GABRIELA NUNES LEAL
Projetos de Pesquisa
Unidades Organizacionais
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina - Médico
6 resultados
Resultados de Busca
Agora exibindo 1 - 6 de 6
- Echocardiographic Follow-up of Perinatally HIV-infected Children and Adolescents Results From a Single-center Retrospective Cohort Study in Brazil(2020) VALLILO, Nathalia Gaspar; DURIGON, Giuliana Stravinskas; LIANZA, Alessandro Cavalcanti; DINIZ, Maria de Fatima Rodrigues; SAWAMURA, Karen Saori Shiraishi; BRITO, Carolina Rocha; MARQUES, Heloisa Helena de Souza; FERRARO, Alexandre Archanjo; LEAL, Gabriela NunesBackground: The effects of HIV and antiretroviral therapy on cardiovascular system of perinatally infected children throughout their development are not fully understood. Objectives: To determine the prevalence of cardiac abnormalities in a retrospective cohort of perinatally HIV-infected patients and to investigate associations between echocardiographic and clinical data during their follow-up. Methods: Review of medical records and echocardiogram reports of 148 perinatally HIV-infected patients between January 1991 and December 2015. Results: Four hundred and eighty echocardiograms were analyzed and 46 (31%) patients showed cardiac abnormalities, frequently subclinical and transient. Nadir CD4 count was higher in patients with consistently normal echocardiogram: 263 (4-1480) versus 202 (5-1746) cells/mu L, P = 0.021. Right ventricular (RV) dilation was detected in 18.9%, left ventricular (LV) dilation in 21.6%, septal hypertrophy in 12.2%, LV posterior wall hypertrophy in 6%, LV systolic dysfunction in 8% and pulmonary hypertension in 8.7% of patients. Opportunistic infections were associated with RV dilation [odds ratio (OR = 4.34; 1.78-10.53; P < 0.01)], pulmonary hypertension (OR = 8.78; 2.80-27.51; P < 0.01) and LV systolic dysfunction (OR = 5.38; 1.55-18.71; P < 0.01). Longer duration of highly active antiretroviral therapy was associated with reduced risk of LV dilation (OR = 0.91; 0.85-0.97; P < 0.01) and systolic dysfunction (OR = 0.71; 0.59-0.85; P < 0.01). Protease inhibitors use was associated with reduced risk of RV dilation (OR = 0.54; 0.30-0.97; P < 0.05), LV dilation (OR = 0.35; 0.21-0.60; P < 0.01) and LV systolic dysfunction (OR = 0.07; 0.02-0.31; P < 0.01). Higher CD4 count was associated with lower risk of LV systolic dysfunction (OR = 0.82; 0.69-0.98; P < 0.05). Conclusions: Echocardiograms identified cardiac abnormalities among children with perinatally acquired HIV infection, and data suggest that immunologic status and therapeutic strategies throughout development can influence cardiac disease burden in this population.
- Mucopolysaccharidosis type VI: case report with first neonatal presentation with ascites fetalis and rapidly progressive cardiac manifestation(2020) HONJO, Rachel Sayuri; VACA, Evelyn Cristina Nunez; LEAL, Gabriela Nunes; ABELLAN, Deipara Monteiro; IKARI, Nana Miura; JATENE, Marcelo Biscegli; MARTINS, Ana Maria; KIM, Chong AeBackground The Mucopolysaccharidosis type VI (MPS VI), also known as Maroteaux-Lamy syndrome (OMIM 253200) is an autosomal recessive lysosomal disorder, caused by the deficiency of the enzyme N-acetylgalactosamine 4-sulfatase (also known as arylsulfatase B) due to mutations of the ARSB gene. Cardiologic features are well recognized, and are always present in MPS VI patients. Generally, the onset and the progression of the cardiologic symptoms are insidious, and just a few patients have developed a rapidly progressive disease. Cardiac involvement in MPS VI is a common and progressive feature. For MPS patients, cardiac evaluations are recommended every 1 to 2 years, including blood pressure measurement, electrocardiography and echocardiography. However, congestive heart failure and valvular surgical repair are not frequently seen, and if so, they are performed in adults. Here we report on an atypical MPS VI case with ascites fetalis and a rapidly progressive cardiac disease. Case presentation A 6-month-old Brazilian male, only child of a Brazilian healthy non-consanguineous couple. During pregnancy, second trimester ultrasonography observed fetal ascites and bilateral hydrocele. Physical exam at 6 months-old revealed a typical gibbus deformity and MPS was suspected. Biochemical investigation revealed a diagnosis of MPS type VI, confirmed by molecular test. Baseline echocardiogram revealed discrete tricuspid regurgitation and a thickened mitral valve with posterior leaflet prolapse, causing moderate to severe regurgitation. The patient evolved with mitral insufficiency and congestive heart failure, eventually requiring surgical repair by the first year of age. Conclusions We report the first case of MPS VI whose manifestations started in the prenatal period with fetal ascites, with severe cardiac valvular disease that eventually required early surgical repair. Moreover, in MPS with neonatal presentation, including fetal hydrops, besides MPS I, IVA and VII, clinicians should include MPS VI in the differential diagnosis.
- Influence of patent ductus arteriosus on left ventricular myocardial deformation in preterm neonates in the early neonatal period(2020) ALMEIDA, Kellen Freitas Silva de; LEAL, Gabriela Nunes; MORHY, Samira Saady; RODRIGUES, Ana Clara Tude; CERRI, Giovanni G.; DORIA-FILHO, Ulysses; ANDRADE, Jose Lazaro de
- Position Statement on Indications for Echocardiography in Fetal and Pediatric Cardiology and Congenital Heart Disease of the Adult-2020(2020) MORHY, Samira Saady; BARBERATO, Silvio Henrique; LIANZA, Alessandro Cavalcanti; SOARES, Andressa Mussi; LEAL, Gabriela Nunes; RIVERA, Ivan Romero; BARBERATO, Marcia Ferreira Alves; GUERRA, Vitor; RIBEIRO, Zilma Vercosa de Sa; PIGNATELLI, Ricardo; ROCHITTE, Carlos Eduardo; VIEIRA, Marcelo Luiz Campos
- Severe clinical spectrum with high mortality in pediatric patients with COVID-19 and multisystem inflammatory syndrome(2020) PEREIRA, Maria Fernanda Badue; LITVINOV, Nadia; FARHAT, Sylvia Costa Lima; EISENCRAFT, Adriana Pasmanik; GIBELLI, Maria Augusta Bento Cicaroni; CARVALHO, Werther Brunow de; FERNANDES, Vinicius Rodrigues; FINK, Thais de Toledo; FRAMIL, Juliana Valeria de Souza; GALLETI, Karine Vusberg; FANTE, Alice Lima; FONSECA, Maria Fernanda Mota; WATANABE, Andreia; PAULA, Camila Sanson Yoshino de; PALANDRI, Giovanna Gavros; LEAL, Gabriela Nunes; DINIZ, Maria de Fatima Rodrigues; PINHO, Joao Renato Rebello; SILVA, Clovis Artur; MARQUES, Heloisa Helena de SousaOBJECTIVES: To assess the outcomes of pediatric patients with laboratory-confirmed coronavirus disease (COVID-19) with or without multisystem inflammatory syndrome in children (MIS-C). METHODS: This cross-sectional study included 471 samples collected from 371 patients (age<18 years) suspected of having severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. The study group comprised 66/371 (18%) laboratory-confirmed pediatric COVID-19 patients: 61 (92.5%) patients tested positive on real-time reverse transcription-polymerase chain reaction tests for SARS-CoV-2, and 5 (7.5%) patients tested positive on serological tests. MIS-C was diagnosed according to the criteria of the Center for Disease Control. RESULTS: MIS-C was diagnosed in 6/66 (9%) patients. The frequencies of diarrhea, vomiting, and/or abdominal pain (67% vs. 22%, p=0.034); pediatric SARS (67% vs. 13%, p=0.008); hypoxemia (83% vs. 23%, p=0.006); and arterial hypotension (50% vs. 3%, p=0.004) were significantly higher in patients with MIS-C than in those without MIS-C. The frequencies of C-reactive protein levels >50 mg/L (83% vs. 25%, p=0.008) and D-dimer levels >1000 ng/mL (100% vs. 40%, p=0.007) and the median D-dimer, troponin T, and ferritin levels (p < 0.05) were significantly higher in patients with MIS-C. The frequencies of pediatric intensive care unit admission (100% vs. 60%, p=0.003), mechanical ventilation (83% vs. 7%, p < 0.001), vasoactive agent use (83% vs. 3%, p <0.001), shock (83% vs. 5%, p<0.001), cardiac abnormalities (100% vs. 2%, p <0.001), and death (67% vs. 3%, p < 0.001) were also significantly higher in patients with MIS-C. Similarly, the frequencies of oxygen therapy (100% vs. 33%, p=0.003), intravenous immunoglobulin therapy (67% vs. 2%, p < 0.001), aspirin therapy (50% vs. 0%, p < 0.001), and current acute renal replacement therapy (50% vs. 2%, p=0.002) were also significantly higher in patients with MIS-C. Logistic regression analysis showed that the presence of MIS-C was significantly associated with gastrointestinal manifestations [odds ratio (OR)=10.98; 95%CI (95% confidence interval)=1.20-100.86; p=0.034] and hypoxemia [OR=16.85; 95%CI=1.34-211.80; p=0.029]. Further univariate analysis showed a positive association between MIS-C and death [OR=58.00; 95%CI=6.39526.79; p <0 .0001]. CONCLUSIONS: Pediatric patients with laboratory-confirmed COVID-19 with MIS-C had a severe clinical spectrum with a high mortality rate. Our study emphasizes the importance of investigating MIS-C in pediatric patients with COVID-19 presenting with gastrointestinal involvement and hypoxemia.
- SARS-CoV-2 in cardiac tissue of a child with COVID-19-related multisystem inflammatory syndrome(2020) DOLHNIKOFF, Marisa; FERRANTI, Juliana Ferreira; MONTEIRO, Renata Aparecida de Almeida; DUARTE-NETO, Amaro Nunes; GOMES-GOUVEA, Michele Soares; DEGASPARE, Natalia Viu; DELGADO, Artur Figueiredo; FIORITA, Carolina Montanari; LEAL, Gabriela Nunes; RODRIGUES, Regina Maria; CHAIM, Khallil Taverna; PINHO, Joao Renato Rebello; CARNEIRO-SAMPAIO, Magda; MAUAD, Thais; SILVA, Luiz Fernando Ferraz da; CARVALHO, Werther Brunow de; SALDIVA, Paulo Hilario Nascimento; CALDINI, Elia Garcia