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KMID : 0386819920020020250
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Journal of the Institute of Health Environmental Sciences
1992 Volume.2 No. 2 p.250 ~ p.261
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A Study on the Diet Therapy in the Treatment of Inborn Errors of Metabolism
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Abstract
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Recent advances in treatment of inherited metabolic disorders include include diet therapy, organ transplantation, enzyme supplement and gene therapy. Diet therapy. Diet therpy is a helpful treatment and alternative or complementary to drugs,
especially
in amino acid abnormality and galactosaemia. With early diagnosis and early treatment with therapeutic diets, the symtoms and signs of the these disease may be prevented or reversed.
Phenylketonuria (PKU) is one of the better known examples of an inborn error of amino acid metabolism. Diet therapy for PKU consists of a combination of low protein foods and a phenylalanine free mixture of other essential amino acid. And the
amounts of
these two are adjusted to maintain plasma phenylalanine neither too high (toxicity) nor too low (inadequate growth).
Galactosaemia may be arrested by eliminating mild and its product from diet. Recent evidence, however, has indicated that despite early diagnosis and the institution of a galactose free diet there appears to be an inability to prevent some degree
of
mental retardation and complicate long term outcome.
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KEYWORD
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