endo Flashcards
PKU management
breast feed or Phe free formula forever (for protein), no meat or dairy, limited fruits and veggies, limit grains
meet nutritional needs for optimum growth, maintain Phe 2-6
PKU cm: untreated - kids
decreased height and weight, musty odor (urine and sweat), hypopigmentation (fair skin, blue eyes, bland hair, eczema), v, irritable, seizure, hyperactive, retardation
PKU cm: untreated - adults
short attention, poor short term memory, vision, decreased motor coordination, mood disorders, decreased grey matter
on diet for life!
PKU nc
test, teach (normal vaccine), refer for genetic counseling, support, flex feeding schedule, no extra developmental assessment, encourage early treatment
galactosemia cm
v, weight loss, jaundice, lethargy, hypotonia, cataracts
long term: learning disability, decreased IQ, short attention, behavior problems
galactosemia tm
lactose free, dont breast feed unless mom is dairy free, soy formula, Ca supplement, med caution - sensitive to penicillin and other meds that contain galactose
hypothyroid s/s
everything slows down
fatigue, exercise intolerance, slower reaction time, weight gain, constipation, sparse, coarse, dry hair and skin, slow pulse, cold intolerance, muscle cramps, sides of eyebrows thin or fall out, dull facial expression, hoarse voice, slow speech, droopy eyelids, puffy and swollen face, enlarged thyroid (goiter), increased menstrual flow and cramping in younger, hypotherm, large protruding tongue, short thick neck, delayed dentition, hypotonia, poor feed, mental defects if delayed diagnosis
hypothyroid nc
recognize early, compliance and periodic monitoring, med amin - teach to avoid heat, dont mix with soy, check interactions, not liquid
goiter nc
airway, noticeable with rapid growth (puberty), large are obvious and small must palpate (swallow)
goiter tm
oral hormone replacement, prompt treatment (brain growth), may admin in increasing amounts over 4-8 weeks to reach euthryroidism, compliance!! with meds for life
hyperthyroidism s/s
everything speeds up
enlarged thyroid (goiter), increased heart rate and BP, slight tremor, lighter and less frequent menstrual, irritated and bulging eyes with redness or visible blood vessles on white part, pain when moving eyes, inability to fully open eyelid, increased activity, restless, poor sleep, fatigue, increased appetite, increased BM, heat intolerance, decreased school performance, difficulty concentrating, lump in throat
hyperthyroidism tm
diagnose with T3 and T4 levels with decreased TSH
drugs (PTU) can cause agranulocytosis: toxic reaction that decreased wbc, if they have sore throat or fever they need to stop and isolate and call hcp to start abx and steroids (they are immunocompromised)
hyperthyroidism nc
identify, limit activity and demands, counsel fam and teachers (low stress), high cal and nutritious diet, meds (SE)
thyrotoxicosis
crisis or storm, can be life threatening
from sudden release of hormone, precipitated by infection, Sx, discont of antithyroid therapy
thyrotoxicosis tm
antithyroid drugs and propanolol
hypopituitarism - gh cm
absences or regression of secondary sex characteristics, normal h and w at birth and progressively deviate, distinguish from - constitutional delay, familial short stature, or genetic disorder (turners syndrome)
<3rd %, w normal-heavy, skeletal proportions normal, retarded bone age, appear younger, delayed perm teeth erupt, emotional issues because so small
hypopituitarism - gh tm
diagnose with history, assessment, x ray, low serum gh (stim test - q 30 min after insulin)
daily SQ GH injection until adult height (growth plate close around 14 or 18), may continue because of effect on bone and muscle, replace other hormones prn
very expensive and painful
hypopituitarism - gh nc
fam support - realistic expectation, response varies
body image
daily injection at bed, 5-7 times/wk
usually school aged
expensive
identify (plot on growth chart), assist with test, emotional support, teach, promote realistic expectation
precoscious puberty
before 9 or 8
precoscious puberty tm
treat cause, lupron (monthly IM) until they are supposed to start puberty, psych support
precoscious puberty - true/complete/central
lurpon
normal P, just happens early
precoscious puberty - premature therlarche
breast, dont treat, resolves on own
precoscious puberty - prematurue pubarche
hair, not a problem
precoscious puberty - premature menarche
period without other development
CAH cm
cortisol down, sex hormones up (androgens)
decreased stress response, hypogly, increased inflam response, hypotension, compromised immune, hyponatremic dehydration s/s
males: precoscious puberty
females: ambiguous genitalia - enlarged clit, fused labia, internal intact
diagnose with untralsound, chromosome
CAH tm
confirm diagnosis, cortisone to decreased ACTH (rest of life), reconstructive sx
CAH nc
parental anxiety, teach parents s/s of dehydration and Na loss crises - injectable hydrocortisone, genetic counseling
DM 1 cm
3Ps, weight decrease, enuresis (urinalysis), irritable, fatigue, abdominal pain
children, insulin dependent, autoimmune Beta cells, rarely overweight or fam history
DM 2 cm
overweight, fatigue, freq infection, acanthosis nigricans (skin folds)
adults, not insulin dependent, body cant produce and use insulin, overweight, fam hx
DM 2 tm
weight loss, exercise, oral meds, insulin
DM 1 tm - mnt
fingerstick, eat whatever, count CHO based on what they ate
avoid high sugar habits
fat increases bg later, dont restrict carbs
extra snack before activity, increase insulin with more food
DM 1 tm - insulin
rapid (15, 1, 3-4)
long acting (4-6, 8-24, all day)
intensive: basal/bolus - give based on fingerstick and how much they eat; pens, meters, sq ports changed q 7-10 days, extra batteries and back up kit, strips, alcohol, bandaids; fingerstick before meal and bedtime, count c post meal, sliding scale, combine, admin (rapid)
blood glucose monitor (4x - meal and bedtime) and feel or display s/s and before phys activity
DM 1 tm - urine ketones
> 240, not enough insulin, when ill, on pump + >240
DM 1 dev - toddler
differentiate misbehavior from hypogly, report funny feeling, expect food jags, give choices (injection site, food choices)
DM 1 dev - preschooler
reassure not punishment, encourage participation, teach to report lows, teach what to eat when low
DM 1 dev - schoolager
educate school personel, age appropriate independence (supervise!), extracuricular, injection
DM 1 dev - adolescence
self care, meal plan and adjust, injection, risk takers (sleep late, skip breakfast, OH decreases BG so need to eat), parental involvement and support, menstruation and eating disorder
DM 1 tm - sick day
test urine ketones
still give insulin and check more frequently
hypogly s/s
<60
low bg, hunger, HA, confusion, shaky, dizzy, sweat
check BS
hypogly tm
check, 15 g rule (1/2c juice or soft drink, 1c milk, glucose tab, cake icing, honey > 1 yr, NO CHOCOLATE), follow with meal/snack (protein)
severe - no swallow: glucagon emergency (expires), IM/SQ, on side bc v, feed when awake
hypergly s/s
> 180
high BG, glucose in urine, polyuria, increased thirst
hypergly tm
check ketones, call hcp, increase fluids w/o caffeine (FIRST), no activity
ketosis and acidosis s/s
ketosis: 0.3 - 7.0 mmol/L
acidosis when >7.0, pH <7.3
s/s: rapid breathing and deep, confusion, lethargy, abd pain
ketosis and acidosis tm
A/V BG, electrolytes, AG (low = low bicarb; high = met acidosis)
dka cm
deep and rapid breathing (kussmal, resp distress w/o lung patho), v dry mouth (dehyd with excessive UOP), , fruity breath, n/v (w/o d), lethargy, drowsy
cerebral edema, hypergly, hypovolemia
insulin deficiency with countereg to enhance gluconeogenesis, glycogenolysis, lipolysis
hyper/hypoK: K loss from shift to extra to exchange with excess H+ in extra, out in urine
dehyd: increased serum osmolarity, h2o shift extra, dilutional hypoNa - out in urine
dka tm
correct dehyd, acidosis and reverse ketosis, restore normal BG, avoid complications
DKA tm - fluid
NS at 10-20 mL/kg/hr, replace fluid deficit evenly ove 48 hr
confirm DKA with labs
DKA tm - insulin
separate IV, prime with insulin, not given in first hour bc r/o cerebral edema, d/c any bodily insulin pump
0.1 U/kg/hr, continue until acidosis clears
this will turn off ketone production, decrease blood sugar, check glucose q1hr
low dose to decrease hypogly or hypok, dont drop more than 50-100 mg/dL/hr (cerebral edema)
dont d/c based only on BG - pH >7.3 and HCO3 > 15 and serum ketones clear
before admin: neuro, VS, BG
continuous IV infusion, regular, high alert, dont give as bolus
once BG 250 - 300, maintain insulin and give dextrose (acidosis takes longer to fix)
DKA tm - K
hour 2 if urinated
start with insulin, consult pharm
monitor! usually significant K deficit
continue throughout IV therapy
max rate is institution specific
DKA tm - dextrose
maintain BG 150 - 250, prevent hypogly
add to IV when BG 250 - 300, change to 5% with .45 NaCl at rate to complete rehydration in 48 hr, check BG q1hr, electrolytes q2-4hr
SQ insulin after DKA resolves
DKA tm - bicarbonate
contraindicated in peds bc cerebreal edema (consult)
severe acidemia, life threatening hyperK
DKA nc - monitor q1
VS, neuro, I+O, poc BG, K
cardiac - continuous
DKA nc - monitor q2
urine ketones, serum beta OH, serum glucose, electrolytes, BUN, Ca, Mg, P, hct, ABG
DKA tm - extra
may need O2, airway, suction, cath (no urine)
need peripheral IV for sampling and insulin drip
altered mental or LOC: airway, NG suction, bladder cath
DKA tm - high risk
icu: <7.1 or 7.2 + young, altered LOC, <5 yr, increased risk of cerebral edema
prep for intubation
caution with meds that alter LOC
DKA tm - transition off IV insulin
pH > 7.3 and HCO3 > 15 -18, AG < 12, eat
SQ, d/c IV, IV dextrose, feed
known DM pt: prior dosing
new DM pt: .7-1 U/kg/day
