NCLEX RN Practice Questions Quizlet Related

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A new mother has some questions about phenylketonuria (PKU). Which of the following
statements made by a nurse is not correct regarding PKU?

  • A. A Guthrie test can check the necessary lab values.
  • B. The urine has a high concentration of phenylpyruvic acid
  • C. Mental deficits are often present with PKU
  • D. The effects of PKU are reversible
Correct Answer: D

Rationale: Phenylketonuria (PKU) is an inherited disorder that increases the levels of phenylalanine (a
building block of proteins) in the blood. If PKU is not treated, phenylalanine can build up
to harmful levels in the body, causing intellectual disability and other serious health
problems. The signs and symptoms of PKU vary from mild to severe. The most severe form
of this disorder is known as classic PKU. Infants with classic PKU appear normal until they
are a few months old. Without treatment, these children develop a permanent intellectual
disability. Seizures, delayed development, behavioral problems, and psychiatric disorders
are also common. Untreated individuals may have a musty or mouse-like odor as a side
effect of excess phenylalanine in the body. Children with classic PKU tend to have lighter skin and hair than unaffected family members and are also likely to have skin disorders
such as eczema. The effects of PKU stay with the infant throughout their life (via Genetic
Home Reference).