Pyridoxine in Homocystinuria

Vitamin B-6 and Sulfur Amino Acid Metabolism

“…Dietary treatment with large doses of vitamin B-6, in the form of pyridoxine hydrochloride , ameliorates or eliminates the biochemical abnormalities of some patients with homocystinuria due to cystathionine B-synthase deficiency (47)(48)(49)(50)(51). Such treatment, which may require 25 to 1500 mg of pyridoxine hydrochloride daily, when successful will eliminate homocystine from plasma and urine, reduce the concentrations of methionine in plasma and urine, and result in the appearance of normal cystine concentrations in plasma and urine within a few days (Fig .…”