JULIANA CAIRES DE OLIVEIRA ACHILI FERREIRA
Projetos de Pesquisa
Unidades Organizacionais
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina
8 resultados
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- Echocardiographic study of juvenile dermatomyositis patients: new insights from speckle-tracking-derived strain(2021) DINIZ, Maria de Fatima Rodrigues; KOZU, Katia Tomie; ELIAS, Adriana Maluf; LIANZA, Alessandro Cavalcanti; SAWAMURA, Karen Saori Shiraishi; MENEZES, Carolina Rocha Brito; FERREIRA, Juliana Caires de Oliveira Achili; SILVA, Clovis Artur; LEAL, Gabriela NunesObjectives This study aimed to investigate subclinical left ventricle (LV) systolic dysfunction in juvenile dermatomyositis (JDM) using two-dimensional speckle-tracking echocardiography (2DST). Possible associations between LV deformation impairment and disease activity/cumulative damage were also evaluated. Methods Thirty-fiveconsecutive JDM patients without cardiac symptoms and 35 healthy volunteers were enrolled. Clinical data were collected from medical records, and echocardiograms were performed by a pediatric cardiologist, unaware of patients' conditions. Results Patients and controls had similar age (12.6 +/- 0.7 vs.12.5 +/- 0.6;p= 0.97) and gender (11F:24M vs.11F:24M;p= 1.0). Median of JDM duration was 4.6 (0.04-17.6) years, and only 6/35 (17%) had active disease (disease activity score (DAS > 3)). Conventional echocardiogram revealed preserved LV ejection fraction (EF) (>= 55%) in all individuals. In JDM, 2DST identified reduction of LV longitudinal [-22(-17.2 to -27.9) % vs. -23(-20.8 to -27.4) %;p= 0.028)] and circumferential -23.9 +/- 2.8% vs. -26.7 +/- 2.9%;p= 0.0002) strain. Lower longitudinal strain was associated with DAS >3 -19.9(-17.2 to -26.5)% vs. -22.1-18.9 to -27.9)%;p= 0.046], MDI extent > 0 [-19(-17.2 to -22.5)% vs. -22.1-19.2 to -27.9)%;p= 0.0008], MDI severity > 0 [-19(-17.2 to -22.1)% vs. -22.3(-20.3 to -27.9)%;p= 0.0001] and calcinosis[-20.6(-17.2 to -23)% vs. -22.3(-20.3 to -27.9)%;p= 0.03]. Lower circumferential strain was associated with MDI extent > 0 (-22.1 +/- 3.87% vs. -24.4 +/- 2.3%;p= 0.039), MDI severity > 0 (-21.7 +/- 3% vs. 24.7 +/- 2.3%;p= 0.004) and calcinosis (-22.5 +/- 3.3% vs. -24.8 +/- 2.1%;p= 0.02). There was a negative correlation between longitudinal strain and cumulative dose of prednisone (r= -0.44;p= 0.009) and methotrexate (r= -0.33;p= 0.0008). Conclusions LV 2DST detected early systolic myocardial compromise in asymptomatic pediatric JDM patients, with preserved EF. Longitudinal strain impairment was associated with disease activity and cumulative damage, whereas circumferential strain impairment was associated exclusively with cumulative damage.
- Health-related quality of life and functionality in primary caregiver of surviving pediatric COVID-19(2023) MARTINS, Fernanda; GONCALVES, Fernanda T.; IMAMURA, Marta; BARBOZA, Daniela S.; MATHEUS, Denise; PEREIRA, Maria Fernanda B.; MARQUES, Heloisa H. S.; CORREA-SILVA, Simone; MONTENEGRO, Marilia M.; FINK, Thais T.; LINDOSO, Livia; BAIN, Vera; FERREIRA, Juliana C. O. A.; ASTLEY, Camilla; MATSUO, Olivia M.; SUGUITA, Priscila; TRINDADE, Vitor; PAULA, Camila S. Y.; LITVINOV, Nadia; PALMEIRA, Patricia; GUALANO, Bruno; DELGADO, Artur F.; CARNEIRO-SAMPAIO, Magda; FORSAIT, Silvana; ODONE-FILHO, Vicente; ANTONANGELO, Leila; BATTISTELLA, Linamara R.; SILVA, Clovis A.ObjectivesTo prospectively assess health-related quality of life (HRQoL), global functionality, and disability in primary caregivers of surviving children and adolescents after COVID-19. MethodsA longitudinal observational study was carried out on primary caregivers of surviving pediatric post-COVID-19 patients (n = 51) and subjects without COVID-19 (n = 60). EuroQol five-dimension five-level questionnaire (EQ-5D-5L) and 12-question WHO Disability Assessment Schedule 2.0 (WHODAS 2.0) were answered for both groups. The univariate regression analysis was carried out using SPSS (v 20) and significance was established at 5%. ResultsThe median duration between COVID-19 diagnosis in children and adolescents and longitudinal follow-up visits was 4.4 months (0.8-10.7). The median age of children and adolescents caregivers with laboratory-confirmed COVID-19 was similar to primary caregivers of subjects without laboratory-confirmed COVID-19 [43.2 (31.6-60.9) vs. 41.5 (21.6-54.8) years, p = 0.08], as well as similar female sex (p = 1.00), level of schooling (p = 0.11), social assistance program (p = 0.28), family income/month U$ (p = 0.25) and the number of household's members in the residence (p = 0.68). The frequency of slight to extreme problems (level & GE; 2) of the pain/discomfort domain according to EQ-5D-5L score was significantly higher in the former group [74% vs. 52.5%, p = 0.03, OR = 2.57 (1.14-5.96)]. The frequency of disability according to WHODAS 2.0 total score was similar to those without disability and unknown (p = 0.79); however, with a very high disability in both groups (72.5% and 78.3%). Further analysis of primary caregivers of children and adolescents with post-COVID-19 condition (PCC) [n = 12/51 (23%)] compared to those without PCC [n = 39/51(77%)] revealed no differences between demographic data, EQ-5D-5L and WHODAS 2.0 scores in both groups (p > 0.05). ConclusionWe longitudinally demonstrated that pain/discomfort were predominantly reported in approximately 75% of primary caregiver of COVID-19 patients, with high disability in approximately three-quarters of both caregiver groups. These data emphasized the prospective and systematic caregiver burden evaluation relevance of pediatric COVID-19.
- Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study(2019) VERDIER, Monica; ANUARDO, Pedro; GORMEZANO, Natali Weniger Spelling; ROMITI, Ricardo; CAMPOS, Lucia Maria Arruda; AIKAWA, Nadia Emi; PEREIRA, Rosa Maria Rodrigues; TERRERI, Maria Teresa; MAGALHAES, Claudia Saad; FERREIRA, Juliana C. O. A.; SILVA, Marco Felipe Castro; FERRIANI, Mariana; SAKAMOTO, Ana Paula; FERRIANI, Virginia Paes Leme; CENTEVILLE, Maraisa; SATO, Juliana; SANTOS, Maria Carolina; BONFA, Eloisa; SILVA, Clovis ArturObjective: 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 = 0297], showed higher frequencies of constitutional involvement (67% vs. 1 0%,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.
- Safety and immunogenicity of the tetravalent, live-attenuated dengue vaccine Butantan-DV in adults in Brazil: a two-step, double-blind, randomised placebo-controlled phase 2 trial(2020) KALLAS, Esper G.; PRECIOSO, Alexander Roberto; PALACIOS, Ricardo; THOME, Beatriz; BRAGA, Patricia Emilia; VANNI, Tazio; CAMPOS, Lucia M. A.; FERRARI, Lilian; MONDINI, Gabriella; SALOMAO, Maria da Graca; SILVA, Anderson da; ESPINOLA, Heloisa M.; SANTOS, Joane do Prado; SANTOS, Cecilia L. S.; TIMENETSKY, Maria do Carmo S. T.; MIRAGLIA, Joao Luiz; GALLINA, Neuza M. F.; WEISKOPF, Daniela; SETTE, Alessandro; GOULART, Raphaella; SALLES, Rafael Tavares; MAESTRI, Alvino; SALLUM, Adriana Maluf Elias; FARHAT, Sylvia Costa Lima; SAKITA, Neusa K.; FERREIRA, Juliana C. O. A.; SILVEIRA, Cassia G. T.; COSTA, Priscilla R.; RAW, Isaias; WHITEHEAD, Stephen S.; DURBIN, Anna P.; KALIL, JorgeBackground The Butantan Institute has manufactured a lyophilised tetravalent live-attenuated dengue vaccine Butantan-DV, which is analogous to the US National Institutes of Health (NIH) TV003 admixture. We aimed to assess the safety and immunogenicity of Butantan-DV. Methods We did a two-step, double-blind, randomised placebo-controlled phase 2 trial at two clinical sites in Sao Paulo, Brazil. We recruited healthy volunteers aged 18-59 years; pregnant women, individuals with a history of neurological, heart, lung, liver or kidney disease, diabetes, cancer, or autoimmune diseases, and individuals with HIV or hepatitis C were excluded. Step A was designed as a small bridge-study between Butantan-DV and TV003 in DENV-naive participants. In step A, we planned to randomly assign 50 dengue virus (DENV)-naive individuals to receive two doses of Butantan-DV, TV003, or placebo, given 6 months apart. In step B, we planned to randomly assign 250 participants (DENV-naive and DENV-exposed) to receive one dose of Butantan-DV or placebo. Participants were randomly assigned, by computer-generated block randomisation (block sizes of five); participants in step A were randomly assigned (2:2:1) to receive Butantan-DV, TV003, or placebo and participants in step B were randomly assigned (4:1) to receive Butantan-DV or placebo. Participants and study staff were unaware of treatment allocation. The primary safety outcome was the frequency of solicited and unsolicited local and systemic adverse reactions within 21 days of the first vaccination, analysed by intention to treat. The primary immunogenicity outcome was seroconversion rates of the DENV-1-4 serotypes measured 91 days after the first vaccination, analysed in the per-protocol population, which included all participants in step A, and all participants included in step B who completed all study visits with serology sample collection. This trial is registered with ClinicalTrials. gov, NCT01696422. Findings Between Nov 5, 2013, and Sept 21, 2015, 300 individuals were enrolled and randomly assigned: 155 (52%) DENV-naive participants and 145 (48%) DENV-exposed participants. Of the 155 DENV-naive participants, 97 (63%) received Butantan-DV, 17 (11%) received TV003, and 41 (27%) received placebo. Of the 145 DENV-exposed participants, 113 (78%) received Butantan-DV, three (2%) received TV003, and 29 (20%) received placebo. Butantan-DV and TV003 were both immunogenic, well-tolerated, and no serious adverse reactions were observed. In step A, rash was the most frequent adverse event (16 [845] of 19 participants in the Butantan-DV group and 13 [76%] of 17 participants in the TV003 group). Viraemia was similar between the Butantan-DV and TV003 groups. Of the 85 DENV-naive participants in the Butantan-DV group who attended all visits for sample collection for seroconversion analysis and thus were included in the per-protocol analysis population, 74 (87%) achieved seroconversion to DENV-1, 78 (92%) to DENV-2, 65 (76%) to DENV-3, and 76 (89%) to DENV-4. Of the 101 DENV-exposed participants in the Butantan-DV group who attended all visits for sample collection for seroconversion analysis, 82 (81%) achieved seroconversion to DENV-1, 79 (78%) to DENV-2, 83 (82%) to DENV-3, and 78 (77%) to DENV-4. Interpretation Butantan-DV and TV003 were safe and induced robust, balanced neutralising antibody responses against the four DENV serotypes. Efficacy evaluation of the Butantan-DV vaccine is ongoing.
- Epidemiology and management practices for childhood-onset systemic lupus erythematosus patients: a survey in Latin America(2018) FERREIRA, Juliana C. O. A.; TRINDADE, Vitor C.; ESPADA, Graciela; MOREL, Zoilo; BONFA, Eloisa; MAGALHAES, Claudia S.; SILVA, Clovis ArturTo assess epidemiology and management practices of Latin America Pediatric Rheumatologists (LAPR) about childhood-onset systemic lupus erythematosus (cSLE). A cross-sectional study was performed in 288 LAPR PANLAR members based on online survey about cSLE practices. The response rate of web-based survey by LAPR was 170/288(59%) and the majority worked in university hospitals (63%). The ACR and/or SLICC classification criteria (99%) and disease activity tools (97%) were almost universally used by LAPR, whereas damage index (70%) and CHAQ (58%) instruments were less frequently used. Laboratory exams, diagnostic imaging, and biopsies were generally available (>75%), however low availability for densitometry (66%). Drug access was excellent for the most common prescribed medications (>75%), except for belimumab (11%). Emerging mosquito-borne diseases were also reported: dengue (20%), chikungunya (11%), and Zika (8%). Groups were further divided in two, according to the median number of cSLE patients followed by LAPR in the last year: groups A and B (25 and <25, respectively). Frequencies of condom in combination with other contraceptive methods were significantly higher in group A than B (p=0.01). The frequencies of reported pregnancy (p<0.001) and non-adherence to therapy were significantly higher in group A (p=0.023). Alcohol intake (p=0.004) and illicit drug use (p=0.007) were also reported more frequently by LAPR of group A in at least one cSLE patient. This first large web-based survey demonstrated an overall excellent access for diagnosis and therapy by LAPR, probably related to their high rate of practices in tertiary care of university hospitals. Adherence to therapy, pregnancy, and substance abuse was identified as major challenges in this population, particularly in larger centers.
- Characteristics of 1555 childhood-onset lupus in three groups based on distinct time intervals to disease diagnosis: a Brazilian multicenter study(2018) V, G. Novak; MOLINARI, B. C.; FERREIRA, J. C.; SAKAMOTO, A. P.; TERRERI, M. T.; PEREIRA, R. M. R.; SAAD-MAGALHAES, C.; AIKAWA, N. E.; CAMPOS, L. M.; LEN, C. A.; APPENZELLER, S.; FERRIANI, V. P.; SILVA, M. F.; OLIVEIRA, S. K.; ISLABDO, A. G.; SZTAJNBOK, F. R.; PAIM, L. B.; BARBOSA, C. M.; SANTOS, M. C.; BICA, B. E.; SENA, E. G.; MORAES, A. J.; ROLIM, A. M.; SPELLING, P. F.; SCHEIBEL, I. M.; CAVALCANTI, A. S.; MATOS, E. N.; ROBAZZI, T. C.; GUIMARACS, L. J.; SANTOS, F. P.; SILVA, C. T.; BONFA, E.; SILVA, C. A.Objective The objective of this study was to compare demographic data, clinical/laboratorial features and disease activity at diagnosis in three different groups with distinct time intervals between onset of signs/symptoms and disease diagnosis. Methods A multicenter study was performed in 1555 childhood-onset systemic lupus erythematosus (American College of Rheumatology criteria) patients from 27 pediatric rheumatology services. Patients were divided into three childhood-onset systemic lupus erythematosus groups: A: short time interval to diagnosis (<1 month); B: intermediate time interval (1 and <3 months); and C: long time interval (3 months). An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2K were evaluated. Results The number of patients in each group was: A=60 (4%); B=522 (33.5%); and C=973 (62.5%). The median age at diagnosis (11.1 (4.2-17) vs. 12 (1.9-17.7) vs. 12.5 (3-18) years, P=0.025) was significantly lower in group A compared with groups B and C. The median number of diagnostic criteria according to SLICC (7 (4-12) vs. 6 (4-13) vs. 6 (4-12), P<0.0001) and SLEDAI-2K (18 (6-57) vs. 16 (2-63) vs. 13 (1-49), P<0.0001) were significantly higher in group A than the other two groups. The frequency of oral ulcers in the palate (25% vs. 15% vs. 11%, P=0.003), pleuritis (25% vs. 24% vs. 14%, P<0.0001), nephritis (52% vs. 47% vs. 40%, P=0.009), neuropsychiatric manifestations (22% vs. 13% vs. 10%, P=0.008), thrombocytopenia (32% vs. 18% vs. 19%, P=0.037), leucopenia/lymphopenia (65% vs. 46% vs. 40%, P<0.0001) and anti-dsDNA antibodies (79% vs. 66% vs. 61%, P=0.01) were significantly higher in group A compared with the other groups. In contrast, group C had a less severe disease characterized by higher frequencies of synovitis (61% vs. 66% vs. 71%, P=0.032) and lower frequencies of serositis (37% vs. 33% vs. 25%, P=0.002), proteinuria >500mg/day (48% vs. 45% vs. 36%, P=0.002) and low complement levels (81% vs. 81% vs. 71%, P<0.0001) compared with groups A or B. Conclusions Our large Brazilian multicenter study demonstrated that for most childhood-onset systemic lupus erythematosus patients, diagnosis is delayed probably due to mild disease onset. Conversely, the minority has a very short time interval to diagnosis and a presentation with a more severe and active multisystemic condition.
- Herpes zoster infection in childhood-onset systemic lupus erythematosus patients: a large multicenter study(2016) FERREIRA, J. C. O. A.; MARQUES, H. H.; FERRIANI, M. P. L.; GORMEZANO, N. W. S.; TERRERI, M. T.; PEREIRA, R. M.; MAGALHAES, C. S.; CAMPOS, L. M.; BUGNI, V.; OKUDA, E. M.; MARINI, R.; PILEGGI, G. S.; BARBOSA, C. M.; BONFA, E.; SILVA, C. A.Objective The aim of this multicenter study in a large childhood-onset systemic lupus erythematosus (cSLE) population was to assess the herpes zoster infection (HZI) prevalence, demographic data, clinical manifestations, laboratory findings, treatment, and outcome. Methods A retrospective multicenter cohort study (Brazilian cSLE group) was performed in ten Pediatric Rheumatology services in SAo Paulo State, Brazil, and included 852 cSLE patients. HZI was defined according to the presence of acute vesicular-bullous lesions on erythematous/edematous base, in a dermatomal distribution. Post-herpetic neuralgia was defined as persistent pain after one month of resolution of lesions in the same dermatome. Patients were divided in two groups for the assessment of current lupus manifestations, laboratory findings, and treatment: patients with HZI (evaluated at the first HZI) and patients without HZI (evaluated at the last visit). Results The frequency of HZI in cSLE patients was 120/852 (14%). Hospitalization occurred in 73 (61%) and overlap bacterial infection in 16 (13%). Intravenous or oral aciclovir was administered in 113/120 (94%) cSLE patients at HZI diagnosis. None of them had ophthalmic complication or death. Post-herpetic neuralgia occurred in 6/120 (5%). After Holm-Bonferroni correction for multiple comparisons, disease duration (1.58 vs 4.41 years, p<0.0001) was significantly lower in HZI cSLE patients compared to those without HZI. Nephritis (37% vs 18%, p<0.0001), lymphopenia (32% vs 17%, p<0.0001) prednisone (97% vs 77%, p<0.0001), cyclophosphamide (20% vs 5%, p<0.0001) and SLE Disease Activity Index 2000 (6.0 (0-35) vs 2 (0-45), p<0.0001) were significantly higher in the former group. The logistic regression model showed that four independent variables were associated with HZI: disease duration<1 year (OR 2.893 (CI 1.821-4.597), p<0.0001), lymphopenia <1500/mm(3) (OR 1.931 (CI 1.183-3.153), p=0.009), prednisone (OR 6.723 (CI 2.072-21.815), p=0.002), and cyclophosphamide use (OR 4.060 (CI 2.174-7.583), p<0.0001). Conclusion HZI is an early viral infection in cSLE with a typical dermatomal distribution. Lymphopenia and immunosuppressive treatment seem to be major factors underlying this complication in spite of a benign course.
- Emotional, hyperactivity and inattention problems in adolescents with immunocompromising chronic diseases during the COVID-19 pandemic(2023) CAMPOS, Reinan T.; LINDOSO, Livia; SOUSA, Renan A. de; HELITO, Alberto C.; IHARA, Bianca P.; STRABELLI, Claudia A. A.; PARADELAS, Levi M. V.; CARNEIRO, Beatriz O. L.; CARDOSO, Maria Paula R.; SOUZA, Jean Paulo V. de; FREIRE, Marianna R. de M.; ASTLEY, Camilla; LAURENTINO, Moises F.; BUSCATTI, Izabel M.; KOZU, Katia; AIKAWA, Nadia E.; SALLUM, Adriana M. E.; FERREIRA, Juliana C. O.; SIMON, Juliana R.; VIANA, Vivianne S. L.; QUEIROZ, Ligia B.; GUALANO, Bruno; ROSCHEL, Hamilton; PEREIRA, Rosa Maria R.; TOMA, Ricardo K.; WATANABE, Andreia; GRANGEIRO, Patricia M.; CASELLA, Caio B.; FARHAT, Sylvia C.; V, Guilherme Polanczyk; CAMPOS, Lucia Maria A.; SILVA, Clovis A.Objective: To assess factors associated with emotional changes and Hyperactivity/Inattention (HI) motivated by COVID-19 quarantine in adolescents with immunocompromising diseases.Methods: A cross-sectional study included 343 adolescents with immunocompromising diseases and 108 healthy adolescents. Online questionnaires were answered including socio-demographic data and self-rated healthcare routine during COVID-19 quarantine and validated surveys: Strengths and Difficulties Questionnaire (SDQ), Pitts-burgh Sleep Quality Index (PSQI), Pediatric Quality of Life Inventory 4.0 (PedsQL4.0).Results: The frequencies of abnormal emotional SDQ scores from adolescents with chronic diseases were similar to those of healthy subjects (110/343 [32%] vs. 38/108 [35%], p = 0.548), as well as abnormal hyperactivity/inat-tention SDQ scores (79/343 [23%] vs. 29/108 [27%], p = 0.417). Logistic regression analysis of independent var-iables associated with abnormal emotional scores from adolescents with chronic diseases showed: female sex (Odds Ratio [OR = 3.76]; 95% Confidence Interval (95% CI) 2.00-7.05; p < 0.001), poor sleep quality (OR = 2.05; 95% CI 1.08-3.88; p = 0.028) and intrafamilial violence during pandemic (OR = 2.17; 95% CI 1.12-4.19; p = 0.021) as independently associated with abnormal emotional scores, whereas total PedsQL score was inversely associated with abnormal emotional scores (OR = 0.95; 95% CI 0.93-0.96; p < 0.0001). Logistic regression analysis associated with abnormal HI scores from patients evidenced that total PedsQL score (OR = 0.97; 95% CI 0.95-0.99; p = 0.010], changes in medical appointments during the pandemic (OR = 0.39; 95% CI 0.19-0.79; p = 0.021), and reliable COVID-19 information (OR = 0.35; 95% CI 0.16-0.77; p = 0.026) remained inversely associated with abnormal HI scores.Conclusion: The present study showed emotional and HI disturbances in adolescents with chronic immunosuppres-sive diseases during the COVID-19 pandemic. It reinforces the need to promptly implement a longitudinal pro-gram to protect the mental health of adolescents with and without chronic illnesses during future pandemics.