Ch 48 lecture (endocrine disorders in children)

Question 1

Teaching highlights for sickday guidelines in Type 1 diabetes

Answer 1

1. Monitor the blood glucose levels every 2 to 3 hours or as instructed.
2. Test urine ketones every 4 hours or as instructed by the healthcare provider.
3. The usual dose of insulin may be increased for high blood glucose levels.
4. Notify the healthcare provider if the child has fever or other signs of infection, is unable to tolerate fluids, has ketones in the urine, has blood glucose levels that are out of target range, or has signs of dehydration.
5. Are there sores on the feet? Do scratches or scrapes take a long time to heal?
6. Has the child had any skin infections?
7. Does the child have changes in mood (depression, unexplained sadness, irritability) or energy level from day to day or throughout the day?
8. Have there been any changes in vision?

Question 2

___ is a common and potentially life-threatening condition that occurs primarily in children with type 1 diabetes.

Answer 2

Diabetic ketoacidosis (DKA)

Potential causes of DKA include:

1. incorrect or missed insulin doses,
2. incorrect administration of insulin, or
3. an illness, trauma, or surgery.
4. DKA may be present in children with new-onset diabetes.

s/s:

1. Polyuria
2. Polydipsia
3. weight loss
4. abdominal pain
5. nausea and vomiting
6. tachycardia
7. signs of dehydration
8. flushed ears and cheeks
9. Kussmaul respirations
10. acetone breath (fruity smell)
11. altered level of consciousness
12. hypotension
13. Hyperglycemia glycosuria and ketonuria are also present.
14. In response to metabolic acidosis, children complain of abdominal or chest pain, nausea, and vomiting.

Question 3

DKA is present with the following findings:

Answer 3

1. blood glucose level greater than 250 mg/dL
   (Many children admitted are undiagnosed, so this is for both T1 and T2 as we don’t know what type they are yet)
2. serum ketones, acidosis (pH less than 7.3 and bicarbonate less than 15 mEq/L) and ketonuria
3. Alteration in electrolytes occur. Correct electrolytes slowly! Especially K -> at a LOW and SLOW rate
4. The blood urea nitrogen (BUN) and creatinine are elevated

Question 4

DKA nursing management:

Answer 4

1. Continuously monitor the child’s vital signs, respiratory status, perfusion, and mental status.
2. Assess for changes in neurologic status, respiratory pattern, blood pressure, and heart rate.
3. Monitor for cardiac arrhythmias associated with hypokalemia.
   (Insulin helps K into cells, so pt will have too much K in blood, but it needs to go back into cells)
4. Assess for signs of dehydration, including dry skin and mucous membranes and depressed fontanelles in infants.
   (Initial fluid is NS)
5. Monitor blood glucose levels hourly or as indicated.
6. Frequently monitor the electrolytes and acid–base status, as well as urine glucose and ketone levels as indicated.
7. Intake and output are monitored hourly.
8. Assess for signs of hypoglycemia that may occur during insulin infusion.

Question 5

Phenylketonuria (PKU)

Answer 5

is an autosomal recessive inherited disorder of amino acid metabolism that affects the body’s use of protein. Children with PKU have a deficiency of the liver enzyme phenylalanine hydroxylase that normally breaks down the essential amino acid phenylalanine into tyrosine.

The defect results in an accumulation of phenylalanine in the blood or phenylalanine metabolites in the urine. If untreated, this disease leads to irreversible brain damage and severe intellectual disability.

Normal is between 2 – 6 mg/dL

Question 6

Phenylalanine levels above __ are indicative of classic PKU.

Answer 6

20 mg/dL

Normal is between 2 – 6 mg/dL

Question 7

What are clinical manifestations of PKU?

Answer 7

phenylalanine accumulates in the blood, causing a musty or mousey body and urine odor, irritability, vomiting, hyperactivity, hypertonia, hyperreflexive deep tendon reflexes, seizures, and an eczema-like rash.

Persistence of elevated phenylalanine leads to disruption of cellular processes of myelination and protein synthesis, and results in a seizure disorder and untreatable intellectual disability.

Babies appear normal at birth except for lighter skin complexion than their nonaffected siblings.

If diagnosis is delayed, a mousy or musty body odor is noticed and intellectual disability may be severe.

Other common findings in untreated children include microcephaly, growth retardation, enamel hypoplasia, prominent maxilla, and widely spaced teeth

Question 8

For best results in a PKU screening, the newborn should have begun __ before specimen collection.

Answer 8

formula or breast milk feeding

Other important screening information includes:

1. Early hospital discharge places newborns at risk for false-negative screening tests if screened within 24 hours of birth.
2. Screening needs to occur no sooner than 48 hours after birth, or the test should be repeated at 1 to 2 weeks of age.
3. If the test shows elevated levels of plasma phenylalanine, a repeat quantitative test is performed.
4. If the second test is positive, the family is referred to an outpatient treatment center.
5. Serum levels of phenylalanine should be measured periodically throughout life. The child will be on a special diet.