BERNADETE DE LOURDES LIPHAUS

(Fonte: Lattes)
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Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/36 - Laboratório de Pediatria Clínica, Hospital das Clínicas, Faculdade de Medicina

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Autor: CARNEIRO-SAMPAIO, Magda × Indexador: MEDLINE × Tipo de acesso: restrictedAccess ×

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Agora exibindo 1 - 6 de 6
  • article 31 Citação(ões) na Scopus
    Inflammasome polymorphisms in juvenile systemic lupus erythematosus
    (2015) PONTILLO, Alessandra; REIS, Edione C.; LIPHAUS, Bernadete L.; SILVA, Clovis A.; CARNEIRO-SAMPAIO, Magda
    Inflammasome is the cytoplasmic complex responsible for pro-IL1 cleavage and secretion of IL-1. Recently our group reported the first association between polymorphisms in the inflammasome receptor NLRP1 and adult-onset systemic lupus erythematosus (SLE) di per se and especially in SLE-associated renal disease, suggesting the involvement of NLRP1-inflammasome in the immune dysregulation characteristic of SLE patients. Considering that juvenile-onset SLE (JSLE) is more severe than adult SLE, and that the genetic background plays a major role in the early development of autoimmune diseases, we analysed selected polymorphisms in inflammasome genes (NLRP1, NLRP3, CARD8, IL1B, TNFAIP3) of children and adolescents with JSLE (n=90) and in healthy controls (n=144). A single polymorphism in IL1B, and not NLRP1, gene resulted in association with JSLE, suggesting that IL-1 is involved in the pathogenesis of SLE, but different genes could play specific role in adult- or early-onset disease.
  • article 1 Citação(ões) na Scopus
    Increased Soluble Cytoplasmic Bcl-2 Protein Serum Levels and Expression and Decreased Fas Expression in Lymphocytes and Monocytes in Juvenile Dermatomyositis
    (2018) LIPHAUS, Bernadete L.; SALLUM, Adriana E. M.; AIKAWA, Nadia E.; KISS, Maria Helena B.; CARRASCO, Solange; PALMEIRA, Patricia; LIMA, Laila; SILVA, Clovis A.; GOLDENSTEIN-SCHAINBERG, Claudia; CARNEIRO-SAMPAIO, Magda
    Objective. To evaluate soluble Fas antigen (sFas), sFas ligand (sFasL), soluble tumor necrosis factor-related apoptosis-inducing ligand, and soluble cytoplasmic Bcl-2 protein (sBcl-2) serum levels, Fas and Bcl-2 expressions in T and B lymphocytes and monocytes and relations with erythrocyte sedimentation rate, C-reactive protein (CRP), Childhood Myositis Assessment Scale, and manual muscle testing in juvenile dermatomyositis (JDM). Methods. Serum levels were determined by ELISA and peripheral cell expressions by flow cytometry for patients with JDM or juvenile idiopathic arthritis (JIA), and healthy controls. Results. Patients with JDM had increased sBcl-2, which correlated with CRP. Expression of Bcl-2 was increased and expression of Fas was decreased in CD3+, CD4+, and CD8+ T lymphocytes compared with JIA and/or healthy controls. Conclusion. Patients with JDM presented a unique apoptosis-related proteins profile, which may contribute to disease development.
  • article 33 Citação(ões) na Scopus
    Low C4, C4A and C4B gene copy numbers are stronger risk factors for juvenile-onset than for adult-onset systemic lupus erythematosus
    (2016) PEREIRA, Kaline M. C.; FARIA, Atila G. A.; LIPHAUS, Bernadete L.; JESUS, Adriana A.; SILVA, Clovis A.; CARNEIRO-SAMPAIO, Magda; ANDRADE, Luis E. C.
    Objective. Complete deficiency of Complement C4 component is a strong genetic risk factor for SLE. C4 is encoded by two different genes, C4A and C4B, which show considerable gene copy number (GCN) variation. This study investigates the association of total C4, C4A and C4B GCN with JSLE. Methods. Ninety JSLE patients, 170 adult-onset SLE (aSLE) patients and 200 healthy individuals were evaluated for C4A and C4B GCN by quantitative real-time PCR. Results. JSLE patients had lower GCN for C4A (mean = 1.7; 95% CI: 1.5, 1.9) and C4B (mean = 1.5; 95% CI: 1.3, 1.6) compared with healthy individuals (mean C4A = 2.3; 95% CI: 2.2, 2.5, P< 0.001; C4B = 2.0; 95% CI: 1.8, 2.1; P< 0.001) or with aSLE patients (mean C4A = 1.9; 95% CI: 1.8, 2.1, P = 0.006; mean C4B = 1.8; 95% CI: 1.7, 1.9, P< 0.001). Low total C4 GCN (< 4 copies) was more frequent in JSLE than in healthy individuals (59% vs 28%; P< 0.001). The same was observed for low C4A (<= 1 copy) (52% vs 18%; P< 0.001) and for low C4B (60% vs 31%; P< 0.001). JSLE had a stronger association with low total C4 (OR = 3.68, 95% CI: 2.19, 6.20), C4A (OR = 4.98, 95% CI: 2.88, 8.62) and C4B (OR = 3.26; 95% CI: 1.95, 5.47) than aSLE (C4 OR= 2.03; 95% CI: 1.32, 3.13; C4A OR= 2.36; 95% CI: 1.46, 3.81; C4B OR= 1.13; 95% CI: 0.73, 1.74). In addition, pericarditis in JSLE patients was associated with low C4 (OR= 4.13; 95% CI: 1.02, 16.68; P = 0.047) and low C4A (OR = 5.54; 95% CI: 1.37, 22.32; P = 0.016). Conclusion. Low total C4, C4A and C4B GCN were associated with a stronger risk for developing JSLE than aSLE. Additionally, low total C4 and C4A GCN are risk factors for pericarditis in JSLE.
  • article 9 Citação(ões) na Scopus
    Increased serum sFas, sTRAIL, and reduced sFasL in juvenile-onset systemic lupus erythematosus
    (2017) LIPHAUS, Bernadete L.; KISS, Maria Helena B.; CARRASCO, Solange; PALMEIRA, Patricia; GOLDENSTEIN-SCHAINBERG, Claudia; CARNEIRO-SAMPAIO, Magda
    The aims of this study were to assess serum Fas, FasL, TRAIL, and Bcl-2 levels in patients with juvenile-onset systemic lupus erythematosus (JSLE) and to evaluate their relations with disease activity parameters and nephritis. Forty-eight JSLE patients, 33 juvenile idiopathic arthritis (JIA, inflammatory controls) patients and 40 healthy controls were enrolled. sFas, sFasL, sTRAIL, and sBcl-2 serum levels were measured by ELISA. Disease activity parameters included SLEDAI score, ESR, anti-dsDNA antibodies, C3, and C4 levels. Thirty-five JSLE patients had nephritis and 32 patients were classified as having active disease (SLEDAI ae4). Statistical analysis methods included Mann-Whitney test and Spearman's rank test. JSLE patients had significantly increased sFas serum levels compared with healthy controls (median 177.6 vs. 117.5 pg/mL; p = 0.0001), higher sTRAIL (median 484.6 vs 270.8 pg/mL; p = 0.02), and reduced sFasL (median 0.05 vs 0.3 ng/mL; p = 0.0002). The same results were observed for JSLE patients with active disease and for patients with nephritis. Additionally, sFas levels in JSLE patients directly correlated with SLEDAI score (r = 0.40; p = 0.009), and sTRAIL levels were increased in JSLE patients with neuropsychiatric disease compared with those without this involvement (median 667.9 vs. 216.2 pg/mL; p = 0.03). Otherwise, sBcl-2 levels of JSLE patients were similar to healthy controls. JIA patients had sFas, sFasL, sTRAIL, and sBcl-2 serum levels similar to JSLE patients and to healthy controls. In summary, this study characterized in JSLE a distinct profile from adult SLE that comprises increased sFas, sTRAIL, and reduced sFasL, notably in patients with active disease and with nephritis.
  • article 5 Citação(ões) na Scopus
    Increased sMer, but not sAxl, sTyro3, and Gas6 relate with active disease in juvenile systemic lupus erythematosus
    (2020) LIPHAUS, Bernadete L.; LIMA, Laila; PALMEIRA, Patricia; SILVA, Clovis A.; GOLDENSTEIN-SCHAINBERG, Claudia; CARNEIRO-SAMPAIO, Magda
    Introduction/objectives Tyro3, Axl, and Mer (TAM) receptors and ligands mediate apoptotic bodies engulfment which alteration has been related with juvenile systemic lupus erythematosus (JSLE) pathogenesis. Thus, the aim was to determine their soluble levels. Methods Serum sTyro3, sAxl, sMer, and Gas6 levels were measured using ELISA in 67 JSLE patients, 12 juvenile idiopathic arthritis (JIA) inflammatory and 20 healthy controls and related with SLEDAI-2K score, anti-dsDNA antibody, ESR, CRP, C3, C4 levels, and nephritis. Results JSLE patients with active disease (SLEDAI-2K> 4) had significantly increased sMer levels compared with healthy controls (median 8.4 vs. 6.0 ng/mL, p = 0.009) and inactive disease patients (5.2 ng/mL, p = 0.0003). sMer levels correlated with SLEDAI-2K (r = 0.44; p = 0.0004) and ESR (r = 0.24; p = 0.04), while sAxl correlated with SLEDAI-2K (r = 0.33; p = 0.008) and C4 levels (r = - 0.24; p = 0.04). JSLE patients taking glucocorticoid had increased sAxl and sMer levels. Moreover, sAxl correlated with sMer and sTyro3 levels. Patients with nephritis and those with focal or diffuse proliferative glomerulonephritis had these protein levels similar to healthy controls and patients without renal involvement. sTyro3 levels of JSLE patients taking glucocorticoid were decreased, and correlated with Gas6 and sAxl, while Gas6 levels correlated with age upon enrollment. JIA controls had protein levels similar to healthy controls and JSLE patients. Conclusions This study reinforces that sMer is increased in active JSLE patients, yet sMer and sAxl correlates with disease activity parameters, and their alterations are disease-specific. However, further studies are needed to determine exact roles of sTyro3 and Gas6 in disease pathogenesis.
  • conferenceObject
    Low Gene Copy Number for C4, C4A and C4B Is a Strong Risk Factor for Developing Systemic Lupus Erythematosus in Childhood
    (2012) ANDRADE, Luis Eduardo C.; PEREIRA, Kaline M. C.; FARIA, Atila G. A.; LIPHAUS, Bernadete; JESUS, Adriana A.; SILVA, Clovis; CARNEIRO-SAMPAIO, Magda
    Background/Purpose: C4 is an important component of the Complement system and plays an essential role in the activation cascade of the classical Complement pathway. Complete C4 deficiency is among the strongest genetic risk factors for systemic lupus erythematosus (SLE). There are two C4 circulating isoforms (C4A and C4B) encoded by C4A and C4B genes, respectively, that differ by only five nucleotides. C4A protein is involved in the clearance of immune complex and apoptotic debris while C4B protein is relevant in the opsonization of microbes. C4A and C4B genes are located at a gene cassette within the MHC class III region and depict gene copy-number variation (CNV). The number of C4A copies may be related to the susceptibility to SLE. This study aimed to investigate the impact of the C4A and C4B gene CNV on juvenile SLE. Methods: We evaluated 90 children and 170 adults with SLE (meeting SLE ACR criteria) sequentially retrieved from the rheumatology outpatient clinic. Two hundred healthy individuals (HI) without evidence of auto-immune diseases were retrieved among blood bank donors. Peripheral blood leukocyte DNA was amplified by quantitative real-time PCR (qPCR) with primers for C4 gene and sequence specific TaqMan® probes for C4A (5FAM-ACCCCTGTCCAGTGTTAG-MGB 3) and C4B (5FAM-ACCTCTCTCCAGTGATAC-MGB 3). Gene copy number (GCN) was determined by the delta-delta cycle threshold (DDCT) method. Results: Children with SLE had lower GCN of total C4 (mean total C4=3.1; 95% CI=2.8–3.4), C4A (mean C4A=1.7; 95% CI=1.5–1.9) and C4B (mean C4B=1.5; 95% CI=1.3–1.6) than HI (C4=4.3 with 95% CI=4.1–4.5, p<0.001; C4A =2.3 with 95% CI=2.2–2.5, p<0.001; C4B=2.0 with 95% CI=1.8–2.1; p<0.001). The frequency of SLE children with total C4 low GCN (<4 copies) was significantly higher than in HI (SLE=59% versus HI=28%; OR=3.68; 95% CI=2.19–6.20; p<0.001). The same was observed for C4A low GCN (2