Phenylketonuria PKU Diet

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Food NameProteinPhenylalanine
Butter, salted0.850.041
Butter, whipped, with salt0.850.041
Butter oil, anhydrous0.280.014
Cheese, blue21.401.087
Cheese, brick23.241.231
Cheese, brie20.751.158
Cheese, camembert19.801.105
Cheese, caraway25.181.326
Cheese, cheddar24.901.311
Cheese, cheshire23.371.231
Cheese, colby23.761.251
Cheese, cottage, creamed, large or small curd11.120.577
Cheese, cottage, creamed, with fruit10.690.572
Cheese, cottage, nonfat, uncreamed, dry, large or small curd10.340.536
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  • Abbreviations: g = gram, mg = milligram, mcg = microgram, kcal = kilocalorie, kJ = kilojoule.






Information from the National Institutes of Health

Phenylketonuria or PKU

Phenylketonuria or PKU is a rare condition in which a baby is born without the ability to properly break down an amino acid called phenylalanine.

Phenylketonuria PKU Causes

Phenylketonuria is inherited, which means it is passed down through families. Both parents must pass on the defective gene in order for a baby to have the condition. This is called an autosomal recessive trait.

Babies with PKU are missing an enzyme called phenylalanine hydroxylase, which is needed to break down an essential amino acid called phenylalanine. The substance is found in foods that contain protein.

Without the enzyme, levels of phenylalanine and two closely-related substances build up in the body. These substances are harmful to the central nervous system and cause brain damage.

Phenylketonuria PKU Treatment

PKU is a treatable disease. Treatment involves a diet that is extremely low in phenylalanine, particularly when the child is growing. The diet must be strictly followed. This requires close supervision by a registered dietitian or doctor, and cooperation of the parent and child. Those who continue the diet into adulthood have better physical and mental health. “Diet for life” has become the standard recommended by most experts. This is especially important before conception and throughout pregnancy.

Phenylalanine occurs in significant amounts in milk, eggs, and other common foods. The artificial sweetener NutraSweet (aspartame) also contains phenylalanine. Any products containing aspartame should be avoided.

A special infant formula called Lofenalac is made for infants with PKU. It can be used throughout life as a protein source that is extremely low in phenylalanine and balanced for the remaining essential amino acids.

Taking supplements such as fish oil to replace the long chain fatty acids missing from a standard phenylalanine-free diet may help improve neurologic development, including fine motor coordination. Other specific supplements, such as iron or carnitine, may be needed.

Phenylalanine

Phenylalanine is an essential amino acid. Phenylalanine can cause mental retardation, brain damage, seizures and other problems in people with PKU.

Phenylalanine occurs naturally in many protein-rich foods, such as milk, eggs and meat. Phenylalanine also is sold as a dietary supplement.

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Phenylketonuria PKU Diet

Avoid foods with phenylalanine.

Besides foods naturally rich in phenylalanine, the artificial sweetener aspartame (Equal, NutraSweet), which is added to many medications, diet foods and diet sodas, contains phenylalanine. Federal regulations require that any food that contains aspartame bear this warning: "Phenylketonurics: Contains phenylalanine."


Below are tables of foods having some protein but lowest in phenylalanine content.
Phenylalanine and protein contents are in milligrams per 100 grams of food weight.



Phe (mg) Protein (mg)
Foods with Protein but Low-Phe (100 g)

0 820
Gerber graduates Fruit Strips Real Fruit Bars

0 100
Frostings, cream cheese-flavor, ready-to-eat

0 1090
Salad dressing, thousand island, commercial, regular

0 830
Salad dressing, thousand island dressing, reduced fat

0 300
Salad dressing, mayonnaise, imitation, soybean

0 100
Salad dressing, mayonnaise, imitation, soybean without cholesterol

0 400
Mollusks, clam, mixed species, canned, liquid

0 460
Alcoholic beverage, beer, regular, all

0 20
Cranberry juice cocktail, bottled, low calorie, with calcium, saccharin and corn sweetener

0 240
Alcoholic beverage, beer, light

0 100
Salad dressing, french, home recipe

1 320
Guava sauce, cooked

1 580
Guavas, strawberry, raw

1 600
Tea, instant, sweetened with sugar, lemon-flavored, with added ascorbic acid, powder

3 100
Puddings, lemon, dry mix, regular, with added oil, potassium, sodium

3 120
Coffee, brewed from grounds, prepared with tap water

3 100
Puddings, lemon, dry mix, regular

3 100
Coffee, brewed from grounds, prepared with tap water, decaffeinated

4 100
Puddings, tapioca, dry mix

4 190
Tapioca, pearl, dry

4 100
Puddings, tapioca, dry mix, with no added salt

5 180
Applesauce, canned, sweetened, with salt

5 190
Pears, canned, water pack, solids and liquids

5 190
Pears, canned, light syrup pack, solids and liquids

5 200
Pears, canned, heavy syrup pack, solids and liquids

5 180
Apples, canned, sweetened, sliced, drained, unheated

5 180
Apples, canned, sweetened, sliced, drained, heated


Phe (mg) Protein (mg)
Foods with Protein but Low-Phe (100 g)

5 190
Pears, canned, extra heavy syrup pack, solids and liquids

5 170
Applesauce, canned, unsweetened, with added ascorbic acid

5 170
Applesauce, canned, unsweetened, without added ascorbic acid

5 160
Applesauce, canned, sweetened, without salt (includes USDA commodity)

5 400
Orange drink, breakfast type, with juice and pulp, frozen concentrate

5 430
Tangerine juice, frozen concentrate, sweetened, diluted with 3 volume water

6 260
Apples, raw, with skin

6 500
Tangerine juice, canned, sweetened

6 200
Babyfood, dessert, dutch apple, junior

6 2550
Guavas, common, raw

6 500
Tangerine juice, raw

6 200
Apples, dried, sulfured, stewed, with added sugar

6 220
Apples, dried, sulfured, stewed, without added sugar

7 270
Apples, raw, without skin

7 540
Soup, SWANSON Chicken Broth 99% Fat Free

7 280
Apples, raw, golden delicious, with skin

8 680
Orange juice, canned, unsweetened

8 370
Pie fillings, canned, cherry

8 680
Orange juice, frozen concentrate, unsweetened, diluted with 3 volume water

8 390
Plums, canned, purple, water pack, solids and liquids

8 360
Plums, canned, purple, extra heavy syrup pack, solids and liquids

8 300
Pears, canned, extra light syrup pack, solids and liquids

8 280
Apples, dehydrated (low moisture), sulfured, stewed

8 290
Apples, frozen, unsweetened, heated

8 370
Plums, canned, purple, light syrup pack, solids and liquids

8 280
Apples, frozen, unsweetened, unheated

8 280
Apples, raw, without skin, cooked, microwave

8 360
Plums, canned, purple, heavy syrup pack, solids and liquids

9 360
Pineapple, canned, light syrup pack, solids and liquids

9 470
Papayas, raw

9 380
Figs, canned, extra heavy syrup pack, solids and liquids


Phe (mg) Protein (mg)
Foods with Protein but Low-Phe (100 g)

9 380
Figs, canned, heavy syrup pack, solids and liquids

9 700
Orange juice, raw

9 350
Pineapple, canned, heavy syrup pack, solids and liquids

9 330
Pickles, cucumber, sour

9 330
Pickles, cucumber, sour, low sodium

9 340
Pineapple, canned, extra heavy syrup pack, solids and liquids

9 340
Pears, canned, juice pack, solids and liquids

10 300
Marmalade, orange

10 480
Grapes, canned, thompson seedless, heavy syrup pack, solids and liquids

10 420
Pineapple, canned, juice pack, solids and liquids

10 160
Noodles, chinese, cellophane or long rice (mung beans), dehydrated

10 400
Figs, canned, water pack, solids and liquids

10 500
Grapes, canned, thompson seedless, water pack, solids and liquids

10 600
Clam and tomato juice, canned

10 370
Pickles, cucumber, sweet, low sodium (includes bread and butter pickles)

10 390
Figs, canned, light syrup pack, solids and liquids

11 300
Honey

11 400
Crabapples, raw

11 430
Pineapple, canned, water pack, solids and liquids

11 420
Lime juice, raw

11 370
Pickle relish, sweet

11 680
Orange juice, chilled, includes from concentrate

11 680
Orange juice, chilled, includes from concentrate, fortified with calcium and vitamin D

11 680
Orange juice, chilled, includes from concentrate, fortified with calcium

11 520
Peaches, canned, heavy syrup, drained

11 410
Salad dressing, italian dressing, commercial, regular

11 390
Pears, raw, bartlett

11 510
Plums, canned, purple, juice pack, solids and liquids

11 360
Pears, raw

12 430
Strawberries, frozen, unsweetened

12 370
Grape juice, canned or bottled, unsweetened, without added ascorbic acid


Phe (mg) Protein (mg)
Foods with Protein but Low-Phe (100 g)

12 400
Pineapple, frozen, chunks, sweetened

12 300
Arrowroot flour

13 770
Grapefruit, raw, pink and red, all areas

13 630
Grapes, american type (slip skin), raw

13 410
Peaches, spiced, canned, heavy syrup pack, solids and liquids

13 300
Puddings, vanilla, dry mix, regular

13 400
Peaches, canned, extra light syrup, solids and liquids

13 600
Beef broth and tomato juice, canned

13 440
Sapodilla, raw

13 300
Frostings, vanilla, creamy, dry mix

13 500
Pears, asian, raw

13 390
Salad dressing, italian dressing, commercial, reduced fat

13 260
Cornstarch

13 300
Puddings, vanilla, dry mix, regular, with added oil

14 280
Butter oil, anhydrous

14 600
Gourd, white-flowered (calabash), cooked, boiled, drained, without salt

14 400
Blueberries, frozen, sweetened

14 650
Grape juice cocktail, frozen concentrate, undiluted, with added ascorbic acid

14 710
Turnips, cooked, boiled, drained, without salt

14 430
Loquats, raw

14 700
Plums, raw

14 600
Gourd, white-flowered (calabash), cooked, boiled, drained, with salt

14 450
Peaches, canned, heavy syrup pack, solids and liquids

14 450
Peaches, canned, light syrup pack, solids and liquids

14 440
Peaches, canned, water pack, solids and liquids

14 710
Turnips, cooked, boiled, drained, with salt

15 470
Peaches, canned, extra heavy syrup pack, solids and liquids

15 340
Frostings, vanilla, creamy, dry mix, prepared with margarine

15 620
Gourd, white-flowered (calabash), raw

15 540
Melons, honeydew, raw

15 450
Tangerines, (mandarin oranges), canned, light syrup pack

15 610
Watermelon, raw

15 420
Blueberries, frozen, unsweetened

15 520
Strawberries, frozen, sweetened, whole

15 530
Strawberries, frozen, sweetened, sliced

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