GABRIELA NUNES LEAL
Projetos de Pesquisa
Unidades Organizacionais
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina - Médico
4 resultados
Resultados de Busca
Agora exibindo 1 - 4 de 4
- Subclinical left ventricular dysfunction in childhood-onset systemic lupus erythematosus: a two-dimensional speckle-tracking echocardiographic study(2016) LEAL, G. N.; SILVA, K. F.; LIANZA, A. C.; GIACOMIN, M. F.; ANDRADE, J. L.; KOZU, K.; BONFA, E.; SILVA, C. A.Objectives: The main purpose of the study was to investigate left ventricular (LV) subclinical systolic and diastolic dysfunction in childhood-onset systemic lupus erythematosus (c-SLE) patients using two-dimensional speckle-tracking (2DST) echocardiography. We also interrogated possible correlations between impairment of myocardial deformation and the SLE Disease Activity Index 2000 (SLEDAI-2K), as well as the presence of traditional and disease-related cardiovascular risk factors (CRFs).Method: A total of 50 asymptomatic patients and 50 controls (age 14.74 vs. 14.82years, p=0.83) were evaluated by standard and 2DST echocardiography.Results: Despite a normal ejection fraction (EF), there was reduction in all parameters of LV longitudinal and radial deformation in patients compared to controls: peak longitudinal systolic strain (PLSS) [-20.3 (-11 to -26) vs. -22 (-17.8 to -30.4)%, p<0.0001], PLSS rate [-1.190.21 vs. -1.3 +/- 0.25s(-1), p=0.0005], longitudinal strain rate in early diastole [1.7 (0.99-2.95) vs. 2 (1.08-3.00) s(-1), p=0.0034], peak radial systolic strain [33.09 +/- 8.6 vs. 44.36 +/- 8.72%, p<0.0001], peak radial systolic strain rate [1.98 +/- 0.53 vs. 2.49 +/- 0.68s(-1), p<0.0001], and radial strain rate in early diastole [-2.31 +/- 0.88 vs. -2.75 +/- 0.97s(-1), p=0.02]. Peak circumferential systolic strain [-23.67 +/- 3.46 vs. -24.6 +/- 2.86%, p=0.43] and circumferential strain in early diastole [0.37 +/- 0.17 vs. 0.41 +/- 0.15, p=0.27] were similar between patients and controls, although peak circumferential systolic strain rate [-1.5 +/- 0.3 vs. -1.6 +/- 0.3s(-1), p=0.036] was reduced in c-SLE. Further analysis of patients revealed a negative correlation between LV PLSS and SLEDAI-2K (r= -0.52, p<0.0001), and also between LV PLSS and the number of CRFs per patient (r= -0.32, p=0.024).Conclusions: 2DST echocardiography has identified subclinical LV deformation impairment in c-SLE patients. Disease activity and cumulative exposure to CRFs contribute to myocardial compromise.
- Strain Echocardiography in Pediatric Sepsis: Direct Guide to Hemodynamic Therapy in the Future?(2016) LEAL, Gabriela Nunes; COLLETI JR., Jose; CARVALHO, Werther Brunow de
- Impact of early enzyme-replacement therapy for mucopolysaccharidosis VI: results of a long-term follow-up of Brazilian siblings(2016) FRANCO, J. F.; SOARES, D. C.; TORRES, L. C.; LEAL, G. N.; CUNHA, M. T.; HONJO, R. S.; BERTOLA, D. R.; KIM, C. A.Mucopolysaccharidosis type VI (MPS VI) is an autosomal recessive multisystem lysosomal storage disorder, which is characterized by the deficiency of the enzyme arylsulfatase B encoded by the ARSB gene. Treatment of this disease with enzyme-replacement therapy (ERT) improves the clinical status of and generates hope for MPS VI patients. However, only few reports on patients with MPS VI treated before 5 years of age have been published. Thus, the objective of this study was to compare the clinical parameters of two sisters affected by MPS VI who started ERT at different ages (9 years and 1 year 5 months, respectively) and to determine the most relevant clinical impacts of early treatment after 85 months of evaluation. The treatment was well tolerated by both siblings. ERT in the younger sibling resulted in increased growth, an improved 6-minute walk test, less coarse face, slower progression of cardiac valve disease, and the absence of compressive myelopathy compared to that in her older sister. On the other hand, the older sibling had typical MPS VI phenotypic features before the commencement of ERT. Corneal clouding, clawed hands, and progressive skeletal changes were observed in both siblings despite the treatment. Both siblings displayed reduced frequencies of upper respiratory infections and apnea indices. This study emphasizes that early diagnosis and treatment of MPS VI are critical for a better disease outcome and to enhance the quality of life for these patients.
- What are the benefits of two-dimensional speckle tracking echocardiography for diagnosis and treatment follow-up of childhood-onset systemic lupus erythematosus myocarditis?(2016) LEAL, Gabriela Nunes; DINIZ, Maria De Fatima; BRUNELLI, Juliana; LIANZA, Alessandro C.; SALLUM, Adriana M. E.; SILVA, Clovis A.