Alagille Syndrome Medication

Updated: Oct 01, 2021
  • Author: Ann Scheimann, MD, MBA; Chief Editor: Carmen Cuffari, MD  more...
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Medication

Medication Summary

Medications are used to manage bile acid-induced pruritus and supplement fat-soluble vitamin stores.

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Antipruritics

Class Summary

Pruritus is often recalcitrant to medical therapy and significantly impacts on the quality of life.

Hydroxyzine (Atarax, Vistaril)

Useful adjunct in the management of pruritus with histamine-mediated triggers. Antagonizes H1-receptors in periphery. May suppress histamine activity in subcortical region of CNS.

Cholestyramine (Questran)

Forms a nonabsorbable complex with bile acids in the intestine, which, in turn, inhibits enterohepatic reuptake of intestinal bile salts. Take other medications at least 1 h before or 4-6 h after cholestyramine.

Not to be administered in dry powder form. Mix with plenty of water or applesauce.

Rifampin (Rifadin, Rimactane)

Precise mechanism of action is unclear. May involve inhibition of bile acid uptake into hepatocytes and facilitation of excretion of dihydroxy and monohydroxy bile acids and toxic bile acids.

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Ileal Bile Acid Transport Inhibitors

Class Summary

Inhibitors of ileal bile acid transport (IBAT) act locally in the distal ileum to decrease reuptake of bile acids and increase clearance of bile acids through the colon, thereby, reducing bile acid serum concentration.

Maralixibat (Livmarli)

Indicated for cholestatic pruritus in patients with Alagille syndrome aged 1 year and older.

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Fat-soluble vitamins

Class Summary

These vitamins are used for supplementation of vitamin A, D, E, and K losses.

Phytonadione (AquaMEPHYTON)

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

Vitamin E (Nutr-E-Sol)

Antioxidant that prevents the oxidation of vitamins A and C. Protects polyunsaturated fatty acids in membranes from attack by free radicals and protects RBCs against hemolysis. Nutr-E-Sol is a specially formulated vitamin E complex with polyethylene glycol 1000 succinate to allow direct absorption without biliary emulsification. Formulation of choice for vitamin E replacement therapy in patients with cholestasis. The formulation contains 400 IU vitamin E/15 mL.

Ergocalciferol (Calciferol, Drisdol)

Also referred to as vitamin D-2. Undergoes metabolic activation in vivo to the biologically active form 1,25-dihydroxyergocalciferol (1,25[OH]2 -D2). Stimulates absorption of calcium and phosphate from the intestines and promotes release of calcium from bone into blood. Ergocalciferol 1 mg provides 40,000 IU of vitamin D activity. Available as liquid drops (8000 IU/mL) and 50,000 IU capsules.

Vitamin A (Palmitate-A 5000, Aquasol-A)

This vitamin is required for bone development, growth, night vision, and gonadal function. It is a biochemical cofactor. In the past, vitamin A has been expressed in units. It is now expressed as retinol equivalents (RE) or mcg of retinol; 1 RE = 1 mcg retinol, and 1 RE of vitamin A = 3.33 units of retinol and 10 u of beta-carotene.

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Trace Element

Class Summary

Zinc deficiency is sometimes seen; zinc is easily replaced via oral compounds.

Zinc (Galzin, Orazinc, Verazinc, Zincate)

Zinc is an essential cofactor for more than 70 enzymes that are important in immune function and cell replication. Dosing guidelines are based on monitoring of levels. The elemental zinc content depends on the particular salt form. Zinc acetate liquid has 5 mg of elemental zinc per mL. Zinc sulfate suspension has 10 mg elemental zinc per mL and zinc sulfate tablets contain 23% elemental zinc.

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Bile Acid

Class Summary

This agent promotes bile salt excretion via direct stimulation of bile flow and via indirect alterations in composition of bile.

Ursodiol (Actigall, Urso Forte)

Decreases cholesterol content of bile.

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