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 ?
It will also not discriminate between FH and non-FH such as secondary hypercholesterolemia, sitosterolemia, or other variants.
10-12) Sitosterolemia causes severe, early atherosclerosis, typically with tendon xanthomas.
El diagnostico diferencial incluye hipercolesterolemia familiar homocigota, sitosterolemia (acumulacion de esteroles de plantas), xantomatosis cerebrotendinosa y sindrome de Alagille (hipoplasia biliar) (13).
Diagnosticos diferenciales de los xantomas eruptivos Enfermedad Sintomas Sitosterolemia Xantomas tendinosos y xantelasmas Artritis Sitosterol aumentado en sangre Xantomatosis Xantomas tendinosos cerebrotendinosa Cataratas juveniles Diarrea cronica Trastornos neurologicos: capacidad intelectual reducido, alteraciones siquiatricas, enfermedad de Parkinson y epilepsia.
Hasta el momento la unica contraindicacion en el consumo de estos compuestos es la existencia de un defecto metabolico poco frecuente como lo es la sitosterolemia, que se caracteriza por una hiperabsorcion intestinal de todos los esteroles consumidos en la alimentacion habitual, y que es asociada a la aterosclerosis prematura (51).
Also, studies of two rare genetic lipid disorders, Tangier disease and sitosterolemia, have revolutionized our understanding of sterol transport (17,18).
Ezetimibe indications: Hypercholesterolemia; Sitosterolemia
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.
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.
Experience in pediatric and adolescent patients (ages 9 to 17) has been limited to patients with homozygous familial hypercholesterolemia (HoFH) or sitosterolemia.
Homozygous Sitosterolemia (Phytosterolemia) A study was conducted to assess the efficacy of ZETIA in the treatment of homozygous sitosterolemia.