childhood Flashcards
agenesis
absent organ, absent primordial tissue
aplasia
absent organ, primodrial tissue present
hypoplasia
incomplete organ development
how infant is exposed to transcervical perinatal infection? disease caused
“inhale” in utero or via birth process; common cause of pneumonia
how infant is exposed to trans placental perinatal infection? disease causes
via blood through chorionic villi, TORCH infections
what is the underlying cause of RDS?
deficient surfactant
what are the risk factors for RDS?
male, maternal DM, prematurity, C-section
why is maternal DM a risk for RDS?
high insulin levels suppress surfactant production
why is C-section a risk for RDS?
decrease in stress-induced steroid release leads to less surfactant
what composes the hyaline membranes?
necrotic epithelial cells and plasma proteins
2 complications associated with O2 therapy for RDS
retinopathy of prematurity
bronchopulmonary dysplasia/dec septation
what are the 2 phases of retinopathy of prematurity?
1- decreased VEGF/apoptosis
2- increased VEGF/neovascularization
what is the typical scenario for NEC?
premature infant who just started enteral feeds
what is the characteristic Xray finding in NEC?
submucosal gas bubbles (pneumatosis intestinalis)
3 presenting symptoms of NEC
bloody stool, abdominal distention, circulatory collapse
definition of SIDS
sudden, unexpected death of infant under 1 that remains unexplained after autopsy, review of clinical hx and investigation of scene
90% of SIDS cases occur between what ages
2-4 mo
risk factors for SIDS (11)
young mom, smoking, male, premature, multiple, short time between babies, parental drug use, family hx SIDS, hyperthermia, soft surface, prone position
Rh immune hydrops is due to
Rh incompatibility (Rh- mon, Rh+ baby)
infant with immune hydrops will have what additional features?
jaundice and kernicterus (due to hemolysis)
which antigen is the focus of immune hydrops?
D antigen
why is ABO incompatibility now a more common cause of immune hydrops?
effective treatment of Rh with RHOgam
what is the mother/baby problem with ABO incompatibility?
O type mother, A or B type fetus (may have anti-A or B IgG that can cross placenta)
immune hydrops sensitization for Rh vs ABO
Rh- requires
ABO- does not require, can’t affect first pregnancy
most common infection to cause hydrops
parvo B19
most common fetal anemia to cause hydrous
alpha thalassemia (Hb barts)
most common benign tumor of infancy
hemangioma
course for hemangiomas
may enlarge as child gets older, but most spontaneously regress
most common germ cell tumor of children
teratoma
neuroblastomas are composed of-
postganglionic sympathetic neurons
main location of neuroblastoma
adrenal medulla
labs in neuroblastoma
elevated urine VMA, HVA
genetic abnormality of neuroblastoma
amplification of N-myc
prognosis is based on/exception
based on stage (higher=worse), except for 4S (infants under 1)
mutation assoc with retinoblastoma
point mutation on chromosome 13 that inactivates RB supressor gene
patients with germ line mutation are at increased risk for-
osteosarcoma later in life
why is retinoic acid teratogenic? (2)
down-regulation of TGFb
interferes with HOX
ureteroplacental insufficiency will affect growth but spares the-
head/brain
classic triad of congenital rubella
PDA, cataracts, blueberry muffin rash
fetal alcohol syndrome presentation (4)
elongated philtrim, frontal bossing, heart murmur, eye problems
first test to do if women has 3+ spontaneous abortions, followed by
chromosomal analysis (followed by testing for maternal antibodies/coagulopathy)
effect of maternal DM on fetal pancreas
islet hyperplasia
cells that make sufactant
type II pneumocytes
L:S ratio that indicates lung maturity
greater than 2:1
PKU is caused by deficiency of
phenylalanine hydroxylase
presumed precursor to wilms tumor
nephrogenic “rests”
NT involved in SIDS
serotonin (respiratory center in the medulla)
