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LIFE SAVING DRUGS IN IEM

Vitamins: B1(Thiamin), B6 (Pyridoxine), Pyridoxal phosphate, Hydroxy B12 (Cobalamin), Folinic acid, Biotin, Carnitine

Cleansing drugs: Copper chelators, Ammonia chelators, sulfur chelators , Acyl CoA chelators

Replacing compounds: L arginine, citrulline, serine, enzymes (mucerase., agalsidase), neurotransmitters (DOPA, BH4, 5 HTP) mannose, Magnesium, Glucose

Inhibitory/Stimulating drugs: Chenodoexycholic acid, NTBC, diazoxide, Carbamyl glutamate, Heam arginate, Dichloroacetate

Special diets: Fructose & Galactose free, Phenylalanine free, Urea Cycle Defects diet, Methionine free diet for Cystathionine synthase deficiency, Branched Chain Aminoacids free, Low protein diets.

 


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