sitosterolemia


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sitosterolemia

 [si-tos″ter-ol-e´me-ah]
the presence of excessive levels of sitosterols in the blood, especially β-sitosterol, absorbed from dietary vegetables due to an unknown intestinal defect. A rare form is associated with xanthomatosis, with tuberous and tendon xanthomas appearing in childhood.

phy·to·ste·ro·lem·i·a

(fī'tō-stē'ro-lēm'ē-ă),
An inherited disorder in which hyperabsorption of phytosterols and shellfish sterols results in tendon and tuberous xanthomata.
Synonym(s): sitosterolemia

sitosterolemia

/si·tos·ter·ol·emia/ (si-tos″ter-ol-e´me-ah) the presence of excessive sitosterols in the blood, especially β-sitosterol, from dietary vegetables. Written also β-sitosterolemia.

sitosterolemia

(sī-tŏs″tĕr-ōl-ē′mē-ă)
A rare, autosomal recessive disorder in which sitosterols are excessively absorbed from the gastrointestinal tract and stored in the body. The disease produces atherosclerotic vascular disease (esp. premature coronary artery disease and premature death), hemolytic anemia, and arthritis.
References in periodicals archive ?
10-12) Sitosterolemia causes severe, early atherosclerosis, typically with tendon xanthomas.
Also, studies of two rare genetic lipid disorders, Tangier disease and sitosterolemia, have revolutionized our understanding of sterol transport (17,18).
Accumulation of dietary cholesterol in sitosterolemia caused by mutations in adjacent ABC transporters.
We show the clinical usefulness of the technique by successfully applying it to 4 inherited disorders in lipid metabolism: SLOS, cerebrotendinous xanthomatosis (CTX), sitosterolemia, and Refsum disease.
Ezetimibe is indicated as monotherapy for the treatment of primary hypercholesterolemia and homozygous sitosterolemia.
Ezetimibe indications: Hypercholesterolemia; Sitosterolemia
Announced June 7) - Announced that ZETIA (ezetimibe), a novel cholesterol-lowering agent that inhibits the absorption of cholesterol in the intestine, was available in Japan for use in patients with hypercholesterolemia, familial hypercholesterolemia or homozygous sitosterolemia.
Treatment experience with ezetimibe in the pediatric population is limited to 4 patients (9 to 17 years) with homozygous sitosterolemia and 5 patients (11 to 17 years) with HoFH.
June 8 /PRNewswire-FirstCall/ -- Schering-Plough Corporation and Bayer HealthCare today announced that ZETIA(R) (ezetimibe), a novel cholesterol-lowering agent that inhibits the absorption of cholesterol in the intestine, is now available in Japan for use in patients with hypercholesterolemia, familial hypercholesterolemia or homozygous sitosterolemia.
Experience in pediatric and adolescent patients (ages 9 to 17) has been limited to patients with homozygous familial hypercholesterolemia (HoFH) or sitosterolemia.
ZETIA is approved in Japan as a monotherapy and co- administered with a statin for use in patients with hypercholesterolemia, familial hypercholesterolemia or homozygous sitosterolemia.