Table 2 Treatment of Hyperhomocysteinemia

From: Impaired Homocysteine Metabolism and Atherothrombotic Disease

Drug

Use

Folates and cobalamin

Folic acid

In all patients with basal and postmethionine-load hyperhomocysteinemia. Nutritional folate deficiency, thermolabile MTHFR mutation, homozygotes for the CBS deficiency who do not respond to pyridoxine, heterozygotes for the CBS deficiency. 400 μg to 1 mg per day in acquired deficiency, 5 mg per day in congenital deficiency.

Folinic acid or N5-methyltetrahydrofolate

Instead of folic acid in patients with altered folate metabolism or thermostable MTHFR mutations.

Cyanocobalamin

Correction of acquired vitamin B12 deficiency before folate therapy. 400 μg to 2 mg per day according to the degree of deficiency.

Pyridoxine

In patients with postmethionine-load hyperhomocysteinemia who do not respond to folate treatment.

 

Nutritional deficiency of vitamin B6, dietary excess of methionine-rich animal proteins, 3 to 15 mg per day.

 

Homozygosity and heterozygosity for CBS deficiency, 30 to 100 mg per day.

Betaine and/or methionine

Betaine

Functional methionine synthase deficiency, thermostable MTHFR mutations.

Methionine

Functional methionine synthase deficiency, thermostable MTHFR mutations.