Emma Holliday Flashcards
acrocyanotic
define
apgar score
blue hands and feet
add 1 to apgar
grimaces to stimulation
apgar score
add 1 to apgar
not full withdrawal to stim, that would be 2
tf
apgar guides therapy and has prognostic value
f
just descriptive basically
erb palsy
appearance
which nerve roots
waiter’s tip
C5-C6
klumpke palsy
appearance
which nerve roots
klumpke claw hand
C7C8T1
scalp edema crosses suture lines is called
vs does not cross suture lines
caput succedaneum
cephalo-hematoma
what makes up mongolian spots
arrested melanocytes
pale pink vascular macules on face or nuchal area
aka
persist or resolve?
(nevus simplex) salmon patch
on the face regress
on the neck may persist into adolescence
firm white papules on face of newborn on day one aka filled with may confuse with... but won't because..
milia
filled with keratin
may confuse with neonatal acne
but won’t because acne presents later (week of life 1 or 2, not day of life 1)
firm yellow/white papules/pustules on an erythematous base on day of life 2
aka
filled with
erythema toxicum
filled with eosinophils
bright red sharply demarcated raised lesion in first few months of life aka
hemangioma
tactile difference nevus simplex vs hemangioma
nevus simplex (salmon patch) is not palpable
hemangioma is raised, palpable, sharp borders
erythematous papules on face in week of life 1 or 2
aka
neonatal acne
why does neonatal acne occur
hormones! same as teens
circulating maternal androgens
biggest clue milia vs neonatal acne
time of presentation
milia - day 1 of life
neonatal acne - week 1 or 2 of life
area of alopecia with orange colored nodular skin
aka
mgmt
nevus sebaceous
remove before adolescence because risk of malignant transformation
newborn seborrheic dermatitis
aka
describe
manage
cradle cap
thick yellow/white oily scale on an inflammatory base
mild shampoo… eg on a soft toothbrush and scrub away… pretty easy to get rid of
2 neonatal disorders screened in every state because disastrous if not caught early and contraindicate breast feeding path time to sx sx mgmt
- PKU Phenylketonuria
- deficient phenylalanine hydroxylase, phe accumulates in brain
- sx take a few months to arise
- developmental delay, mental retardation, vomiting, athetosis writhing muscle contractions, fair hair eyes skin, musty smell
- low phenylalanine diet
Galactosemia
- deficient G1p-uridyl-transferase, G1p accumulates and damages kidney liver brain
- right at birth (galactose can cross placenta)
- mental retardation, jaundice hyperbilirubinemia, hypoglycemia, cataracts, seizures
- no lactose diet (no breast milk!)
tf
phenylalanine and lactose are present in breast milk
T
that’s why important to screen for PKU and Galactosemia in neonates
physiologic jaundice usually resolved by…
day of life 5
4 red flags for pathologic jaundice
and next best test
1st day of life (usually pathologic)
total bili ^12
d bili ^2
rate of rise ^5/day
get coombs for ABO Rh incompatibility
-if negative… lots of other stuff it could be…
does sepsis cause neonatal indirect or direct hyperbilirubinemia
direct
does galactosemia cause neonatal indirect or direct hyperbilirubinemia
direct
does hypothyroidism cause neonatal direct or indirect hyperbilirubinemia
direct
pathogenesis of dubin johnson and rotors
secretion… absorption… or something… get conjugated hyperbili… not a conjugation defect (that’s gilbert and crigler najar unconjugated hyperbili)
dubin johnson vs rotor liver color
dubin johnson black liver
rotor red (normal) liver
where does indirect bilirubin deposit in the brain in kernicterus
basal ganglia
cranial nerve nuclei
tf
phototherapy conjugates bilirubin
FALSE
it ionizes the unconjugated bili so that it becomes soluble and can be excreted… but does not conjugate it… whatever that means
diaphragmatic hernia identified prenatally
the big worry
how to manage delivery and repair
big worry is pulmonary hypoplasia
ECMO (extracorporeal membrane oxygenation) after delivery to support for a few days and let lungs mature… THEN operate 3-4 days after delivery…
drooling newborn who can’t breath think…
esophageal atresia
tracheo-esophageal fistula, etc
VACTERL
Vertebral defects Anal atresia Cardiac defects Tracheo-Esophageal fistula Renal anomalies Limb anomalies
VACTERL association has 3+ of the above
feeding tube coils, dianosing esophageal atresia
…what else to look for?
VACTERL association has 3+ of
Vertebral defects Anal atresia Cardiac defects Tracheo-Esophageal fistula Renal anomalies Limb anomalies
what is the choana
back of the nasal passage
1 week old baby cyanotic with feeds but pinks with crying
think…
also look for…
choanal atresia (failed recanalization of nasal fossa)
CHARGE association Coloboma (hole in an eye structure) Heart defects Atresia of choana Retarded growth GU anomalies Ear anomalies and deafness
CHARGE association
Coloboma (hole in an eye structure) Heart defects Atresia of choana Retarded growth GU anomalies Ear anomalies and deafness
32 wk premie w dyspnea rr 80 w nasal flaring
cxr ground glass opacities, air bronchograms, atelactasis
dx
pathophys
tx
RDS
L:S ratio v2 prenatally
not enough surfactant to keep alveoli open
corticosteroid prenatally to stim lung maturation
O2 therapy with nasal CPAP to keep alveoli open
tf
theophyline for RDS
false
theophylline for problems with respiratory drive (central sleep apnea)
RDS is a problem of oxygenation, not respiration
38wk LGA infant porn by csection to mom with gestational diabetes has dyspnea and grunting
dx
pathophys
tx/prognosis
TTN
transient tachypnea of the newborn
cxr perihilar streaking from retained fluid in lung fissures
retained fluid from not having it expulsed during vaginal passage because delivered by c section
minimal O2 needed usually, self resolves in hours-dats
baby born after rupture of membranes yielded green/brown fluid
dx
next best step
complications
meconium aspiration syndrome
intubate and suction BEFORE typical stimulation maneuvers to prevent further aspiration into lungs
pneumonitis, pulmonary htn (lungs not expanding to decrease resistance)
newborn periumbilical intestinal hernia, defect lateral of midline, no sac
dx
associations
complications
gastroschisis
^maternal AFP prenatally
NO disease associations
(as opposed to omphalocele….big baby big tongue ear pits low glucose Beckwith Wiedemann Syndrome)
dehydration and scarring requiring resection, short gut syndrome (malnutrition etc)
newborn periumbilical intestinal hernia covered by sac
dx
associations
omphalocele
assoc w Beckwith Wiedemann Syndrome - big baby w big tongue, ear pits, low glucose, omphalocele
newborn umbilical hernia
(midline defect WITHOUT BOWEL present)
assoc
tx
assoc w Congenital Hypothyroidism
newborn
big tongue
umbilical hernia
think…
assoc
tx
congenital hypothyroidism
vs big tongue and ompalocele… and ear pits, low glucose assoc w Beckwith Wiedemann Syndrome
big tongue and umbilical hernia think…
not..
congenital hypothyroid
if big tongue and Omphalocele think beckwith wiedemann syndrome… espec if with ear pits, low glucose
metabolic complication of pyloric stenosis
hypochloremic metabolic alkalosis (because vomiting up HCL)
Polyhydramnios is typically caused by
decreased fetal swallowing or increased fetal urination
causes of necrotizing enterocolitis
premature gut (premie) maybe introducing food too soon maybe formula...
treat necrotizing enterocolitis
NPO TPN abx resection of necrotic bowel
prune belly syndrome
define
association
failure of abdominal musculature to form so belly looks pruny because intestines underneath skin more easily visualized
assoc w cryptorchidism
tf
orchopexy for cryptorchidism decreases risk of cancer
tish fish
does not decrease rate of cancer… but increases detection of testicular changes so id and tx faster
newborn w ambiguous genitalia, one month later with vomiting, hyponatremia, hyperkalemia, and acidosis
dx
most common cause
most definitive test
tx
CAH
congenital adrenal hyperplasia
21 hydroxylase deficiency
test: 17OHP 17hydroyprogesterone level, ACTH bolus afterward
give cortisone and fludricortisone (replace cortisol and aldosterone)
anterior midline abdominal mass and no pee in newborn… most common cause… treat
posterior urethral valves
(mass is distended bladder)
cath then surg
infant complications of maternal type 1 diabetes
how to prevent
placental insufficiency/IUGR, congenital heart disease, neural tube defects, caudal regression syndrome, small left colon syndrome
control insulin during pregnancy, especially 1st trimester, take 4mg of folate per day
infant complications of gestational diabetes (gimme6)
LGA (from fetal hyperinsulinemia), birth trauma clavicle fx BPBP risk, TTN risk because csection rates higher
hypoglycemia (from fetal hyperinsulinemia), neonatal seizures
hypocalcemia, neonatal seizures
polycythemia (big baby needs more O2 delivery so makes more EPO), renal or splenic vein thromboses
jaundice (more RBCs to break down), kernicterus
RDS (hyperinsulinemia interferes with cortisol surge prior to birth that normally stimulates lung maturation)
treat neonatal hypoglycemia due to gestational diabetes
feed frequently (eg breast milk) if glucose v40, IV dextrose of glucose v20
L:S ratio in mature vs immatuer lungs
mature ^2
immature v2
risk factors for neonatal sepsis (gimme6)
- prematurity
- prolonged rupture of membranes ^18hours
- chorioamnionitis (e.g with prolonged rupture of membranes)
- GBS+ mom
- intrapartum fever
- maternal leukocytosis
most common bugs of neonatal sepsis
empiric abx
GBS
Ecoli
lysteria
amp and gent til 48 hours negative cx
amp and cefotaxime if meningitis suspected
congenital TORCH infections and treatment:
maculopapular rash on palms and soles, snuffles, periostitis
congenital syphilis
penicillin
congenital TORCH infections and treatment:
hydrocephalus, intracranial calcifications, and chorioretinitis
congenital toxoplasmosis
sulfadiazine and leucovorin
congenital TORCH infections and treatment:
cataracts, deafness, PDA VSD heart defects, extramedullary hematopoesis
congenital rubella syndrome
no treatment… so vaccinate!
congenital TORCH infections and treatment:
microcephaly, periventricular calcifications, deafness, thrombocytopenia, petechiae
congenital CMV
gancyclovir… but will not prevent mental retardation
congenital TORCH infections and treatment:
limb hypoplasia, cutaneous scars, cataracts, chorioretinitis, cortical atrophy
congenital varicella (an HSV)
vaccinate! baby gets IVIG against VZV if mom exposed 5 days before or 2days after delivery
neonatal conjunctivitis: cause, complication, and treatment
DOL 1-3, red conjunctiva and tearing
DOL 3-5, purulent conjunctivitis
DOL 7-14, mucopurulent conjunctivitis and lid swelling
chemical conjunctivis… from silver nitrate drops… so use erythromycin instead
gonococcal conjunctivitis… can cause corneal ulceration… give topical erythromycin and IV 3rd gen cephalosporin
chlamydial conjunctivitis… can cause chlamydial PNA… give oral erythromycin
down syndrome complications heart gi endocrine msk neuro cancer
heart - endocardial cushon defects
gi - hirschpung, intestinal atresia, annular pancreas, imperforate anus
endocrine - hypothyroidism
msk - cervico-atlanto instability (important preop for anesthesia intubation!)
neuro - alzheimers (APP on ch21)
cancer - ALL
why inc AD in Downs
APP amyloid precursor protein on chromosome 21
omphalocele, rocker bottom feet, hammer toe, microcephaly, clenched hand
think…
edward syndrome
(trisomy 21)
“edward scissor hands rocker feet small head spilled intestines”
holoprosencephaly, severe mental retardation, microcephaly, cleft lip/palate
think…
patau syndrome
trisomy 13
14yo F no breast dev, short stature, high FSH think... genotype... assoc vasc and renal anomalies... tx...
Turner Syndrome
XO genotype
coarcted aorta / bicuspid aortic valce
estrogen replacement for secondary sex characteristics and avoidance of osteoporosis
18yo tall lanky boy with mild mental retardation with gynecomastia and hypogonadism
- has what syndrome
- increased risk for what malignancy
Klinefelter syndrome
increased risk of gonadal malignancy
cafe-au-lait spots, seizures, large head
think…
inheritance pattern…
neurofibromatosis
autosomal dominant
glossoptosis means
downward displacement and retraction of tongue
mandibular hypoplasia, glossoptosis, cleft soft palate. with fetal alcohol syndrome or edwards syndrome is called…
pierre robin sequence…
broad square face, rhot stature, self-injurious behavior, deletion on ch17 is called…
smith-magenis
prader willi signs and symptoms and genetics
hypotonia hypogonadism hyperphagia skin picking aggression deletion on paternal ch15
angelman syndrome signs and symptoms and genetics
seizures strabismus sociable with episodic laughter deletion on maternal ch15
williams syndrome signs symptoms and genetics
elfin facies friendly high empathy and verbal reasoning deletion on ch7
fragile x syndrome
population significance
genetics
sings and symptoms
most common cause of mental retardation in boys
CGG repeats on X chromosome with anticipation
macrocephaly, large ears, macroorchidism
2yo M w multiple ear infections, diarrheal episodes, and pneumonias. no tonsils on exam
what dx
why no tonsils
labs
bruton agammaglobulinemia
x-linked, infections @ 6-9 mos
no b cells, no tonsils
no b cells on flow, low Igs
17yo F w decreased Igs but normal number of B cells
what dx
complication
combined variable immunodeficiency
(acquired)
lymphoma risk because increased lymphoid tissue trying to make functional b cells
most common B-cell defect
symptoms
complication
selective IgA deficiency
recurrent URIs, diarrhea
anaphylaxis if blood transfusion with IgA!!
no thymus and no tonsils, severe lymphopenia
what dx
tx
SCID
x-linked and autosomal recessive forms
bone marrow transplant emergently
child w recurrent swollen infected lymph nodes in groin and staph aureus skin abscesses
what dx
how dx
CGD
x-linked recessive
(staph aureus abscesses because susceptible to catalase positive organisms)
dx w nitrotetrazolium blue (yellow means they have the dz) also flow cytometry w DHR-123
baby w severe eczema petechiae and recurrent ear infections
what dx
what Ig make up
other common presentation
wisckott aldrich syndrome
high IgE IgA, low IgG
often present w prolonged bleeding after circumcision
(thrombocytopenic too)
why do newborns lose birth weight
they pee
baby should regain birth weight by
double weight by
triple weight by
increase length 50% by
double length by
regain birth weight by 2wks
double weight by 6mos
triple weight by 12mos
increase length 50% by 1y
double length by
contraindications for breast feeding
maternal
fetal
maternal - HIV, TB? chemo, radioactive iodine… alcohol…
fetal - PKU, galactossemia
tf
maternal hepC is a contraindication to breast feeding
F
contraindications include
maternal - HIV, TB? chemo, radioactive iodine… alcohol…
fetal - PKU, galactossemia
breast milk vs formula
breast milk
more whey, more lactose, more long chain fatty acids, less iron but better absorbed
predominant protein in breast milk
whey > casein
bone age vs time age eg around puberty parents worried about child growth
constitutional delay
familial short stature
obesity
constitutional delay
bone v time
familial short stature
bone = time
obesity
bone ^ time (estrogen)
pathologic short stature
aka
tests to consider, why
aka falling off the growth curve at puberty
chest ct - craniopharyngioma
TFTs - hypothyroid
IgF1 - hypopituitarism
karyotype - Turners if female
primitive reflexes
when do they go away
moro grasp rooting stepping tonic neck / fencing (all present at birth - 4-6mos)
parachute
(appears 6-8 mos and NEVER GOES AWAY)
parachute reflex
what is it
when does it appear
when does it go away
baby lifted from tummy, arms go out
appears 6-8 mos and NEVER GOES AWAY
which primitive reflex never goes away
parachute
(appears 6-8 mos and NEVER GOES AWAY)
grasp rooting stepping tonic neck / fencing (all present at birth - 4-6mos)
pincer grasp at what age
9 mos
gross and fine motor at 9 mos
sit up without support
pincer grasp
half of speech is comprehensible at what age
2 years
cooing starts at what age
2 mos
with social smile
when should kids be potty trained
stool continence by 4 years
urinary continence by 5 years
(pathologic after.. can attain well before)
primary vs secondary urinary incontinence in child
primary is never continent
secondary is recurrence after 6 mos of continence
medical causes to rule out and how, in peds pt still incontinent of urine after 5yo
UTI - UA
constipation - disimpact
diabetes - check glucose
enuresis
define
aka in peds
treat
urinary incontinence (bed-wetting = nocturnal enuresis)
behavioral reward system, pee before bed, bell-alarm pad
pharm if behavioral fails, DDAVP or imipramine
most common cause of peds fecal incontinence
treat
constipation, fecal retention
disempatct, stool softeners, high fiber diet, behavioral mod eg put on pot after eat to take advantage of gastrocolic reflex
when does baby get hepB IVIG in addition to hepB vaccine
when mom hepB positive
vaccines due at 2 4 6 mos
hep B DTap PCV hib IPV rotavirus
vaccine starting at 6 mos and yearly after
flu
vaccines due at 12 mos
MMRV and hepA
live attenuated
drug allergy that contraindicates MMR vaccine
neomycin/streptomycin allergy… because it is in vaccine…
vaccines due before age 2
before kindergarden
at age 12
Dtap HepA boosters before 2
IPV last Dtap MMRV boosters before kindergarden
Tdap booster, meningococcal, HPV at age 12…. ish
% of kids with benign heart murmurs
30%
benign peds heart murmurs
Stills - v2/6 systolic @ lower mid-sternum
Venous hum - heard @ anterior neck, disappears when jugular vein compressed
peds v2/6 systolic murmur @ lower mid-sternum
Stills
benign
peds murmur heard @ anterior neck, disappears when jugular vein compressed
Venous hum
benign
never normal heart murmur in peds
what to do if these heard
ANYTHING
DIASTOLIC
ANYTHING ^2/6
get an echo
any murmur worse with inspiration is defect on what side of heart
R side
any murmur worse with inspiration
bipolar mom has baby with holosytolic murmur worse on inspiration… why
what associated arrhythmia
ebstein anomaly from lithium
wpw wolff parkinson white arrhythmia
when is surgery indicated for 2/6 systolic murmur caused by VSD in peds
if sympromatic (failure to thrive, pulmonary htn)
otherwise wait till 2yo
what does common cardiac defect in turner syndrome look like on cxr
reverse 3 sign.. aortic arch looks weird
notching at inferior rib borders from increased collateral flow thru intercostal arteries
(from aortic coarctation)
heart stuff
joint stuff
rash
think…
tx
compx
acute rheumatic fever
oral penicillin or erythromycin for 10 days, then prophylactic till 20 yo
mitral stenosis, then aortic or tricuspid
