One-year prospective nerve conduction study of thalidomide neuropathy in lupus erythematosus: Incidence, coasting effect and drug plasma levels.

BACKGROUND: Few prospective studies in cutaneous and systemic lupus erythematosus (CLE/SLE) assessed thalidomide-induced peripheral neuropathy (TiPN) incidence/reversibility, and most have not excluded confounding causes neither monitored thalidomide plasma levels. OBJECTIVES: To evaluate TiPN incidence/reversibility, coasting effect and its association with thalidomide plasma levels in CLE/SLE. METHODS: One-year prospective study of thalidomide in 20 CLE/SLE patients without pregnancy potential, with normal nerve conduction study (NCS), and excluded other PN causes. Thalidomide levels were determined by high-performance liquid chromatography/tandem mass spectrometry. RESULTS: Twelve patients (60%) developed TiPN: 33.3% were symptomatic and 66.6% asymptomatic. Half of this latter group developed coasting effect (TiPN symptoms 1-3 months after drug withdrawal). The main predictive factors for TiPN were treatment duration ≥6 months (p = 0.025) and cumulative dose (p = 0.023). No difference in plasma thalidomide levels between patients with/without TiPN was observed (p = 0.464). After drug withdrawal, 75% symptomatic TiPN patients improved their symptoms. Seven TiPN patients underwent an additional NCS after drug withdrawal: 42.8% worsened NCS, 14.2% was stable, and 42.8% had improved NCS. CONCLUSION: Our data provides novel evidence of coasting effect in half of asymptomatic patients with TiPN. The irreversible nature of this lesion in 25% of TiPN patients reinforces the relevance of early NCS monitoring, and suggests thalidomide use solely as a bridge for other effective therapy for refractory cutaneous lupus patients.

Safety and immunogenicity of the quadrivalent human papillomavirus vaccine in patients with childhood systemic lupus erythematosus: a real-world interventional multi-centre study.

OBJECTIVE: This study aimed to assess the safety and immunogenicity of the quadrivalent human papillomavirus (qHPV) vaccination in childhood-onset systemic lupus erythematosus (cSLE) patients. METHODS: Volunteer cSLE patients aged 9-20 years and healthy controls (HC) were enrolled to receive a two- or three-dose qHPV vaccination schedule from March 2014 to March 2016. Study visits were performed before the first dose, one month after the second and third doses and one year after the first dose. In each study visit, disease activity and adverse events following vaccination were analyzed, and a serum sample was collected for testing antibody concentrations. Participant recruitment was conducted in 15 Brazilian paediatric rheumatology units. Of the 256 cSLE patients included, 210 completed the two- or three-dose schedules; 15 had previously received one dose, and 18 had received two doses of the vaccine. The analysis was based on intention-to-treat so that participants who did not complete the entire study protocol were also included. RESULTS: No severe adverse events were related to the vaccination. Disease activity was generally low and remained stable or even improved. The HC presented 100% seropositivity to HPV16 and HPV18, whereas the two- and three-dose cSLE groups presented 93% and 83% versus 97% and 91%, respectively. One year after the first dose, seropositivity of the three-dose cSLE group was 91% to HPV16 and 84% to HPV18. CONCLUSIONS: HPV vaccination in cSLE patients is safe and immunogenic. Since the seropositivity to HPV16 and HPV18 was higher for the three-dose schedule group, this regimen should be recommended for cSLE patients.

Influence of air pollution on renal activity in patients with childhood-onset systemic lupus erythematosus.

BACKGROUND: Childhood-onset systemic lupus erythematosus (cSLE) is an autoimmune and multifactorial disease that can affect the renal system. Exposure to air pollution can trigger systemic inflammation in cSLE patients and increase risk of disease activity. We evaluated effects of individual real-time exposure to air pollutants on renal activity in cSLE patients using the Systemic Lupus Erythematosus Disease Activity Index 2000. METHODS: Longitudinal panel study of 108 repetitive measures from 9 pediatric lupus patients. Over three consecutive weeks, daily individual levels of fine particulate matter (PM2.5) and nitrogen dioxide (NO2) were measured, as well as weekly clinical evaluation and laboratory tests. This was repeated every 10 weeks over a 1-year period. Specific generalized estimating equation models were used to evaluate the impact of these pollutants on risk of nephritis and anti-dsDNA > 20 UI/mL and on 24-h urine protein and serum complement (C3) levels. RESULTS: An interquartile range (IQR) increase of 18.12 µg/m3 in PM2.5 daily concentration was associated with increased risk of nephritis and positive results for anti-dsDNA. Moreover, increase in 24-h urine protein and decrease in C3 serum levels also associated with exposure to pollutants. An IQR increase in PM2.57-day moving average was associated with increased risks of leukocyturia (3.4; 95% CI 2.6:4.3), positive anti-dsDNA (3.1; 95% CI 2.1:4.0), and 36.3-mg increase (95% IC 20.2:52.3) in 24-h urine protein. An IQR increase (63.1 µg/m3) in 7-day cumulative NO2 levels was associated with decreased serum C3 levels. CONCLUSIONS: This prospective study suggests exposure to air pollution can trigger renal activity in cSLE patients.

Description of Malignancy Rates in Childhood- and Adult-Onset Systemic Lupus Erythematous by Proportional Meta-analysis.

OBJECTIVE: Describe malignancy rates in childhood onset and adult onset systemic lupus erythematous (SLE) by proportional meta-analysis. METHODS: Two reviewers screened data from PubMed (1966-2015), EMBASE (1980-2015), and LILACS (1982-2015) for SLE-associated malignancy. Proportional meta-analysis with a random-effects model and 95% confidence intervals (CIs) were calculated according to SLE onset age and mean follow-up time. Statistical difference was defined by 95% CI overlap. RESULTS: Overall the malignancy rate reported in 30 case series with 96,578 subjects was 3.4% (95% CI, 0.0260-0.0442; I = 97.6%; P < 0.0001). The malignancy rate was 4.2% (95% CI, 0.0318-0.0531; I = 98%; P < 0.0001) in 25 adult-onset SLE series, compared with 0.5% (95% CI, 0.0003-0.0154; I = 62.6%; P = 0.03) in 5 childhood-onset SLE series. Overall, in those with less than 5 years' follow-up, the malignancy rate was 2.8% (95% CI, 0.013-0.047; I = 91%; P < 0.0001) compared with 3.6% (95% CI, 0.0226-0.0531; I = 98.3%; P < 0.0001) in those with more than 5 years' follow-up, which was not significant, with 95% CI overlap. CONCLUSIONS: The meta-analysis indicated lower malignancy rates in pediatric-onset SLE compared with adult-onset SLE, but accrued data from childhood-onset SLE are still needed.

Childhood-onset systemic lupus erythematosus (cSLE) and malignancy: a nationwide multicentre series review.

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.

Robust immunogenicity to the H3N2 component of influenza A vaccine in primary Sjögren syndrome.

INTRODUCTION: Influenza A (H3N2) virus is the major cause of morbidity/mortality due to seasonal influenza over 50 years. Data about the safety/immunogenicity of influenza A/Singapore (H3N2) vaccine are scarce in primary Sjögren syndrome (pSS). METHODS: Twenty-one consecutive pSS patients and 42 HC (healthy control individuals) were immunized with influenza A/Singapore/INFIMH-16-0019/2016 (H3N2)-like virus. Rates of SP (seroprotection) and SC (seroconversion), GMT (geometric mean titers), FI-GMT (factor increase in GMT), ESSDAI (EULAR Sjögren's Syndrome Disease Activity Index), and adverse events were appraised before and 4 weeks post-vaccination. RESULTS: pSS and HC had similar mean age (51.2 ± 14.2 vs. 50.6 ± 12.1 years, p = 0.886). Pre-vaccination SP rates were high in pSS and HC (90.5% vs. 71.4%, p = 0.114), and GMT were higher in pSS [80.0 (52.4-160.0) vs. 40.0 (20.0-80.0), p = 0.001]. The percentage of influenza vaccination in the preceding two years was elevated and similar in pSS and HC (94.1% vs. 94.6%, p = 1.000). GMT values augmented in both groups four weeks after vaccination and persisted higher in the first group [160.0 (80.0-320.0) vs. 80.0 (40.0-80.0), p < 0.001] with equivalent FI-GMT [1.4 (1.0-2.8) vs. 1.4 (1.0-2.0), p = 0.410]. Both groups had low and similar SC rates (19.0% vs. 9.5%, p = 0.423). ESSDAI values persisted steadily during the study (p = 0.313). No serious adverse events have occurred. CONCLUSION: The novel demonstration that the influenza A/Singapore (H3N2) vaccine induces a different pattern of immunogenicity from other influenza A constituents in pSS, featured by a desirable high pre- and post-vaccination immunogenicity, is in line with reported differences in immune responses between strains in trivalent vaccines and may be related to pre-existing immunity. CLINICALTRIALS: gov: #NCT03540823. Key Points • This prospective study demonstrated a robust pre- and post-vaccination immunogenicity to influenza A/Singapore/INFIMH-16-0019/2016 (H3N2)-like virus in primary Sjögren's syndrome (pSS). • This high immunogenicity pattern may be related to pre-existing immunization, or else it is related to immunogenicity differences of each strain. • This vaccine had an adequate safety profile in pSS, with no impact on disease activity.

TELEHEALTH FOR CHILDREN AND ADOLESCENTS WITH PHYSICAL DISABILITIES DURING THE COVID-19 PANDEMIC.

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 .

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 .

Inactivated SARS-CoV-2 vaccine in primary Sjögren's syndrome: humoral response, safety, and effects on disease activity.

INTRODUCTION: There is no study specifically focused on SARS-CoV-2 vaccine in primary Sjögren's syndrome (pSS). OBJECTIVES: To assess the immunogenicity, safety, possible effects on disease activity, and autoantibody profile of the Sinovac-CoronaVac vaccine in pSS. METHODS: Fifty-one pSS patients and 102 sex- and age-balanced controls without autoimmune diseases were included in a prospective phase 4 trial of the Sinovac-CoronaVac vaccine (two doses 28 days apart, D0/D28). Participants were assessed in three face-to-face visits (D0/D28 and six weeks after the 2nd dose (D69)) regarding adverse effects; clinical EULAR Sjögren's Syndrome Disease Activity Index (clinESSDAI); anti-SARS-CoV-2 S1/S2 IgG (seroconversion (SC) and geometric mean titers (GMT)); neutralizing antibodies (NAb); and pSS autoantibody profile. RESULTS: Patients and controls had comparable female sex frequency (98.0% vs. 98.0%, p = 1.000) and mean age (53.5 ± 11.7 vs. 53.4 ± 11.4 years, p = 0.924), respectively. On D69, pSS patients presented moderate SC (67.5% vs. 93.0%, p < 0.001) and GMT (22.5 (95% CI 14.6-34.5) vs. 59.6 (95% CI 51.1-69.4) AU/mL, p < 0.001) of anti-SARS-CoV-2 S1/S2 IgG but lower than controls, and also, moderate NAb frequency (52.5% vs. 73.3%, p = 0.021) but lower than controls. Median neutralizing activity on D69 was comparable in pSS (58.6% (IQR 43.7-63.6)) and controls (64% (IQR 46.4-81.1)) (p = 0.219). Adverse events were mild. clinESSDAI and anti-Ro(SS-A)/anti-La(SS-B) levels were stable throughout the study (p > 0.05). CONCLUSION: Sinovac-CoronaVac vaccine is safe in pSS, without a deleterious impact on disease activity, and has a moderate short-term humoral response, though lower than controls. Thus, a booster dose needs to be studied in these patients. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT04754698. Key Points • Sinovac-CoronaVac vaccine is safe in pSS, without a detrimental effect on systemic disease activity, and has a moderate short-term humoral response • A booster dose should be considered in these patients.

SARS-CoV-2 and rhinovirus infections: are there differences in clinical presentation, laboratory abnormalities, and outcomes in the pediatric population?

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.

Late response to anti-TNF-α therapy in refractory mucocutaneous lesions of Behçet's disease.

Behçet's disease (BD) is a multisystem chronic inflammatory disorder characterized by oral and genital ulceration and ocular involvement. Recurrent oral and genital ulcers are the most common symptoms of BD and occur in more than 80% of patients. The treatments of these disease manifestations include colchicine, corticosteroids and immunosuppressive drugs in severe cases. Anti-TNF-α therapy may be useful in refractory severe BD, particularly for ocular, central nervous system, gastrointestinal and refractory mucocutaneous lesions. During a 2-year period, 280 patients suffering from rheumatic diseases received anti-TNF-α agents at the infusion center of our University Hospital. Two patients (0.7%) presented BD; one of them had celiac disease as well, with recalcitrant mucocutaneous lesions that were not responsive to immunosuppressive drugs. We reported those patients who were successfully treated with infliximab and adalimumab, despite their late response.

Gaps on rheumatologists' knowledge of physical activity.

To assess the rheumatologists' knowledge and willingness to prescribe physical activity, we conducted a nationwide survey. All adult and paediatric rheumatologist members of the Brazilian Rheumatology Society were invited to fulfil a questionnaire on their knowledge and willingness to promote physical activity. Four hundred twenty-eight rheumatologists participated in the survey, representing approximately 25% of the society's members. Forty-five percent of the rheumatologists reported having had training to prescribe physical activity, and 68% believe that physical activity is a part of patients' treatment. Most reported assessing physical activity levels (86%) and recommending physical activity (98%) always or most of the time. However, 48% do not know the minimum physical activity recommendations for health maintenance, nor do they know how much vigorous activity should be done in replacement of moderate activity. In addition, only 20% are aware of physical activity recommendation for paediatric patients, whereas 31% know that very light-intensity physical activity promotes health-related beneficial effects. Year of graduation, having been trained on physical activity prescription, and regularly recommending physical activity to patients did not associate with the overall score of correct answers (OR: 1.00 [0.99, 1.02], p = 0.391; OR: 0.99 [0.69, 1.44], p = 983; OR: 0.90 [0.61, 1.32], p = 0.576; respectively). Rheumatologists are highly willing to promote physical activity, but major gaps in their knowledge were identified. Given the widespread recognition of physical activity as a key element on the management of rheumatic patients, these data hint to the need of incorporating physical activity in the rheumatologist' training. Key Points • This survey showed that rheumatologists are highly prone to promote physical activity. • However, rheumatologists have major gaps in knowledge of physical activity. • Adding physical activity to rheumatologist's training programs is key to improve physical activity promotion.

Interleukin-1 receptor antagonist gene (IL1RN) polymorphism possibly associated to severity of rheumatic carditis in a Brazilian cohort.

AIMS: To evaluate the IL1RN polymorphism as a possible marker for Rheumatic Fever (RF) susceptibility or disease severity. METHODS: The genotypes of 84 RF patients (Jones criteria) and 84 normal race-matched controls were determined through the analysis of the number of 86-bp tandem repeats in the second intron of IL1RN. The DNA was extracted from peripheral-blood leukocytes and amplified with specific primers. Clinical manifestations of RF were obtained through a standardized questionnaire and an extensive chart review. Carditis was defined as new onset cardiac murmur that was perceived by a trained physician with corresponding valvae regurgitation or stenosis on echocardiogram. Carditis was classified as severe in the presence of congestive heart failure or upon the indication for cardiac surgery. The statistical association among the genotypes, RF and its clinical variations was determined. RESULTS: The presence of allele 1 and the genotype A1/A1 were found less frequently among patients with severe carditis when compared to patients without this manifestation (OR=0.11, p=0.031; OR=0.092, p=0.017). Neither allele 1 nor allele 2 were associated with the presence of RF (p=0.188 and p=0.106), overall carditis (p=0.578 and p=0.767), polyarthritis (p=0.343 and p=0.313) and chorea (p=0.654 and p=0.633). CONCLUSION: In the Brazilian population, the polymorphism of the IL-1ra gene is a relevant factor for rheumatic heart disease severity.

Comparison of urinary parameters, biomarkers, and outcome of childhood systemic lupus erythematosus early onset-lupus nephritis.

BACKGROUND: Urinary parameters, anti-dsDNA antibodies and complement tests were explored in patients with childhood-Systemic Lupus Erythematosus (cSLE) early-onset lupus nephritis (ELN) from a large multicenter cohort study. METHODS: Clinical and laboratory features of cSLE cases with kidney involvement at presentation, were reviewed. Disease activity parameters including SLEDAI-2 K scores and major organ involvement at onset and follow up, with accrued damage scored by SLICC-DI, during last follow up, were compared with those without kidney involvement. Autoantibodies, renal function and complement tests were determined by standard methods. Subjects were grouped by presence or absence of ELN. RESULTS: Out of the 846 subjects enrolled, mean age 11.6 (SD 3.6) years; 427 (50.5%) had ELN. There was no significant difference in the ELN proportion, according to onset age, but ELN frequency was significantly higher in non-Caucasians (p = 0.03). Hematuria, pyuria, urine casts, 24-h proteinuria and arterial hypertension at baseline, all had significant association with ELN outcome (p < 0.001). With a similar follow up time, there were significantly higher SLICC-DI damage scores during last follow up visit (p = 0.004) and also higher death rates (p < 0.0001) in those with ELN. Low C3 (chi-square test, p = 0.01), but not C3 levels associated significantly with ELN. High anti-dsDNA antibody levels were associated with ELN (p < 0.0001), but anti-Sm, anti-RNP, anti-Ro, anti-La antibodies were not associated. Low C4, C4 levels, low CH50 and CH50 values had no significant association. High erythrocyte sedimentation rate (ESR) was associated with the absence of ELN (p = 0.02). CONCLUSION: The frequency of ELN was 50%, resulting in higher morbidity and mortality compared to those without ELN. The urinary parameters, positive anti-dsDNA and low C3 are reliable for discriminating ELN.

Childhood-onset systemic lupus erythematosus (cSLE) and malignancy: a nationwide multicentre series review

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.

Sexuality in teenagers with epilepsy.

OBJECTIVES: In adults with epilepsy it is well known that the epileptic syndrome, seizure frequency and antiepileptic drug use may influence sexual function and behavior. However, knowledge acquired with adult populations has been extrapolated to teenagers, based on the supposition that these patients are influenced by similar factors. This study aimed to evaluate aspects related to sexuality obtained from female adolescents with epilepsy. METHODS: We carried out a prospective study of 35 female adolescents, with epilepsy, with ages from 10 to 20 years, and epileptic syndromes diagnosed according to ILAE criteria (1989). Information on sexual function and behavior of adolescents with epilepsy was evaluated by use of a standard questionnaire. Exclusion criteria were lack of menarche, previous endocrine or chronic clinical disorders, and moderate to severe mental retardation. RESULTS: No differences were observed between the age at first sexual intercourse, sexual activity, libido and orgasm of adolescents with epilepsy when compared to controls. Epilepsy clinical variables such as age of onset, duration and severity had no significant relationship with distinct aspects of sexual function and behavior. CONCLUSION: Adolescents with epilepsy represent a special patient group because, even with their chronic disorder, they have an active sexual life, despite the severity of their disorder. Therefore, aspects related to sexuality require special attention by health professionals when attending to adolescents with epilepsy.

[Musculoskeletal involvement as a first manifestation of neoplasm disease]. / Comprometimento musculoesquelético como primeira manifestação de neoplasias.

OBJECTIVE: To describe the clinical and laboratory findings that contributed for the diagnosis of neoplasia in patients with musculoskeletal symptoms at presentation. METHODS: Retrospective analysis of medical records from patients with final diagnosis of neoplasia attended at the "Unidade de Reumatologia do Instituto da Criança - FMUSP" between January 1983 and December 2006. Data on musculoskeletal complaints, clinical examination, laboratory tests, radiological studies and diagnostic procedures were obtained. RESULTS: From 4876 patients, 25 (0.5%) children were studied (52% with acute lymphoid leukemia and 24% with neuroblastoma). Twenty children (80%) presented arthritis and/or arthralgia at onset of the disease. All patients presented systemic symptoms, such as fever (22 cases - 88%). The initial blood cell count was abnormal in 16 patients (64%), showing anemia and thrombocytopenia (12 and 5 cases, respectively). Blast cells were present in only two patients and eleven patients developed blood cell count abnormalities during follow-up. X-ray studies showed abnormalities in 11/14 patients, ultrasound in 12/18, scintigraphy in 5/5, CT in 7/9 and MRI in 3/3. Bone marrow smear was abnormal in 18/22 patients, but in three of them the abnormalities were not detected by the first test. CONCLUSION: Musculoskeletal symptoms are common at the onset of neoplasia, especially for acute lymphoid leukemia, and this possibility should be considered in the differential diagnosis of rheumatic diseases. Laboratory tests may be normal at the onset of the disease, therefore serial exams should be performed. For a correct diagnosis. radiological studies and bone marrow aspiration have proven to be essential.

Risk Factors Associated with Juvenile Idiopathic Arthritis: Exposure to Cigarette Smoke and Air Pollution from Pregnancy to Disease Diagnosis.

OBJECTIVE: To evaluate exposure to environmental factors inhaled during pregnancy and after birth until juvenile idiopathic arthritis (JIA) diagnosis among residents of a large city. METHODS: This is an exploratory case-control study that consists of 66 patients with JIA and 124 healthy controls matched by age and sex, living in the São Paulo, Brazil, metropolitan area until JIA diagnosis, and whose mothers had resided in this region during pregnancy. A structured and reliable questionnaire (κ index for test-retest was 0.80) assessed demographic data, gestational and perinatal-related factors, and exposure to inhalable environmental elements during pregnancy and after birth (occupational exposure to inhalable particles and/or volatile vapor, exposure to cigarette smoke, and the presence of industrial activities or gas stations near the home, work, daycare, or school). Tropospheric pollutants included particulate matter (PM10), sulfur dioxide (SO2), nitrogen dioxide (NO2), ozone (O3), and carbon monoxide (CO). RESULTS: During pregnancy, intrauterine cigarette smoke exposure (OR 3.43, 95% CI 1.45-8.12, p = 0.005) and maternal occupational exposure (OR 13.69, 95% CI 4.4-42.3, p < 0.001) were significant independent risk factors for JIA diagnosis. In contrast, maternal employment (OR 0.06, 95% CI 0.02-0.2, p < 0.001) and ideal maternal weight gain (OR 0.36, 95% CI 0.2-0.8, p = 0.017) presented negative associations. Secondhand smoke exposure from birth to JIA diagnosis (OR 3.6, 95% CI 1.8-7.3, p < 0.001) and exposure to O3 during the second year of life (OR 2.76, 95% CI 1.20-6.37, p = 0.017) were independent and significant risk factors for the pathogenesis of JIA. CONCLUSION: In our study, cigarette smoke exposure (intrauterine and after birth), exposure to O3 in the second year of life, and maternal occupational exposure were identified as potential risk factors for JIA, warranting further study.

Care delivery for the child to grow up despite the pain: the family's experience.

This study aimed to understand the meaning of the experience of families having a child experiencing pain due to Juvenile Rheumatoid Arthritis and to construct a theoretical model representing this experience. Grounded Theory and Symbolic Interactionism were used as methodological framework and theoretical framework, respectively. Data were collected by semistructured interviews with 12 families. Data analysis allowed for the construction of the theoretical model Caring for the child to grow despite the pain, which describes an experience based on motivational elements: wanting to see the child without pain and wanting to see the child live a normal life, reviewing how the family lives the transition in its development cycles, retaking and integrating them in the family dynamic with the appearance of the disease and pain in the child. This theoretical model provides a framework for teaching, research and care, permitting advances in terms of theoretical nursing knowledge.

Telehealth for children and adolescents with physical disabilities during the covid-19 pandemic / Teleatendimento a crianças e adolescentes com deficiências físicas durante a pandemia covid-19

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 .

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 .

SARS-CoV-2 and rhinovirus infections: are there differences in clinical presentation, laboratory abnormalities, and outcomes in the pediatric population?

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.