Table 4.

Treatment of Manifestations in Individuals with Sitosterolemia

ManifestationTreatmentConsiderations/Other
Elevated plant
sterol levels
  • Diet low in shellfish sterols & plant sterols (i.e., avoidance of vegetable oils, margarine, nuts, seeds, avocados, chocolate, & shellfish)
  • Treatment w/sterol absorption inhibitor ezetimibe (10 mg/day in adults)
  • Bile acid sequestrants such as cholestryramine (8-15 g/day) may be considered in those w/incomplete response to ezetimibe.
Partial ileal bypass surgery (i.e., shortening of the ileum) has been used to ↑ intestinal bile acid loss.Partial or complete ileal bypass surgery in persons w/sitosterolemia has resulted in ≥50% ↓ of plasma & cellular sterol & stanol levels but should be used only as a last resort now that ezetimibe is available.

From: Sitosterolemia

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