Term
What values are charted on a growth chart? |
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Definition
infants= length, weight, and head circumference children over 2= hieght, weightand BMI |
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Term
What components of the psychosocial history should you ask teenagers? |
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Definition
home life, education, employment, activities, drugs, depression, safety (seat belts, guns in the home, abuse), sexual activity |
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Term
How should you examine a pt whom you suspect has epiglotitis? |
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Definition
don't inspect the pharynx, leave the pt in the parents arm during the physical exam |
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Term
What's the difference between rectal and oral temps? |
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Definition
rectal is the gold standard; oral is generally 1 degree below rectal |
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Term
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Definition
anterior fontanelle, open, soft, and flat |
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Term
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Definition
cyanosis, clubbing, edema |
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Term
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Definition
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Definition
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Term
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Definition
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Definition
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Definition
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
normocephalic, atraumatic |
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
usual state of good health |
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Term
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Definition
well developed, well nourished |
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Term
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Definition
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Term
How should you maneuver the auricle if you want to see the tympanic membrane? |
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Definition
in an infant, pull the auricle backward and downward; in an older child, pull backward and upward |
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Term
What is the average heart rate of a newborn? |
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Definition
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Term
Why is it important to check femoral pulses in an infant? |
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Definition
to exclude coarctation of the aorta |
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Term
What percent of infants have innocent murmurs? |
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Definition
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Term
What is a normal heart rate in a child? |
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Definition
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Term
What are characteristics of an innocent murmur? |
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Definition
low intensity (I-II/VI), occurence in systole, variation with position and respiration, and a musical quality |
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Term
What is a normal respiratory rate for a newborn? |
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Definition
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Term
What is a normal respiratory rate in a child? |
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Definition
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Term
How is infant breaths different from adult breaths? |
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Definition
expiration is more prolonged in infants; respiratory movements are produced by abdomenal movements |
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Term
Describe the Barlow maneuver. |
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Definition
stabilize the pelvis with one hand, and then flex and adduct the opposite hip and apply gental posterior pressure on the thigh |
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Term
What is the name of the maneuver used to dislocate the hip in developmental hip dysplasia? |
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Definition
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Term
What is the maneuver used to reduce a hip dislocation of developmental hip dislocation? |
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Definition
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Term
Describe the ortolani maneuver? |
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Definition
place one finger on the greater trochanter and one on the inner thigh, flex and abduct thehip and lift the femoral head anteriorly, feeling for a clunk as it relocates into the acetabulum |
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Term
When is a pediatrician called to a birth? |
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Definition
multiple gestations, preterm deliveries, cesarean sections, possible meconium aspirations, and known fetal anomalies |
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Term
What should be done to a baby who is born? |
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Definition
dried off, nose and mouth are suctioned, and the baby is rubbed and stimulated and placed under a warmer; apgar scores are assessed at one and five minutes and baby recieves an initial head to toe evaluation |
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Term
At what age do children get heel sticks? |
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Definition
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Term
Most neonatal reflexes disapear by what age? |
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Definition
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Term
What is the significance of persistent primitive reflex? |
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Definition
those infants are at greater risks for developmental disability |
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Term
At what age should children be crawling? |
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Definition
crawling is not a developmental milestone because some children skip this step |
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Term
What is a premature baby? |
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Definition
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Term
How long do you correct for prematurity for? |
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Definition
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Term
What is the definition of failure to thrive? |
|
Definition
weight below third percentile for age, weight less than 80% of ideal, or falling off the growth curve |
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Term
What are the traditional two categories of causes of FTT? |
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Definition
medical (organic) and psychosocial (nonorganic) |
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Term
What are the gross motor developmental milestones? |
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Definition
1 month=lifts head when prone; 4 months= rolls front to back; 5 months= rolls back to front; 6 month= sits upright; 12 months= walks |
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Term
What are the fine motor milestones? |
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Definition
birth= visually fixes; 1 month= tracks to midline; 2 months= tracks past midline; 6= transfers objects; 12= two finger pincer grasp |
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Term
What are the language developmental milestones? |
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Definition
1 month= alerts to sound; 3 months= coos; 4 months= orients to voice; 6 months= babbles; 9 months= mama, dada-nonspecific; 12 months= mama, dada-specific; 15 months= uses 4 to 6 words |
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Term
What are the social milestones? |
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Definition
2 months= smile; 4 months= laughs; 6 months= stranger anxiety; 12 months= imitates actions and comes when called; 18 months= may start toliet training |
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Term
What is the timing of the moro startle? |
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Definition
present at birth; disappears by 3 to 6 months |
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Term
Describe the moro startle. |
|
Definition
head extension leads to extension, adduction, and then abduction of the upper extremities |
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Term
Name some neonatal reflexes. |
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Definition
moro startle, palmar grasp, rooting, stepping, asymmetric tonic neck, parachute, galant, and babinski |
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Term
What is the timing of the stepping reflex? |
|
Definition
present at birth; disappears by 2 to 4 months |
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Term
Describe the stepping reflex? |
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Definition
infants move thier legs in a walking movement when they are held upright and leaning forward |
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Term
When does the asymmetric tonic neck reflex disappear? |
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Definition
turning the head while leads to ipsilateral extremity extension and contralateral flexion (fencer position) |
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Term
What is the parachute reflex? |
|
Definition
infants extend the ipsilateral arm to support the body when tilted to one side while sitting |
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Term
When does the galant reflex disapear? |
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Definition
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Term
Describe the galant reflex? |
|
Definition
stroking theparavertebral region while the infant is prone causes the pelvis to move toward the stimulated side |
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Term
Describe a brief differential of FTT. |
|
Definition
GI= pyloric stenosis, duodenal atresia, malabsorption, celiacs infection= HIV, TB, intestinal parasites chronic disease= CF, BPD, CHD reduced growth potential= congenital syndromes, skeletal dysplasias |
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Term
What is "falling off the growth curve"? |
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Definition
crossing down two major percentile lines on a growth chart |
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Term
How should you workup failure to thrive? |
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Definition
assess psychosocial issues; CBC, electrolytes, creatinine, albumin, protein, UA, and urine culture; if GI symptoms= stool guaiac, culture and O&P; sweat chloride test, assessment of bone age, malabsorption tests (stool pH and reducing substances) |
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Term
In failure to thrive, in what order to kids fall of their growth charts? |
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Definition
weight first, then height, then head circumference |
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Term
What much do babies grow in the first year? |
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Definition
triple in weight and double in length |
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Term
What is the average birth weight? |
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Definition
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Term
How much weight should neonates gain in the first few days of life? |
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Definition
generally they loose 5-10% of their body weight over the first few days; formula fed babies return to BW by the second week of life, whereas breast-fed babies should return to BW by the third week of life |
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Term
How much weight should babies gain per day? |
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Definition
30 g/day for 3-4 months; 10-20 g/day for 4-12 months |
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Term
At what point should the birth weight double? triple? quadruple? |
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Definition
doubles at 4-5 months; triples by one year; and quadruples by two years |
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Term
How much weight should a child gain on average per year? |
|
Definition
5 lbs per year from age 2 to puberty |
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Term
What is the average birth length? |
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Definition
50 centimeters (20 inches) |
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Term
What is the average height of a one year old? |
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Definition
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Term
What is the average height of a three year old? |
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Definition
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Term
What is the average height of a four year old? |
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Definition
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Term
When do children triple their birth height? |
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Definition
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Term
How much do children grow per yea? |
|
Definition
2-3 inches per year from age four to puberty |
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Term
What is the average birth head circumference? |
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Definition
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|
Term
At what rate should the head circumference enlarge in the first year? |
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Definition
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Term
What percent of head growth has occurred by age 2? |
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Definition
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Term
What are the contraindications to all types of vaccination? |
|
Definition
current moderate to severe acute illness; severe allergy to a vaccine component |
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Term
What are the contraindications to influenza vaccine? |
|
Definition
anaphylactic reaction to eggs, current moderate to severe acute illness |
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Term
What are the CI to MMR vaccination? |
|
Definition
anaphylactic reaction to gelatin or neomycin (perform prior skin testing); current moderate to severe acute illness; preganancy, immune compromise, or use of high dose steroids |
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Term
What are the contraindications to IPV vaccination? |
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Definition
current moderate to severe acute illness; anaphylactic reaction to neomycin, polymyxin B, or streptomycin (perform prior skin testing); |
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Term
What are the contraindications to DTaP vaccination? |
|
Definition
concurrent moderate to severe acute illness; encephalopathy within seven days of prior pertussis vaccination (use DT instead of DTaP) |
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Term
What are the contraindications for oral polio vaccination? |
|
Definition
current moderate to severe acute illness; pregnancy, immune compromise, high dose steroids |
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Term
What are the CIs for varicella?? |
|
Definition
current moderate to severe acute illness; pregnancy, immune compromise or use of high dose steroids |
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Term
What kind of reactions to pertussis vaccines necessitate the use of precaution in administering future vaccines? |
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Definition
fever greater than 40.5 (105F), shocklike state, persistent crying for > 3 hrs within the first 48 hours of vaccination, or seizure within three days of vaccination |
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Term
What vaccine is administered at birth? |
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Definition
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Term
What vaccine is administered at 2 months? |
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Definition
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Term
What vaccines should be administered at 4 months? |
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Definition
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Term
What vaccines should be administered at 6 months? |
|
Definition
HBV, DTaP, Hib, IPV, influenza, PCV |
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Term
What vaccines should be administered at 12-15 months? |
|
Definition
Hib, influenza, PCV, MMR, varicella, HAV |
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Term
What are the 15-18 month shots? |
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Definition
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Term
What are the two year shots? |
|
Definition
influenza, HAV, meningococcal |
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Term
What are the 4-6 yoa shots? |
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Definition
DTaP, IPV, influenza, MMR, varicella |
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Term
What are the 11 to 12 yoa shots? |
|
Definition
DTaP, HPV, MCV4, PPV, influenza |
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Term
What are the 13 to 14 yoa shots? |
|
Definition
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|
Term
What are the 15 yoa shots? |
|
Definition
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|
Term
What vaccinations are needed at 16 to 18 yoa? |
|
Definition
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|
Term
At what ages are vaccinations needed? |
|
Definition
birth, 2 months, 4 months, 6 months, 12-15 months, 2 years, 4-6 years, 11-12 years, 13-14 years, 15 years, 16-18 years |
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Term
At what age can a child first recieve the flu vaccine? |
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Definition
6 months for the trivalent inactivated influenza vaccine and five years for the live attenuated vaccine; children less than 9 who are receiving the influenza vaccine for the first time should recieve two doses, separated by > 4 weeks for inactivated and > 6 weeks for live attenuated vaccine |
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Term
How far apart should the HAV vaccine be administered? |
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Definition
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Term
What is teh dosing of HPV vaccine? |
|
Definition
first dose to females 11 to 12 yoa; second dose two months after the first and third dose 6 months after the first dose |
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Term
How long should women breast feed? |
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Definition
first six months of life infant should be exclusively breast fed; continuation of breast feeding plus formula/food should be continued for optimal infant nutrition |
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Term
After 6 months of breast feeding why might it be advantageous to start adding supplemental diet? |
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Definition
because the maternal stores of iron are often depleted by that time and infants may require flouride and iron supplementation |
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Term
What are the benefits of breast feeding? |
|
Definition
faster mother-infant bonding, decrased risk of eczema and cow's milk protein allergy; decreased risk of serious infections due to the presence of maternal IgA antibodies; decreased risk of breast and ovarian cancer in the mom and faster return to prepregnancy weight |
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Term
When is breast feeding NOT recommended? |
|
Definition
mother has been infected with HIV or is taking antiretrovirals, mother has active, untreated TB, mother is infected with HTLV I or II, using or dependent on illicit drugs, taking chemo or undergoing radiation |
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Term
All breast fed infants should recieve what supplementation? |
|
Definition
vitamin D supplementaiton (200 IU) to prevent rickets |
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Term
What is formula made up of? |
|
Definition
can be soy or cow's milk based; with long chain polyunsaturated fatty acids to promote growth, neurodevelopment and visual acuity; can be supplemented with iron |
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Term
|
Definition
in the first two months of life babies will eat 2-3 ounces (approximately 10-20 minutes breast feeding) every 2-3 hours |
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Term
When should new foods be introduced to babies? |
|
Definition
after six months of age at a rate of one per week to allow for the identification of potential allergies |
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Term
What food should never be fed to infants under 1 yoa? |
|
Definition
honey; also foods that may lead to choking like nuts, raisens, and hot dogs |
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Term
What foods should be avoided in an infant/toddlers diet to avoid food allergies? |
|
Definition
avoid cow's milk until 1 yoa, eggs until 2 yoa, and peanuts, tree nuts, and fish until 3 yoa |
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|
Term
What is the normal progression of puberty in males? |
|
Definition
testicular enlargement --> penile enlargement --> growth spurt --> pubic hair |
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Term
What is the progression of puberty in females? |
|
Definition
thlarche --> growth spurt --> pubic hair --> menarche |
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Term
What is considered precocious puberty? |
|
Definition
2ndary sexual chars in males less than 9, black girls less than 6, white girls less than 7 |
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Term
At 4 to 6 months, a baby should be eating... |
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Definition
iron-fortified single-grain cereal; supplemental water |
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|
Term
At 6 to 7 months, an infants should be eating... |
|
Definition
strained fruit, consider flouride supplementation (depends on local water supply) |
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|
Term
At seven to eight months you should introduce what into a childs diet? |
|
Definition
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|
Term
When can you introduce well chopped meats into an infants diet? |
|
Definition
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|
Term
When can you add cheese, egg yolk, and protein rich foods into a childs diet? |
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Definition
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|
Term
When should children starteating soft finger foods? |
|
Definition
|
|
Term
|
Definition
males= childhood size external genitalia; females= preadolescent; elevation of papilla only; no pubic hair |
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|
Term
|
Definition
males= enlargement of testes and scrotum, females= breast buds, elevation of breast and papilla; pubic hair= spares, straight downy hair on labia/penile base |
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|
Term
What is tanner stage III? |
|
Definition
males= enlargement of the penis mainly in length; females= enlargement of the breast and areola, single contour; pubic hair= darker, coarse, curled hair |
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|
Term
|
Definition
male= continued penile enlargement, especially in breadth, scrotal skin darkens, rugations are present; females= projection of the aerola and papilla; separate contour (the secondary mound); pubic hair is adult type but limited to the genital area |
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|
Term
|
Definition
male genitalia is adult sie and shape; female breasts are mature (areola recesses and nipple projects); pubic hair is adult quality and distribution (spread to thighs) |
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|
Term
Why is it important not to leave formula bottles in the crib overnight? |
|
Definition
can cause milk bottle tooth caries |
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|
Term
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Definition
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|
Term
|
Definition
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|
Term
What is idiopathic early puberty called? in which gender is it more common? |
|
Definition
true central (gonadotropin-dependent) precocious puberty; more common in females |
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|
Term
What is the most common cause of gonadotropin dependent precocious puberty? |
|
Definition
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|
Term
How do you workup a patient with precocious puberty secondary to elevated gonadotropin? |
|
Definition
although this is most commonly idiopathic it is important to get a CT/MRI to rule out CNS pathology as a potentially treatable cause |
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|
Term
What is the incidence of congenital heart diesease? |
|
Definition
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|
Term
What determines whether a cardiac defect is a cyanotic heart defect or an acyanotic heart defect? |
|
Definition
cyanotic has right to left shunt; acyanotic is a left to right shunt |
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|
Term
How do you workup a congenital heart disease? |
|
Definition
CXR, ECG and an echo; sometimes angiography |
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|
Term
Name some more common acyanotic heart diseases? |
|
Definition
PDA, ASD, VSD, and coarctation of the aorta |
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|
Term
What are the symptoms of acyanotic congenital heart conditions? |
|
Definition
lesions are often asymptomatic in early childhood but as fetal circulation transitions into adult circulation over the first several weeks of life, left to right shunting increases and symptoms of CHF develop, usually between one and three months; large ASD or VSD may lead to Eisenmenger's dynrome |
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|
Term
What is eisenmenger's syndrome? |
|
Definition
when longstanding acyanotic congenital heart conditions eventually result in shunt reversal and pulmonary hypertension |
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|
Term
What are the major causes of cyanotic heart disease? |
|
Definition
hypoplastic left heart syndrome, truncus arteriosus, transposition of the great vessels, tricuspid atresia, tetralogy of fallot, total anomalous pulmonary venous return |
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|
Term
How do cyanotic congenital heart conditions present? |
|
Definition
typically in the first week of life because they have been dependent on the ductus arteriosus, which typically closes within the first week of life |
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|
Term
What determines the presentation of tetrology of fallot? |
|
Definition
the severity of the pulmonary artery stenosis |
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|
Term
What medication is given to most patients with cyanotic congenital heart conditions? |
|
Definition
PGE1 to maintain a patent PDA |
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|
Term
What are diaper rashes commonly secondarily infected with? |
|
Definition
candida albicans; 80% of diaper rashes lasting more than 4 days is colonized with candida |
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|
Term
What does a diaper rash due to irritant dermatitis look like? |
|
Definition
ill-defined erythematous patches or plaques, often with scaling; usually spare the inguinal folds |
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|
Term
What does a candidal infection look like? |
|
Definition
well-demarcated, beefy-red erytematous patches surrounded by satellite erythematous papules or pustules; can be found within the inguinal folds |
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|
Term
How do you diagnose candida infection of a diaper rash? |
|
Definition
usually clinically but you can scrape a satellite lesion, stain with 10% KOH, and observe psuedohyphae under the microscope |
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Term
How do you treat diaper rash? |
|
Definition
keep diaper area clean and dry; use barier cream in moist areas; treat candidal infection with topical antifungal agents (nystatin is first line treatment for infants, but clotrimazole is an alternative for older children); severe candidal infections can be treated with low potency topical steroid (e.g. 1% hydrocortisone cream) in conjunction with topical antifungals |
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|
Term
What is the general pathophys of Reye's syndrome? |
|
Definition
aspirin causes mitochondrial dysfunction characterized by acute severe encephalopathy along with degenerative liver disease |
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|
Term
What is the most common cause of bowel obstruction in the first two years of life? |
|
Definition
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|
Term
In what gender is intussusception more common? |
|
Definition
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|
Term
What is the most common location for intussusception? |
|
Definition
when one portion of the bowel telescopes into an adjacent portion; usually proximal to the ileocecal valve |
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|
Term
What are the risk factors for intussusception? |
|
Definition
polyps, meckel's diverticulum, adenovirus or rotavirus infection, henoch-shonlein purpura, intestinal lymphoma, celiac disease and CF |
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|
Term
Whatis the classic triad of intussuception? |
|
Definition
intermittent colicky abdominal pain, vomiting, and bloody mucous stools= found in only one third of patients but most will at least have one of the signs |
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|
Term
What signs of intussuception are ominous? |
|
Definition
red "currant jelly" stools, lethargy and fever |
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|
Term
What should you think if you feel a "sausage-shaped" abdominal mass on palpation? |
|
Definition
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|
Term
How do you workup/treat intussusception? |
|
Definition
assess and correct any volume or electrolyte abnormalities; AXR/US to establish diagnosis in equivocal cases; air contrast or barium enema is both diagnostic and therapeutic but DO NOT do them if there are peritoneal signs |
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|
Term
What should you do if a child has signs of intussusception and peritoneal signs? |
|
Definition
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|
Term
What percent of intussusceptions are cured by barium/air enemas? |
|
Definition
|
|
Term
What virus causes rubeola (measles)? |
|
Definition
|
|
Term
What are the symptoms of measles (rubeola)? |
|
Definition
cough, coryza, conjunctivitis for 2 to 3 days; koplike spots that resolve before the appearance of the rash; erythematous maculopapular rash that beings on the head and spreads to the body and fades in the same pattern; high fever is present |
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|
Term
What are the complications associated with rubeola (measles)? |
|
Definition
otitis media is very common, encephalitis, pneumonia, subacute sclerosing panencephalitis (rare) |
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|
Term
What are the three day measles? |
|
Definition
german measles or rubella |
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|
Term
What virus causes rubella? |
|
Definition
|
|
Term
What re the symptoms of rubella? |
|
Definition
prodrome of malaise followed by posterior cervical and suboccipital LAD (adolescents may developtransient polyarthralgias); maculopapular rash that begins on the face and then generalizes resolving in 3-5 days; fever on the first day of the rash only; petechieae on the palate (forschheimer spots) |
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|
Term
What are petechiae on the palate called/associated with? |
|
Definition
forschheimer spots= associated with rubella |
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|
Term
What are the complications associated with rubella? |
|
Definition
devastating results if a fetus is infected during gestation; rarely encephalitis and thrombocytopenia |
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|
Term
What diseases are caused by HHV-6 and HHV-7? |
|
Definition
roseola infantum (exanthem subitum) |
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|
Term
What are teh symptoms of roseola infantum (exanthem subitum)? |
|
Definition
abrupt onset of high temperature (more than 40 C or 104F) for 3 to 5 days but the child does not feel ill; as teh fever drops a maculopapular rash appears on the trunk, spread peripherally and resolvesin 24 hours |
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|
Term
What are the complications associated with roseola infantum (exanthem subitum)? |
|
Definition
rapid temperature increases associated with febrile seizures |
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|
Term
How is varicella transmitted? |
|
Definition
hightlycontagious via respiratory droplets and contactwith lesions until crusted over |
|
|
Term
What are the symptoms of varicella? |
|
Definition
prodrome of fever and malaise for one day; pruritic tear drop vesicles on an erythematous base that start on the face and trunk and spread peripheraly; lesions break and crust over in one week with different stages of healing |
|
|
Term
What are the complications associated with varicella? |
|
Definition
secondary skin infection is most common but in immunocompromised children or neonates; rarely, fatal disseminated disease may occur= meningoencphalitis, hepatitis, pneumonitis |
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|
Term
What exanthem is caused by parvovirus B19? |
|
Definition
erythema infectiosum (fifth disease) |
|
|
Term
How is erythema infectiosum (fifth disease) transmitted? |
|
Definition
epidemics occur in the spring; contagious via respiratory secretions |
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|
Term
What are the symptoms of erythema infectiosum? |
|
Definition
mild flu like illness for 7 to 10 days (but a lot of times kids don't have this) then slapped cheek rash with circumoral pallor; then an erythematous maculopapular rash spreads to the trunk and legs in a lacy-reticular pattern (usuallyu lasts 2 to 3 weeks); low grade or no fever |
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|
Term
What are the complication sof erythema infectiosum? |
|
Definition
aplastic crisis (sickle cell disease and other anemias) fetal anema/hydrops fetalis (in utero infection), arthritis, encephalopathy |
|
|
Term
What is the virus that causes hand, foot and mouth disease? |
|
Definition
|
|
Term
How is hand, foot and mouth disease transmitted? |
|
Definition
contagious by direct contact |
|
|
Term
What are the symptoms of hand, foot and mouth disease? |
|
Definition
fever, anorexia and oral pain= prodrome; followed by football-shaped" vesicles with surrounding erythema on the hands and feet and oral ulcerations that resolves in one week; fever present |
|
|
Term
What should also be in your differential if you suspect intususception? |
|
Definition
constipation, GI infection Meckels diverticulum, lymhpoma in children >6 yoa, meconium ileus in neonates |
|
|
Term
What is the male to female ratio of pyloric stenosis? |
|
Definition
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|
Term
What is the incidence of pyloric stenosis? |
|
Definition
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|
Term
When does pyloric stenosis present? |
|
Definition
first 2 to 8 weeks of life |
|
|
Term
What are teh findings on physical exam for pyloric stenosis? |
|
Definition
olive shaped mass in the peigastric area; gastric peristalsis may be visible |
|
|
Term
What complication is common with pyloric stenosis? |
|
Definition
hypochloremic, hypokalemic metabolic alkylosis with dehydration secondary to persistent emesis |
|
|
Term
What is the differential diagnosis for pyloric stenosis? |
|
Definition
pylorospasm, overfeeding, gastroenteritis, hiatal hernia, duodenal atresia, esophageal stenosis, malrotation/volvulus, incarcerated hernias, meconium ileus, milk protein allergy, GERD |
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|
Term
What antibiotic is associated with pyloric stenosis? |
|
Definition
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|
Term
How do you treat pyloric stenosis? |
|
Definition
NG tube placement; correction of dehydration and electrolyte abnormalities; surgical pyloromyotomy |
|
|
Term
What are the associated diseases/problems with Down syndrome? |
|
Definition
cardiac defects (endocardial cushion/septal defects); increased incidence of ALL, alzheimer's disease seen in adulthood; GI anomalies (duodenal atresia, hirschspurng's disease) |
|
|
Term
What is the incidence of Down syndrome? |
|
Definition
|
|
Term
What causes down syndrome? |
|
Definition
95% are due to maternal nondysjunction; 4% due to unbalanced translocation |
|
|
Term
What is the life expectancy of Down syndrome? |
|
Definition
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|
Term
What are the features/associated anomalies of Edwards' syndrome? |
|
Definition
severe mental/grwoth retardation, prominent occiput, rocker-bottom feet, low-set ears, horseshoe kidney |
|
|
Term
What are the features/associated symptoms of Patau's syndrome? |
|
Definition
severe mental/growth retardation, scalp defects, microencephaly, polycactyly, agenesis of corpus callosum |
|
|
Term
What is the incidence and male:female of Edward's syndrome? |
|
Definition
incidence of 1 in 300; male to female ratio of 1:3 |
|
|
Term
What is the incidence and male: female of Patau's syndrome? |
|
Definition
incidence of 1 in 5000; 2:3 |
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|
Term
What is the most common cause of hospital admissions for sickle cell patients? 2nd most common? |
|
Definition
vaso-occlusive or pain crisis; acute chest syndrome |
|
|
Term
Where do vaso-occlusive crises in sickle cell patients occur? |
|
Definition
hand-foot syndrome (dactylitis), priaprism, avascular necrosis of the femoral head; pain crises typically occur in bones |
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|
Term
What is the definition of acute chest syndrome? |
|
Definition
in sickle cell patients; radiologic appearance of new pulmonary infiltrate and fever; hypoxia may be present but is not required for diagnosis; etiology is often unknown but it can be caused by infection and fat emboli (from bone marrow infarcts) |
|
|
Term
What is the treatment for sickle cell patients with aplastic anemia? |
|
Definition
most patients need transfusions for 1 to 2 weeks |
|
|
Term
At what age is acute splenic sequestration most common? |
|
Definition
between 6 months and 2 years of age |
|
|
Term
Do you ever do a splenectomy for patients with acute splenic sequestration? |
|
Definition
yes; if they have had two or more events of acute splenic sequestration |
|
|
Term
What organisms are sickle cell patients at greater risk of being infected by? |
|
Definition
streptococcus pneumoniae, haemophilus influenzae, salmonella, neisseria menigitidis (bc of functional asplenia) |
|
|
Term
How common is it to have strokes/CVAs in sickle cell patients? |
|
Definition
11% have an overt stroke by age 20; silent ifarcts in an additional 22% |
|
|
Term
What are the symptoms of heterozygotes with sickle cell trait? |
|
Definition
no manifestations of disease but may show painless hematuria or inability to concentrate urine |
|
|
Term
What are the complications associated with sickle cell? |
|
Definition
vaso-occlusive crises, acute chest syndrome, aplastic anemia, hemolytic crisis, acute splenic sequestration, serious infection, CVA, pulmonary artery hypertension, renal papillary necrosis, hematuria, cholelithiasis, retinopathy |
|
|
Term
What are teh first and second most common organisms responsible for osteomyelitis in a sickle cell patient? |
|
Definition
salmonella then S. aureus |
|
|
Term
At what age do patients first show signs of sickle cell? |
|
Definition
afterfour months of age as fetal hemoglobin decreases |
|
|
Term
How does sickle cell present? |
|
Definition
progressive hemolytic anemia that develops after four months of age= pallor, splenomegaly, jaundice, systolic ejection murmur and growth retardation; dactylitis is common at initial presentation |
|
|
Term
Any sickle cell patient with a temperature >38 C must be evaluated for... |
|
Definition
bacterial sepsis, septic joints or osteomyelitis |
|
|
Term
What are triggers for acute decompensation of sickle cell patients? |
|
Definition
hypoxia, changes in temperature and dehydration |
|
|
Term
Why is sickledex inappropriate for use in newborns? |
|
Definition
large amounts of fetal hemoglobin can lead to a false negative result |
|
|
Term
How do you treat sickle cell pain crises? |
|
Definition
NSAIDS and/or opiods (morphine or hydromorphone), hydration (1.5 to 2.0x maintenance), and O2 for hypoxia |
|
|
Term
What is the treatement for acute chest syndrome? |
|
Definition
BS abx (cepahlosporin + macrolide); O2, fluids, analgesics, incentive spirometry, and exchange transfusion in the presence of hypoxia or if hematocrit is less than 18% |
|
|
Term
Howdo you treat complications of sickle cell like stroke and priapism? |
|
Definition
chronic exchange transfusions to keep HbS below 30% |
|
|
Term
How do you treat sickle cell episodes of aplastic anemia, sequestration, hemolytic crisis? |
|
Definition
|
|
Term
What is teh median life expectancy of sickle cell patients? |
|
Definition
|
|
Term
What prophylactic medications should be given to sickle cell patients? |
|
Definition
oral penicillin VK BID at diagnosis until child is at least five yoa; vaccinate against S pneumoniae, H flu, HBV, influenza, N meningitidis; give folic acid QD (somewhat controversial); retinal exams yearly starting at age eight; hip radiographs starting at age 10; echos for pulmonary artery pressure every other year starting at age 10; hydroxyurea in children over 5yoa with hx of severe complications |
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|
Term
What should you do to prophylax sickle cell pts with a history of stroke? |
|
Definition
place them on chronic exchange transfusion protocols ot prevent future events |
|
|
Term
What should you give to sickle cell patients with transfusions? |
|
Definition
deferoxamine (desfereal) or the new oral iron chelator deferasirox (exjade) to prevent hemochromotosis |
|
|
Term
What are teh most common childhood cancers? |
|
Definition
1. leukemia, 2. CNS tumors, 3. lymphoma, 4. neuroblastoma; also STS, Wil'ms and bone |
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|
Term
At what age does ALL peak? |
|
Definition
|
|
Term
What are the signs and symptoms of ALL? |
|
Definition
lethargy, fever, bone pain, limp orrefusal to bear weight and CNS manifestations (including headache); petechiae, purpura and bleeding from thrombocytopenia, pallor from anemia, LAD, HSM, and testicular swelling |
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|
Term
HOw do you work up a child with ALL? |
|
Definition
peripheral smear to reveal large immature lymphoblasts, bone marrow bx to establish diagnosis as morphology of peripheral blasts may not reflect the true bone marrow morphology; obtain a baseline CMP along with calcium, mg, and phos to define baseline values prior to chemo (to monitor for tumor lysis syndrome); flow cytometry, CXR, LP and CT to screen for metastasis |
|
|
Term
What two lab values are often elevated in patients with ALL? |
|
Definition
|
|
Term
What are the typical WBC counts in children with ALL? |
|
Definition
although SLL is a lymphocyte-proliferative disorder; WBC counts can be low, normal or high |
|
|
Term
How long do you treat ALL? |
|
Definition
2 years for girls and 3 years for boys |
|
|
Term
What are the three steps of ALL treatment? |
|
Definition
induction, consolidation, and maintenance |
|
|
Term
What is involved in the induction phase of ALL treatment? |
|
Definition
prednisone, vincristine and L-asparaginase |
|
|
Term
What is involved in the consolidation phase of ALL treatment? |
|
Definition
intrathecal methotrexate with or without cranial irradiation |
|
|
Term
What is involved in the maintenance phase of ALL treatment? |
|
Definition
6-MP, methotrexate, vincristine |
|
|
Term
What is tumor lysis syndrome? |
|
Definition
induction phase of chemo induces rapid killing of tumor cells, releasing cytosol directly into the blood; cells contain high concentrations of K, phos, and DNA and thus hyperkalemia, hyperphosphatemia and increased uric acid results in acute renal failure |
|
|
Term
What is the treatment for tumor lysis syndrome? |
|
Definition
fluids, diuretics, allopurinol, alkalinization of urine and reduction of phosphate |
|
|
Term
What is teh tumor marker common for ALL? |
|
Definition
common ALL antigen= CALLA |
|
|
Term
What is teh overall cure rate of Wilm's tumor? |
|
Definition
|
|
Term
What is Wilm's tumor associated with? |
|
Definition
family hx of wilm's tumor, Beckwith-Wiedemann syndrome, Denys-Drashsyndrome, WAGR syndrome, and neurofibromatosis |
|
|
Term
What is Beckwith-Wiedemann syndrome? |
|
Definition
hemihypertrophy, macroglossia, visceromegally, and embryonal tumors |
|
|
Term
What is denys-Drash syndrome? |
|
Definition
nephropathy and genital abnomralities; assoc with wilms |
|
|
Term
|
Definition
Wilm's tumor, aniridia, genitourinary abnomralities and mental retardation |
|
|
Term
What is the most common presentation of Wilm's tumor? |
|
Definition
85% are diagnosed after incidental discoverty of a painless abdominal mass |
|
|
Term
What are possible symptoms of Wilms tumor? |
|
Definition
nausea, emesis, bone pain, weight loss, dysuria, polyuria |
|
|
Term
What are common PE/lab findings in patients with wilms tumor? |
|
Definition
abdominal mass, fever, hematuria, hypertension, varicocele |
|
|
Term
What causes hypertension in kids with WIlms? |
|
Definition
secretion of renin by tumor cells or compression of renal artery by tumor |
|
|
Term
How do you workup wilms tumor? |
|
Definition
Abdominal u/s reveals solid intrarenal mass, CT reveals hematogenous metastasis, CBC, LFTs, BUN/Cr, UA; Cest CT or CXR to screen for metastasis |
|
|
Term
What percent of patients have metastasis when they are diagnosed with wilms? |
|
Definition
10-15% of patients have it |
|
|
Term
How does wilm's tumor metastasize? |
|
Definition
|
|
Term
What is the treatment for Wilms? |
|
Definition
abdominal exploration with tumor excision and nephrectomy, postsurgical chemotherapy with vincristine and dactomycin |
|
|
Term
|
Definition
tumor of neural crest cells that make up the adrenal medulla nd sympathetic nervous system |
|
|
Term
What is teh most common malignant tumor of infants? |
|
Definition
|
|
Term
When does neuroblastoma typically present? |
|
Definition
|
|
Term
What is neuroblastoma associated with? |
|
Definition
neurofibromatosis, Hirschsprung's disease, beckwith-Wiedemann syndrome and fetal hydantoin syndrome |
|
|
Term
What are the symptoms of neuroblastoma? |
|
Definition
abdominal distension, anorexia, weight loss, malaise, an dmuscular symptoms; firm smooth nontender abdominal or flank mass |
|
|
Term
What are physical exam findings of neuroblastoma? |
|
Definition
hypertension (from compression of renal vasculature), fever, pallor and periorbital bruising (raccoon eyes); metastases to the liver, bone and lymph nodes can lead to HSM, leg swelling, bone pain or LAD; a skin rash, watery diarrhea (from secretion of VIP), and opsoclonus/myoclonus (dancing eyes/dancing feet) may also be present |
|
|
Term
How do you work up neuroblastoma? |
|
Definition
CT of the abdomen, chest and pelvis, bone scan and bone marrow aspirate; LP; 24 hr urine collection for catecholamines (VMA and HVA) are elevated in 95% of patients; CBC, LFTs, coagulation panel, BUN/Cr |
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|
Term
What percent of patients with neuroblastoma have distant metastasis at time of diagnosis? |
|
Definition
|
|
Term
What is another name for Wilms tumor? |
|
Definition
|
|
Term
What is the treatment for neuroblastoma? |
|
Definition
excision of localized tumors; for intermediate to high-risk stages combination chemo and/or adjunctive radiation may be used |
|
|
Term
What is the definition of JIA? |
|
Definition
collagen vascular disease that is defined by persistent inflammation in > 1 joint for > 6 weeks in a patient <16 yoa |
|
|
Term
What is the most common age of onset of JIA? |
|
Definition
|
|
Term
T/F JIA is more common in girls. |
|
Definition
true; except for stills disease in which girls=boys |
|
|
Term
What are the three types of JIA? |
|
Definition
pauciarticular, polyarticular and systemic |
|
|
Term
What percent of children with systemic JIA develop macrophage activation syndrome? |
|
Definition
|
|
Term
What is macrophage activating syndrome? |
|
Definition
life-threatening illness characterized by sudden-onset fever, pancytopenia, HSM, liver dysfunction, DIC, hypofibrinogenemia, and hypertriglyceridemia |
|
|
Term
What is the most common type of JIA? |
|
Definition
|
|
Term
What is pauciarticular JIA? |
|
Definition
affects four or fewer joins, usually large joints (knees and ankles) |
|
|
Term
What is teh prognosis of pauciarticular JIA? |
|
Definition
good, 70% go into remission after several years |
|
|
Term
What is a serious complication of pauciarticular JIA? |
|
Definition
chronic asymptomatic uveitis can lead to blindness in young children if not diagnosed by slit-lamp examination; acute onset uveitis is more common in older children |
|
|
Term
How can you tell if a pauciarticular JIA pt is at higher risk for chronic uveitis? |
|
Definition
|
|
Term
What is the definition of polyarticular JIA? |
|
Definition
affects five or more joints, usually large (knees, ankles)and small joints (hand, feet), as well as the TMJ and cervical vertebrae |
|
|
Term
What is the second most common form of JIA? |
|
Definition
|
|
Term
RF seropositivity in polyarticular JIA is associated with... |
|
Definition
ANA+, more severe disease, and older age of onset |
|
|
Term
What is another name for systemic JIA? |
|
Definition
|
|
Term
|
Definition
|
|
Term
What are the characteristics of systemic JIA? |
|
Definition
high, spiking fevers to more than 39.4; a "salmon" colored rash that comes and goes with fever, and an unremiting and severe arthritis; other symptoms include myalgias, LAD, growth retardation, HSM, pericarditis, and pleuritis |
|
|
Term
What is the prognosis of systemic JIA? |
|
Definition
complete resolution is rare and 1/2 develop destructive arthritis |
|
|
Term
What are the serologies of systemic JIA? |
|
Definition
RF and ANA are both negative |
|
|
Term
What do the lab results of systemic JIA show? |
|
Definition
anemia of chronic disease; increased WBC count and increased acute phase reactants (ESR, CRP, platelets) |
|
|
Term
What do the involved joints of JIA look like? |
|
Definition
warm with limited ROM but rarely erythematous |
|
|
Term
HOw do you workup a pt with suspected JIA? |
|
Definition
CBC, ESR, RF/ANA, radiographs, MRI, synovial fluid analysis |
|
|
Term
What do radiographs of JIA show? |
|
Definition
soft tissue swellin, osteopenia, joint space narrowing or bony erosions, |
|
|
Term
What does synovial fluid analysis of JIA joints show? |
|
Definition
leukocytosis (5000-30,000 WBCs/mm3) and elevated protein |
|
|
Term
What drugs are used to treat JIA? |
|
Definition
most respond to NSAIDS, methotrexate for severe disease, antiTNF therapy (etanercept or infliximab) for refractory polyarticulardisease; intraarticular steroids; systemic steroids for severe systemic disease or severe uveitis, steroid eye drops and dilating agents are administered for most cases of uveitis |
|
|
Term
What are the nonpharmacologic treatments of JIA? |
|
Definition
calcium supplements and weight bearing exercise to prevent osteoporosis, stretching and morning baths are helpful for morning stiffness |
|
|
Term
What is the definition of fever? |
|
Definition
rectal temp over 38 or more than 100.4 |
|
|
Term
For fever management of an infant, it is important to narrow youre differential by first determining... |
|
Definition
the age of the child: <28 days, 28-90 days, and 3- 36 months |
|
|
Term
T/F Acute otitis media can cause fever. |
|
Definition
|
|
Term
What does it mean to say a patient is toxic appearing? |
|
Definition
lethargic, signs of poor perfusion, marked hypoventilation or hyperventilation, or cyanosis |
|
|
Term
What does it mean to call a child lethargic? |
|
Definition
altered level of consciousness characterized by poor or absent eye contact or failure of the child to recognize the parents or to interact with the environment |
|
|
Term
What is a full sepsis workup for infants? |
|
Definition
LP and CSFculture, gram stain, cells, glucose and protein, and possibly HSV PCR, urine culture and UA; CBC with diff, blood culture and BMP (electrolytes, glucose and BUN/CR) |
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|