Q. Therapeutic dose of pyridoxine?
Therapeutic dose of pyridoxine (lg/day) is usually tried in all patients with hypermethioninernia and homocystinemia. For patients who do not respond to pyridoxine, methionine restricted diet supplemented with L-cysteine is used. If plasma folate concentrations are below normal, folate should be given as a supplement. What are the special preparations and foods low in or free of methionine? This list can be referred to while planning diet plans for patients suffering from homocystinuria. While planning the diet for homocystinuria one must consider energy, protein, methionine, cysteine, folate, vitamins B, and B and fluid needs.
The discussion so far focused on the disorders linked to protein mettabolism. Disorders of carbohydrate metabolism also exist. One such disorder is galactosemia which is also an inherited disorder characterized by an ability of the body to utilize galactose.