Pediatric Nursing Demystified

Three relatively rare metabolism disorders are galactosemia, maple syrup

urine disease, and phenylketonuria. Galactosemia is the inability of the patient

to metabolize galactose, which is necessary to convert carbohydrates into glucose

for energy because the patient is missing the GALT hepatic enzyme.

Maple syrup urine disease is a defective or absence of the branched-chain

amino acid, causing a buildup of the amino acid and ketoacids in urine result-

ing in urine smelling like burnt sugar.

Phenylketonuria is a disorder that is the result of a dysfunctional phenylala-

nine hydroxylase, leading to a buildup of phenylalanine in the body that can

cause mental retardation.

(^194) Pediatric Nursing Demystified
FINAL CHECKUP

1. Why would a patient diagnosed with Cushing syndrome experience delayed
   wound healing?
   a. Excessive corticosteroids reduce the inflammation process.
   b. Decreased corticosteroids reduce the inflammation process.
   c. Frequent bruising.
   d. Changes in mental status.


2. A parent of a child who is recently diagnosed with type 1 diabetes mellitus is
   concerned that her child still exhibits hyperglycemic and hypoglycemic symp-
   toms. What is your best response?
   a. You must carefully monitor your child’s blood glucose level.
   b. The health-care provider adjusts the insulin dose based on the child’s activities
   and diet.
   c. The health-care provider is still in the process of adjusting your child’s insulin
   dose and diet to determine the proper balance for your child.
   d. You must carefully monitor your child’s urine glucose level.


3. The mother of a child who has been diagnosed with type 1 diabetes mellitus asks
   what effect a glucagon injection has on her child. What is the best response?
   a. Glucagon transports glucose into the cell where glucose is metabolized.
   b. Glucagon causes the liver to release glucose.
   c. Glucagon is used for signs of hyperglycemia.
   d. Glucagon is a form of long-lasting insulin.


4. What test should frequently be performed on a child who is being treated for
   Graves disease?
   a. A urine test to determine if the patient has developed type 1 diabetes mellitus.
   b. A urine test to determine if the patient has developed type 2 diabetes mellitus.
   c. A blood test to determine if the patient has developed type 1 diabetes mellitus.
   d. A blood count because the treatment may result in leucopenia and thrombocytopenia.

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