NUR331 Exam 3 Flashcards
phenylketonuria (PKU)
- inborn genetic error of metabolism where there is an absence of the enzyme phenylalanine hydroxylase (causes inability to metabolize the amino acid, phenylalanine)
- can build up and cause brain damage
- will ALWAYS be on a PA+ free formula
- limited fruits, vegetables, and grains, no meat
- must keep PA+ in a safe range of 2-6
CM of untreated PKU
- restricted height and weight
- musty odor to urine and sweat
- hypopigmentation
- vomiting
- irritability
- seizures
- hyperactivity
- mental delay
- adults have: short attention span, poor short term memory, visual perception problems, poor motor coordination, mood disorders, brain structural changes, etc
NC for PKU
- ensure proper testing as a newborn
- teaching
- referral for genetic counseling
- supportive
- flexible feeding schedule
- encourage early treatment
galactosemia
- inborn error of carb metabolism
- lack of enzyme needed to convert galactose to glucose
- CM - vomiting, weight loss, jaundice, lethargy, hypotonia, cataracts
- long term consequences - learning disabilities, lower IQ, short attention span, behavior problems
- lactose-free diet, soy formula
- calcium supplements, sensitive to medications
- don’t allow mom to breastfeed unless she is dairy free
juvenile hypothyroidism
- s/s - hypothermia, large protruding tongue, short and thick neck, delayed detention, hypotonia, poor feeding, prolonged jaundice, constipation, goiter
- englarged thyroid could obstruct airway
- levothyroxine for life, prompt treatment needed for brain growth in infants, dose changes with growth
- teach med administration - avoid heat exposure, do NOT mix with soy formula
hyperthyroidism
- mimics s/s of adults
- diagnosed based on high T3 and T4
- given anti thyroid drugs - most likely will receive remission in 1-2 years
- subtotal thyroidectomy or ablation with radioiodine
- high cal and nutritious diet, limit activity
agranulocytosis
toxic reaction of anti-thyroid drugs that causes sore throat and fever
- should isolate and call HCP
thyrotoxicosis
- thyroid storm
- can be life threatening
- can be precipitated by infection, surgery, or discontinuation of antithyroid therapy
- treated with antithyroid drugs and propanolol
hypopituitarism
- causes - tumors, encephalitis, radiation, head trauma, congenital, and idiopathic
- absence or regression of secondary sex characteristics
- normal height and weight at birth but patterns deviate
- diagnosed by ruling out short stature and Turner’s
- CM - short, normal to heavy weight, slow bone age, appear younger than chronological age, delayed growth of permanent teeth
- given growth hormone until reaching adult height, may need thyroid extract, cortisone, testosterone/estrogen (expensive and painful)
- injections are given at bedtime, requires frequent blood draws
precocious puberty
- sexual development before age 9 in boys or 8 in girls
- cause - disorder of gonads, adrenal glands, or HPA axis
- included are premature thelarche, premature pubarche, and premature menarche
- treat specific cause if known
- Lupron - monthly IM until puberty that slows growth
congenital adrenal hyperplasia
- decreased enzyme activity that is required for cortisol production in the adrenal cortex
- overproduction of adrenal androgens results in virilization of female fetus
- decreased cortisol and aldosterone
- CM - ambiguous genitalia, decreased stress response, hypoglycemia, increased inflammatory response, hypotension, compromised immune system, dehydration
- ultrasound to determine organs, chromosome typing for positive sex determination
- given cortisone to suppress abnormally high secretion of ACTH
- teach parents of s/s of salt-losing crisis and dehydration
diagnostic criteria of diabetes
- FPG >126
- PG >200
- casual >200 with 3 p’s
CM of DM1
- hyperglycemia and acidosis
- weight loss
- 3 p’s
- enuresis
- irritability
- unusual fatigue
- abdominal pain
CM DM2
- overweight
- fatigue
- frequent infections
- acanthosis nigrocans
diabetic medical management
- nutrition
- medication
- developmental issues
- glucose and urine monitoring
- hypoglycemia management
- hyperglycemia management
- sick day management
snack management with DM
- balance of carbs, fats, and proteins
- extra food during exercise
- consistent meal times
- avoid high sugar
- decrease insulin with exercise
- eat snacks when drinking alcohol
carb-free sources
meat, jello, cheese
onset, peak, duration of insulin lispro
- 15min
- 1 hr
- 3-4 hr
onset, peak, duration of insulin glargine
- 4-6 hours
- 8-20 hrs
- 24 hours
When should urine ketones be monitored?
- any time glucose is 240+
- during illness
- when on an insulin pump and sugar is 240+
What is bolus insulin matched with?
carb intake
correction is everything above the desired level
developmental issues with 3-6years DM
- reassure child who thinks it’s punishment
- encourage child to participate
- teach child to report lows
- teach child what to eat when they get low
developmental issues with 6-12 years DM
- educate school personel about DM
- encourage independence, but all activities must be supervised
- encourage extra curricular activities
adolescent DM developmental issues
- know which foods fit into meal plan and how to adjust
- they like to take risks
- need continued parental support