Dataset Information

Oral Fat Tolerance Test for Sitosterolemia and Familial Hypercholesterolemia: A Study Protocol.

ABSTRACT: AIM:Sitosterolemia is an extremely rare, autosomal recessive disease characterized by high plasma cholesterols and plant sterols because of increased absorption of dietary cholesterols and sterols from the intestine, and decreased excretion from biliary tract. Previous study indicated that sitosterolemic patients might be vulnerable to post-prandial hyperlipidemia, including high remnant-like lipoprotein particles (RLP) level. Here we evaluate whether a loading dietary fat increases a post-prandial RLP cholesterol level in sitosterolemic patients compared to heterozygous familial hypercholesterolemic patients (FH). METHODS:We recruit total of 20 patients: 5 patients with homozygous sitosterolemia, 5 patients with heterozygous sitosterolemia, and 10 patients with heterozygous FH as controls from May 2015 to March 2018 at Kanazawa University Hospital, Japan. All patients receive Oral Fat Tolerance Test (OFTT) cream (50 g/body surface area square meter, orally only once, and the cream includes 34% of fat, 74 mg of cholesterol, and rich in palmitic and oleic acids. The primary endpoint is the change of a RLP cholesterol level after OFTT cream loading between sitosterolemia and FH. We measure them at baseline, and 2, 4, and 6 hours after the oral fat loading. RESULTS:This is the first study to evaluate whether sitosterolemia patients have a higher post-prandial RLP cholesterol level compared to heterozygous FH patients. CONCLUSION:The result may become an additional evidence to restrict dietary cholesterols for sitosterolemia. This study is registered at University Hospital Medical Information Network (UMIN) Clinical Trials Registry (UMIN ID: UMIN000020330).

SUBMITTER: Nomura A

PROVIDER: S-EPMC6099073 | biostudies-literature | 2018 Aug

REPOSITORIES: biostudies-literature

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Oral Fat Tolerance Test for Sitosterolemia and Familial Hypercholesterolemia: A Study Protocol.

Nomura Akihiro A Tada Hayato H Nohara Atsushi A Kawashiri Masa-Aki MA Yamagishi Masakazu M

Journal of atherosclerosis and thrombosis 20180120 8

<h4>Aim</h4>Sitosterolemia is an extremely rare, autosomal recessive disease characterized by high plasma cholesterols and plant sterols because of increased absorption of dietary cholesterols and sterols from the intestine, and decreased excretion from biliary tract. Previous study indicated that sitosterolemic patients might be vulnerable to post-prandial hyperlipidemia, including high remnant-like lipoprotein particles (RLP) level. Here we evaluate whether a loading dietary fat increases a po ...[more]

PMID: 29353827

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Project description:Background Atherosclerotic cardiovascular diseases (ASCVD) and dyslipidemia are associated to a higher risk of cardiovascular events, mortality, use of healthcare resources and costs. In Spain, the evidence about the administration of lipid-lowering treatments in clinical practice, and their clinical effectiveness in patients with ASCVD and hypercholesterolemia and patients with FH is scarce. Therefore, a multidisciplinary working group of cardiologists, family physicians, internal medicine specialists and neurologists was gathered for the Reality study. The aim of this study is to describe the demographic and clinical characteristics, comorbidities, and concomitant medication of patients with ASCVD and hypercholesterolemia and of patients with familial hypercholesterolemia (FH). The use of healthcare resources and costs associated to the management of these diseases after their diagnosis were also considered. Methods This is an observational and retrospective study, based on the BIG-PAC® database, which includes the electronic medical registries (EMRs) of 1.8 million people from 7 Autonomous Communities in Spain (including public primary care centers and hospitals). The study includes patients who had a new or recurrent episode of ASCVD during the recruitment period (from 01/01/2017 to 31/12/2018). The index date will be defined as the date of the ASCVD event, and the follow-up period will be 24 months. According to their first diagnosis in the database, patients will be classified as ASCVD (5 groups: stable/unstable angina, acute myocardial infarction, ischemic stroke, transient ischemic attack, and peripheral arterial disease) or FH. Discussion This study aims to analyze the treatment patterns and use of healthcare resources of ASCVD and FH in Spain. The prevalence of these disorders will also be estimated. Due to the high morbidity and mortality associated with these diseases, it is expected that our study will provide useful information for healthcare systems and decision makers to improve the management of these disabling diseases.

Dataset Information

Oral Fat Tolerance Test for Sitosterolemia and Familial Hypercholesterolemia: A Study Protocol.

Publications

Oral Fat Tolerance Test for Sitosterolemia and Familial Hypercholesterolemia: A Study Protocol.

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