Acanthocytosis Medication

Updated: Mar 24, 2021
  • Author: Pedro A de Alarcon, MD; Chief Editor: Hassan M Yaish, MD  more...
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Medication

Medication Summary

Treatment of abetalipoproteinemia includes dietary restriction of triglycerides, supplementation with medium-chain triglycerides, and supplementation of lipid-soluble vitamins A, D, E, and K in high doses. Occasionally, patients have associated iron or folic acid deficiencies, necessitating supplementation with oral iron and folic acid.

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Vitamins and Cofactors

Class Summary

These agents are organic substances required by the body in small amounts for various metabolic processes. They are classified as fat-soluble or water-soluble. Vitamins A, D, E, and K are fat-soluble; biotin, folic acid, niacin, pantothenic acid, the B vitamins (ie, B-1, B-2, B-6, B-12), and vitamin C are generally water-soluble. These agents are clinically used for the prevention and treatment of specific vitamin-deficiency states.

Vitamin A (Aquasol A, Palmitate-A 5000)

Required for bone development, growth, night vision, and gonadal function and is a biochemical cofactor.

Ergocalciferol (Drisdol)

Vitamin D stimulates the absorption of calcium and phosphate from the intestines and decreases bone resorption.

Vitamin E (Nutr-E-Sol)

Vitamin E protects polyunsaturated fatty acids in membranes from free radical injury and stabilizes RBC membranes. Nutr-E-Sol is a specially formulated vitamin E complexed with polyethylene glycol 1000 succinate to allow direct absorption without biliary emulsification. This is the formulation of choice for vitamin E–replacement therapy in patients with cholestasis. The formulation contains 400 IU vitamin E/15 mL.

Phytonadione (Mephyton)

Vitamin K-1 is necessary for the production of clotting factors II, VII, IX, and X by serving as a cofactor during carboxylation of glutamic acid residues.

Folic acid

Important cofactor for enzymes used in production of RBCs.

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Trace Elements

Class Summary

These agents are inorganic substances found in small amounts in the tissues and are required for various metabolic processes.

Polysaccharide-iron complex (Niferex, Nu-Iron)

A nutritionally essential inorganic substance. Polysaccharide-iron complex is a product that contains ferric iron. 150 mg equals 150 mg of elemental iron. Also available as elixir containing 100 mg elemental iron per 5 mL.

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