GLAUCIA VANESSA NOVAK
Projetos de Pesquisa
Unidades Organizacionais
LIM/36 - Laboratório de Pediatria Clínica, Hospital das Clínicas, Faculdade de Medicina
19 resultados
Resultados de Busca
Agora exibindo 1 - 10 de 19
- Serum lipids in Brazilian children and adolescents: determining their reference intervals(2015) SLHESSARENKO, Natasha; JACOB, Cristina M. A.; AZEVEDO, Raymundo S.; FONTES, Cor J. F.; NOVAK, Glaucia V.; ANDRIOLO, AdagmarBackground: Demographic, geographic, environmental and genetic factors influence lipids. In many countries, the normal lipid ranges for laboratory tests are based on references from American children and adolescents. In this work, we determined the reference intervals (RIs) for total cholesterol (TC), high-density lipoprotein cholesterol (HDL-c), non-high-density lipoprotein cholesterol (nHDL-c), low-density lipoprotein cholesterol (LDL-c) and triglycerides (TG) in Brazilian healthy children and adolescents. Methods: A cross-sectional study was conducted of 1,866 randomly sampled healthy children and adolescents from kindergartens and schools. Blood samples were collected after a variable period of fasting based on the age of the participant. The upper cut-off points were the 75th and 95th percentiles for TC, nHDL-c, LDL-c and TG. The 10th percentile (low) was used as the bottom level for HDL-c. Non-parametric tests were used for statistical analyses. Results: The following RI and 75th and 95th percentiles were observed for each age interval. The 95th percentile values obtained for TC were: 1 to 2 years, 189 mg/dL, 3 to 8 years, 199 mg/dL; 9 to 12 years, 205 mg/dL. For the nHDL c, the only age group 1 to 12 years, this percentile value was 150 mg/dL. For the LDL-cholesterol, the values corresponding to the percentiles above, aged 1 to 8 years and 9 to 12 years, were 132 mg/dL 139 mg/dL, respectively. For the triglycerides, the values corresponding to 95th percentile were: 1 year, 189 mg/dL; 2 to 5 years, 139 mg/dL; 6 to 12 years, 139 mg/dL. The 10th percentiles for HDL-c were 24 mg/dL, 28 mg/dL, 32 mg/dL and 36 mg/dL for children 1, 2, 3 and 4-12 years old, respectively. Conclusions: The lipid reference intervals defined in the studied Brazilian children and adolescents differ from those recommended by the international literature and should be used for clinical decisions contributing to improve the diagnosis in this particular group in our country.
- CANDLE syndrome: chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature-a rare case with a novel mutation(2016) CAVALCANTE, Miria Paula V.; BRUNELLI, Juliana B.; MIRANDA, Clarissa C.; NOVAK, Glaucia V.; MALLE, Louise; AIKAWA, Nadia E.; JESUS, Adriana A.; SILVA, Clovis ArturWe described herein a patient with chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome and a novel mutation in PSMB8 gene. This patient had multiple visceral inflammatory involvements, including rare manifestations, such as Sweet syndrome and pericarditis. A 3-year-old male, Caucasian, was born to consanguineous healthy parents. At the age of 11 months, he presented daily fever (temperature > 40 A degrees C), irritability, hepatomegaly, splenomegaly; and tender and itching, erythematous papular and edematous plaque lesions. Echocardiogram showed mild pericarditis. Skin biopsy revealed a neutrophil infiltrate without vasculitis suggesting Sweet syndrome. Mutational screening of PSMB8 gene revealed homozygous c.280G > C, p.A94P mutation. He responded partially to high doses of oral glucorticoid and intravenous methylprednisolone. Colchicine, azathioprine, methotrexate, cyclosporine, and intravenous immunoglobulin were not efficacious. At the age of 3 years and 1 month, tocilizumab was administered resulting in remission of daily fever and irritability. However, there was no improvement of the skin tenderness and itching lesions. Conclusion: A new mutation in a CANDLE syndrome patient was reported with pericarditis and mimicking Sweet syndrome. The disease manifestations were refractory to immunosuppressive agents and partially responsive to tocilizumab therapy.
- Esophageal abnormalities in juvenile localized scleroderma: is it associated with other extracutaneous manifestations?(2017) VALOES, Clarissa C. M.; NOVAK, Glaucia V.; BRUNELLI, Juliana B.; KOZU, Katia T.; TOMA, Ricardo K.; SILVA, Clovis A.Objective: To assess esophageal involvement (EI) in juvenile localized scleroderma (JLS) population and the possible association between this gastrointestinal manifestation and demographic data, clinical features, laboratory exams, treatments and outcomes. Methods: For a period of 30 years, 5881 patients with rheumatic diseases were followed in our Pediatric Rheumatology Division. EI was defined by the presence of symptoms (solid/liquid dysphagia, heartburn, esophageal regurgitation, nausea/vomiting and epigastralgia) and confirmed by at least one EI exam abnormality: barium contrast radiography, upper gastrointestinal endoscopy and 24-hour esophageal pH-monitoring. Results: JLS was observed in 56/5881 patients (0.9%), mainly linear morphea subtype. EI was observed in 23/56(41%) of JLS patients. Eight(35%) of 23 EI patients with JLS were symptomatic and presented heartburn(5/8), solid and liquid dysphagia(3/8), nausea and epigastralgia(1/8). The frequency of any cumulative extracutaneous manifestations (calcinosis, arthritis/arthralgia, central nervous system, interstitial pneumonitis, mesangial nephritis and/or arrhythmia) was significantly higher in JLS patients with EI compared to those without this complication (56% vs. 24%, p = 0.024). No differences were evidenced in demographic data, JLS subtypes and in each extracutaneous manifestation in both groups (p > 0.05). The frequency of methotrexate use was significantly higher in JLS patients with EI compared to those without (52% vs. 12%, p = 0.002). Autoantibody profile (antinuclear antibodies, antiSCL-70, rheumatoid factor, anticentromere, anti-cardiolipin, anti-Ro/SSA and anti-La/SSB) was similar in both groups (p > 0.05). Conclusions: Our study demonstrated that EI was frequently observed in JLS patients, mainly in asymptomatic patients with linear subtype. EI occurred in JLS patients with other extra cutaneous manifestations and required methotrexate therapy. (C) 2016 Published by Elsevier Editora Ltda.
- Symptomatic polyautoimmunity at diagnosis of 1463 childhood-onset lupus: A Brazilian multicenter study(2018) SETOUE, Debora N.; PITTA, Ana C.; FIOROT, Fernanda J.; NASTRI, Mariana M.; NOVAK, Glaucia V.; MOLINARI, Beatriz C.; OLIVEIRA, Juliana C.; GORMEZANO, Natali W.; SAKAMOTO, Ana P.; TERRERI, Maria T.; PEREIRA, Rosa M.; SAAD-MAGALHAES, Claudia; SALLUM, Adriana M.; KOZU, Katia; FRAGA, Melissa M.; PIOTTO, Daniela P.; CLEMENTE, Gleice; MARINI, Roberto; GOMES, Hugo R.; RABELO-JUNIOR, Carlos N.; FELIX, Marta M.; RIBEIRO, Maria C.; ALMEIDA, Rozana G.; ASSAD, Ana P.; SACCHETTI, Silvana B.; BARROS, Leandra C.; BONFA, Eloisa; SILVA, Clovis A.Objective: To evaluate symptomatic polyautoimmunity (PA) at childhood-onset systemic lupus erythematosus (cSLE) diagnosis, and its association with demographic data, disease activity, clinical manifestations and laboratorial abnormalities in a large Brazilian cSLE population. Methods: A multicenter retrospective study was performed in 1463 cSLE(ACR criteria) patients from 27 Pediatric Rheumatology services. Symptomatic PA was defined according to the presence of more than one concomitant autoimmune disease(AD) and symptomatic multiple autoimmune syndrome(MAS) was defined as three or more AD. An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2K were evaluated. Results: At cSLE diagnosis symptomatic PA was observed in 144/1463(9.8%) and symptomatic MAS occurred in solely 10/1463(0.7%). In the former group the more frequently observed associated AD were Hashimoto thyroiditis n = 42/144(29%), antiphospholipid syndrome n = 42/144(29%), autoimmune hepatitis n = 26/144(18%) and type 1 diabetes mellitus n = 23/144(15.9%). Further comparisons between cSLE patients with and without PA showed a higher median age(p = 0.016) and lower mean SLICC criteria (p = 0.039) in those with PA. Additionally, these cSLE patients had less renal involvement(35% vs. 44%, p = 0.038) and red blood cell cast(6% vs. 12%, p = 0.042) and more antiphospholipid antibodies(29% vs. 15%, p < 0.0001). Conclusions: Approximately 10% of cSLE had symptomatic PA at diagnosis, particularly endocrine autoimmune disorders and antiphospholipid syndrome. Lupus was characterized by a mild disease onset and MAS was infrequently evidenced. Further studies are necessary to determine if this subgroup of cSLE patients have a distinct genetic background with a less severe disease and a better long-term outcome.
- Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome(2018) BLAY, Gabriela; RODRIGUES, Joaquim C.; FERREIRA, Juliana C. O.; LEAL, Gabriela N.; GORMEZANO, Natali W.; V, Glaucia Novak; PEREIRA, Rosa M. R.; TERRERI, Maria T.; MAGALHAES, Claudia S.; MOLINARI, Beatriz C.; SAKAMOTO, Ana P.; AIKAWA, Nadia E.; CAMPOS, Lucia M. A.; FERNANDES, Taciana A. P.; CLEMENTE, Gleice; PERACCHI, Octavio A. B.; BUGNI, Vanessa; MARINI, Roberto; SACCHETTI, Silvana B.; CARVALHO, Luciana M.; FRAGA, Melissa M.; CASTRO, Tania C. M.; RAMOS, Valeria C.; BONFA, Eloisa; SILVA, Clovis A.Objective: To evaluate prevalence, clinical manifestations, laboratory abnormalities and treatment in a multicenter cohort study including 847 childhood-onset systemic lupus erythematosus (cSLE) patients with and without diffuse alveolar hemorrhage (DAH), as well as concomitant parameters of severity. Methods: DAH was defined as the presence of at least three respiratory symptoms/signs associated with diffuse interstitial/alveolar infiltrates on chest x-ray or high-resolution computer tomography and sudden drop in hemoglobin levels. Statistical analysis was performed using Bonferroni correction (p<0.0022). Results: DAH was observed in 19/847 (2.2%) cSLE patients. Cough/dyspnea/tachycardia/hypoxemia occurred in all cSLE patients with DAH. Concomitant parameters of severity observed were: mechanical ventilation in 14/19 (74%), hemoptysis 12/19 (63%), macrophage activation syndrome 2/19 (10%) and death 9/19 (47%). Further analysis of cSLE patients at DAH diagnosis compared to 76 cSLE control patients without DAH with same disease duration [3 (1-151) vs. 4 (1-151) months, p = 0.335], showed higher frequencies of constitutional involvement (74% vs. 10%, p < 0.0001), serositis (63% vs. 6%, p < 0.0001) and sepsis (53% vs. 9%, p < 0.0001) in the DAH group. The median of disease activity score(SLEDAI-2 K) was significantly higher in cSLE patients with DAH [18 (5-40) vs. 6 (0-44), p < 0.0001]. The frequencies of thrombocytopenia (53% vs. 12%, p < 0.0001), intravenous methylprednisolone (95% vs. 16%, p < 0.0001) and intravenous cyclophosphamide (47% vs. 8%, p < 0.0001) were also significantly higher in DAH patients. Conclusions: This was the first study to demonstrate that DAH, although not a disease activity score descriptor, occurred in the context of significant moderate/severe cSLE flare. Importantly, we identified that this condition was associated with serious disease flare complicated by sepsis with high mortality rate.
conferenceObject Validation of Flare Criteria for Children and Adolescents with Systemic Lupus Erythematosus(2017) BRUNNER, Hermine I.; HOLLAND, Michael J.; BERESFORD, Michael W.; RUPERTO, Nicolino; ARDOIN, Stacy P.; APPENZELLER, Simone; SILVA, Clovis A.; NOVAK, Glaucia V.; LOURENCO, Daniela M.; FLORES, Francisco; GOILAV, Beatrice; WENDERFER, Scott E.; LEVY, Deborah M.; RAVELLI, Angelo; KHUBCHANDANI, Raju; AVCIN, Tadej; KLEIN-GITELMAN, Marisa S.; FELDMAN, Brian M.; YING, JunconferenceObject Predictors of Mortality in a Series of 1528 Brazilian Childhood-onset Systemic Lupus Erythematosus Patients(2021) SAKAMOTO, Ana Paula; SILVA, Clovis Artur; ISLABAO, Aline; NOVAK, Glaucia; MOLINARI, Beatriz; PEREIRA, Rosa; SAAD-MAGALHAES, Claudia; CLEMENTE, Gleice; FRAGA, Melissa; PIOTO, Daniela Petry; LEN, Claudio; PRADO, Rogerio do; TERRERI, Maria- The new 2019-EULAR/ACR classification criteria specific domains at diagnosis can predict damage accrual in 670 childhood-onset systemic lupus erythematosus patients(2021) PITTA, Ana C.; SILVA, Clovis A.; INSFRAN, Carlos E.; PASOTO, Sandra G.; TRINDADE, Vitor C.; V, Glaucia Novak; SAKAMOTO, Ana P.; TERRERI, Maria T.; PEREIRA, Rosa M. R.; MAGALHAES, Claudia S.; FONSECA, Adriana R.; ISLABAO, Aline G.; ASSAD, Ana P. L.; BUSCATTI, Izabel M.; ELIAS, Adriana M.; PIOTTO, Daniela P.; FERRIANI, Virginia P.; CARVALHO, Luciana M.; RABELO JUNIOR, Carlos N.; MARINI, Roberto; SZTAJNBOK, Flavio R.; SACCHETTI, Silvana B.; BICA, Blanca E.; MORAES, Ana J.; ROBAZZI, Teresa C.; LOTUFO, Simone; CAVALCANTI, Andre S.; NAKA, Erica N.; CARNEIRO-SAMPAIO, Magda; BONFA, Eloisa; AIKAWA, Nadia E.Objective To evaluate if the 2019-European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) criteria at diagnosis of childhood-onset systemic lupus erythematosus (cSLE) are associated with higher rates of early damage scored by Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (SDI). Methods This retrospective multicenter study included 670 cSLE patients with <= 5 years of disease duration. All patients fulfilled both 2019-EULAR/ACR and 1997-ACR classification criteria. Total score of 2019-EULAR/ACR criteria and each of its specific domains were assessed at diagnosis as predictors of damage accrual at the last visit, according to the presence of any organ damage (defined by SDI >= 1). Results Median disease duration was 2.8 (IQR 1.8-3.8) years and 200 (29.9%) patients had at least one organ damage (SDI >= 1). The most frequent domains were neuropsychiatric (12%), renal (7%), and musculoskeletal (6%). There was a higher frequency of renal (58% vs 43%, p = 0.0004) and neuropsychiatric domain (21% vs 7%, p < 0.0001) of 2019-EULAR/ACR criteria in patients with damage (SDI >= 1) compared to those without damage (SDI = 0). Patients scoring renal or neuropsychiatric domains of the 2019-EULAR/ACR criteria at diagnosis were associated with renal damage (odds ratio 9.701, 95% confidence interval 3.773-24.941, p < 0.001) or neuropsychiatric damage (OR 9.480, 95% CI 5.481-16.399, p<0.0001) at latest visit, respectively. cSLE patients with positive anti-dsDNA at diagnosis were also associated with renal damage by the latest visit (OR 2.438, 95% CI 1.114-5.3381, p = 0.021). Constitutional, hematologic, mucocutaneous, serosal, and musculoskeletal domains and specific criteria as well as other immunologic criteria were not associated with damage accrual. Median of SLEDAI-2K was significantly higher in patients with global damage (19.5 (2-51) vs 14 (0-51), p<0.001). 2019-EULAR/ACR score >25 was associated with more overall (SDI >= 1) (38% vs 25%, p = 0.0002) and renal damage (11% vs 5%, p = 0.023). Conclusions The 2019-EULAR/ACR criteria at diagnosis were associated with a higher rate of early damage in cSLE patients, especially for renal and neuropsychiatric damage. Of note, damage was particularly associated with high disease activity at diagnosis and 2019-EULAR/ACR score >25.
conferenceObject Renal Outcomes in 1528 Childhood-onset Systemic Lupus Erythematosus Patients: A Brazilian Multicenter Study(2021) SAKAMOTO, Ana Paula; SILVA, Clovis; ISLABAO, Aline; NOVAK, Glaucia; MOLINARI, Beatriz; PEREIRA, Rosa; SAAD-MAGALHAES, Claudia; CLEMENTE, Gleice; PIOTO, Daniela Petry; AIKAWA, Nadia; PITA, Ana; TRINDADE, Vitor; APPENZELLER, Simone; CARVALHO, Luciana; RABELO-JUNIOR, Carlos; FONSECA, Adriana; SZTAJNBOK, Flavio; SANTOS, Maria; BICA, Blanca Elena Rios Gomes; SENA, Evaldo; MORAES, Ana; FRAGA, Melissa; ROBAZZI, Teresa; SPELLING, Paulo; SCHEIBEL, Iloite; CAVALCANTI, Andre; MATOS, Erica; GUIMARAES, Luciano; SANTOS, Flavia; MOTA, Licia Maria Henrique; BONFA, Eloisa; TERRERI, Maria- Disease presentation of 1312 childhood-onset systemic lupus erythematosus: influence of ethnicity(2019) FIOROT, Fernanda J.; ISLABAO, Aline G.; PEREIRA, Rosa M.; TERRERI, Maria T.; SAAD-MAGALHAES, Claudia; V, Glaucia Novak; MOLINARI, Beatriz C.; SAKAMOTO, Ana P.; AIKAWA, Nadia E.; CAMPOS, Lucia M.; PERACCHI, Octavio A.; APPENZELLER, Simone; FERRIANI, Virginia P.; SILVA, Marco F.; FONSECA, Adriana R.; SZTAJNBOK, Flavio R.; PAIM, Luciana B.; FRAGA, Melissa M.; OKUDA, Eunice M.; BICA, Blanca E.; SENA, Evaldo G.; MORAES, Ana J.; ROLIM, Ana M.; SPELLING, Paulo F.; SCHEIBEL, Iloite M.; CAVALCANTI, Andre S.; MATOS, Erica N.; ROBAZZI, Teresa C.; GUIMARAES, Luciano J.; SANTOS, Flavia P.; RAMOS, Valeria C.; CARNEIRO-SAMPAIO, Magda; BONFA, Eloisa; SILVA, Clovis A.Objective To evaluate the influence of ethnicity in presentation of childhood-onset systemic lupus erythematosus (cSLE) patients. Methods This multicenter study included cSLE patients (American College of Rheumatology criteria) followed in 27 Pediatric Rheumatology services of Brazil. Ethnicities were classified in four groups according to the parents' and all four grandparents' self-reported ethnicity. The statistical analysis was performed using the Bonferroni's correction (p < 0.0027). Results According to ethnic groups, 1537 cSLE patients were classified in Caucasian (n = 786), African-Latin American (n = 526), Asian (n = 8), and others/unknown (n = 217). Comparisons between 1312 African-Latin American and Caucasian revealed similar median age at cSLE diagnosis [12.2(2.6-18) vs. 12.1(0.3-18) years, p = 0.234], time interval to diagnosis [0.25(0-12) vs. 0.3(0-10) years, p = 0.034], and SLEDAI-2K score [14(0-55) vs. 14(0-63), p = 0.781] in both groups. The mean number of diagnostic criteria according to SLICC (6.47 +/- 1.911 vs. 5.81 +/- 1.631, p < 0.0001) and frequencies of maculopapular lupus rash (8% vs. 3%, p < 0.0001), palate oral ulcers (17% vs. 11%, p = 0.001), tongue oral ulcers (4% vs. 1%, p = 0.001), and nonscarring alopecia (29% vs. 16%, p < 0.0001) were significantly higher in African-Latin American, whereas malar rash (45% vs. 58%, p < 0.0001) was more frequent in Caucasian. The presence of anti-phospholipid antibody (23% vs. 12%, p < 0.0001), low complement levels (58% vs. 41%, p < 0.0001), and isolated direct Coombs test (10% vs. 5%, p = 0.001) was also significantly higher in the former group. Conclusions Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable with Caucasian. Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of the former group. The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients.