@article{oai:kanazawa-u.repo.nii.ac.jp:00046678,
 author = {野村, 章洋 and 多田, 隼人 and 野原, 淳 and 川尻, 剛照 and 山岸, 正和 and Nomura, Akihiro and Tada, Hayato and Nohara, Atsushi and Kawashiri, Masa-aki and Yamagishi, Masakazu},
 issue = {8},
 journal = {Journal of Atherosclerosis and Thrombosis},
 month = {},
 note = {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).
\nMethods: 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.
\nResults: This is the first study to evaluate whether sitosterolemia patients have a higher post-prandial RLP cholesterol level compared to heterozygous FH patients.
\nConclusion: 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)., This article distributed under the terms of the latest version of CC BY-NC-SA defined by the Creative Commons Attribution License., 金沢大学附属病院先端医療開発センター},
 pages = {741--746},
 title = {Oral Fat Tolerance Test for Sitosterolemia and Familial Hypercholesterolemia: A Study Protocol},
 volume = {25},
 year = {2018}
}