PKU is a genetic disorder that affects about 1 in 10,000 babies born in the US and Europe. It occurs when the liver cannot break down an amino acid called phenylalanine. If left untreated, a buildup of phenylalanine can cause intellectual disabilities, seizures, and other issues. However, with early treatment and lifelong adherence to a strict low-protein diet supplemented by medical formulas, prognosis is good for managing the disorder. The article discusses PKU in more detail and the importance of treatment and dietary management for positive outcomes.

1. Team Member Database/ Journal Article
Citation
Summary Statement
Myra (Ashley) McVay Gale Health & Wellness
Resource Center
Phenylketonuria: outcomes
and treatment
Pediatric Health
v2 i2; p225(10)
Fickie, M.R.,
Gentile, J.,
Waisbren, S.
2008
PKU is a genetic disorder
affecting nearly 1 in every
10,000 babies born in the US
and Europe. It occurs when
the liver can not metabolize
phenylalanine. Unless
treated early, a buildup of
phenylalanine in the body
can be toxic, resulting in
severe mental retardation,
seizures, and eczema,
among other things.
Prognosis is relatively good
with this disorder providing
that the patient follows a
life-long strict dietary
regiment.

2. Nutrient Total Rec. %Rec
Calories 584.97 2200 26.59%
Pro (g) 13.01 48 27.10%
Fat (g) 5.89 73.33 8.03%
Carb (g) 127.56 --
Fiber (g) 8.38 30 27.93%
Cal (mg) 90.38 1000 9.04%
Iron (mg) 7.54 10 75.4%
Na (mg) 1100.91 2400 45.87%
Pot (mg) 2032.62 --
Phos (mg) 322.24 700 46.03%
Ash (g) 7.82 330 2.37%
vitA (IU) 2477.13 4000 61.93%
vitC (mg) 142.21 75 189.61%
Thia (mg) 0.69 1.1 62.73%
Ribo (mg) 0.87 1.1 79.09%
Nia (mg) 12.33 14 88.07%
H2O % 58.74 Female 19-30 --
satF (g) 3.59 24.44 14.69%
monoF (g) 0.39 24.44 1.6%
polyF (g) 0.93 24.44 3.81%
Chol (mg) 0 300 0%
Food Item
# of
Servings
Serving
Size
Total
Grams
BROCCOLI-CKD-BOILED-DRAINED
-W/SALT
0.5
1 CUP
CHOPPED
78 grams
MUSHROOMS-CKD-BOILED
-DRAINED-W/SALT
0.5
1 CUP
PIECES
78 grams
POTATOES-BAKED-FLESH AND SKIN
-WO/SALT
1
1 POTATO2-
1/3X4-3/4IN
202
grams
BANANAS-RAW 1
1
FRUITWO/S
KN&SEEDS
114
grams
SWEETS-CANDIES-JELLYBEANS 10 1 SM 11 grams
SOUP-TOMATO-CND-PREP W/EQ VOL
WATER
-COMMERCIAL
0.5
1 CUP (8 FL
OZ)
122
grams
WATER-MUNICIPAL 1 1 c
682.67
grams
CREAM SUBSTITUTE-NONDAIRY
-POWDERED
0.33 1 oz
9.3555
grams
CEREALS READY-TO-EAT
-CORN FLAKES-KELLOGG'S
-(CORN)
0.75 1 OZ BOX
21.3
grams

3. Explanation of Diet
• The previously shown meal plan hinges on supplementation
of a PKU Formula taken 3 times a day. The formula used in my
calculations is Phenex-2.
• Based on the low-protein diet and supplementation of the
formula, I have compiled a revised summary of some basic
nutrients.
• Total Calories: 1814.97 Protein (g): 103.01
• Fat (g): 47.89 Sodium (mg): 3740
• A complete list of nutrition facts for Phenex-2 can be found at
http://abbottnutrition.com/brands/products/phenex-2
• The basis for my diet plan came from
http://www.mayoclinic.com/health/phenylketonuria/DS0051
4/DSECTION=lifestyle-and-home-remedies