Phenylketonuria (PKU)

Theresa Koneski NUFD 255

Medical Condition Presentation

Background - Theory

• A rare disorder which is inherited only if both parents carry the gene

• Newborn babies are screened to prevent serious health problems

• PKU disorder hinders the enzyme needed to breakdown phenylalanine

• Phenylalanine = amino acid found in foods high in protein

• If phenylalanine builds up in the body it causes intellectual disability, emotional, behavioral, psychological, social, and neurological problems

• Delays and/or hinders normal development

Background – Nutrition Concerns

• Lack of grains, beans, nuts, and animal products causes deficiencies in numerous micronutrients

• Zinc, selenium, iron, Vitamin B12, and folate are generally lower

• Calcium, Vitamin A, C, D, and E supplements are required• Recommended to focus on calcium, phosphorus, & Vitamin D

for optimal bone health • Manganese is frequently recorded as being too high or too low

Background - Treatment• Dietary:

• Limit foods high in protein• Consume higher carbohydrate diet • Avoid food & beverages containing aspartame, which releases phenylalanine in the body• Consult with dietitian for a meal plan• Consume PKU formula as a protein supplement

• Medical:• Can try neutral amino acid therapy (tablet or powder form)

• May block some phenylalanine absorption in the body and prevent brain damage• Be cautious about medications and other oral supplements, some contain phenylalanine • Regular blood tests to monitor PKU levels, review diet records, chart growth, & assess

development

Individual with PKU• Anthropometry:

• Height: 167.64 cm Weight: 60 kg Age: 23 Gender: female

• Activity factor: moderate exercise 3-5 days/week: 1.55• 4 meals per day • BEE: 655 + (9.6 x 60 kg) + (1.8 x 167.6 cm) – (4.7 x 23 years)

BEE = 1,424.60 kcal• TEE = 1,424.60 x 1.55

TEE = 2,208.13 kcal• Calorie needs: 2,208.13/4 = 552.03 kcal per meal

Recipes:Sweet Veggie Pizza servings: 4 1 lb low-protein pizza dough (see recipe below) 2 cups tomato sauce 18-oz artichoke hearts 0.8 cup black olives 1 cup shiitake mushrooms 34 mL olive oil 2 medium sweet onions 1 cup spinach 4 small apples 4-oz mozzarella cheese Directions:

1. Preheat oven to 450℉ . 2. Caramelized onions: In a large skillet heat 2 tbsp. olive oil over medium-high heat until

very hot but not smoking. Add sliced onions and drizzle with remaining olive oil. Reduce heat to low, cover and cook, stirring every 10 minutes to prevent burning and sticking, for 25-30 minutes or until onions are soft and brown.

3. Meanwhile, on prepared baking sheet or pizza stone, spread dough using your fingertips to gently press the dough out so it covers the entire sheet. Bake in preheated oven for 6 to 8 minutes or until dough starts to harden but not brown. Let cool in pan on a rack for about 10 minutes.

4. Spread tomato sauce over crust and scatter caramelized onions, artichokes, olives, mushrooms, spinach, apples, and mozzarella.

5. Bake for 10 to 12 minutes or until crust is browned around the edges.

Low Protein Pizza Dough servings: 4 1 cup warm water 1/2 tsp brown sugar 120 gm bread mix ½ tsp olive oil 1/8 tsp garlic powder .56 tsp active dry yeast Directions:

1. Combine in a small bowl ½ cup warm water, dry yeast, and the brown sugar, and mix well. Let it go frothy, about 5 minutes.

2. In a large bowl, combine garlic powder, bread mix, ½ tsp olive oil, and frothy yeast mixture. Adding more warm water, beat with a wooden spoon or dough cook until mixture forms a sticky dough.

3. Transfer to a greased bowl and let the dough rise for 20 minutes. Spread dough using your fingertips to cover baking sheet or pizza stone.

4. Bake pizza dough for 6 to 8 minutes before filling to prevent a soggy crust.

Analysis:Pertinent

Macronutrient, Vitamin, and/or

Mineral

Daily Recommended Intake and/or AMDR

Range

Your Recipe % of DRI or AMDR %

Total Calories 2,208.13 kcal/day; 552.03 kcal/meal

540 kcal 97.8%

Protein 10-20% 21.38 g; 85.52 kcal

15.8%

Carbohydrates 50-60% 86.25 g;345 kcal

63.9%

Total Fat 20-35% 15.14 g;136.26 kcal

25.2%

Saturated Fat <7% 2.86 g;25.74 kcal

4.8%

Fiber 25 g/day 21.45 g 343.2%Sodium 1.5 g/day 1.35 g 360%Manganese 1.8 g/day 1.13 g 251.1%Selenium 55 μg/day 25.73 μg 187.1%

Folate 400 μg/day 232 μg 232%Vitamin B12 2.4 μg/day .536 μg 87.7%

Calcium 1000 mg/day 461 mg 184.4%Iron 18 mg/day 5.25 mg 116.7%Zinc 8 mg/day 3.06 mg 153%

Vitamin A 700 μg/day 648.3 μg 370.5%Vitamin C 75 mg/day 34.92 mg 186.2%Vitamin D 15 μg/day 6 μg 160%Vitamin E 15 mg/day 5.36 mg 142.9%

Modified:

• Originally, recipe contained red bell peppers

• Caused too high levels of Vitamins A & C

• Substituted with shiitake mushrooms

• Also increased Vitamin B12 • Added apples, spinach, and a little

mozzarella cheese to balance micronutrients

• Decreased amount of bread mix because carbohydrate content was too high

• Decreased amount of tomato sauce because Vitamin A and Manganese was too high

Sources:

• http://www.sciencedirect.com/science/article/pii/S1096719213003211

• http://pediatrics.aappublications.org/content/104/6/e68.full

• http://www.biomedcentral.com/content/pdf/1743-7075-9-7.pdf

• http://www.mayoclinic.org/diseases-conditions/phenylketonuria/basics/symptoms/con-20026275

• http://www.vitamins-supplements.org/amino-acids/phenylalanine.php