Peds Final Review - Metabolic and Endocrine Disorders Flashcards
CONGENITAL HYPOTHYROIDISM
Description: A congenital condition resulting from inadequate thyroid tissue development in utero.
Mental retardation and growth failure occur if it is not detected and treated in early infancy.
CONGENITAL HYPOTHYROIDISM
Nursing Assessment
Newborn screening reveals low T4 (thyroxine) and high TSH (thyroid-stimulating hormone).
Symptoms in the newborn: --Long gestation (>42 weeks) --Large hypoactive infant --Delayed meconium passage --Feeding problems (poor suck) --Prolonged physiologic jaundice --Hypothermia
Symptoms in early infancy
Large, protruding tongue Coarse hair Lethargy, sleepiness Flat expression Constipation
CONGENITAL HYPOTHYROIDISM
NCLEX Hint: An infant with hypothyroidism is often described as a good, quiet baby by the parents.
CONGENITAL HYPOTHYROIDISM
Nursing Diagnoses:
Delayed growth and development related to
Deficient knowledge of medication program related to
CONGENITAL HYPOTHYROIDISM
Nursing Interventions:
–Perform newborn screening programs before discharge.
–Assess newborn for signs of congenital hypothyroidism.
Teach family about replacement therapy with thyroid hormone:
Explain that child will have a lifelong need for the therapy
Tell parents to give child a single dose in the morning
Teach family to check child’s pulse daily before giving thyroid medication
Signs of overdose include rapid pulse, irritability, fever, weight loss, and diarrhea
Signs of underdose include lethargy, fatigue, constipation, and poor feeding
Periodic thyroid testing is necessary
PKU (Phenylketonuria)
An autosomal recessive disorder in which the body cannot metabolize the essential amino acid phenylalanine.
The buildup of serum phenylalanine leads to CNS damage, most notably mental retardation.
Decreased melanin produces light skin and blond hair.
PKU (Phenylketonuria)
Nursing Assessment:
Newborn screening using the Guthrie test; positive result: serum phenylalanine level of 4 mg/dL
Frequent vomiting, failure to gain weight
Irritability, hyperactivity
Musty odor of urine
PKU (Phenylketonuria)
Nursing Diagnoses:
Delayed growth and development related to
Deficient knowledge of disease process and diet related to
PKU (Phenylketonuria)
Nursing Interventions:
—Perform newborn screening at birth and again at about 3 weeks of age
Teach family dietary management.
Stress the importance of strict adherence to prescribed low-phenylalanine diet
Instruct family to provide special formulas for infant: Lofenalac, Phenex-1
Instruct family to provide phenyl-free milk substitute after the age of 2 years
Teach family to avoid foods high in phenylalanine– that is high-protein foods, such as meat, milk, dairy products, and eggs.
Teach family to offer foods low in phenylalanine, that is vegetables, fruits, juices, cereals, breads, and starches
Encourage family to work with nutritionist
Teach that diet must be maintained at least until brain growth is complete (6 – 8 yr) or for life
Refer for genetic counseling
PKU (Phenylketonuria)
NCLEX Hint: NutraSweet (aspartame) contains phenylalanine and should not therefore be given to a child with PKU
PKU (Phenylketonuria)
NCLEX Hint: NutraSweet (aspartame) contains phenylalanine and should not therefore be given to a child with PKU
Idiopathic hypopituitarism
Nursing Assessment:
Short
Delayed bone age
Permanent tooth eruption delayed
Sexual maturity delayed
Skin fine with delicate features
Idiopathic hypopituitarism
Nursing Interventions:
Management of replacement therapies such as growth hormone, thyroid hormone, cortisol, and sex hormones.
Family teaching to treat child as chronological age. Child will be younger looking than peers.
Central Precocious puberty
Nursing Assessment:
Defined as manifestation of sexual development before 9yr for boys
Before 7yr for Caucasian girls
Before 6yr for African-American girls
Central Precocious puberty
Nursing Interventions:
Teach family about leuprolide injections.
Child will look much older than chronological age.
Urge family to treat child and dress child age-appropriately.
