Teste de caminhada de seis minutos como um marcador de capacidade funcional na Lipodistrofia Generalizada Congênita

Abstract

Background: Congenital Generalized Lipodystrophy (CGL) is a rare disease characterized by metabolic outcomes such as hypertriglyceridemia, hyperinsulinemia, hypoleptinemia, hypoadiponectinemia and diabetes mellitus. Cardiovascular and respiratory disorders have been noted previously. Considering clinical aspects of CGL patients, especially cardiovascular complications, functional capacity can be considered an important clinical and prognostic marker. The aim of the study was to evaluate the functional capacity of CGL patients, the role of metreleptin (MLP) function and the occurrence of peripheral arterial disease (PAD) in this population. Methods: cohort study conducted between 2018 and 2019 with 12 subjects with CGL compared to a healthy control group. Functional capacity and the occurrence of PAD were assessed using the six-minute walk test (6MWT) and the ankle-brachial index (ABI), respectively. Genetic diagnoses, sociodemographic and anthropometric data were obtained. Correlations between metabolic parameters, ABI and 6MWT were also performed. Results: CGL patients had a reduction in predicted distance walked (DTC6) (p = 0.009) and increased heart rate (HR), systolic (SBP) and diastolic (DBP) blood pressure after the 6MWT compared to healthy individuals (p <0 .05). We found that only 25% (n = 3) of the CGL subjects had ABI 0.9 and were classified as PAD. Individuals with CGL had no changes in ABI and blood pressure 12 months after MLP replacement but walked a higher 6MWD compared to baseline (p = 0.04). In addition, 6MWD and right ABI measurements were positively correlated in individuals with CGL (p = 0.03). The right ABI was negatively correlated with glucose, triglycerides and VLDL-c (p < 0.05). Conclusions: the data suggest that CGL patients have lower functional capacity and only 25% of the sample has PAD. Individuals with higher ITB had higher 6MWD.