Dermatologic Manifestations of Homocystinuria Medication

Updated: Jun 21, 2018
  • Author: Janette Baloghova, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Medication

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

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Dietary supplements

Class Summary

These agents are used to correct nutritional deficiencies. In addition to the supplements listed below, cysteine is a sulfur-containing amino acid. It is generally considered an essential amino acid in infants.

Betaine anhydrous (Cystadane)

Betaine anhydrous is an antihomocystinuric that acts as a methyl-group donor in the remethylation of homocysteine to methionine, removing excess homocysteine from the body.

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Vitamins

Class Summary

Vitamins are essential for normal DNA synthesis.

Pyridoxine (Pyri 500, Neuro-K)

Pyridoxine is a precursor to pyridoxal, which is important in the metabolism of proteins, carbohydrates, and fats. It also aids in the release of liver- and muscle-stored glycogen. It is involved in the synthesis of GABA in the CNS.

Cyanocobalamin (Pysicians EZ use B-12, B-12 Compliance injection)

Cyanocobalamin deoxyadenosylcobalamin and hydroxocobalamin are active forms of vitamin B-12 in humans. Vitamin B-12 is synthesized by microbes but not by humans or plants. It is a coenzyme in various metabolic functions, including carbohydrate and fat metabolism and protein synthesis, which are responsible for cell replication and hematopoiesis.

Folic acid (FA-8)

Folic acid is an important cofactor for enzymes used in nucleoprotein synthesis and maintenance of erythropoiesis. It stimulates white blood cells and platelet production in folate deficiency anemia.

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