Ten-year Follow-up of Familial Hypercholesterolemia Patients After Intensive Cholesterol-lowering Therapy

Masaki, Nobuyuki; Tatami, Ryozo; Kumamoto, Teruhiko; Izawa, Akira; Shimada, Yasuhiro; Takamatsu, T.; Katsushika, Shuichi; Ishise, Shozo; Maruyama, Yoshiyuki; Yoshimoto, Nobuo

doi:10.1536/ihj.46.833

Cited by 34 publications

(37 citation statements)

References 31 publications

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“…36) Once diagnosed, heterozygous FH patients can readily be treated with cholesterol-lowering medication to attenuate the development of atherosclerosis and prevent the development of CAD. [37][38][39][40][41] Study limitations: This study had several limitations. First, the possibility of underdiagnosing heterozygous FH in patients with ACS cannot be denied because we found a considerable number of patients with ATT of 9 mm or more without diagnosing heterozygous FH.…”

Section: Discussionmentioning

confidence: 99%

Estimated Prevalence of Heterozygous Familial Hypercholesterolemia in Patients With Acute Coronary Syndrome

et al. 2017

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SummaryHeterozygous familial hypercholesterolemia (FH) represents a strong risk for development of premature coronary artery disease (CAD). However, the majority of patients with FH are undiagnosed and the prevalence likely represents an underestimate in most countries. In Japan, the possible contribution of FH to the development of CAD may be higher because of the low incidence of CAD among the general population. We estimated the prevalence of heterozygous FH by measuring Achilles tendon thickness (ATT) in patients with acute coronary syndrome (ACS).A total of 359 patients suffering from ACS were enrolled in this multicenter registration study. Heterozygous FH was defined according to the diagnostic criteria proposed by the Japan Atherosclerosis Society. After excluding 63 patients because of missing ATT data or plasma triglyceride levels that were 4.5 mmol/L or more, 296 patients were eligible for inclusion in the study. The number of patients with ATT of 9 mm or more was 53 (17.9%). They were significantly younger and had significantly higher LDL cholesterol levels than patients with an ATT less than 9 mm. The prevalence of heterozygous FH was 5.7% (1/17.5) and more prominent in younger patients who were less than 60 years old (7.8%). In patients with ATT of 9 mm or more, approximately 1 in 3.5 fulfilled the criteria for heterozygous FH.We demonstrated the usefulness of measuring ATT by radiography and the high prevalence of heterozygous FH in patients with ACS in Japan, especially in younger patients who were less than 60 years old. (Int Heart J 2017; 58: 88-94)

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Section: Discussionmentioning

confidence: 99%

Estimated Prevalence of Heterozygous Familial Hypercholesterolemia in Patients With Acute Coronary Syndrome

et al. 2017

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“…Its activity is extremely potent and markedly higher than (about 10 folds) that of probucol: one of the most potent antioxidants used in antioxidant therapy [5]. Transfection of Hp cDNA into Chinese hamster ovary (CHO) cells protects the cells against oxidative stress [4].…”

Section: Introductionmentioning

confidence: 99%

Immunochemical property of human haptoglobin phenotypes: Determination of plasma haptoglobin using type-matched standards

Cheng

Pan

Lai

et al. 2007

Clinical Biochemistry

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“…78 In a prospective, 10-year follow-up study of 18 patients receiving the combination of apheresis and statin therapy, a delay was observed in the progression of coronary artery disease, with evidence for the prevention of major cardiac events. 79 Little change was observed in coronary stenosis and ejection fraction. However, the use of apheresis is limited by high cost, difficult access, and the inconvenience of treatment (> 3 hours required for treatment every 1-2 weeks).…”

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confidence: 99%

Management of Familial Hypercholesterolemia: A Review of the Recommendations from the National Lipid Association Expert Panel on Familial Hypercholesterolemia

Robinson

2013

JMCP

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suMMaRyFamilial hypercholesterolemia (FH) is a genetic disorder of lipid metabolism that is characterized by a significant elevation in levels of low-density lipoprotein cholesterol (LDL-C), and patients are at very high risk for premature coronary heart disease (CHD). The etiology of FH includes known mutations in the gene of the LDL receptor, LDLR ; the gene of apolipoprotein B, apo B ; and the proprotein convertase subtilisin/kexin type 9 gene, PCSK9. The National Lipid Association Expert Panel on Familial Hypercholesterolemia has provided recommendations for the screening and treatment of patients with FH. Early identification and aggressive treatment of FH in individual patients, as well as screening of all first-degree relatives, are recommended to minimize the risk for premature CHD. Similar to patients with conventional hypercholesterolemia, patients with FH should receive statins as initial treatment, but patients with FH may require higher doses of statins, more potent statins, statin-based combination therapy, or adjunctive therapies. Patients with FH who have additional risk factors for, or existing, cardiovascular disease or those with an inadequate response to initial statin therapy should have access to higher doses of the most efficacious statins; statins used in combination with other LDL-C-lowering agents should also be supported by formularies; additional treatments, such as LDL-C apheresis or novel therapies, may also be required to achieve acceptable LDL-C levels. New treatment approaches include mipomersen, which was approved by the FDA in January 2013. Mipomersen is an oligonucleotide inhibitor of apolipoprotein B-100 synthesis (called an antisense inhibitor) indicated as an adjunct to lipid-lowering medications and diet to reduce LDL-C, apolipoprotein B, total cholesterol, and non-high density lipoproteincholesterol (non-HDL-C) levels in patients with homozygous FH (HoFH). The microsomal transfer protein lomitapide has also received FDA approval for use only in patients with HoFH. Other novel treatments currently in development include PCSK9 inhibitors. Therapies such as apheresis are likely more expensive than simple therapy with a statin but may be needed to achieve long-term reductions in complications from nonfatal and fatal cardiovascular events and hospitalizations related to myocardial infarction, cardiac revascularization, and stroke in FH patients. The cost-effectiveness of this more aggressive therapy has not been determined and should be studied. Utilization of published guidelines and the recommendations from the National Lipid Association will help to optimize the management of patients with FH.

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Ten-year Follow-up of Familial Hypercholesterolemia Patients After Intensive Cholesterol-lowering Therapy

Cited by 34 publications

References 31 publications

Estimated Prevalence of Heterozygous Familial Hypercholesterolemia in Patients With Acute Coronary Syndrome

Estimated Prevalence of Heterozygous Familial Hypercholesterolemia in Patients With Acute Coronary Syndrome

Immunochemical property of human haptoglobin phenotypes: Determination of plasma haptoglobin using type-matched standards

Management of Familial Hypercholesterolemia: A Review of the Recommendations from the National Lipid Association Expert Panel on Familial Hypercholesterolemia

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