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Background: Familial hypercholesterolemia (FH) significantly increases the risk of atherosclerotic cardiovascular disease (ASCVD); however, recent data from ambulatory care centers suggests that prescription rates for statins remain low in patients with severe dyslipidemia or diagnosed FH. National rates of screening, awareness, and treatment with statins among individuals with FH or severe dyslipidemia are unknown.Methods: Data from the 1999 to 2014 National Health and Nutrition Examination Survey (NHANES) were used to estimate prevalence rates of self-reported screening, awareness, and statin therapy among U.S. adults ≥20 years of age (n=42,471 weighted to represent 212 million U.S. adults) with FH (defined using the Dutch Lipid Clinic criteria) and with severe dyslipidemia (defined as low-density lipoprotein cholesterol (LDL-C) levels ≥190mg/dL). Logistic regression was used to identify sociodemographic and clinical correlates of hypercholesterolemia awareness and statin therapy. Results were extrapolated to the U.S. adult population.Results: The US prevalence of definite/probable FH was 0.47% (standard error 0.03%) and of severe dyslipidemia was 6.59% (SE 0.17%). Rates of cholesterol screening and awareness were high (>80%) among adults with definite/probable FH or severe dyslipidemia; however, statin use was uniformly low (52.3% (SE 8.2%) of adults with definite/probable FH and 37.6% (SE 1.2%) of adults with severe dyslipidemia). Less than half of those on statins were prescribed a high-intensity statin. The prevalence of statin use in adults with definite/probable FH and severe dyslipidemia increased slightly over time but not faster than trends in the general population. Older age, insurance, having a usual source of care, diabetes, hypertension, and having a personal history of early ASCVD were associated with statin use. The discrepancy between cholesterol screening and treatment rates was most pronounced in younger patients, uninsured patients, and patients without a usual source of care.Conclusions: Despite high rates of cholesterol screening and awareness, only about half of U.S adults with FH are on statin therapy and even fewer are prescribed a high-intensity statin; young and uninsured patients are at the highest risk for under treatment. A low rate of statin use in young adults is of particular relevance given the early onset of ASCVD in adults with FH. This study highlights an opportunity and an imperative to improve statin treatment rates in this high-risk population. Additional studies are needed to better understand how to close the gap between screening and treatment among adults with FH and improve treatment rates among those with limited access to care.