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J Clin Endocrinol Metab. 2012 Oct;97(10):3399-407. doi: 10.1210/jc.2012-2185.

Approach to the patient with extremely low HDL-cholesterol.

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  • 1Cardiovascular Institute, Perelman School of Medicine at the University of Pennsylvania, 11-125 Translational Research Center, 3400 Civic Center Boulevard, Philadelphia, Pennsylvania 19104, USA.


Patients with extremely low high-density lipoprotein-cholesterol (HDL-C) pose distinct challenges to clinical diagnosis and management. Confirmation of HDL-C levels below 20 mg/dl in the absence of severe hypertriglyceridemia should be followed by evaluation for secondary causes, such as androgen use, malignancy, and primary monogenic disorders, namely, apolipoprotein A-I mutations, Tangier disease, and lecithin-cholesterol acyltransferase deficiency. Global cardiovascular risk assessment is a critical component of comprehensive evaluation, although the association between extremely low HDL-C levels and atherosclerosis remains unclear. Therapeutic interventions address reversible causes of low HDL-C, multiorgan abnormalities that may accompany primary disorders and cardiovascular risk modification when appropriate. Uncommon encounters with patients exhibiting extremely low HDL-C provide an opportunity to directly observe the role of HDL metabolism in atherosclerosis and beyond the vascular system.

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