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[Cardiometabolic abnormalities in patients with Berardinelli-Seip syndrome].

2010; Sociedade Brasileira de Cardiologia (SBC); Volume: 94; Issue: 1 Linguagem: Inglês

10.1590/s0066-782x2010000100017

ISSN

1678-4170

Autores

Antonio Guedes do Rêgo, Evandro Tinoco Mesquita, Carlos Alberto de Faria, Marcel Alvares Guedes do Rêgo, Maria de Fátima Paiva Baracho, Maria Goretti do Nascimento Santos, Eryvaldo Sócrates Tabosa do Egito, José Brandão Neto,

Tópico(s)

Erythrocyte Function and Pathophysiology

Resumo

Berardinelli-Seip syndrome (BSS) or Generalized Congenital Lipodystrophy often affects the cardiovascular system and also promotes metabolic abnormalities involving glycidic and lipid metabolisms.To assess the prevalence of cardiometabolic abnormalities in patients with BSS.Twenty-two patients from the state of Rio Grande do Norte, Brazil, diagnosed with BSS, underwent clinical evaluation, resting electrocardiogram, echodopplercardiogram, chest X-ray, 24-hour ambulatory electrocardiogram monitoring, exercise testing and laboratory analysis.The patients were predominantly young adults, most of whom women. The whole sample showed insulin resistance, acanthosis nigricans and diminished HDL-cholesterol. The presence of splenomegaly, hepatomegaly, type II diabetes and elevated triglycerides was constant. Metabolic syndrome was characterized in most patients, which were predominantly women and with a high degree of paternal consanguinity. SAH and prehypertension blood pressure were found in more than half of the patients (77.3%). The echodopplercardiogram showed the presence of CLVH (50%), eccentric left ventricular hypertrophy (4.5%), and normal left ventricular geometry (45.5%). High arrhythmia rates were observed by Holter monitoring, such as ventricular ectopic beats, supraventricular ectopic beats and sustained supraventricular tachycardia. Chronotropic incompetence (54.5%) was observed during exercise testing.A high prevalence of cardiovascular and metabolic abnormalities was observed in young asymptomatic individuals with BSS. These findings point to the need for systematic cardiological follow-up and of preventive measures in this high-risk group.

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