PEDs Metabolic/Thyroid/Endocrine Alterations

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ashlynn4787
ID:
299832
Filename:
PEDs Metabolic/Thyroid/Endocrine Alterations
Updated:
2015-04-03 19:03:13
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PEDs metabolic alterations endocrine thyroid
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PEDs Metabolic/Thyroid/Endocrine Alterations
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  1. What is Galactosemia?
    • Prevents conversion of galactose to glucose.
    • Can't digest milk or sugar.
    • Infants exhibit growth, retardation, hypotonia, liver damage, cataracts, and infections. Vomiting and diarrhea after feeding.
  2. Galactosemia Management
    • Lactose restricted diet
    • close monitoring for infection
    • untreated equals death
    • treated may have learning deficits
  3. What is Maple Syrup Urine Disease?
    • Affects metabolism of certain amino acids
    • buildup causes ketoacidosis (48-72hrs after birth)
    • Infant is lethargic, poor feeding, vomiting, weight loss, seizures, and loss of reflexes.
    • Urine smells like maple syrup
  4. Maple Syrup Urine Disease Management
    • Dialysis is needed
    • low protein, low amino acid diet
    • untreated can die quickly
    • treated can have neurologic deficits
  5. Tay-Sachs Disease
    • Affects the Jewish population
    • after 6 months infant begins to display neurplogic deterioration
    • Macrocephaly, seizures, blindness, deafness and death in early childhood
  6. Tay-Sachs Disease Management
    symptomatic and supportive
  7. Congenital Hypothyroidism S/S
    • mottled skin
    • large frontanel
    • large tounge
    • hypotonic muscle tone
    • slow reflexes
    • distended abdomen
    • prolonged jaundice
    • lethargy
    • feeding problems
    • coldness to touch
    • umblilical hernia
    • hoarse cry
    • excessive sleeping
    • Can cause mental retardation that cannot be reversed
  8. Management for Congenital Hypothyroidism
    • Synthetic thyroid hormone replacement (levothyroxine, synthroid)
    • Sick day rules-repeat dose if throw up within one hour of med admin. Repeat dose
    • Monitor Pulse for changes
  9. Hypothyroidism S/S
    • Fatigue
    • constipation
    • cold intolerance
    • weight gain
    • dry thick skin
    • edema of face, eyes, hands
    • decreased growth
    • decreased energy
    • muscle hypertrophy
    • decreased heart rate
    • delayed skeletal maturation
    • delayed puberty
  10. Hypothyroidism Management
    Synthetic thyroid hormone (levothyroxine, synthroid)

    Teach s/s of hyperthyroidism
  11. Hyperthyroidism S/S
    • Nervousness, anxiety
    • Diarrhea
    • heat intolerance
    • weight loss
    • smooth, velvet skin
    • prominent eyes
    • accelerated linear growth
    • emotional lability
    • muscle weakness
    • increased heart rate
    • high BP
    • tremor
    • increased appetite
  12. Hyperthyroidism Management
    • Antithyroid drug therapy (propylthiouracil, methimazole)
    • oral radioactive iodine treatment
    • subtotal or total thyroidectomy

    • Can cause changes in bone marrow suppression
    • *Call Dr if baby is sick*
    • watch for s/s of hypothyroidism
  13. What is Congenital Adrenal Hyperplasia?
    • Autosomal recessive
    • Not enough glucocorticoid
    • Lack of cortisol leas to male hormones produced
  14. Congenital Adrenal Hyperplasia S/S
    • ambiguous genitalia
    • Salt washing-depleted Na, increased K, dehydrated, HTN
    • short stature
    • early pubic hair
  15. Congenital Adrenal Hyperplasia (CAH) Management
    • glucocorticoid (hydrocortisol, fludrocortisone)
    • Sick day rules- 2-3x more
  16. Precocious Puberty
    Signs of secondary development before age 8 in girls & age 9 in boys

    • Concerning because during puberty get last growth spurt
    • Want adequate time for growth
  17. Precocious Puberty Girls S/S
    • Breast development
    • pubic hair
    • axillary hair
    • enlargement of vagina, uterus, ovaries
    • acne
    • growth spurt
    • adult body odor
    • onset of mentruation
    • moodiness
  18. Precocious Puberty Boys S/S
    • Testicular enlargement
    • Penile enlargement
    • Pubic hair
    • Facial hair
    • acne
    • adult body odor
    • deepening of voice
    • moodiness
  19. Precocious Puberty Management
    • Gonadotropin releasing hormone agonist (GnRH)
    • Given intranasally or by monthly IM injection

    • Psychosocial support
    • child needs to be treated based on age
  20. Growth Hormone Deficiency
    • height less than 5th percentile for age
    • diminished growth rate (<5cm/year)

    must consider family height
  21. Growth Hormone Deficiency S/S
    • Immature or cherub face
    • delayed puberty
    • hypoglycemia
    • diminished muscle mass with increased adipose tissue
    • micropenis
  22. Growth Hormone Deficiency Management
    • Synthetic growth hormone replacement
    • reconstituted, refridgerated, subQ
    • administer at bedtime
    • Until adequate height or until growth plates fuse

    • Psychosocial support
    • Talk tot hem as their age not appearance

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