GABRIELA NUNES LEAL
Projetos de Pesquisa
Unidades Organizacionais
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina - Médico
3 resultados
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article 13 Citação(ões) na Scopus Long-term evaluation of cardiac function in juvenile idiopathic arthritis under anti-TNF therapy(2014) LIANZA, A. C.; AIKAWA, N. E.; MORAES, J. C. B.; LEAL, G. N.; MORHY, S. S.; ANDRADE, J. L.; BONFA, E.; SILVA, C. A.Objective This paper aims to perform global assessment of long-term cardiac function in juvenile idiopathic arthritis (JIA) patients under TNF blockage therapy. Methods Twenty-five polyarticular-course JIA patients pre-anti-TNF and 22 healthy controls underwent conventional/tissue Doppler echocardiography and cardiac biomarkers measurements (N-terminal pro-brain natriuretic peptide [NT-pro-BNP] and troponin T) at baseline (BL). Twenty-one JIA patients completed six evaluations during two consecutive years. Clinical/laboratorial evaluations were assessed before and during TNF blockage therapy. Results JIA patients and controls were comparable regarding current age (p=0.898) and female gender (p=0.38). At BL isoyolumetric relaxation time of left ventricle (p=0.03), ventricular septum (VS), E' wave (p=0.014) and VS S wave velocity (p=0.03) were significantly reduced in JIA patients compared to controls. Frequencies of elevated NT-pro-BNP and troponin T levels were similar in JIA and controls (p=0.297 and p=0.756) and levels remained within normal range throughout the study, except for one patient with mild troponin T elevation. During TNF blockage therapy, none of the 21 participants had heart failure, ejection fraction or other parameters alterations in conventional and tissue Doppler. Only one had mild pulmonary hypertension. Further analysis revealed that JIA patients with elevated levels of NT-pro-BNP at BL had significantly more active joints (p=0.025) and higher ESR (p=0.034). Conclusion Long-term TNF blockage safety was demonstrated in JIA patients in spite of the observed subclinical diastolic involvement. Elevated cardiac biomarker in these patients was associated with inflammatory parameters reinforcing the need for a careful interpretation of this finding in patients with active disease.- Advantages of early replacement therapy for mucopolysaccharidosis type VI: echocardiographic follow-up of siblings(2014) LEAL, Gabriela N.; PAULA, Ana C. de; MORHY, Samira S.; ANDRADE, Jose L.; KIM, Chong A.Mucopolysaccharidosis type VI (Marateaux-Lamy syndrome) is an autosomal recessive disorder caused by deficient activity of the enzyme N-acetylgalactosamine-4-sulphatase (arylsulphatase B). Cytoplasmic vacuoles full of dermatan sulphate are observed in endothelial cells, myocyte, and fibroblasts, compromising the function of cardiovascular structures and contributing significantly towards morbidity and mortality. The primary objective of this study was to assess the advantages of early replacement therapy with recombinant human arylsulphatase B through the echocardiographic follow-up of sisters who started treatment at quite different ages: one at 9 years and the other at 1 year and 7 months. The older sibling showed striking mitral and aortic valve compromise when she was only 2 years old and finally needed cardiac surgery at the age of 8, even before starting enzyme replacement. Differently, the younger one has developed only mild mitral and aortic lesions throughout the follow-up period of 3 years. The two siblings had left ventricle cardiomyopathy, but partial reverse remodelling was induced by enzyme replacement therapy in both cases. The younger sibling has never received any cardiovascular drugs, whereas the older one has been using beta-blockers and diuretics in addition to enzyme therapy to cope with heart failure. Comparing the outcomes of these two sisters with a very aggressive phenotype of mucopolysaccharidosis type VI, the conclusion was that early onset of therapy may slow down the disease progression and prevent severe cardiac lesions to be established. Moreover, patients' compliance is essential for the success of treatment, as sequential echocardiographic evaluation demonstrated worsening of some cardiac lesions whenever infusions were missed.
conferenceObject Subclinical Right Ventricle Systolic Dysfunction By Two-Dimensional Speckle-Tracking Echocardiography in Childhood-Onset Systemic Lupus Erythematosus Patients(2014) LEAL, Gabriela N.; SILVA, Kellen F.; FRANCA, Camila M. P.; LIANZA, Alessandro C.; ANDRADE, Jose L.; CAMPOS, Lucia M. A.; BONFA, Eloisa; SILVA, Clovis A.