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