Peds Exam 3a - Endo (minor)

Question 1

PKU clinical manifestations

Answer 1

-restricted growth
-musty odor and sweat
-fair skin, blue eyes, blonde
-Vomiting, irritabile
-seizure
-cognitive impairment

Question 2

pku mgt

Answer 2

-breast feed or phen free formula
-phen free diet for life (no meat, no dairy, limited fruits & veg, limited grains)

Question 3

galactosemia

Answer 3

cannot convert galactose to glucose (autosomal recessive)
not lactose intolerant

Question 4

galactosemia clinical manifestations

Answer 4

-vomiting
-wt loss
-jaundice
-lethargy
-hypotonia
-cataracts

Question 5

long term consequence of galatosemia

Answer 5

-learning disabilities
-lower IQ
-short attention span
-behavior problems

Question 6

galactosemia mgt

Answer 6

-lactose free diet
-calcium supplements
-medication cautions (BC has lactose in it)
cannot be breastfed

Question 7

hypothyroidism symptoms

Answer 7

everything slows down & high TSH
-constipated
-wt gain
-hypothermia & tonia
-poor feeding & delayed dentition
-prolonged jaundice
-short, thick neck
-long protruding tongue
-goiter; can be an airway problem

Question 8

hyperthyroidism symptoms (graves)

Answer 8

everything speeds up & low TSH
-sweaty
-hair loss
-wt loss
-eyes bulge

Question 9

therapeutic mgt of hypothyroidism

Answer 9

oral thyroid hormone replacement (start low and slowly move up)

Question 10

nursing considerations for hypothyroidism

Answer 10

-early recognition
-encourage compliance and periodic monitoring (med doses change as they age)
-teach medication admin (avoid heat exposure & do not mix w/ soy based formula)

Question 11

therapeutic mgt of hyperthyroidism

Answer 11

-dx w/ t3 & t4 w/ suppressed TSH
-anti thyroid drugs (PTU & MTZ, tapazole)
-subtotal thyroidectomy
-ablation w/ radioiodine

Question 12

what do you do if you have a child with hyperthyroidism and they are put on PTU then develop a sore throat or an infection

Answer 12

very series take them to the doctors immediately & keep them away from people because PTU makes them immunocompromised

Question 13

nursing considerations for hyperthyroidism

Answer 13

-identify early
-limit activity & demands placed on child
-counsel family & teachers
-high kcal, nutritious diet
-medications (PTU)

Question 14

thyrotoxicosis

Answer 14

may occur from sudden release of hormone a life threatening thyroid crisis
everything is in overdrive

Question 15

thyrotoxicosis treatment

Answer 15

-antithyroid drugs
-propranolol

Question 16

growth hormone deficiency

Answer 16

-born normal wt then just do not grow
-takes a lot to dx, need parents height & predictions
-grow proportional, just small
appears younger than their age but cognitive function develops normally, causing psych/social issues

Question 17

growth hormone deficiency treatment

Answer 17

administer growth hormones at night subQ 5-7x/wk , usually do school age children

Question 18

growth hormone deficiency education

Answer 18

-when giving shot squeeze fat & can use ice before to reduce pinch
-make sure parents know timing of doses
-not making extremely tall kids, just normal for their predicted
-treat as their age, not their appearance

Question 19

how do you treat true/complete precocious puberty

Answer 19

lupron injection 1x/mon IM at a clinic until normal pubertal changes occur
G&D question for age and how to engage/prep the child in their care and give some control

Question 20

congenital adrenal hyperplasia

Answer 20

too much sex hormone production & not enough cortisol production causing increase urine Na, ambiguous genitalia (sometimes cannot tell gender), wt gain problems & dehydration

Question 21

in congenital adrenal hyperplasia, infants specifically have a problem because

Answer 21

they have a decreased stress response causing hypotension, compromised immune systems, hypoNa dehy, and increase inflammatory response

Question 22

in congenital adrenal hyperplasia, later in life when you have a decreased stress response, what do you do

Answer 22

cortisol replacement for the rest of their life

Question 23

congenital adrenal hyperplasia therapeutic mgt

Answer 23

-confirm diagnosis & assign a sex according to genotype
-cortisone to suppress abnormally high secretion of ACTH
-reconstructive surgery as required