PEDIATRIC: THYROID GLAND DISORDER
CONGENITAL HYPOTHYROIDISM (CRETENISM)
Data Base
A. General information: Etiology and Pathophysiology
 Disorder related to absent or nonfunctioning thyroid
 Failure of the embryonic development of the thyroid
gland or inborn enzyme defect in the formation of
thyroxine
 Impaired development of nervous system leads to
mental retardation; level depends on degree of
hypothyroidism and interval before therapy is begun
 Appears at 3 to 6 months of age in formula-fed babies;
may be delayed in breastfed babies; newborns are
supplied with maternal thyroid hormones that last up to
3 months
 Characteristic infant facies: short forehead; wide, puffy
eyes; wrinkled eyelids; broad, short, upturned nose;
large, protruding tongue; hair is dry, brittle, and
lusterless with low hairline
 Possible causes
 Antithyroid drugs taken during pregnancy (in
infants)
 Chromosomal abnormalities
 Chronic autoimmune thyroiditis (in children older
than age 2)
 Defective embryonic development that causes
congenital absence or underdevelopment of the
thyroid gland (most common cause in infants)
 Inherited enzymatic defect in the synthesis of
thyroxine (T4) caused by an autosomal recessive
gene (in infants)
B. Clinical findings
 General findings
 Delayed dentition
 Legs shorter in relation to trunk size
 Cognitive impairment (develops as the disorder
progresses)
 Short stature with the persistence of infant
proportions
 Short, thick neck
 Skin is yellowish from carotenemia resulting from
decreased conversion of carotene to vitamin A
 Prolonged physiologic jaundice, feeding
difficulties, inactivity (excessive sleeping, little
crying), anemia, problems resulting from hypotonic
abdominal muscles (constipation, protruding
abdomen, and umbilical hernia)
 With slow basal metabolic rate
 Cool body and skin temperature
 Decreased perspiration
 Dry, scaly skin; skin is mottled because of
decreased heart rate and circulation
 Easy weight gain (decreased growth and decreased
metabolic rate)
 Slow pulse
 Untreated hypothyroidism in infants
 Hoarse crying
 Persistent jaundice
 Respiratory difficulties
 Untreated hypothyroidism in older children
 Bone and muscle dystrophy
 Cognitive impairment
 Stunted growth (dwarfism)
C. Diagnostic evaluation
 Electrocardiogram shows bradycardia and flat or
inverted T waves in untreated infants
 Hip, knee, and thigh x-rays reveal absence of the
femoral or tibial epiphyseal line and delayed skeletal
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development that’s markedly inappropriate for the
child’s chronological age
 In myxedema coma, laboratory tests may also show low
serum sodium levels, decreased pH, and increase partial
pressure of arterial carbon dioxide, indicating
respiratory acidosis
 Increased gonadotropin levels accompany sexual
precocity in older children and may coexist with
hypothyroidism
 Serum cholesterol, alkaline phosphatase, and
triglycerides levels are elevated
 Normocytic normochromic anemia is present
 Radioimmunoassay confirms hypothyroidism with low
triiodothyronine and T4 levels
 Thyroid scan and 131I uptake tests show decreased
uptake levels and confirm the absence of thyroid tissue
in athyroid children
 Thyroid-stimulating hormone (TSH) level is decreased
when hypothyroidism is due to hypothalamic or
pituitary insufficiency
D. Medical management
1. Prevention: neonatal screening blood test
2. Drug therapy option: thyroid hormone replacement
 Oral thyroid hormone: levothyroxine (Synthroid)
 Supplemental vitamin D (to prevent rickets
resulting from rapid bone growth)
 Without treatment mental retardation and
developmental delay will occur after age 3 months
E. Therapeutic interventions
1. Detection: neonatal screening for thyroxine (T4) and
thyroid-stimulating hormone (TSH)
a. Test is routine and mandatory in many areas
b. Performed by heel-stick blood test at the same time
as other neonatal metabolic tests
2. Treatment: replacement therapy with thyroid hormone;
if therapy is begun before 3 months of age, chances for
normal growth and normal IQ are increased
General Nursing Care of Children with
Inborn Errors of Metabolism
A. ASSESSMENT
1. Verification of test results
2. Parent’s understanding of disorder
3. Growth and development
B. ANALYSIS/NURSING DIAGNOSES
1. Interrupted family processes related to situational crisis;
genetic illness
2. Anticipatory grieving related to loss of perfect child
3. Delayed growth and development related to changes in
metabolism
4. Imbalanced nutrition: less than body requirements
related to restrictive diet
C. PLANNING/IMPLEMENTATION
1. During early management of infantile hypothyroidism,
monitor blood pressure and pulse rate; report
hypertension and tachycardia immediately (normal
infant heart rate is approximately 120 beats per minute).
These signs of hyperthyroidism indicate that the dose of
thyroid replacement medication is too high.
2. Check rectal temperature every 2 to 4 hours. Keep the
infant warm and his skin moist to promote
normothermia and reduce metabolic demands
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 3. If the infant’s tongue is unusually large, position him/her on his/her side and observe him/her frequently to prevent airway
obstruction
4. Provide parents with support, referrals, and counseling as necessary to help them cope with the possibility of caring for a physically
and cognitively impaired child
5. Adolescent girls require future-oriented counseling that stresses the importance of adequate thyroid replacement during pregnancy.
Ideally, females should have excellent control before conception to prevent congenital hypothyroidism in their child
6. Specific nursing care for children with hypothyroidism
a. Instruct parents regarding administration of thyroid replacement and signs of overdose (rapid pulse, dyspnea, insomnia,
irritability, sweating, fever, and weight loss)
b. Teach parents to take pulse
7. Provide client teaching and discharge planning concerning
 Medication administration and side effects
 Importance of continued therapy

F. EVALUATION/OUTCOMES
1. Achieves satisfactory growth and development
2. Consumes adequate nutrients for growth
3. Child and family verbalize necessity of diet and acceptable modifications
4. Child and family verbalize and demonstrate prescribed diet/medications

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