Purpose: Congenital generalized lipodystrophy (CGL) is a rare autosomal recessive disorder characterized by the absence of functional adipocytes resulting in ectopic lipid storage, metabolic disorders and early cardiovascular disease. Two-dimensional speckle-tracking (2D-STE) allows the detection of early abnormalities in myocardial function. We aimed to evaluate myocardial deformation in a large sample of CGL patients using 2D-STE. Patients and Methods: A cross-sectional study of 22 patients with CGL and 22 healthy subjects, matched for sex and age, was conducted from 2013 to 2018. All participants had undergone standard conventional echocardiography (ECHO) and 2D-STE. Determination of blood glucose, lipids, insulin, and leptin were performed in all CGL patients. Results: In the CGL group the mean age was 14.6±10.7 years where 68.2% (n=15) were younger than 18 years old. All the patients had hypoleptinemia, 95.4% (21/22) low HDL-c, 86.4% (19/22) hypertriglyceridemia, 68.2% (15/22) diabetes, 50% (11/22) hepatic steatosis, 41% (9/22) insulin resistance, 41% (9/22) hypercholesterolemia, and 18.2% (4/22) hypertension. ECHO showed that 36.6% (8/22) of CGL patients presented diastolic dysfunction, 31.8% (7/22) left ventricular hypertrophy (LVH), 27.3% (6/22) increased left atrial volume index (LAVI), and 18.2% (4/22) increased left ventricular systolic diameter (LVDS) but normal ejection fraction (EF), whether using 2D-STE, 68.2% (15/22) of CGL patients showed abnormal global longitudinal strain (GLS) (p<0.01), and in almost LV segments. Positive association between abnormal GLS and A1c (r=0.57, p=0.005), glucose (r=0.5, p=0.018) and basal insulin (r= 0.69, p= 0.024), and negative association with leptin (r = −0.51, p = 0.005) were found in these patients. Conclusion: The 2D-STE revealed precocious left ventricular systolic dysfunction in a young CGL population with normal systolic function by ECHO. Early exposure to common metabolic abnormalities as insulin resistance, hyperglycemia, and hypoleptinemia must be involved in myocardial damage in these patients.
Resumo: Este artigo apresenta o desenvolvimento de um estudo de caso, voltado ao desenvolvimento do Pensamento Computacional. O estudo de caso compreendeu a utilização da ferramenta Scratch, permitindo ampliar o conhecimento dos alunos da Escola Estadual de Ensino Médio Lucila Nogueira, localizada na cidade de Boa Vista das Missões/RS. Foram realizadas oficinas práticas utilizando o Scratch para que os alunos desenvolvessem noções de lógica de programação, estimulando o desenvolvimento do Pensamento Computacional e, também, despertando o interesse pela área de Tecnologia da Informação. Os resultados obtidos neste estudo de caso permitiram comprovar que o uso da ferramenta Scratch potencializou os processos de ensino e de aprendizagem relacionados ao Pensamento Computacional, por meio da observação realizada e, também, com base nos resultados obtidos a partir da aplicação de um instrumento de pesquisa.
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