Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
106 Cards in this Set
- Front
- Back
|
Gestation
|
37-42 weeks
expected birth weight 2500 grams |
|
|
define premature
|
infant born before 37 weeks
|
|
|
define postmature
|
infant born after 42 weeks
|
|
|
does a LBW mean an infant is premature
|
no
|
|
|
SGA typically have
|
intrauterine growth restreiction.
|
|
|
to use a nomogram
|
you need accurate assessment of maternal dates
|
|
|
What type of complications develop as a consequence of prematurity
|
1. RDS
2 NEC 3/ Subependyme hemorrhage 4. Periventricular leukomalcia |
|
|
symmetric growth restriction
|
when there is proportional reductoin in the size and weight of all organs.. including the brain
|
|
|
what are the causes of symmetric growth restriction
|
they are intrinic to the developing fetus....CX DISORDER/MALFORMATION SYNDROM/CONGENTIAL INFECTION
AND THE IMPACT FETAL GROWTH OCCURS EARLY IN GESTATION |
|
|
asymmetric growth restriction
|
when the brain is spared relative to other organs...
|
|
|
what causes asymmetric growth restriction
|
comprimise of ureteroplacental blood supply or maternal factors such as chronic HTN, severe malnutrition and heavy smoking
|
|
|
assymetric growth restriction has its greatest impact on..
|
late in gestation.. thus thats why its sparing of the brain.
|
|
|
growth restricted infacnts are at increased risk for
|
hypoglycemia, hypothermia and nerulogic dysfxns such as learning and hearing or visual impairment.
also at increased risk of adult onset Dbs and obesity... HTN and CAD |
|
|
What are the risk factors for prematurity
|
PROM
ACUTE CHORIAOAMNIONITIS MULTIPLE GESTATIONS STRUCTUAL ABNORMALITIES OF THE UTERUS OR PLACENTA |
|
|
RDS IS INVERSLY PROPORTIONAL TO
|
GESTATIONAL AGE
|
|
|
WHAT FACTORS INCREASE THE INCIDENCE OF RDS
|
MATERNAL Dbs
TWIN GESTATOINS C-SECTION prior to the onset of labor. |
|
|
IRDS
|
different from regular RDS which is caused HMD. IRDS is casued by respiratory distress in the neonate, such meconium aspirate syndrome, that results from acute intrauterin hypoxia or intrauterine pneumonia
|
|
|
RDS is a defienciy of what
|
surfactatn. which is made by type 2 pneumocytes
|
|
|
surfactant is primarly made up of
|
dipalmitoyl lecithin... and and several associated surfactant proteins SPA SPB SPC
|
|
|
surfactant syntheiss occurs duirng what time
|
during the third trimester... and it increases rapidly after 37 weeks.
|
|
|
what happens if surfactant defiecinet
|
the patient has to increase work of breathing to open and maintan the patency of the alveoi. ou can get aleoli collapase
|
|
|
alveoliar collapse produce a charatesitic type of pattern on chest xray
|
fine granelar patter.. goundglass
|
|
|
why RDS CALLED HMD
|
b/c hypoxic injruy to the aveolar epi and endo allows plasma to leak from the capillaires into the alveoli, whre it mixes with necrotic cellular debire to form fibrin rich pink-staining membranes along the surface of alveolar dcts and small respiratory bronchioles
|
|
|
pulmonary atelectasis requires
|
it requires increased work of breathing b the infacnt that is manifested clinically by gruntin, tachypnea, and nasal flaring and intercostal retractions.
|
|
|
how to treat RDS
|
artificial surfacant... cirulating corticosteroids stimulare surfactant production.
|
|
|
Can NEC occur in term infants..
|
yes but is largley a dx of prematurity
|
|
|
NEC is inversly proportional to
|
gestational age
|
|
|
What is the pathophys of NEC
|
bowel ischema with secondary bacteral overgrowth is the suspected casue abbetted by varous cytokines including TGF-beta and Platelet Activation Factor
|
|
|
what may contribute to te development of NEC in infants
|
early formula feeding..
|
|
|
what part of the bowel are the most commonly affected
|
distal smal bowel and proximal colon.. and the reason for this is b/c probably these areas are more sucetpbiel to hypotension
|
|
|
in NEC coagulative necrosis involves
|
it invovles the mucosa and may etnd o inovle the entire thickness of the wall
|
|
|
In NEC where do bacteral proliferate
|
in necrotic tissue and produce gas.. and its visbe as gas bubbles in the bowell wall
|
|
|
in NEC, when gas is oberved in the portal vein...
|
its is a higly advers prognostic sign.
|
|
|
Subependymal hemorrhage occurs in what part of the brain
|
this area overlies the basal ganglia at the base of the lateral ventricls in premature infants and is composed of immature neruonal matrix
|
|
|
what is immature (germinal) neruonal matris
|
these conatain delicate veonlus channels that can be damaged when there is increase intravascular pressure when systemic stresses increase systemic blood pressure and or cause cereral venous congestion in prematures.
|
|
|
what is periventrcualr leukomalacia?
|
it is important cerebral complication that can occur uring prematurity...
|
|
|
what are some of the long term consequences of periventricular leukomalcia
|
cerebral palsy
|
|
|
why do you get periventricular leudomalcia and where in the brain
|
it occurs in the deep hemispheric white matter of the frontoparital regions is a zone of marginal perfusion in prematures.. you can get reduced oxygen delievery to this area resulting from hypotension.. and hypoxemia...
|
|
|
malformation
|
is defect of morphogeniss that resutls from an intrinsically anormal developmentla process
|
|
|
syndrome
|
a recuring group of malfomrations that is thought to have a single underlying cause
|
|
|
most single malformations are..
|
multifactorial
|
|
|
a malformation syndrome is thought to have
|
been du to a chromosmal abnormality
|
|
|
disruption
|
it resluts from a secondary event that damages an intrinsically normal developing structure
|
|
|
a common example of disruption is
|
intestinal atreisa
|
|
|
are disruptions localized
|
yes
|
|
|
deformation
|
is a congetnial anomaly caused by distortion of an otherwise normal struture by mechanical formes and is usually develops late in gestations....e.g CLUB FOOT
|
|
|
defomration is most often caused by...
|
it is most often caused by uterine constraint on the fetus resulting from reduced amniotic fluid in the 3 rd trimester (oligohydramnos)
|
|
|
what is an importnat component of amniotic fluid
|
fetal urine
|
|
|
what are some conditions that reduce fetal urine outupt
|
genitourinary anomalies are an imortant cause of olgohydramnios
|
|
|
sequence
|
is used for cascade of anomalies that follows secondarily from oligohydramnios regardless of its cause and includes
1. equinovarus deformity of the feet 2. spade like hands 3. dysmorphic facies 4. pulmonary hypoplasia |
|
|
SIDS
|
IS DEFINED AS A THE SUDDEN UNEXPECTED EATH OF AN INFANT LESS THAN ONE YEAR OF AGE. with onset of the fatal episode apparently occuring during sleep that remains unexplained after a thorough death scene investigation
|
|
|
85% OF CASSES OF SIDS OCCUR B/W WHAT AGES
|
2-4 months
|
|
|
SIDS is a diagnoisis of ...
|
exclusion
|
|
|
What is the triple risk model in regards to SIDS
|
1. subtue structual abonrmalities in the ventral meddulla (arucate nucle).. that are parts of the brain that affects sleep arousal and cardio resp. regulation
2. external stressors such as a prone sleep positiion, hypethermia/ minor infection or passive smoke inhalation 3. a critical windo of cardiorespiaratory vulnerability in the developing infant that peaks b/w 2-4 months |
|
|
what is the back to sleep program
|
it is a program designed to redicne the incidence of sids by have the babying lye on there back.
|
|
|
what are some the paternal factors that increase the risk of SIDS
|
1.young maternal age
2. maternal smoking during preg. 3. paretnal drug abuse 4 short inter-gestational intervals 5. poor prenatal care 6. low socioeconmomic status 7. AA and american indian |
|
|
WHat are factors in the infant that increase the risk of SIDS
|
1. preamturuity or small ofr gestatinoal age.
2 male sex 3. Product of multpie gestations 4. SIDS IN PRIOR SIBLINGS 5. ANTECEDENT RES. IFN 6. PRONE TO SLEEPING POSTIIONS 7. STRUCTUAL ABONR. IN BRAIN STEM. |
|
|
what are some of te postmortem findings of SIDS
|
congestion in lungs, and petechia on the pleurla suruface, thymus and epicar
|
|
|
what is more common bening tumors or malingant tumors
|
benign
|
|
|
what is a heterotopia
|
is a focus of normal appearing tissue located in a abnormal site.
|
|
|
what is a harmatorma
|
it is a a mass formed by localied overgrowth of one or more mature tissue components native to the site in which it is foud but lacking normal orgnization sturcture.
|
|
|
what is the difference b.w a hemangioma and vascular malformatoin
|
the former appears as a blush on a skin of a neonate that grows rapidly over several months into a distinct mass and then involutes slowly over the ensuing years. it isintally highly celluar with increased mitotic activity but the cellularity diminhes over tim and the capillaires regress with replacement of fibroadpiose tissue.
|
|
|
in evaulating hemangioma or in vascualr malformation what best way to approach this dx
|
a watch and wait approach..
|
|
|
what is a vascular malformatoin
|
it is a harmartoma that grow slowly in conert with teh child can can cause significant disfigurment
|
|
|
lyphatic malformatoin (lymphangioma)
|
these are common during child hood. they are harmartomas rather than true neoplasm.
|
|
|
tertatomas
|
with the exception of gonaldal lesions that occur in odler children and adults, teratomas are located in the midline from the head to the coccyx, possible the result of incomplete migration of some germ cell precurosr cells during embroygensiss.
|
|
|
a common site for a tertoma in young female infants is ..
|
the sacral region... sacrococcygeal teratoma...
|
|
|
termotomsa contain what type of tissues
|
these tumors contain a varitey of tissue derived from all three embyronic germl layers..endoderm.. mesoderm.. and ectoderm.
|
|
|
when a frankly malignat componet is also present with a tertoma it is almost always...
|
yolk sac tumor... and they are very sensitive to chemo....
|
|
|
whats the difference b/w a malignat tumor in adult an that of a child...
|
1. sites and cells of orgin are different
2. often rcaptiulate embyonal development. 3. moare likely to regress or mature 4. have a overall higher cure rate 5. progonosis can vary by the age of the child.... 6. getntic aberrations are well orgnanized. |
|
|
what is the largest group of pediatirc malignancies
|
lymphomas and leukemias.. then its brain tumors .. and neuroblatoma... wilms tumor
|
|
|
neuroblastoma
|
it is the most common solid tumor of infancy and childhood outside the CNS
|
|
|
over half of the neuroblastomas are..
|
abdominal, and halof of those arise in the adrenal gland..
|
|
|
extra-adrenal neuroblastomas tend to be ...
|
paraspinous and arise from either the sympathetic gangila or organ of zuckerkandal
|
|
|
neuroblastomoa tend to arise from
|
primintive nerual crest cells that are destineng to for the SNS
|
|
|
what is the morpho of immature nerublastoma
|
soft and dark reddish grey, often with puctate white foci of calcification. they also contain HOMER-WRIGHT PSEUDO-ROSETTES
|
|
|
WHAT ARE HOMER-WRIGHT PSEUO-ROSETTES
|
rossettes of tumor cells surrounding mall aggregates of fibrillar pink neuropil.
|
|
|
as the neruoblasts matture some will differatie into what
|
ganglion cells or into schwann cells
|
|
|
ganglionerublastoma
|
is the nerm used for tumor sthat contain both mature and immature elemtetns
|
|
|
ganglineruoma
|
only contain mature ganglion cells and schwannian stroma
|
|
|
the prognossi of neruoblastoma depends on the ..
|
the age of the patient and the stage of the dx
|
|
|
infants < 1 year of age with neuroblastoma have
|
good prognoisis
|
|
|
over 90 % of neruoblastomas produce one or more what
|
catecholamine precursors: epi VMA, HVA. serum or urine levels can be measured and are usefull for making the intal diagnossi and for monitiring therapy.
|
|
|
Wilms tumor
|
most common renal tumor in childhood
|
|
|
wilms tumor is most frequent in what age group
|
2-5 years and usually presents as a an abdominal mass discovered by the parent.
|
|
|
what are some of the underlying syndromes of wilms tumor
|
BECKWITH-WIEDEMANN SYNDROME
WAGR SYNDROME DENYS-DRASH SYNDROME ALL OF WHICH ARE ASSOCIATED WITH MUTATOINS ON CX 11P WHERE THE WT1 GENES RESIDES. |
|
|
IF A PERSON HAS A WILMS TUMOR BUT HAS A NORMAL WT1 GENE WHERE IS THE LIKLEY MUTATION
|
ON THE WTX ON THE X CX
|
|
|
What is the morpho of wilms tumor...
|
is large spherical and sharply circumscribed from the adjacent kidney by a fivrous pseudocapsule
|
|
|
what is the classic histo app.of wilms tumor
|
it ia a triphaasic embroyanl neoplasm with blastemal, epi, and stromal compartments
|
|
|
wilms have a high rate of mitotic activit and with prominent apoptosis and that means...
|
they have an excellent response to chemo.
|
|
|
what is te most common site of distant mets..
|
is the lung
|
|
|
Rhabdomyosarcoma
|
is a soft tissue sarcoma of childhood. they mostly occur during the first decade of life.
|
|
|
what are the common sites affetet by Rhabdomyosarcoma
|
head and neck... then genitourinary tract. and then the extremites
|
|
|
embyronal rhabdomyosarcoma
|
over 60% of all rhabod are classified as this... it is sheet of moderately pleomorphic hyperchromatic mall cells witha focla or diffuse myxoid quality ... tadpole cels ith densely eosinophilic cytoplasm may give you a big clude
|
|
|
botryoid rhabodmsarcoma
|
these are embyonal tmors that arise beneath an epi sruface such as the bladder or vagina and project into the lumen as a grape like mass
|
|
|
alveolar rhaomysarcoma
|
has a lesser prognosiss that rhabdomyosarcoma...
|
|
|
alveolar rhabdo is frequently associated with what transloacation...
|
FKHR GENE ON CX 13 AND EITHER PAX3 ON CX 2 OR PAX7 ON CX 1
|
|
|
RETINOBLASTOMA HAS A PEAK INCIDENCE OF
|
18-24 MONTHS
|
|
|
TUMOR DEVELOPMENT OF RETINOBLASTOMA RESUULTS
|
FROM LOS OF HETEROZYGOSITY OF THE RB TUMOR SUPPRESSOR GENE...
|
|
|
knduoson two hit model is from
|
retionblastoma
|
|
|
retinoblastoma has both spordiac and familarl fomrs
|
l
|
|
|
30% of retion blastomas are.... while 70% are...
|
bilater..unilateral
|
|
|
familial retionblastomas tumors are often..
|
develop in earlir age <1 yer of age and are more often bilateral.
|
|
|
what is the common presentation of retionblasotma
|
white pupil (leukocoria)
|
|
|
flexner wintersteiner rosettes
|
can see this in retinoblastoma..... true rossettes with a central lumen
but you can see HOMER=WRIGHT PEDOROSETTES WICH LACK A CENTRAL LUMEN. |
|
|
THE MOST OMMON MET. SITES OF RETINOBLASTOMA ARE
|
CNS, SKULL, DISTAL BONES, AND LN
|
