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First Aid for Wards: Peds
Part 1
288
Medical
Professional
10/03/2012

Additional Medical Flashcards

 


 

Cards

Term
What values are charted on a growth chart?
Definition
infants= length, weight, and head circumference
children over 2= hieght, weightand BMI
Term
What components of the psychosocial history should you ask teenagers?
Definition
home life, education, employment, activities, drugs, depression, safety (seat belts, guns in the home, abuse), sexual activity
Term
How should you examine a pt whom you suspect has epiglotitis?
Definition
don't inspect the pharynx, leave the pt in the parents arm during the physical exam
Term
What's the difference between rectal and oral temps?
Definition
rectal is the gold standard; oral is generally 1 degree below rectal
Term
AFOSF=
Definition
anterior fontanelle, open, soft, and flat
Term
C/C/E=
Definition
cyanosis, clubbing, edema
Term
c/w=
Definition
consistent with
Term
What is DOA?
Definition
day of admission
Term
F/C=
Definition
fever/chills
Term
FM=
Definition
fine motor
Term
GU=
Definition
genitourinary
Term
HC=
Definition
head circumference
Term
IUTD=
Definition
immunizations up to date
Term
MMM=
Definition
mucous membranes moist
Term
NBNB=
Definition
nonbilious, nonbloody
Term
NC/AT=
Definition
normocephalic, atraumatic
Term
nl=
Definition
normal
Term
O/P=
Definition
oropharynx
Term
PTA=
Definition
prior to admission
Term
USGH=
Definition
usual state of good health
Term
WD/WN=
Definition
well developed, well nourished
Term
W/R/R=
Definition
wheezes/rhonchi/ralse
Term
How should you maneuver the auricle if you want to see the tympanic membrane?
Definition
in an infant, pull the auricle backward and downward; in an older child, pull backward and upward
Term
What is the average heart rate of a newborn?
Definition
140-160
Term
Why is it important to check femoral pulses in an infant?
Definition
to exclude coarctation of the aorta
Term
What percent of infants have innocent murmurs?
Definition
50%
Term
What is a normal heart rate in a child?
Definition
less than 120
Term
What are characteristics of an innocent murmur?
Definition
low intensity (I-II/VI), occurence in systole, variation with position and respiration, and a musical quality
Term
What is a normal respiratory rate for a newborn?
Definition
40-60
Term
What is a normal respiratory rate in a child?
Definition
15-25
Term
How is infant breaths different from adult breaths?
Definition
expiration is more prolonged in infants; respiratory movements are produced by abdomenal movements
Term
Describe the Barlow maneuver.
Definition
stabilize the pelvis with one hand, and then flex and adduct the opposite hip and apply gental posterior pressure on the thigh
Term
What is the name of the maneuver used to dislocate the hip in developmental hip dysplasia?
Definition
barlow maneuver
Term
What is the maneuver used to reduce a hip dislocation of developmental hip dislocation?
Definition
ortolani maneuver
Term
Describe the ortolani maneuver?
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
Term
When is a pediatrician called to a birth?
Definition
multiple gestations, preterm deliveries, cesarean sections, possible meconium aspirations, and known fetal anomalies
Term
What should be done to a baby who is born?
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
Term
At what age do children get heel sticks?
Definition
less than 2 month
Term
Most neonatal reflexes disapear by what age?
Definition
6 months
Term
What is the significance of persistent primitive reflex?
Definition
those infants are at greater risks for developmental disability
Term
At what age should children be crawling?
Definition
crawling is not a developmental milestone because some children skip this step
Term
What is a premature baby?
Definition
born before 38 weeks
Term
How long do you correct for prematurity for?
Definition
2 years
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
Term
What are the traditional two categories of causes of FTT?
Definition
medical (organic) and psychosocial (nonorganic)
Term
What are the gross motor developmental milestones?
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
Term
What are the fine motor milestones?
Definition
birth= visually fixes; 1 month= tracks to midline; 2 months= tracks past midline; 6= transfers objects; 12= two finger pincer grasp
Term
What are the language developmental milestones?
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
Term
What are the social milestones?
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
Term
What is the timing of the moro startle?
Definition
present at birth; disappears by 3 to 6 months
Term
Describe the moro startle.
Definition
head extension leads to extension, adduction, and then abduction of the upper extremities
Term
Name some neonatal reflexes.
Definition
moro startle, palmar grasp, rooting, stepping, asymmetric tonic neck, parachute, galant, and babinski
Term
What is the timing of the stepping reflex?
Definition
present at birth; disappears by 2 to 4 months
Term
Describe the stepping reflex?
Definition
infants move thier legs in a walking movement when they are held upright and leaning forward
Term
When does the asymmetric tonic neck reflex disappear?
Definition
turning the head while leads to ipsilateral extremity extension and contralateral flexion (fencer position)
Term
What is the parachute reflex?
Definition
infants extend the ipsilateral arm to support the body when tilted to one side while sitting
Term
When does the galant reflex disapear?
Definition
disappears by 6-9 months
Term
Describe the galant reflex?
Definition
stroking theparavertebral region while the infant is prone causes the pelvis to move toward the stimulated side
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
Term
What is "falling off the growth curve"?
Definition
crossing down two major percentile lines on a growth chart
Term
How should you workup failure to thrive?
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)
Term
In failure to thrive, in what order to kids fall of their growth charts?
Definition
weight first, then height, then head circumference
Term
What much do babies grow in the first year?
Definition
triple in weight and double in length
Term
What is the average birth weight?
Definition
7.7 lbs or 3.5 kg
Term
How much weight should neonates gain in the first few days of life?
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
Term
How much weight should babies gain per day?
Definition
30 g/day for 3-4 months; 10-20 g/day for 4-12 months
Term
At what point should the birth weight double? triple? quadruple?
Definition
doubles at 4-5 months; triples by one year; and quadruples by two years
Term
How much weight should a child gain on average per year?
Definition
5 lbs per year from age 2 to puberty
Term
What is the average birth length?
Definition
50 centimeters (20 inches)
Term
What is the average height of a one year old?
Definition
30 inches
Term
What is the average height of a three year old?
Definition
3 feet
Term
What is the average height of a four year old?
Definition
40 inches
Term
When do children triple their birth height?
Definition
13 yoa
Term
How much do children grow per yea?
Definition
2-3 inches per year from age four to puberty
Term
What is the average birth head circumference?
Definition
35 cm
Term
At what rate should the head circumference enlarge in the first year?
Definition
1 cm per year
Term
What percent of head growth has occurred by age 2?
Definition
90%
Term
What are the contraindications to all types of vaccination?
Definition
current moderate to severe acute illness; severe allergy to a vaccine component
Term
What are the contraindications to influenza vaccine?
Definition
anaphylactic reaction to eggs, current moderate to severe acute illness
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
Term
What are the contraindications to IPV vaccination?
Definition
current moderate to severe acute illness; anaphylactic reaction to neomycin, polymyxin B, or streptomycin (perform prior skin testing);
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)
Term
What are the contraindications for oral polio vaccination?
Definition
current moderate to severe acute illness; pregnancy, immune compromise, high dose steroids
Term
What are the CIs for varicella??
Definition
current moderate to severe acute illness; pregnancy, immune compromise or use of high dose steroids
Term
What kind of reactions to pertussis vaccines necessitate the use of precaution in administering future vaccines?
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
Term
What vaccine is administered at birth?
Definition
HBV
Term
What vaccine is administered at 2 months?
Definition
HBV, DTaP, Hib, IPV, PCV
Term
What vaccines should be administered at 4 months?
Definition
DTaP, Hib, IPV, PCV
Term
What vaccines should be administered at 6 months?
Definition
HBV, DTaP, Hib, IPV, influenza, PCV
Term
What vaccines should be administered at 12-15 months?
Definition
Hib, influenza, PCV, MMR, varicella, HAV
Term
What are the 15-18 month shots?
Definition
DTaP and influenza
Term
What are the two year shots?
Definition
influenza, HAV, meningococcal
Term
What are the 4-6 yoa shots?
Definition
DTaP, IPV, influenza, MMR, varicella
Term
What are the 11 to 12 yoa shots?
Definition
DTaP, HPV, MCV4, PPV, influenza
Term
What are the 13 to 14 yoa shots?
Definition
MCV4, PPV, and influenza
Term
What are the 15 yoa shots?
Definition
MCV, PPV, influenza
Term
What vaccinations are needed at 16 to 18 yoa?
Definition
MCV4, influenza, HAV
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
Term
At what age can a child first recieve the flu vaccine?
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
Term
How far apart should the HAV vaccine be administered?
Definition
at least 6 months apart
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
Term
How long should women breast feed?
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
Term
After 6 months of breast feeding why might it be advantageous to start adding supplemental diet?
Definition
because the maternal stores of iron are often depleted by that time and infants may require flouride and iron supplementation
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
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
Term
All breast fed infants should recieve what supplementation?
Definition
vitamin D supplementaiton (200 IU) to prevent rickets
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
Term
How much do babies eat?
Definition
in the first two months of life babies will eat 2-3 ounces (approximately 10-20 minutes breast feeding) every 2-3 hours
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
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
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
Term
What is the normal progression of puberty in males?
Definition
testicular enlargement --> penile enlargement --> growth spurt --> pubic hair
Term
What is the progression of puberty in females?
Definition
thlarche --> growth spurt --> pubic hair --> menarche
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
Term
At 4 to 6 months, a baby should be eating...
Definition
iron-fortified single-grain cereal; supplemental water
Term
At 6 to 7 months, an infants should be eating...
Definition
strained fruit, consider flouride supplementation (depends on local water supply)
Term
At seven to eight months you should introduce what into a childs diet?
Definition
strained vegetables
Term
When can you introduce well chopped meats into an infants diet?
Definition
8 to 9 months
Term
When can you add cheese, egg yolk, and protein rich foods into a childs diet?
Definition
9-10 months
Term
When should children starteating soft finger foods?
Definition
10-12 months
Term
What is Tanner stage I?
Definition
males= childhood size external genitalia; females= preadolescent; elevation of papilla only; no pubic hair
Term
What is tanner stage II?
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
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
Term
What is tanner stage IV?
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
Term
What is tanner stage V?
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)
Term
Why is it important not to leave formula bottles in the crib overnight?
Definition
can cause milk bottle tooth caries
Term
What is pubarche?
Definition
pubic hair development
Term
What is thelarche?
Definition
breast development
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
Term
What is the most common cause of gonadotropin dependent precocious puberty?
Definition
idiopathic
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
Term
What is the incidence of congenital heart diesease?
Definition
0.8%
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
Term
How do you workup a congenital heart disease?
Definition
CXR, ECG and an echo; sometimes angiography
Term
Name some more common acyanotic heart diseases?
Definition
PDA, ASD, VSD, and coarctation of the aorta
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
Term
What is eisenmenger's syndrome?
Definition
when longstanding acyanotic congenital heart conditions eventually result in shunt reversal and pulmonary hypertension
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
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
Term
What determines the presentation of tetrology of fallot?
Definition
the severity of the pulmonary artery stenosis
Term
What medication is given to most patients with cyanotic congenital heart conditions?
Definition
PGE1 to maintain a patent PDA
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
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
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
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
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
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
Term
What is the most common cause of bowel obstruction in the first two years of life?
Definition
intussusception
Term
In what gender is intussusception more common?
Definition
males
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
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
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
Term
What signs of intussuception are ominous?
Definition
red "currant jelly" stools, lethargy and fever
Term
What should you think if you feel a "sausage-shaped" abdominal mass on palpation?
Definition
intussusception
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
Term
What should you do if a child has signs of intussusception and peritoneal signs?
Definition
straight to surgery
Term
What percent of intussusceptions are cured by barium/air enemas?
Definition
75%
Term
What virus causes rubeola (measles)?
Definition
paramyxovirus
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
Term
What are the complications associated with rubeola (measles)?
Definition
otitis media is very common, encephalitis, pneumonia, subacute sclerosing panencephalitis (rare)
Term
What are the three day measles?
Definition
german measles or rubella
Term
What virus causes rubella?
Definition
RNA virus= togavirus
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)
Term
What are petechiae on the palate called/associated with?
Definition
forschheimer spots= associated with rubella
Term
What are the complications associated with rubella?
Definition
devastating results if a fetus is infected during gestation; rarely encephalitis and thrombocytopenia
Term
What diseases are caused by HHV-6 and HHV-7?
Definition
roseola infantum (exanthem subitum)
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
Term
What are the complications associated with roseola infantum (exanthem subitum)?
Definition
rapid temperature increases associated with febrile seizures
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
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
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
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
coxsackie A virus
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
male: female= 4:1
Term
What is the incidence of pyloric stenosis?
Definition
1/500 births
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
Term
What antibiotic is associated with pyloric stenosis?
Definition
erythromycin
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
1/600
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
30 to 40s
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
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
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
simple transfusion
Term
What is teh median life expectancy of sickle cell patients?
Definition
42 for men; 28 for women
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
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
Term
At what age does ALL peak?
Definition
4 yoa
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
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
uric acid and LDH
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
>85%
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
What is WAGR syndrome?
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
hematogenously
Term
What is the treatment for Wilms?
Definition
abdominal exploration with tumor excision and nephrectomy, postsurgical chemotherapy with vincristine and dactomycin
Term
What is a neuroblastoma?
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
neuroblastoma
Term
When does neuroblastoma typically present?
Definition
less than 5 yoa
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
Term
What percent of patients with neuroblastoma have distant metastasis at time of diagnosis?
Definition
50%
Term
What is another name for Wilms tumor?
Definition
nephroblastoma
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
1-3 yoa
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
5-8%
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
pauciarticular
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
if they are ANA+
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
polyarticular
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
Still's disease
Term
What is Still's disease?
Definition
systemic JIA
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
false
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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