Nutritional Therapy

Question 1

Nutritional overview of PKU

Answer 1

Give minimal Phe (intact protein) required for anabolism

Give adequate protein + calories for growth

Metabolic formula (phe removed, can add in tyrosine with vitamins and minerals that a pateints needs

**PKU patients need MORE protein than normal population when newly born?

Question 2

Enfamil formula

Answer 2

Phe + protein + Kcal + Osm

Question 3

Periflex

Answer 3

Protein + fat + carbs

Question 4

Duocal

Answer 4

Fat and carbs

Question 5

Diet for PKU

Answer 5

Fruits and veggies should be the staple

Pure sugar adn pure fat food can be eaten

Most cannot have regular starches

• if they eat one high PKU meal they will most likely be fine a few days later
• *but IVA patient will probably go acidemic in a few hours

Question 6

Monitoring PKU

Answer 6

Blood lab tests

Routine clinic visits

• monitor growth
• additional lab tests
• counseling and teaching

Dietary prescroption individualized
-phe tolerance ranges
New therapies

Question 7

Kuvan (BH4 supplementation)

Answer 7

Alternative treatment

Makes PAH enzyme work better (BH4 = cofactor)