City, DF, MexicoHosp Infantil Mexico Fed Gomez, Mexico City, DF, MexicoHosp San Juan Dios, Barcelona, SpainHosp Univ Valle Hebron, Barcelona, SpainMt Sinai Med Ctr, New York, NY 10029 USAMt Sinai Med Ctr, Miami Beach, FL 33140 USAComplejo Hosp Univ Ruiz & Paez, Bolivar, VenezuelaHacettepe Univ, Dept Pediat, Ankara, TurkeyIstanbul Univ, Cerrahpasa Med Sch, Istanbul, TurkeyFMF Arthrit Vasculitis & Orphan Dis Res Ctr, Inst Hlth Sci, Ankara, TurkeyUniv Calgary, Dept Pediat, Alberta Childrens Hosp, Res Inst, Calgary, AB T2N 1N4, CanadaUniversidade Federal de São Paulo, Dept Pediat, São Paulo, BrazilWeb of Scienc

Search for eccentric black hole coalescences during the third observing run of LIGO and Virgo

Despite the growing number of confident binary black hole coalescences observed through gravitational waves so far, the astrophysical origin of these binaries remains uncertain. Orbital eccentricity is one of the clearest tracers of binary formation channels. Identifying binary eccentricity, however, remains challenging due to the limited availability of gravitational waveforms that include effects of eccentricity. Here, we present observational results for a waveform-independent search sensitive to eccentric black hole coalescences, covering the third observing run (O3) of the LIGO and Virgo detectors. We identified no new high-significance candidates beyond those that were already identified with searches focusing on quasi-circular binaries. We determine the sensitivity of our search to high-mass (total mass M&gt;70 M⊙) binaries covering eccentricities up to 0.3 at 15 Hz orbital frequency, and use this to compare model predictions to search results. Assuming all detections are indeed quasi-circular, for our fiducial population model, we place an upper limit for the merger rate density of high-mass binaries with eccentricities 0&lt;e≤0.3 at 0.33 Gpc−3 yr−1 at 90\% confidence level

Ultralight vector dark matter search using data from the KAGRA O3GK run

Among the various candidates for dark matter (DM), ultralight vector DM can be probed by laser interferometric gravitational wave detectors through the measurement of oscillating length changes in the arm cavities. In this context, KAGRA has a unique feature due to differing compositions of its mirrors, enhancing the signal of vector DM in the length change in the auxiliary channels. Here we present the result of a search for U(1)B−L gauge boson DM using the KAGRA data from auxiliary length channels during the first joint observation run together with GEO600. By applying our search pipeline, which takes into account the stochastic nature of ultralight DM, upper bounds on the coupling strength between the U(1)B−L gauge boson and ordinary matter are obtained for a range of DM masses. While our constraints are less stringent than those derived from previous experiments, this study demonstrates the applicability of our method to the lower-mass vector DM search, which is made difficult in this measurement by the short observation time compared to the auto-correlation time scale of DM