Phenylketonuria

Swati Kolpuru
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Phenylketonuria - Treatment
The only treatment for this disease is diet restricted in phenylalanine. Phenylalanine must be restricted starting in the first few weeks of life. Infants with PKU should be breast fed along with special formula which are phenylalanine free. A diet should be planned with adequate calories and amino acids to ensure adequate growth and development.

Certain vegetables, fruits, grains and other low phenylalanine foods should be added later. Regular milk, cheese, eggs, meat fish should never be allowed. Individuals with PKU must remain on a restricted diet throughout life. A restricted diet, started early and well maintained, makes normal development possible and prevents mental retardation.

Individuals with phenylketonuria should be adequately followed so that phenylalanine blood levels are monitored and the importance of dietary restriction emphasized.

Blood levels are monitored weekly during infancy. After that they can be done every 3 months. Plasma amino acid concentrations are determined once or twice a year to ensure adequate tyrosine levels.


Phenylketonuria Phenylketonuria 02/28/2001
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