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More than 70 years have passed since the first description of Klinefelter Syndrome (KS), the most frequent chromosome disorder causing male infertility and hypogonadism. KS is associated with increased cardiovascular (CV) mortality due to several comorbidities, including hypogonadism, as well as metabolic syndrome and type 2 diabetes, which are highly prevalent in these patients. Aside from metabolic disturbances, patients with KS suffer from both acquired and congenital CV abnormalities, cerebrovascular thromboembolic disease, subclinical atherosclerosis and endothelial dysfunction, which may all contribute to increased CV mortality. The mechanisms involved in this increased risk of CV morbidity and mortality are not entirely understood. More research is needed to better characterise the CV manifestations, elucidate the pathophysiological mechanisms and define the contribution of testosterone replacement to restoring CV health in KS patients. This review explores the complex association between KS, metabolic syndrome and CV risk in order to plan future studies and improve strategies to reduce mortality in this high-risk population.

Original publication

DOI

10.2174/1381612826666201102105408

Type

Journal article

Journal

Current pharmaceutical design

Publication Date

01/2020

Volume

26

Pages

5556 - 5563

Addresses

Department of Experimental Medicine, Policlinico Umberto I, Sapienza University of Rome, 00161, Rome, Italy.

Keywords

Humans, Klinefelter Syndrome, Diabetes Mellitus, Type 2, Hypogonadism, Testosterone, Male, Metabolic Syndrome