Nur 311 Exam 2 Study Guide/Practice
Questions
PKU diet to follow ANS; Breast Feeding or PA+ Free Formula. Restricted PA + Intake Throughout Life; No
meat, limited fruits & vegetables, limited grains
What happens if people with PKU don't follow their diet ANS; Short Attention Span
Poor short term memory
Visual perception problems
Poor motor coordination
Mood disorders
Brain structural changes (reduced grey matter)
Safe PKU level ANS; 2-6mg/dl
PKU nursing Considerations ANS; Assist with testing
Teach parents about diet and need for follow up blood levels throughout life
Child may have trouble adhering to diet as ages
Provide emotional support
Genetic counseling
What kind of diet should a child with Galactosemia Follow ANS; lactose free diet; soy formula
Calcium supplementation.
Dark green leafy green veggies are good
Can galactosemia breastfeed? ANS; NO!!! AVOID!!! Need soy formula and calcium supplementation
,Long-term consequences if diet not followed for galactosemia ANS; learning disabilities, lower IQs, short
attention span, behavior problems
Clinical Manifestations of galactosemia ANS; vomiting, weight loss, jaundice (hepatomegaly), lethargy,
hypotonia, cataracts (galactose accumulation)
When should growth hormone be given? ANS; NIGHT TIME!!! Sub Q injections given @ night 5x a week.
Is stopped once when child is where they are supposed to be height wise.
Realistic expectations for Growth hormone ANS; Growth hormone will only make them grow to what
they were supposed to grow. So giving it to them won't make them a 7'0 Center for UK.
Nursing considerations for Growth Hormone therapy ANS; serial blood draws q 30 minutes for 3-hours
following stimulation with insulin or glycogen.
Treat with chronological age not developmental age
Incomplete puberty ANS; Premature thelarche
Premature pubarche
What is considered True or complete precocious puberty ANS; When Puberty HAPPENS EARLY!!!
What drug is for Precocious puberty ANS; Lupron. Slows prepubertal growth to normal rates
Given IM monthly
When to Stop Lupron Treatment ANS; Treatment is discontinued at age for normal pubertal changes to
resume
,Precocious Puberty ANS; a condition in which puberty begins before the age of 8 in girls and 9 in boys.
Testicular enlargement in boys and breast enlargement in girls 1st sign
What drug is used for Treatment of CAH ANS; Cortisone to suppress abnormally high secretion of ACTH
for LIFE. Reconstructive surgery as required.
What is something a person with CAH should always be wearing? ANS; Medical alert Bracelet
Detection of CAH in males ANS; precocious genital development
Detection of CAH in females ANS; -Enlarged clitoris appears as a small phallus
-Fused labia produce saclike structure without testes
-Internal female sexual organs are intact
Diagnostic Evaluation for CAH ANS; -Ultrasonography to visualize pelvic organs
-Chromosome typing for positive sex determination and to rule out any other genetic anomalies
Teaching for CAH ANS; Teach parents signs and symptoms of dehydration and salt-losing crises. Have
injectable hydrocortisone available and teach how to use it. MONITOR FOR HYPONATREMIC
DEHYDRATION!!!
Nursing considerations for CAH ANS; Hyponatremic dehydration for CAH most at risk, sodium less than
135, blood sugar drops, do an ultra sound for gender, genetics for CAH status, always have to have
cortisol replacement.
what medication is for hypothyroidism ANS; Levothyroxine (Synthroid) ANS; take in the AM. Needs to
be crushed but can not be given in soy based formula. Keep out of sun. May administer in increasing
amounts over 4 to 8 weeks to reach euthyroidism. Too much will lead to hyperthyroidism
, Goiter ANS; Hypertrophy of the thyroid gland. Occurs with hypothyroidism. Congenital cause harms the
airway of newborn and results from maternal ingestion of antithyroid drugs during pregnancy.
Juvenile Hypothyroidism ANS; Congenital hypothyroidism is the major cause of childhood mental
retardation. Everything Slows down!!!
problems with miss diagnosis for hypothyroidism ANS; Missed or delayed diagnosis may lead to mental
defects
Signs & Symptoms Hypothyroidism (congenital) ANS; EVERYTHING SLOWS DOWN!!!
Hypothermia
Large protruding tongue
Short, thick neck,
Delayed dentition
Hypotonia
Poor feeding
Prolonged jaundice
Constipation
Clinical Manifestations Hypothyroidism (acquired) ANS; EVERYTHING SLOWS DOWN!!!
Decreased growth
Myxedema (dry skin, puffiness around eyes, sparse hair)
Sleepiness
Goiter
Altered body proportions; short stature, legs proportionately short compared to trunk
Hyperthyroidism (Graves Disease) ANS; Most common in females 12-14
Questions
PKU diet to follow ANS; Breast Feeding or PA+ Free Formula. Restricted PA + Intake Throughout Life; No
meat, limited fruits & vegetables, limited grains
What happens if people with PKU don't follow their diet ANS; Short Attention Span
Poor short term memory
Visual perception problems
Poor motor coordination
Mood disorders
Brain structural changes (reduced grey matter)
Safe PKU level ANS; 2-6mg/dl
PKU nursing Considerations ANS; Assist with testing
Teach parents about diet and need for follow up blood levels throughout life
Child may have trouble adhering to diet as ages
Provide emotional support
Genetic counseling
What kind of diet should a child with Galactosemia Follow ANS; lactose free diet; soy formula
Calcium supplementation.
Dark green leafy green veggies are good
Can galactosemia breastfeed? ANS; NO!!! AVOID!!! Need soy formula and calcium supplementation
,Long-term consequences if diet not followed for galactosemia ANS; learning disabilities, lower IQs, short
attention span, behavior problems
Clinical Manifestations of galactosemia ANS; vomiting, weight loss, jaundice (hepatomegaly), lethargy,
hypotonia, cataracts (galactose accumulation)
When should growth hormone be given? ANS; NIGHT TIME!!! Sub Q injections given @ night 5x a week.
Is stopped once when child is where they are supposed to be height wise.
Realistic expectations for Growth hormone ANS; Growth hormone will only make them grow to what
they were supposed to grow. So giving it to them won't make them a 7'0 Center for UK.
Nursing considerations for Growth Hormone therapy ANS; serial blood draws q 30 minutes for 3-hours
following stimulation with insulin or glycogen.
Treat with chronological age not developmental age
Incomplete puberty ANS; Premature thelarche
Premature pubarche
What is considered True or complete precocious puberty ANS; When Puberty HAPPENS EARLY!!!
What drug is for Precocious puberty ANS; Lupron. Slows prepubertal growth to normal rates
Given IM monthly
When to Stop Lupron Treatment ANS; Treatment is discontinued at age for normal pubertal changes to
resume
,Precocious Puberty ANS; a condition in which puberty begins before the age of 8 in girls and 9 in boys.
Testicular enlargement in boys and breast enlargement in girls 1st sign
What drug is used for Treatment of CAH ANS; Cortisone to suppress abnormally high secretion of ACTH
for LIFE. Reconstructive surgery as required.
What is something a person with CAH should always be wearing? ANS; Medical alert Bracelet
Detection of CAH in males ANS; precocious genital development
Detection of CAH in females ANS; -Enlarged clitoris appears as a small phallus
-Fused labia produce saclike structure without testes
-Internal female sexual organs are intact
Diagnostic Evaluation for CAH ANS; -Ultrasonography to visualize pelvic organs
-Chromosome typing for positive sex determination and to rule out any other genetic anomalies
Teaching for CAH ANS; Teach parents signs and symptoms of dehydration and salt-losing crises. Have
injectable hydrocortisone available and teach how to use it. MONITOR FOR HYPONATREMIC
DEHYDRATION!!!
Nursing considerations for CAH ANS; Hyponatremic dehydration for CAH most at risk, sodium less than
135, blood sugar drops, do an ultra sound for gender, genetics for CAH status, always have to have
cortisol replacement.
what medication is for hypothyroidism ANS; Levothyroxine (Synthroid) ANS; take in the AM. Needs to
be crushed but can not be given in soy based formula. Keep out of sun. May administer in increasing
amounts over 4 to 8 weeks to reach euthyroidism. Too much will lead to hyperthyroidism
, Goiter ANS; Hypertrophy of the thyroid gland. Occurs with hypothyroidism. Congenital cause harms the
airway of newborn and results from maternal ingestion of antithyroid drugs during pregnancy.
Juvenile Hypothyroidism ANS; Congenital hypothyroidism is the major cause of childhood mental
retardation. Everything Slows down!!!
problems with miss diagnosis for hypothyroidism ANS; Missed or delayed diagnosis may lead to mental
defects
Signs & Symptoms Hypothyroidism (congenital) ANS; EVERYTHING SLOWS DOWN!!!
Hypothermia
Large protruding tongue
Short, thick neck,
Delayed dentition
Hypotonia
Poor feeding
Prolonged jaundice
Constipation
Clinical Manifestations Hypothyroidism (acquired) ANS; EVERYTHING SLOWS DOWN!!!
Decreased growth
Myxedema (dry skin, puffiness around eyes, sparse hair)
Sleepiness
Goiter
Altered body proportions; short stature, legs proportionately short compared to trunk
Hyperthyroidism (Graves Disease) ANS; Most common in females 12-14
