Transcript What Do You Know About PKU?

What Do You Know About PKU?
1. What is phenylketonuria or PKU?
2. What causes PKU?
3. What are the effects of PKU on infants and
children?
4. How is PKU diagnosed? When is testing
conducted?
5. What is phenylalanine? Why is it important?
Copyright © Notice: The materials are copyrighted © and trademarked ™ as the property of The Curriculum Center for Family and Consumer Sciences, Texas Tech University.
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What Do You Know About PKU?
6. How is PKU treated?
7. What is aspartame? By what trade names is it
known?
8. Why must a person with PKU not use aspartame?
9. What special diet must a person with PKU follow?
10. What alternate food sources could help a person
with PKU meet the daily requirements for protein?
Copyright © Notice: The materials are copyrighted © and trademarked ™ as the property of The Curriculum Center for Family and Consumer Sciences, Texas Tech University.
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What Do You Know About PKU?
Answer Key
1. What is phenylketonuria or PKU?
•
Phenylketonuria (PKU) is a disorder in
which there is a deficiency of the enzyme
that metabolizes phenylalanine in the liver.
2. What causes PKU?
•
PKU is inherited and is present at birth.
Symptoms of PKU result from the buildup of
phenylalanine to toxic levels in the blood.
Copyright © Notice: The materials are copyrighted © and trademarked ™ as the property of The Curriculum Center for Family and Consumer Sciences, Texas Tech University.
What Do You Know About PKU?
Answer Key
3. What are the effects of PKU on infants and
children?
•
If not treated, babies born with PKU will
experience abnormal brain development
resulting in mental retardation. Once PKU is
diagnosed, newborns are given a special diet to
prevent buildup of phenylalanine. Infants with
PKU, for example, cannot be breast-fed.
Copyright © Notice: The materials are copyrighted © and trademarked ™ as the property of The Curriculum Center for Family and Consumer Sciences, Texas Tech University.
What Do You Know About PKU?
Answer Key
4. How is PKU diagnosed? When is testing
conducted?
•
Tests are available to diagnose PKU; the most
common test currently used is the Guthrie test.
In Texas, all newborns are required by state
law to be tested for PKU a few days after birth.
Copyright © Notice: The materials are copyrighted © and trademarked ™ as the property of The Curriculum Center for Family and Consumer Sciences, Texas Tech University.
What Do You Know About PKU?
Answer Key
5. What is phenylalanine? Why is it
important?
•
Phenylalanine is one of the essential
amino acids, which make up protein. All
essential amino acids must be present
for protein synthesis to occur; therefore,
some phenylalanine is required for the
body to synthesize proteins.
Copyright © Notice: The materials are copyrighted © and trademarked ™ as the property of The Curriculum Center for Family and Consumer Sciences, Texas Tech University.
What Do You Know About PKU?
Answer Key
6. How is PKU treated?
•
PKU is treated through a special diet,
which must begin before the infant is
two years old and continue throughout
the individual’s life.
Copyright © Notice: The materials are copyrighted © and trademarked ™ as the property of The Curriculum Center for Family and Consumer Sciences, Texas Tech University.
What Do You Know About PKU?
Answer Key
7. What is aspartame? By what trade
names is it known?
•
Aspartame is an artificial sweetener
composed of methanol and two amino
acids—phenylalanine and aspartic acid.
It is known by the trade names
NutraSweet® and Equal®.
Copyright © Notice: The materials are copyrighted © and trademarked ™ as the property of The Curriculum Center for Family and Consumer Sciences, Texas Tech University.
What Do You Know About PKU?
Answer Key
8. Why must a person with PKU not
use aspartame?
•
Aspartame cannot be used by a
person with PKU because it contains
phenylalanine as an additive.
Copyright © Notice: The materials are copyrighted © and trademarked ™ as the property of The Curriculum Center for Family and Consumer Sciences, Texas Tech University.
What Do You Know About PKU?
Answer Key
9. What special diet must a person
with PKU follow?
•
The diet must avoid high-protein foods
and include fruits, vegetables, and
cereals. No soft drinks or other foods
to which aspartame has been added
can be consumed.
Copyright © Notice: The materials are copyrighted © and trademarked ™ as the property of The Curriculum Center for Family and Consumer Sciences, Texas Tech University.
What Do You Know About PKU?
Answer Key
10. What alternate food sources could
help a person with PKU meet the
daily requirements for protein?
•
Answers will vary. Infants must use a
special formula since milk is not
allowed.
Copyright © Notice: The materials are copyrighted © and trademarked ™ as the property of The Curriculum Center for Family and Consumer Sciences, Texas Tech University.