title: Evaluation of epicardial adipose tissue in familial partial lipodystrophy.
abstract: BACKGROUND
  Dunnigan type Familial Partial Lipodystrophy (FPLD) is characterized by loss of subcutaneous fat from the limbs and excessive accumulation on the visceral adipose tissue (VAT). Affected individuals have insulin resistance (IR), diabetes, dyslipidemia and early cardiovascular (CV) events, due to their imbalanced distribution of total body fat (TBF). Epicardial adipose tissue (EAT) is correlated with VAT. Hence, EAT could be a new index of cardiac and visceral adiposity with great potential as a marker of CV risk in FPLD.
  
  
  OBJECTIVE
  Compare EAT in FPLD patients versus healthy controls. Moreover, we aimed to verify if EFT is related to anthropometrical (ATPM) and Dual-Energy X-ray Absorptiometry (DEXA) measures, as well as laboratory blood findings. We postulated that FPLD patients have enlarged EAT.
  
  
  METHODS
  This is an observational, cross-sectional study. Six patients with a confirmed mutation in the LMNA gene for FPLD were enrolled in the study. Six sex, age and BMI-matched healthy controls were also selected. EFT was measured by transthoracic echocardiography (ECHO). All participants had body fat distribution evaluated by ATPM and by DEXA measures. Fasting blood samples were obtained for biochemical profiles and also for leptin measurements.
  
  
  RESULTS
  Median EFT was significantly higher in the FPLD group than in matched controls (6.0 ± 3.6 mm vs. 0.0 ± 2.04 mm; p = 0.0306). Additionally, FPLD patients had lower leptin values. There was no significant correlation between EAT and ATPM and DEXA measurements, nor laboratory findings.
  
  
  CONCLUSIONS
  This study demonstrates, for the first time, that EAT measured by ECHO is increased in FPLD patients, compared to healthy controls. However, it failed to prove a significant relation neither between EAT and DEXA, ATPM or laboratory variables analyzed.
authors: Metabolism Unit, State Institute of Diabetes and Endocrinology, IEDE, Rio de Janeiro, Brazil.
authors: Amélio Fernando
authors: AF
authors: Godoy-Matos
authors: Metabolism Unit, State Institute of Diabetes and Endocrinology, IEDE, Rio de Janeiro, Brazil.
authors: Cynthia M
authors: CM
authors: Valério
authors: Metabolism Unit, State Institute of Diabetes and Endocrinology, IEDE, Rio de Janeiro, Brazil.
authors: Juliana Bonadiman E
authors: JB
authors: Bragança
authors: Metabolism Unit, State Institute of Diabetes and Endocrinology, IEDE, Rio de Janeiro, Brazil.
authors: Ricardo de Andrade
authors: Rde A
authors: Oliveira
authors: Metabolism Unit, State Institute of Diabetes and Endocrinology, IEDE, Rio de Janeiro, Brazil.
authors: Roberto Luis
authors: RL
authors: Zagury
authors: National Cardiology Institute of Laranjeiras, Rio de Janeiro, Brazil.
authors: Rodolfo de Paula
authors: Rde P
authors: Lustosa
authors: National Cardiology Institute of Laranjeiras, Rio de Janeiro, Brazil.
authors: Gabriel Cordeiro
authors: GC
authors: Camargo
authors: National Cardiology Institute of Laranjeiras, Rio de Janeiro, Brazil.
authors: César Augusto da Silva
authors: CA
authors: Nascimento
authors: Metabolism Unit, State Institute of Diabetes and Endocrinology, IEDE, Rio de Janeiro, Brazil.
authors: Rodrigo O
authors: RO
authors: Moreira
keywords: Dual energy x-ray absorptiometry; Echocardiography; Epicardial adipose tissue; Lipodystrophy; Metabolic syndrome