Sitosterolemia is an autosomal recessive disorder characterized by increased plant sterol levels, xanthomas, and accelerated atherosclerosis. Although it was originally reported in patients with normolipemic xanthomas, severe hypercholesterolemia have been reported in patients with sitosterolemia, especially in children. Sitosterolemia is caused by increased intestinal absorption and decreased biliary excretion of sterols resulting from biallelic mutations in either ABCG5 or ABCG8, which encode the sterol efflux transporter ABCG5 and ABCG8. Patients with sitosterolemia show extreme phenotypic heterogeneity, ranging from almost asymptomatic individuals to those with severe hypercholesterolemia leading to accelerated atherosclerosis and premature cardiac death. Hematologic manifestations include hemolytic anemia with stomatocytosis, macrothrombocytopenia, splenomegaly, and abnormal bleeding. The mainstay of therapy includes dietary restriction of both cholesterol and plant sterols and the sterol absorption inhibitor, ezetimibe. Foods rich in plant sterols include vegetable oils, wheat germs, nuts, seeds, avocado, shortening, margarine and chocolate. Hypercholesterolemia in patients with sitosterolemia is dramatically responsive to low cholesterol diet and bile acid sequestrants. Plant sterol assay should be performed in patients with normocholesterolemic xanthomas, hypercholesterolemia with unexpectedly good response to dietary modifications or to cholesterol absorption inhibitors, or hypercholesterolemia with poor response to statins, or those with unexplained hemolytic anemia and macrothrombocytopenia. Because prognosis can be improved by proper management, it is important to find these patients out and diagnose correctly. This review article aimed to summarize recent publications on sitosterolemia, and to suggest clinical indications for plant sterol assay.
Absorption ; Anemia, Hemolytic ; Atherosclerosis ; Bile ; Cacao ; Child ; Cholesterol ; Death ; Diagnosis* ; Diet ; Food Habits ; Hemorrhage ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; Hypercholesterolemia ; Intestinal Absorption ; Margarine ; Nuts ; Persea ; Phytosterols ; Plant Oils ; Plants ; Population Characteristics ; Prognosis ; Splenomegaly ; Sterols ; Triticum ; Xanthomatosis

The purpose of this study was to compare the eating habit, food intake frequency and preferred food and taste of juvenile delinquents (100 numbers) and male high school students (100 numbers), using questionnaires from August to October 2004. 1) It was shown that the juvenile delinquents took more unbalanced diet (p <0.05), smoking (p <0.001) and drinking (p <0.001) than general students, while general students had more eating between meals (p <0.01). 2) The intake of food was a significant difference in white rices (p <0.01), bibimbab (p <0.001), kimbab (p <0.001), fried rice (p <0.001), ramen (p <0.001), jajangmyun (p <0.001), jambong (p <0.001), hamburger (p <0.001), pizza (p <0.001) and breads (p <0.05). The juvenile delinquents took more major food than that of general students in all the items except for white rice. It was observed, then, that the juvenile delinquents showed more intake of such subsidiary foods as bulgoki (p <0.001), roasted thin pork chops (p <0.001), grilled pork belly (p <0.001), pork cutlet (p <0.01), sweet and sour pork (p <0.001), chicken with ginseng (p <0.001), fried chicken (p <0.001), kimchi pot stew (p <0.01) and zucchini (p <0.05) than general students, with general students taking more kimchi (p <0.01) and fried or boiled soybean curd (p <0.01). The intake of dessert was a significant difference in yogurt (p <0.01), orange juice (p <0.001), cola (p <0.001), soft drinks (p <0.001), ion drinks (p <0.001), coffee (p <0.001), apple (p <0.01), banana (p <0.001), orange (p <0.001), cake (p <0.001), hot dog (p <0.001), ddokbokgi (p <0.01), mandu (p <0.001), fried fish paste (p <0.05), steamed korean sausage (p <0.001), fried foods (p <0.05), hem & sausage (p <0.001), cheese (p <0.001), ice-cream (p <0.001), candy (p <0.01), chocolate (p <0.001), mayonnaise (p <0.05), jam (p <0.01) and butter & margarine (p <0.01). Then, the juvenile delinquents were shown to entirely have more intake of all the desserts than those of general students. 3) Regarding preference of foods, it was shown that the juvenile delinquents preferred cereals (p <0.05), grilled pork belly (p <0.001), chicken with ginseng (p <0.01), anchovies and small fish (p <0.05), chicken with ginseng (p <0.05), seasoned spinach (p <0.001), seasoned zucchini (p <0.001), milk (p <0.05), coffee (p <0.001) and butter & margarine (p <0.05) while general students did egg (p <0.01), soybean sprout soup (p <0.001), boiled or fried potato (p <0.05), seasoned soybean sprout (p <0.05), boiled or fried soybean curd (p <0.01), fried foods (p <0.01) and snack (p <0.05). For their preference of tastes, hot taste (p <0.01) was higher in the juvenile delinquents. 4) Under general environments there was a change of the difference in their behaviour of pork cutlet (p <0.01) intake in accordance with other family and of milk (p <0.001) and butter & margarine (p <0.05) preference in accordance with growth in a big city between the two groups.
Animals ; Bread ; Butter ; Cacao ; Candy ; Carbonated Beverages ; Edible Grain ; Cheese ; Chickens ; Citrus sinensis ; Coffee ; Cola ; Diet ; Dogs ; Drinking ; Eating* ; Food Habits* ; Food Preferences ; Humans ; Male* ; Margarine ; Meals ; Milk ; Musa ; Ovum ; Panax ; Seasons ; Smoke ; Smoking ; Snacks ; Solanum tuberosum ; Soybeans ; Spinacia oleracea ; Steam ; Surveys and Questionnaires ; Yogurt