CLOVIS ARTUR ALMEIDA DA SILVA

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Departamento de Pediatria, Faculdade de Medicina - Docente
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina
LIM/36 - Laboratório de Pediatria Clínica, Hospital das Clínicas, Faculdade de Medicina - Líder

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  • article 4 Citação(ões) na Scopus
    Antiphospholipid syndrome plus rheumatic fever: a higher risk factor for stroke?
    (2012) CAMARGO, Elisa Watanabe; FREIRE, Paula Vieira; SILVA, Clovis Artur; SANTOS, Nelita Rocha dos; MOTA, Licia Maria Henrique da; PEREIRA, Rosa Maria Rodrigues; CARVALHO, Jozelio Freire de
    To compare clinical and laboratory findings between patients with primary antiphospholipid syndrome (PAPS) versus secondary APS due to rheumatic fever (APS-RF) (according to Jones criteria). Seventy-three APS patients (Sapporo criteria) were enrolled, and demographic, clinical, and laboratory data were collected. Exclusion criteria were heart congenital abnormalities and previous infectious endocarditis. Patients were divided into two groups: PAPS (n = 68) and APS-RF (n = 5). The mean current age, disease duration, frequencies of female gender, and Caucasian race were similar in APS-RF and PAPS patients (P > 0.05). Remarkably, the frequency of stroke was significantly higher in APS-RF compared to PAPS patients (80% vs. 25%, P = 0.02). Of note, echocardiogram of these patients did not show intracardiac thrombus. No significant differences were found in peripheral thromboembolic events (P = 1.0), pulmonary thromboembolism (P = 1.0), miscarriage (P = 0.16), thrombocytopenia (P = 0.36), arterial events (P = 0.58), and thrombosis of small vessels (P = 1.0). There were no differences in the frequencies of comorbidities such as diabetes mellitus, hypertension, smoking, and hyperlipidemia in both groups (P > 0.05). The frequencies of lupus anticoagulant, IgG, and IgM anticardiolipin were similar in two groups. APS patients associated with rheumatic fever without infective endocarditis may imply a high stroke risk as compared with PAPS, and future studies are needed to confirm this finding.
  • conferenceObject
    EFFECT OF LONG-TERM TNF BLOCKAGE ON LIPID PROFILE IN ANKYLOSING SPONDYLITIS PATIENTS
    (2012) MORAES, J. C.; SOUZA, F. H. C.; RIBEIRO, A. C. D. M.; SAAD, C. G. S.; CALICH, A. L.; SILVA, C. A.; BONFA, E.
  • conferenceObject
    A Brazilian Multicenter Study of 71 Children and Adolescents with Takayasu's Arteritis
    (2012) TERRERI, Maria Teresa; CLEMENTE, Gleice; SILVA, Clovis; SACCHETTI, Silvana; SALLUM, Adriana M.; CAMPOS, Lucia M. A.; SANTOS, Maria Carolina; SZTAJNBOK, Flavio; ALMEIDA, Rozana Gasparello de; FERRIANI, Virginia P.; BICA, Blanca E.; ROBAZZI, Teresa; BANDEIRA, Marcia; CAVALCANTI, Andre; LESSA, Marise; OLIVEIRA, Sheila K. Feitosa de; HILARIO, Maria Odete
    Background/Purpose: Takayasu’s arteritis is a chronic granulomatous disease that affects the vascular wall of the large arteries and can result in end organ damage. It is more prevalent in young women, but can also occur in the childhood. The disease is rare and there are few reports about the clinical features at this age. Our aim was to describe the clinical features of Takayasu’s arteritis in children and adolescents in our population. Methods: In this Brazilian multicenter retrospective study including 10 pediatric rheumatology centers we identified 71 children and adolescents with Takayasu’s arteritis. Patients’ demographic, clinical, laboratory, angiographic, therapeutic data and disease outcome were recorded. Results: Of the 71 patients, 51 (72%) were girls, with a mean age at onset of 9.2 years (range 4 months to 17.2 years); the average time to diagnosis was 1.2 years; and the mean follow-up time was 5.4 years. The most frequent angiographic type was type IV (41%) followed by type V (27%) and the abdominal aorta was the most affected vessel (63.4%). The main lesion was arterial stenosis (84.5% of patients). At initial presentation 80.6% of patients had increased acute phase reactants and 41% of patients had a positive Mantoux test. The predominant clinical symptoms at onset were constitutional (77.5%), followed by neurological (70.4%) and musculoskeletal symptoms (64.8%). The main cardiovascular manifestation was arterial hypertension (84.5%). At the final evaluation, neurological symptoms were predominant (22.7%) and decrease of peripheral pulses (66.7%) was the main cardiovascular manifestation. Sixty four (90.1%) patients were treated with corticosteroids, 30 patients (42.3%) with methotrexate as the first immunosuppressive treatment and 18 (25.4%) were treated with cyclophosphamide as initial therapy. Infliximab was used in only 4 patients throughout the follow-up. At the final evaluation, 55% of patients were in disease remission, 28% had active disease, 7% died and in 10% the outcome was unknown. Conclusion: Takayasu’s arteritis is a rare childhood disease. In this multicenter study we observed a high rate of disease remission, however prospective studies are needed in order to better define overall disease outcome.
  • article 30 Citação(ões) na Scopus
    Hereditary Autoinflammatory Syndromes: A Brazilian Multicenter Study
    (2012) JESUS, Adriana A.; FUJIHIRA, Erika; WATASE, Mariana; TERRERI, Maria T.; HILARIO, Maria O.; CARNEIRO-SAMPAIO, Magda; LEN, Claudio A.; OLIVEIRA, Sheila K.; RODRIGUES, Marta C.; PEREIRA, Rosa M.; BICA, Blanca; SILVA, Nilzio A.; CAVALCANTI, Andre; MARINI, Roberto; SZTAJNBOK, Flavio; QUINTERO, Maria V.; FERRIANI, Virginia P.; MORAES-VASCONCELOS, Dewton; SILVA, Clovis A.; OLIVEIRA, Joao B.
    To evaluate the prevalence of genetic defects in clinically suspected autoinflammatory syndromes (AIS) in a Brazilian multicenter study. The study included 102 patients with a clinical diagnosis of Cryopyrin Associated Periodic Syndromes (CAPS), TNF Receptor Associated Periodic Syndrome (TRAPS), Familial Mediterranean Fever (FMF), Mevalonate Kinase Deficiency (MKD) and Pediatric Granulomatous Arthritis (PGA). One of the five AIS-related genes (NLRP3, TNFRSF1A, MEFV, MVK and NOD2) was evaluated in each patient by direct DNA sequencing, based on the most probable clinical suspect. Clinical diagnoses of the 102 patients were: CAPS (n = 28), TRAPS (n = 31), FMF (n = 17), MKD (n = 17) and PGA (n = 9). Of them, 27/102 (26 %) had a confirmed genetic diagnosis: 6/28 (21 %) CAPS patients, 7/31 (23 %) TRAPS, 3/17 (18 %) FMF, 3/17 (18 %) MKD and 8/9 (89 %) PGA. We have found that approximately one third of the Brazilian patients with a clinical suspicion of AIS have a confirmed genetic diagnosis.
  • conferenceObject
    TESTICULAR SERTOLI CELL FUNCTION IN ANKYLOSING SPONDYLITIS: THE POSSIBLE EFFECT OF TNF BLOCKAGE
    (2012) SAAD, C. G. S.; ALMEIDA, B. P. de; SOUZA, F. H. C.; MORAES, J. C. B.; NUKUMIZU, L. A.; SAMPAIO-FARROS, P. D.; VIANA, V. S. T.; BONFA, E.; SILVA, C. A.
    Introduction: Inhibin B is an important testicular Sertoli cell function marker allowing a global evaluation of testicular tissue. However there are no data regarding this cell function in ankylosing spondylitis (AS) patients.in this hormone production. Materials and Methods: 20 consecutive male AS patients and 24 healthy controls were evaluated. At study entry, AS patients were not receiving sulfasalazine/methotrexate and they never used biological/cytotoxic agents. Serum dimeric inhibin B levels were measured by a double-antibody ELISA. Demographic, disease parameters and urologic evaluation were systematically performed. The latter included testicular Döppler ultrasound, hormone profile and semen analysis. Ten of these patients received anti-TNF treatment and they were re-evaluated for inhibin B and disease parameters at 6 months(6M). Four of them also repeated sperm analysis. Results: At study entry, the median of current and spermarche age were similar in AS patients and controls [33(17-53) vs. 28.5(15-54) years, p=0.175; 13(9-18) vs. 12(11-15)years, p=0.358; respectively]. The median of inhibin B [68(23-265) vs. 112.9(47.8-231.9)pg/mL, p=0.111], FSH levels and the other hormones were comparable in both groups (p>0.05). All patients and controls had normal sperm motility and concentration with two AS patients presenting borderline low inhibin B levels. Further analysis at 6M of the 10 patients referred for anti-TNF therapy, including one with borderline inhibin B, revealed that median inhibin B levels remained largely stable [126.5(24-316) vs. 116.5(28-265)pg/mL, p=0.431]. Sperm motility/concentration were preserved in the four patients that performed this analysis after anti-TNF. Conclusions: This is the first study to demonstrate, through a specific marker, a normal testicular Sertoli cell function associated with preserved sperm quality in AS patients. We further identified that anti-TNF drugs do not seem to have a deleterious effect in inhibin B production reinforcing its safety for testicular function in this disease.
  • article 12 Citação(ões) na Scopus
    Skeletal muscle major histocompatibility complex class I and II expression differences in adult and juvenile dermatomyositis
    (2012) SHINJO, Samuel Katsuyuki; SALLUM, Adriana Maluf Elias; SILVA, Clovis Artur; MARIE, Suely Kazue Nagahashi
    OBJECTIVE: To analyze major histocompatibility complex expression in the muscle fibers of juvenile and adult dermatomyositis. METHOD: In total, 28 untreated adult dermatomyositis patients, 28 juvenile dermatomyositis patients (Bohan and Peter's criteria) and a control group consisting of four dystrophic and five Pompe's disease patients were analyzed. Routine histological and immunohistochemical (major histocompatibility complex I and II, StreptoABComplex/HRP, Dakopatts) analyses were performed on serial frozen muscle sections. Inflammatory cells, fiber damage, perifascicular atrophy and increased connective tissue were analyzed relative to the expression of major histocompatibility complexes I and II, which were assessed as negatively or positively stained fibers in 10 fields (200X). RESULTS: The mean ages at disease onset were 42.0 +/- 15.9 and 7.3 +/- 3.4 years in adult and juvenile dermatomyositis, respectively, and the symptom durations before muscle biopsy were similar in both groups. No significant differences were observed regarding gender, ethnicity and frequency of organ involvement, except for higher creatine kinase and lactate dehydrogenase levels in adult dermatomyositis (p<0.050). Moreover, a significantly higher frequency of major histocompatibility complex I (96.4% vs. 50.0%, p<0.001) compared with major histocompatibility complex II expression (14.3% vs. 53.6%, p = 0.004) was observed in juvenile dermatomyositis. Fiber damage (p = 0.006) and increased connective tissue (p<0.001) were significantly higher in adult dermatomyositis compared with the presence of perifascicular atrophy (p<0.001). The results of the histochemical and histological data did not correlate with the demographic data or with the clinical and laboratory features. CONCLUSION: The overexpression of major histocompatibility complex I was an important finding for the diagnosis of both groups, particularly for juvenile dermatomyositis, whereas there was lower levels of expression of major histocompatibility complex II than major histocompatibility complex I. This finding was particularly apparent in juvenile dermatomyositis.
  • article 14 Citação(ões) na Scopus
    Kawasaki disease and juvenile systemic lupus erythematosus
    (2012) DINIZ, J. C.; ALMEIDA, R. T.; AIKAWA, N. E.; SALLUM, A. M. E.; SAKANE, P. T.; SILVA, C. A.
    Kawasaki disease (KD) is a common vasculitis in childhood. To the authors' knowledge, only one case of juvenile systemic lupus erythematosus (JSLE)-like onset mimicking KD and another case of KD and JSLE association have previously been described. However, the prevalence of this association of the two diseases was not reported. Therefore, over 27 consecutive years, 5419 patients were followed at the Pediatric Rheumatology Unit and 271 (5%) of them met the ACR classification criteria for JSLE. Two (0.7%) of them were female. These also had KD according to European League against Rheumatism / Paediatric Rheumatology European Society (EULAR/PReS) consensus criteria and are described in this report. One case was a 13-year-old who presented all six KD criteria. Echocardiogram showed pericardial effusion, dilatation and tortuosity of right and left coronary, and her symptoms promptly improved after treatment with intravenous immunoglobulin (IVIG). Lupus diagnosis was established a few days later. Another case was a 4-year-old who had also met all six KD criteria, with improvement after IVIG, and lupus diagnosis was made 1 year later. In conclusion, the frequency of the association between these two autoimmune diseases was rare. The occurrence of a second autoimmune systemic disease in a patient with a history of KD should also be considered. Furthermore, the initial presentation of lupus may mimic KD.
  • article 15 Citação(ões) na Scopus
    Pandemic unadjuvanted influenza A (H1N1) vaccine in dermatomyositis and polymyositis: Immunogenicity independent of therapy and no harmful effect in disease
    (2012) SHINJO, Samuel Katsuyuki; MORAES, Julio Cesar Bertacini de; LEVY-NETO, Mauricio; AIKAWA, Nadia Emi; RIBEIRO, Ana Cristina de Medeiros; SAAD, Carla Goncalves Schahin; PRECIOSO, Alexander; SILVA, Clovis Artur; BONFA, Eloisa
    The goal of the present study was to evaluate the influence of the influenza A H1N1/2009 vaccine on dermatomyositis/polymyositis (DM/PM) disease parameters and the potential deleterious effect of therapy on immune response. Thirty-seven DM and 21 PM patients (Bohan and Peter's criteria) were gender- and age-matched to 116 healthy controls. Seroprotection, seroconversion, the geometric mean titers (GMTs) and the factor increase (FI) in the GMTs were calculated. Disease safety was determined from a muscle enzyme analysis and the DM/PM scores [patient's visual analog scale (VAS), physician's VAS, manual muscle strength (MMT-8)] evaluated pre- and post-vaccination. The mean age (43.1 +/- 9.9 vs. 43.8 +/- 8.4 years, p = 0.607) and gender distribution (p = 1.00) were comparable between the patients and controls. After 21 days, seroconversion (p = 0.394), seroprotection (p = 0.08), GMT (p = 0.573) and the FI in the GMT (p = 0.496) were similar in both groups. The disease and muscle parameters remained stable throughout the study, including the creatine kinase (p = 0.20) and aldolase levels (p = 0.98), the physicians' VAS (p = 1.00), the patients' VAS (p = 1.00) and the MMT-8 (p = 1.00). Regarding the influence of treatment, the seroconversion rates were comparable between the controls and patients undergoing treatment with glucocorticoid (GC) (p = 0.969), GC >0.5 mg/kg/day (p = 0.395) and GC + immunosuppressors (p = 0.285). Vaccine-related adverse events were mild and similar in the DM/PM and control groups (p > 0.05). Our data support the administration of the pandemic influenza A H1N1/2009 vaccination in DM/PM, as we found no short-term harmful effects related to the disease itself and adequate immunogenicity in spite of therapy. Further studies are necessary to identify any long-term adverse effects in patients with these diseases.(c) 2012 Elsevier Ltd. All rights reserved.
  • article 38 Citação(ões) na Scopus
    Subclinical impairment of ovarian reserve in juvenile systemic lupus erythematosus after cyclophosphamide therapy
    (2012) AIKAWA, N. E.; SALLUM, A. M. E.; PEREIRA, R. M. R.; SUZUKI, L.; VIANA, V. S. T.; BONFA, E.; SILVA, C. A.
    Objective To perform systematic assessment of ovarian reserve markers using a combination of tests in juvenile systemic lupus erythematosus (JSLE) patients without amenorrhoea. Methods Twenty-seven consecutive JSLE female patients and 13 healthy controls without amenorrhoea were evaluated for 6 months. Ovarian reserve was assessed during early follicular phase by serum levels of follicle stimulating hormone (FSH), luteinising hormone (LH), estradiol, inhibin A, inhibin B and anti-Mullerian hormone (AMH). Ovarian size was measured by abdominal ultrasonography. Demographic data, disease activity, damage and treatment were also analysed. Results The median of current age was similar in ISLE patients and controls (16.5 vs. 15years, p=0.31) with a significantly higher age at menarche (13 vs. 12years, p=0.03). A trend of lower median total antral follicle count was observed in JSLE compared to controls (9 vs. 14.5, p=0.062) with similar median of other ovarian reserve parameters (p>0.05). Further evaluation of patients treated with cyclophosphamide and those without this treatment revealed a higher median FSH levels (6.4 vs. 4.6 IU/L, p=0.023). Inhibin B, AMH levels and ovarian volume were also lower but did not reach statistical significance (10.8 vs. 27.6 pg/mL, p=0.175; 0.6 vs. 1.5 ng/mL, p=0.276; 3.4 vs. 5 cm(3), p=0.133; respectively). LH (2.7 vs. 2.9 IU/L, p=0.43), estradiol (50 vs. 38 pg/mL, p=0.337) and inhibin A (1.1 vs. 0 pg/mL, p=0.489) levels were comparable in both groups. Conclusions Our study suggests that ovarian reserve after cyclophosphamide treatment may be hampered in spite of the presence of menstrual cycles emphasising the relevance of gonadal protection during the use of this alkylating agent.
  • article 36 Citação(ões) na Scopus
    Immune response and tolerability of varicella vaccine in children and adolescents with systemic lupus erythematosus previously exposed to varicella-zoster virus
    (2012) BARBOSA, C. M. P. L.; TERRERI, M. T. R. A.; ROSARIO, P. O.; MORAES-PINTO, M. I. de; SILVA, C. A. A.; HILARIO, M. O. E.
    Objectives The aim of the present paper is to evaluate the immune response and tolerability of varicella vaccine in children and adolescents with systemic lupus erythematosus previously exposed to varicella-zoster virus. Methods We performed a prospective and controlled study on a group of 54 SLE patients that were chosen at random to be or not to be vaccinated (28 were vaccinated and 26 were not). Twenty-eight healthy controls, of matching age and sex were also vaccinated. All were submitted to a questionnaire, physical evaluation and laboratory assays: lymphocyte immuno-phenotyping by flow cytometry, plasma varicella zoster virus (VZV) serology by ELISA and in vitro interferon gamma (IFN gamma) production by T-cells after stimulus with VZV antigen. They were evaluated before vaccination and at 30, 45, 180 and 360 days afterwards. Results We did not observe any differences in the frequency of adverse events in both vaccinated groups. At study entry, all individuals were seropositive for VZV antibodies. The serum VZV antibody titres similarly increased after vaccination. The frequency of flares and the SLEDAI score were also similar among the patients. Thirty days after vaccination the production of IFN gamma specific to VZV was lower in the SLE group compared to healthy, controls. In the follow-up we observed 4 cases of herpes zoster in the SLE unvaccinated group, but no zoster in the vaccinated group. Conclusion The varicella vaccine was well tolerated in SLE group, who had pre-existing immunity to varicella. The varicella vaccine immunogenicity measurement by serum antibody titres was appropriate. The incidence of HZ was lower in the vaccinated lupus group.