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 Increased malignancy frequency is well documented in adult-systemic lupus erythematosus (SLE), but with limited reports in childhood-onset SLE (cSLE) series. We explored the frequency of malignancy associated with cSLE, describing clinical and demographic characteristics, disease activity and cumulative damage, by the time of malignancy diagnosis. Method A retrospective case-notes review, in a nationwide cohort from 27 Pediatric Rheumatology centres, with descriptive biopsy-proven malignancy, disease activity/damage accrual, and immunosuppressive treatment were compiled in each participating centre, using a standard protocol. Results Of the 1757 cSLE cases in the updated cohort, 12 (0.7%) developed malignancy with median time 10 years after cSLE diagnosis. There were 91% females, median age at cSLE diagnosis 12 years, median age at malignancy diagnosis 23 years. Of all diagnosed malignancies, 11 were single-site, and a single case with concomitant multiple sites; four had haematological (0.22%) and 8 solid malignancy (0.45%). Median (min–max) SLEDAI-2 K scores were 9 (0–38), median (min–max) SLICC/ACR-DI (SDI) score were 1 (1–5) Histopathology defined 1 Hodgkin's lymphoma, 2 non-Hodgkin's lymphoma, 1 acute lymphoblastic leukaemia; 4 gastrointestinal carcinoma, 1 squamous cell carcinoma of the tongue and 1 anal carcinoma; 1 had sigmoid adenocarcinoma and 1 stomach carcinoid; 3 had genital malignancy, being 1 vulvae, 1 cervix and 1 vulvae and cervix carcinomas; 1 had central nervous system oligodendroglioma; and 1 testicle germ cell teratoma. Conclusion Estimated malignancy frequency of 0.7% was reported during cSLE follow up in a multicentric series. Median disease activity and cumulative damage scores, by the time of malignancy diagnoses, were high; considering that reported in adult series. adultsystemic systemic SLE, , (SLE) childhoodonset childhood onset (cSLE series characteristics casenotes notes review centres biopsyproven biopsy proven activitydamage accrual centre protocol 175 07 0 7 (0.7% 91 females malignancies singlesite, singlesite site, site single-site sites 0.22% 022 22 (0.22% 0.45%. 045 0.45% . 45 (0.45%) min–max minmax min max (min–max SLEDAI2 SLEDAI SLEDAI- 0–38, 038 0–38 38 (0–38) SLICC/ACRDI SLICCACRDI SLICC/ACR DI SLICC ACR SDI (SDI 1–5 15 5 (1–5 Hodgkins Hodgkin s lymphoma nonHodgkins non leukaemia carcinoid carcinomas oligodendroglioma teratoma 0.7 diagnoses high (SLE 17 (0.7 0.22 02 (0.22 04 0.45 (0.45% 03 0–3 (0–38 ACRDI SLICCACR 1– (1– 0. (0. 0.2 (0.2 0.4 (0.45 0– (0–3 (1 (0 (0.4 (0– (

Resumo Objetivo: Avaliar os parâmetros de saúde física e mental, de qualidade de vida relacionada à saúde (QVRS), em adolescentes com deficiência física matriculados em organização não governamental (ONG) esportiva vs. em adolescentes sem deficiência, durante a pandemia da doença do coronavírus 2019 (COVID-19). Métodos: Este estudo transversal incluiu 30 adolescentes com deficiência e 86 adolescentes sem deficiência que responderam a questionário online com dados sociodemográficos e informações de rotina de saúde autoavaliadas durante a quarentena da COVID-19. Versões validadas de autorrelato do Strengths and Difficulties Questionnaire (SDQ), Pediatric Quality of Life Inventory 4.0 (PedsQL4.0), Pittsburgh Sleep Quality Index (PSQI) e Pediatric Outcome Data Collection Instrument (PODCI) também foram aplicadas. Resultados: A mediana de problemas emocionais [4(0–10) vs. 5(0–10),p=0,018] e pró-social [7(0–10) vs. 9(3–10),p=0,006] foi menor em adolescentes com deficiência vs. adolescentes sem deficiência. Adolescentes com deficiência tiveram função global significantemente inferior [68(21–99) vs. 94(67–100),p <0,001] e pontuações de felicidade mais altas do PODCI [90(65–100) vs. 80(0–100),p=0,016] em comparação com o grupo sem deficiências. A análise de regressão logística demonstrou que a atividade física/semana (odds ratio — OR=1,03; intervalo de confiança — IC95%=1,01–1,05,p=0,002) foi maior nos adolescentes com deficiência. No entanto, atividades domésticas (OR=0,14; IC95%=0,04–0,43,p=0,001) e tempo de tela ≥3 horas/dia (OR=0,09; IC95%=0,02–0,38,p=0,001) foram menores nos adolescentes com deficiência. Conclusões: Os adolescentes com deficiência que frequentam uma organização não governamental (ONG) esportiva não tiveram maior risco de apresentar indicadores adversos à saúde; apesar de apresentarem função física reduzida, relataram mais atividade física, maior felicidade e menos tempo de tela em comparação com adolescentes sem deficiência durante a pandemia da COVID-19.

Abstract Objective: This study aimed to assess physical and mental health, and health-related quality of life (HRQL) parameters in adolescents with physical disabilities enrolled in a sports nongovernmental organization (NGO) versus adolescents without disabilities during coronavirus disease 2019 (COVID-19) pandemic. Methods: This cross-sectional study included 30 adolescents with disabilities and 86 adolescents without disabilities who responded to an online questionnaire with sociodemographic data and self-rated healthcare routine information during the COVID-19 quarantine. Validated self-report versions of the Strengths and Difficulties Questionnaire (SDQ), Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0), Pittsburgh Sleep Quality Index (PSQI), and Pediatric Outcome Data Collection Instrument (PODCI) were also applied. Results: The median of emotional [4 (0–10) vs. 5 (0–10), p=0.018] and prosocial [7 (0–10) vs. 9 (3–10), p=0.006] problems was lower in adolescents with disabilities versus adolescents without disabilities. Adolescents with disabilities had significantly lower global function [68 (21–99) vs. 94 (67–100), p<0.001] and higher happiness scores in the PODCI scale [90 (65–100) vs. 80 (0–100), p=0.016] compared to controls. Logistic regression analysis demonstrated that physical activity/week (OR=1.03; 95%CI 1.01–1.05, p=0.002) was higher in adolescents with disabilities compared to adolescents without disabilities. However, housework activities (OR=0.14; 95%CI 0.04–0.43, p=0.001) and screen time ≥3 h/day (OR=0.09; 95%CI 0.02–0.38, p=0.001) were lower in adolescents with disabilities compared to adolescents without disabilities. Conclusion: Adolescents with disabilities attending a sports NGO were not at higher risk of adverse health-related indicators; despite showing reduced physical function, they reported more physical activity, higher happiness, and less screen time compared to adolescents without disabilities during the COVID-19 pandemic.

Abstract Introduction Seasonal influenza A (H3N2) virus is an important cause of morbidity and mortality in the last 50 years in population that is greater than the impact of H1N1. Data assessing immunogenicity and safety of this virus component in juvenile systemic lupus erythematosus (JSLE) is lacking in the literature. Objective To evaluate short-term immunogenicity and safety of influenza A/Singapore (H3N2) vaccine in JSLE. Methods 24 consecutive JSLE patients and 29 healthy controls (HC) were vaccinated with influenza A/Singapore/ INFIMH-16-0019/2016(H3N2)-like virus. Influenza A (H3N2) seroprotection (SP), seroconversion (SC), geometric mean titers (GMT), factor increase in GMT (FI-GMT) titers were assessed before and 4 weeks post-vaccination. Disease activity, therapies and adverse events (AE) were also evaluated. Results JSLE patients and controls were comparable in current age [14.5 (10.1–18.3) vs. 14 (9–18.4) years, p = 0.448] and female sex [21 (87.5%) vs. 19 (65.5%), p = 0.108]. Before vaccination, JSLE and HC had comparable SP rates [22 (91.7%) vs. 25 (86.2%), p = 0.678] and GMT titers [102.3 (95% CI 75.0–139.4) vs. 109.6 (95% CI 68.2–176.2), p = 0.231]. At D30, JSLE and HC had similar immune response, since no differences were observed in SP [24 (100%) vs. 28 (96.6%), p = 1.000)], SC [4 (16.7%) vs. 9 (31.0%), p = 0.338), GMT [162.3 (132.9–198.3) vs. 208.1 (150.5–287.8), p = 0.143] and factor increase in GMT [1.6 (1.2–2.1) vs. 1.9 (1.4–2.5), p = 0.574]. SLEDAI-2K scores [2 (0–17) vs. 2 (0–17), p = 0.765] and therapies remained stable throughout the study. Further analysis of possible factors influencing vaccine immune response among JSLE patients demonstrated similar GMT between patients with SLEDAI < 4 compared to SLEDAI ≥ 4 ( p = 0.713), as well as between patients with and without current use of prednisone ( p = 0.420), azathioprine ( p = 1.0), mycophenolate mofetil ( p = 0.185), and methotrexate ( p = 0.095). No serious AE were reported in both groups and most of them were asymptomatic (58.3% vs. 44.8%, p = 0.958). Local and systemic AE were alike in both groups ( p > 0.05). Conclusion This is the first study that identified adequate immune protection against H3N2-influenza strain with additional vaccine-induced increment of immune response and an adequate safety profile in JSLE. ( www.clinicaltrials.gov , NCT03540823). H3N2 HN H N (H3N2 5 H1N1 (JSLE literature shortterm short term ASingapore Singapore (HC INFIMH160019/2016H3N2like INFIMH1600192016H3N2like INFIMHHNlike INFIMH 16 0019/2016 like 0019 2016 SP, (SP) SC, (SC) GMT, (GMT) FIGMT FI (FI-GMT postvaccination. postvaccination post vaccination. vaccination post-vaccination activity (AE evaluated 14.5 145 [14. 10.1–18.3 101183 10 1 18 3 (10.1–18.3 vs 9–18.4 9184 (9–18.4 0.448 0448 0 448 21 87.5% 875 87 (87.5% 65.5%, 655 65.5% 65 (65.5%) 0.108. 0108 0.108 . 108 0.108] 22 91.7% 917 91 7 (91.7% 86.2%, 862 86.2% 86 (86.2%) 0.678 0678 678 102.3 1023 102 [102. 95% 95 (95 75.0–139.4 7501394 75 139 1096 109 6 109. 68.2–176.2, 6821762 68.2–176.2 68 176 68.2–176.2) 0.231. 0231 0.231 231 0.231] D30 D 100% 100 (100% 96.6%, 966 96.6% 96 (96.6%) 1.000, 1000 1.000 000 1.000)] [ 16.7% 167 (16.7% 31.0%, 310 31.0% 31 (31.0%) 0.338, 0338 0.338 338 0.338) 162.3 1623 162 [162. 132.9–198.3 13291983 132 198 (132.9–198.3 2081 208 208. 150.5–287.8, 15052878 150.5–287.8 150 287 8 (150.5–287.8) 0.143 0143 143 1.6 [1. 1.2–2.1 1221 (1.2–2.1 1. 1.4–2.5, 1425 1.4–2.5 (1.4–2.5) 0.574. 0574 0.574 574 0.574] SLEDAI2K SLEDAIK 2K K 0–17 017 17 (0–17 0–17, 0.765 0765 765 0.713, 0713 0.713 713 0.713) 0.420, 0420 0.420 420 0.420) 1.0, 1.0 1.0) 0.185, 0185 0.185 185 0.185) 0.095. 0095 0.095 095 0.095) 58.3% 583 58 (58.3 44 44.8% 0.958. 0958 0.958 958 0.958) 0.05. 005 0.05 05 0.05) H3N2influenza HNinfluenza vaccineinduced induced wwwclinicaltrialsgov www clinicaltrials gov NCT03540823. NCT03540823 NCT NCT03540823) H3N (H3N H1N INFIMH160019 2016H3N2like 00192016 0019/201 001 201 (SP (SC (GMT 14. [14 10.1–18. 10118 (10.1–18. 9–18. 918 (9–18. 0.44 044 87.5 (87.5 65.5 (65.5% 010 0.10 91.7 (91.7 86.2 (86.2% 0.67 067 67 102. [102 (9 75.0–139. 750139 13 682176 68.2–176. 023 0.23 23 D3 (100 96.6 (96.6% 1.00 00 1.000) 16.7 (16.7 31.0 (31.0% 033 0.33 33 162. [162 132.9–198. 1329198 (132.9–198. 20 1505287 150.5–287. 15 (150.5–287.8 0.14 014 [1 1.2–2. 122 (1.2–2. 142 1.4–2. (1.4–2.5 057 0.57 57 0–1 01 (0–1 0.76 076 76 071 0.71 71 042 0.42 42 018 0.18 009 0.09 09 58.3 (58. 44.8 0.95 0.0 NCT0354082 INFIMH16001 HNlike 0019201 0019/20 10.1–18 1011 (10.1–18 9–18 (9–18 0.4 04 87. (87. 65. (65.5 0.1 91. (91. 86. (86.2 0.6 06 [10 75.0–139 75013 68217 68.2–176 02 0.2 (10 96. (96.6 16. (16. 31. (31.0 03 0.3 [16 132.9–198 132919 (132.9–198 150528 150.5–287 (150.5–287. 1.2–2 12 (1.2–2 1.4–2 (1.4–2. 0.5 0– (0– 0.7 07 58. (58 44. 0.9 0. NCT035408 INFIMH1600 001920 0019/2 10.1–1 101 (10.1–1 9–1 (9–1 (87 (65. (91 (86. 75.0–13 7501 6821 68.2–17 (1 (96. (16 (31. 132.9–19 13291 (132.9–19 15052 150.5–28 (150.5–287 1.2– (1.2– 1.4– (1.4–2 (0 (5 NCT03540 INFIMH160 00192 0019/ 10.1– (10.1– 9– (9– (8 (65 (86 75.0–1 750 682 68.2–1 (96 (31 132.9–1 1329 (132.9–1 1505 150.5–2 (150.5–28 1.2 (1.2 1.4 (1.4– NCT0354 INFIMH16 10.1 (10.1 (6 75.0– 68.2– (3 132.9– (132.9– 150.5– (150.5–2 (1. (1.4 NCT035 INFIMH1 10. (10. 75.0 68.2 132.9 (132.9 150.5 (150.5– NCT03 75. 68. 132. (132. 150. (150.5 NCT0 (132 (150. (13 (150 (15

Abstract Background Data on post-acute COVID-19 in autoimmune rheumatic diseases (ARD) are scarce, focusing on a single disease, with variable definitions of this condition and time of vaccination. The aim of this study was to evaluate the frequency and pattern of post-acute COVID-19 in vaccinated patients with ARD using established diagnosis criteria. Methods Retrospective evaluation of a prospective cohort of 108 ARD patients and 32 non-ARD controls, diagnosed with SARS-CoV-2 infection (RT-PCR/antigen test) after the third dose of the CoronaVac vaccine. Post-acute COVID-19 (≥ 4 weeks and > 12 weeks of SARS-CoV-2 symptoms) were registered according to the established international criteria. Results ARD patients and non-ARD controls, balanced for age and sex, had high and comparable frequencies of ≥ 4 weeks post-acute COVID-19 (58.3% vs. 53.1%, p = 0.6854) and > 12 weeks post-acute COVID-19 (39.8% vs. 46.9%, p = 0.5419). Regarding ≥ 4 weeks post-acute COVID-19, frequencies of ≥ 3 symptoms were similar in ARD and non-ARD controls (54% vs. 41.2%, p = 0.7886), and this was also similar in > 12 weeks post-acute COVID-19 (68.3% vs. 88.2%, p = 0.1322). Further analysis of the risk factors for ≥ 4 weeks post-acute COVID-19 in ARD patients revealed that age, sex, clinical severity of COVID-19, reinfection, and autoimmune diseases were not associated with this condition (p > 0.05). The clinical manifestations of post-acute COVID-19 were similar in both groups (p > 0.05), with fatigue and memory loss being the most frequent manifestations. Conclusion We provide novel data demonstrating that immune/inflammatory ARD disturbances after third dose vaccination do not seem to be a major determinant of post-acute COVID-19 since its pattern is very similar to that of the general population. Clinical Trials platform (NCT04754698). postacute post acute COVID19 COVID 19 COVID-1 (ARD scarce disease criteria 10 nonARD non SARSCoV2 SARSCoV SARS CoV 2 SARS-CoV- RTPCR/antigen RTPCRantigen RT PCR/antigen PCR antigen test vaccine Postacute Post ( 1 sex 58.3% 583 58 (58.3 vs 531 53 53.1% 0.6854 06854 0 6854 39.8% 398 39 8 (39.8 469 46 9 46.9% 0.5419. 05419 0.5419 . 5419 0.5419) COVID19, 19, 54% 54 (54 412 41 41.2% 0.7886, 07886 0.7886 , 7886 0.7886) 68.3% 683 68 (68.3 882 88 88.2% 0.1322. 01322 0.1322 1322 0.1322) reinfection 0.05. 005 0.05 05 0.05) 0.05, immuneinflammatory immune inflammatory population NCT04754698. NCT04754698 NCT (NCT04754698) COVID1 COVID- SARS-CoV RTPCR PCRantigen 58.3 5 (58. 53.1 0.685 0685 685 39.8 (39. 46.9 0541 0.541 541 (5 41.2 0788 0.788 788 68.3 6 (68. 88.2 0132 0.132 132 00 0.0 NCT0475469 (NCT04754698 58. (58 53. 0.68 068 39. (39 46. 054 0.54 41. 078 0.78 78 68. (68 88. 013 0.13 13 0. NCT047546 (NCT0475469 0.6 06 (3 0.5 07 0.7 7 (6 01 0.1 NCT04754 (NCT047546 NCT0475 (NCT04754 NCT047 (NCT0475 NCT04 (NCT047 NCT0 (NCT04 (NCT0 (NCT

Abstract Objective: To evaluate physical and mental health indicators in adolescents with preexisting chronic immunocompromised conditions during coronavirus disease 2019 (COVID-19) quarantine. Methods: A cross-sectional study included 355 adolescents with chronic conditions and 111 healthy adolescents. An online self-rated survey was used to investigate socio-demographic features, healthcare routine, and the quarantine impact on physical and mental health. The validated self-reported version of the Strengths and Difficulties Questionnaire (SDQ) was also applied. Results: The median of age [14 (10–18) vs. 15 (10–18) years, p = 0.733] and frequencies of female (61% vs. 60%, p = 0.970) were similar between adolescents with preexisting chronic conditions and healthy adolescents during quarantine of COVID-19 pandemic. The frequencies of abnormal total difficulties score of SDQ were similar in patients and controls (30% vs. 31%, p = 0.775). Logistic regression analysis showed that being female (OR = 1.965; 95% CI = 1.091–3.541, p = 0.024), fear of underlying disease activity/complication (OR = 1.009; 95%CI = 1.001–1.018, p = 0.030) were associated with severe psychosocial dysfunction in adolescents with chronic conditions, whereas school homework (OR = 0.449; 95% CI = 0.206–0.981, p = 0.045) and physical activity (OR = 0.990; 95% CI = 0.981–0.999, p = 0.030) were protective factors. Further analysis of patients with chronic immunocompromised conditions and previous diagnosis of mental disorders (9%) compared with patients without diagnosis showed higher median of total difficulties score (p = 0.001), emotional (p = 0.005), conduct (p = 0.007), peer problems (p = 0.001) and hyperactivity (p = 0.034) in the former group. Conclusion: Adolescents with preexisting chronic immunocompromised conditions during COVID-19 quarantine were not at higher risk of adverse health indicators. Being female, fear of underlying disease activity/complication, and household members working outside of the home were relevant issues for adolescents with preexisting chronic conditions. This study reinforces the need to establish mental health strategies for teens with chronic conditions, particularly during the pandemic.

Abstract Objective: To evaluate idiopathic musculoskeletal pain, musculoskeletal pain syndromes, and use of electronic devices in adolescents with asthma and healthy controls. Methods: Cross-sectional study was conducted on 150 asthmatic adolescents and 300 controls. Adolescents completed a self-administered questionnaire regarding painful symptoms, use of electronic devices, and physical activity. Seven musculoskeletal pain syndromes were evaluated, and Asthma Control Test (ACT) was assessed. Results: Musculoskeletal pain (42% vs. 61%, p = 0.0002) and musculoskeletal pain syndromes (2.7% vs. 15.7%, p = 0.0006) were significantly lower in asthmatic adolescents than in controls. The frequency of pain in the hands and wrists was reduced in asthmatic than in controls (12.6% vs. 31.1%, p = 0.004), in addition to cell phone use (80% vs. 93%, p < 0.0001), simultaneous use of at least two electronic media (47% vs. 91%, p < 0.0001), myofascial syndrome (0% vs. 7.1%, p = 0.043), and tendinitis (0% vs. 9.2%, p = 0.008). Logistic regression analysis, including asthma with musculoskeletal pain as the dependent variable, and female sex, ACT > 20, simultaneous use of at least two electronic devices, cell phone use, and weekends and weekdays of cell phone use, as independent variables, showed that female sex (odds ratio [OR], 2.06; 95% confidence interval [CI], 1.929–6.316; p = 0.0009) and ACT ≥ 20 (OR, 0.194; 95% CI, 0.039–0.967; p = 0.045) were associated with asthma and musculoskeletal pain (Nagelkerke R2 = 0.206). Conclusion: Musculoskeletal pain and musculoskeletal pain syndromes were lower in adolescents with asthma. Female sex was associated with musculoskeletal pain in asthmatic, whereas patients with asthma symptoms and well-controlled disease reported a lower prevalence of musculoskeletal pain.

RESUMO Objetivo: Avaliar a dor abdominal intermitente em pacientes com vasculite por IgA e sua relação com dados demográficos, manifestações clínicas e tratamentos. Métodos: Um estudo de coorte retrospectiva incluiu 322 pacientes com vasculite por IgA (critérios EULAR/PRINTO/PRES) em uma Unidade de Reumatologia Pediátrica durante 32 anos. Dezesseis pacientes foram excluídos em razão de dados incompletos. A dor abdominal intermitente foi caracterizada por nova dor abdominal difusa após resolução completa no primeiro mês da doença. Resultados: Dor abdominal intermitente foi observada em 35/306 (11%) dos pacientes com vasculite por IgA. A mediana entre a primeira e a segunda dor abdominal foi 10 dias (3–30 dias). O principal tratamento incluiu glicocorticoide [n=26/35 (74%)] e/ou ranitidina [n=22/35 (63%)]. Análises adicionais mostraram que a frequência da púrpura/petéquia intermitente (37 vs. 21%; p=0,027) e a mediana da duração da púrpura/petéquia [20 (3–90) vs. 14 (1–270) dias; p=0,014] foram significativamente maiores em pacientes com vasculite por IgA com dor abdominal intermitente em comparação com aqueles sem essa condição. Sangramento gastrointestinal (49 vs. 13%; p<0,001), nefrite (71 vs. 45%; p=0,006), uso de glicocorticoides (74 vs. 44%; p=0,001) e de imunoglobulina endovenosa (6 vs. 0%; p=0,036) também foram maiores no primeiro grupo. A frequência do uso de ranitidina foi significativamente maior em pacientes com vasculite por IgA com dor abdominal intermitente versus sem dor (63 vs. 28%; p<0,001), ao passo que a mediana da duração do uso de ranitidina foi reduzida no primeiro grupo [35 (2–90) vs. 60 (5–425) dias; p=0,004]. Conclusões: Dor abdominal intermitente ocorreu em, aproximadamente, um décimo dos pacientes com vasculite por IgA, nos primeiros 30 dias da doença, e foi associada a manifestações clínicas graves. Este estudo sugere, portanto, que esses pacientes devem ser seguidos rigorosamente com avaliação clínica e laboratorial, principalmente durante o primeiro mês da doença.

ABSTRACT Objective: To assess intermittent abdominal pain in IgA vasculitis patients and its relation to demographic data, clinical manifestations and treatments. Methods: A retrospective cohort study included 322 patients with IgA vasculitis (EULAR/PRINTO/PRES criteria) seen at the Pediatric Rheumatology Unit in the last 32 years. Sixteen patients were excluded due to incomplete data in medical charts. Intermittent abdominal pain was characterized by new abdominal pain after complete resolution in the first month of disease. Results: Intermittent abdominal pain was observed in 35/306 (11%) IgA vasculitis patients. The median time between first and second abdominal pain was 10 days (3–30 days). The main treatment of intermittent abdominal pain included glucocorticoid [n=26/35 (74%)] and/or ranitidine [n=22/35 (63%)]. Additional analysis showed that the frequency of intermittent purpura/petechiae (37 vs. 21%; p=0.027) and the median of purpura/petechiae duration [20 (3–90) vs. 14 (1–270) days; p=0.014] were significantly higher in IgA vasculitis patients with intermittent abdominal pain compared to those without. Gastrointestinal bleeding (49 vs. 13%; p<0.001), nephritis (71 vs. 45%; p=0.006), glucocorticoid (74 vs. 44%; p=0.001) and intravenous immunoglobulin use (6 vs. 0%; p=0.036) were also significantly higher in the former group. The frequency of ranitidine use was significantly higher in IgA vasculitis patients with intermittent abdominal pain versus without (63 vs. 28%; p<0.001), whereas the median of ranitidine duration was reduced in the former group [35 (2–90) vs. 60 (5–425) days; p=0.004]. Conclusions: Intermittent abdominal pain occurred in nearly a tenth of IgA vasculitis patients, in the first 30 days of disease, and was associated with other severe clinical features. Therefore, this study suggests that these patients should be followed strictly with clinical and laboratorial assessment, particularly during the first month of disease course.

RESUMO Crianças e adolescentes com deficiência física apresentam desafios motores e socioemocionais que interferem na qualidade de vida relacionada à saúde e os colocam em maior risco de desenvolver doenças secundárias. Além disso, estão mais sujeitos a restrições de serviços adequados que ofereçam terapias, principalmente para a população de baixa renda social. Torna-se necessário oferecer ações voltadas para a promoção da saúde em um sentido mais amplo, oferecendo não só meios de habilitação e reabilitação física, mas também de ganhos sociais e emocionais. Este objetivo pode ser atingido com esportes adaptados e atividades recreativas nos quais o condicionamento físico é acompanhado por ganhos em autoestima e benefícios sociais. Com a pandemia de COVID-19 e o isolamento social, crianças e adolescentes com deficiência física ficaram ainda mais privados de assistência. Relatamos aqui o esforço de uma organização não-governamental esportiva em manter o atendimento físico e psicológico por meio de consultas virtuais, e avaliamos as percepções das partes interessadas no processo. Nível de evidência IV; série de casos .

ABSTRACT Children and adolescents with physical disabilities have motor and social-emotional challenges that interfere with their health-related quality of life and put them at greater risk of developing secondary conditions. Moreover, services that provide them therapies are more likely to be restricted, especially for the low-income population. There must be broader actions towards health promotion, offering not only means for physical habilitation and rehabilitation but for social and emotional improvements as well. This goal is attainable by adaptive sports and recreational activities where physical conditioning is accompanied by an improvement in self-esteem and social benefits. With the COVID-19 pandemic and social isolation, children and adolescents with physical disabilities were even further deprived of assistance. Our aim was to report the efforts of a non-governmental sports organization in maintaining physical and psychological care through virtual consultations and to analyze the perceptions of those affected by the process. Level of evidence IV; case series .

Objetivo: Avaliar violência interpessoal não sexual contra crianças e adolescentes em um hospital universitário terciário. Métodos: Foi realizado um estudo transversal com 240 pacientes em situação de violência não sexual por 15 anos. A análise incluiu: dados demográficos, local de referência do hospital, tipo e autor da violência, encaminhamento legal, exames laboratoriais e de imagem, e desfechos graves. Resultados: Situações de violência não sexual foram diagnosticadas em 240 de 295.993 (0,1%) pacientes durante os 15 anos do período: 148/240 (61,7%) em crianças e 92/240 (38,3%) em adolescentes. Os tipos de violências mais frequentes foram negligência em 156/240 (65,0%), violência física 62/240 (25,8%), agressão psicológica/emocional 52/240 (21,7%), síndrome de Münchausen por procuração 4/240 (1,7%) e bullying/cyberbullying em 3/240 (1,3%). As condições crônicas pediátricas mais frequentes foram: doença renal crônica 24/123 (19,5%), vírus da imunodeficiência humana 14/123 (11,4%), prematuridade 9/123 (7,3%), paralisia cerebral 8/123 (6,5%) e asma 8/123 (6,5%). Comparações entre crianças e adolescentes em situação de violência não sexual revelaram que houve diferença significativa entre os locais de referência do hospital. A frequência de pacientes sob violência encaminhados de ambulatórios foi significantemente reduzida em crianças versus adolescentes (27,7 vs. 62%), enquanto encaminhamentos de pronto-socorro foram mais numerosos no primeiro grupo (57,4 vs. 25%, p<0,001). Os tipos de violências e condições pediátricas crônicas foram semelhantes nos dois grupos(p>0,05). Conclusões: Violência não sexual na nossa população pediátrica foi raramente diagnosticada em um hospital terciário, principalmente negligência, agressão física e psicológica/emocional. Aproximadamente dois terços dos diagnósticos de violência ocorreram em crianças, preferencialmente encaminhadas pelo departamento de emergência. Em contrapartida, cerca de um terço dos diagnósticos de violência ocorreu em adolescentes, encaminhados principalmente pelos ambulatórios.

Abstract Objective: The objective of this study was to assess interpersonal nonsexual violence against children and adolescents in a tertiary university hospital. Methods: A cross-sectional study was performed in 240 patients under nonsexual violence situation for 15 consecutive years. Data analyses included demographic data, hospital referral site, type and author of nonsexual violence, legal referral, laboratorial and imaging examinations, and outcomes. Results: Nonsexual violence situation was diagnosed in 240 (0.1%) of 295,993 patients for 15 years: 148 (61.7%) in children and 92 (38.3%) in adolescents. Out of 240, the most frequent types of violence were negligence in 156 (65.0%), physical 62 (25.8%), psychological/emotional aggression 52 (21.7%), Munchausen by proxy syndrome 4 (1.7%), and bullying/cyberbullying in 3 (1.3%). Out of 123, the most common pediatric chronic conditions were chronic kidney disease 24 (19.5%), human immunodeficiency virus 14 (11.4%), prematurity 9 (7.3%), cerebral palsy 8 (6.5%), and asthma 8 (6.5%). Further comparison between children versus adolescent under nonsexual violence situation revealed significant difference between the hospital referral sites. The frequency of patients under violence referred from outpatient clinics was significantly reduced in children versus adolescents (27.7 vs. 62%), whereas emergency department was higher in the former group (57.4 vs. 25.0%; p<0.001). All types of violence situations and pediatric chronic conditions were similar in both groups (p>0.05). Conclusions: Nonsexual violence against our pediatric population was rarely diagnosed in a tertiary hospital, mainly negligence, physical, and psychological/emotional aggression. Approximately two-thirds of violence diagnosis occurred in children, referred mainly by the emergency department. In contrast, approximately one-third of violence diagnosis occurred in adolescents, referred mostly by outpatient clinics.

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.

ABSTRACT This study aims to assess COVID-19 and other respiratory viruses in pediatric patients. Between April 17 and September 30, 2020, we collected 1,566 respiratory samples from 1,044 symptomatic patients who were younger than 18 years old to assess SARS-CoV-2 infection. Of these, 919 were analyzed for other respiratory pathogens (ORP). Patients with laboratory-confirmed COVID-19 or ORP were included. We evaluated 76 pediatric COVID-19 infections and 157 other respiratory virus infections. Rhinovirus occurred in 132/157 (84%). COVID-19 patients who were significantly older, had more fevers, headaches and pneumonia than those with ORP. The median white blood cell count was lower in patients with SARS-CoV-2 than in those with ORP (6,470 versus 8,170; p=0.02). COVID-19 patients had significantly worse symptoms than those with ORP.