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01.12.2018 | Research article | Ausgabe 1/2018 Open Access

BMC Cardiovascular Disorders 1/2018

Early commitment of cardiovascular autonomic modulation in Brazilian patients with congenital generalized lipodystrophy

BMC Cardiovascular Disorders > Ausgabe 1/2018
Clarisse Mourão Melo Ponte, Virgínia Oliveira Fernandes, Maria Helane Costa Gurgel, Izabella Tamira Galdino Farias Vasconcelos, Lia Beatriz de Azevedo Souza Karbage, Christiane Bezerra Rocha Liberato, Carlos Antônio Negrato, Marília de Brito Gomes, Ana Paula Dias Rangel Montenegro, Renan Magalhães Montenegro Júnior
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Electronic supplementary material

The online version of this article (https://​doi.​org/​10.​1186/​s12872-017-0738-4) contains supplementary material, which is available to authorized users.



Metabolic abnormalities in congenital generalized lipodystrophy (CGL) are associated with microvascular complications. However, the evaluation of different types of neuropathy in these patients, including the commitment of cardiovascular autonomic modulation, is scarce. The objective of the present study was to determine the prevalence of cardiovascular autonomic neuropathy (CAN) in patients with CGL compared with individuals with type 1 diabetes and healthy subjects.


Ten patients with CGL, 20 patients with type 1 diabetes and 20 healthy subjects were included in the study. Controls were paired 1:2 for age, gender, BMI and pubertal stage. Heart rate variability (HRV) was analyzed using cardiovascular autonomic reflex tests, including postural hypotension test, Valsalva (VAL), respiratory (E/I) and orthostatic (30/15) coefficients, and spectral analysis of the HRV, determining very low (VLF), low (LF) and high (HF) frequencies components. The diagnosis of CAN was defined as the presence of at least two altered tests.


CAN was detected in 40% of the CGL patients, 5% in type 1 diabetes patients and was absent in healthy individuals (p < 0.05). We observed a significant reduction in the E/I, VLF, LF and HF in CGL cases vs. type 1 diabetes and healthy individuals and lower levels of 30/15 and VAL in CGL vs. healthy individuals. A significant positive correlation was observed between leptin and 30/15 coefficient (r = 0.396; p = 0.036) after adjusting for insulin resistance and triglycerides. Autonomic cardiovascular tests were associated with HbA1c, HOMA-IR, triglycerides and albumin/creatinine ratio in CGL cases.


We observed a high prevalence of CAN in young patients with CGL, suggesting that insulin resistance, hypertriglyceridemia and hypoleptinemia, may have been involved in early CAN development. Additional studies are needed to evaluate the role of leptinemia in the physiopathogenesis of the condition.
Additional file 1: Table S1. Molecular results of the AGPAT2 and BSCL2 genes in patients with generalized congenital lipodystrophy. Table S2. Results of the cardiovascular autonomic tests of patients with congenital generalized lipodystrophy. Table S3. Symptoms of dysautonomia in patients with congenital generalized lipodistrophy and type 1 diabetes as well as healthy individuals. Table S4. Evaluation of the cardiometabolic parameters and cardiovascular autonomic tests in patients with lipodystrophy and type 1 diabetes. (DOCX 36 kb)
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