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Path Pedatric Disease
KYCOM Block 10
82
Pathology
Graduate
09/29/2013

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Cards

Term
what arethe 7 types of congenital abnormalities, name one example that goes with each
Definition
malformations- polydactly
disruption- amniotic bands
deformation- oligohydraminos
sequence- abberation in oogenesis
malformation syndrome- trisomy 13
agenesis- missing orgsn
atesia- hollow organ is solid
Term
wht are the three causes of genetic abnormality
Definition
genetic
enivornmental
multifactorial
Term
define malformation
Definition
INTRINICALLY abnormal developmental process
Term
what are 2 causes of malformations
Definition
single gene mutation
multifactoral
Term
define disruption
Definition
EXTRINSIC disturbance results in secondary destruction of an organ or body region
Term
define deformation
Definition
EXTRINSIC disturbance in morphogenesis due to localized or generalized compression of the fetus
Term
define sequence
Definition
multiple congenital abnormalities that result from secondary effects of a signle localized abberation in organogenesis
Term
define malformation syndrome
Definition
presence of several defects from a single causation
Term
define agenesis
Definition
complete absence of an organ or its anlage
Term
define atresia
Definition
absence of an opening, commonly by enterance of exit
Term
oligohydraminosis: aka, define
Definition
potter syndrome

too little amniotic fluid causes generalized compression of fetus and other complications
Term
what complications are caused by oligohydraminosis (4), exlain each
Definition
permanent contracted position: grew in bad position, altered faces, positioning of hands and feet

breech presentation: baby has no room to flip head first for delivery with no fluid

pulmonary hypoplasia: most common, lungs need practice breaths with fluid to mature and are not ready by delivery due to too little fluid

aminion nodosum: plaque like lesions in amnion
Term
what caues oligohydraminosis (3)
Definition
anything that effects the production of pee

no kidney, obstruction, leaking amniotic fluid
Term
what is the most important factor in enivornmental insult to babys development
Definition
timing, where the baby is at in development
Term
what is the LNMP for the first week of baby's development
Definition
3 weeks before

LMNP wk3 = baby wk1
Term
what is the consqeuence of environemtal exposure in LNMP weeks 3 and 4
Definition
all or nothing. no abnormalities will form, it anything happens it will terminate the pregnacy.

usually not susceptible to teratogens at this time
Term
when do enivornmental factors get more sensitive for developing baby, why is this a concern clinically
Definition
LNMP week 5. because the LNMP was not that fat away, mom could just think oh I'm a day late and take a pregnacy test and at this time it could still be negative so she goes out drinking thinking she isnt pregnant and a few weeks later she takes a test and its positive..
tell people trying to get pregnant not to drink at all.
Term
how do teratogens (drugs, alcohol) cause problems with baby. what are two examples of drug teratogens
Definition
genes and proteins that regulate morphogenesis may be targets

thalamadide, acutane (retinoic Acid)
Term
what does acutane do to babies, what is it made of
Definition
retinoic acid changes HOX gene expression causing retinoic acid embryopathy
Term
what are the 3 signs of retinoic acid embryopathy
Definition
CNS, cardiac, crainofacial
Term
what does thalamadide do to babies
Definition
helps prevent miscarriage but causes developmental defects
Term
prematurity: classification, relation to birth weight
Definition
gestational age <37 weeks
may have low birth weight but appropirate for gestational age
Term
what classifies low birth weight
Definition
<2500 g
Term
what is the most common cause of neonatal mortality
Definition
premature infants are more suceptible to morbidity and mortality later in life
Term
what are the 4 major risk factors for premature baby birth
Definition
premature rupture of membranes

intrauterina infection causes choriomanionitis

structural abnormalities of uterus, cervic, placenta

multiple gestation
Term
what are common complications in baby when premature (3)
Definition
hayline membrane disease (respiratory distress syndrome)
necrotizing endocarditis
inraventricular and germinal matrix hemorrhage (brain hemorrhage)
Term
define small for gestational age
Definition
undergrown rather than immature
<2500g full term
Term
what are the later in life risk for babies small for gestational age (3)
Definition
cerebral dysfunction
learning dissabilities
sensory impairments
Term
what are the three factors that determine if a baby will be small for gestational age
Definition
fetal
placental
maternal
Term
what are the 3 things that can happen in the fetus that can make it be small for gestational age
Definition
congenital abnormalities
congenital infections
chromosomal disorders
Term
what are the placental causes of small for gestational age babies
Definition
brain is spared relative to visceral organs (asymmetric distribution)
Term
what are the material causes of small for gestational age babies (4)
Definition
maternal disease
drugs
enivornmental agents
malnutrition
Term
what is the most common cause of small for gestational age
Definition
maternal factors
Term
how can you determine if small for gestational age was due to fetal or placental factors
Definition
fetal: growth retardation will affect all organs symmetrically

placental: brain and heart get more resources to stay alive so their head will be appropirate for their age and their body small with little fat
Term
what is another name for respiratory distress syndrome of the newborn, why
Definition
hayline membrane disease
plasma leaks into alveolar space (mostly the fibrin) can makes glassy appearance (hayline)
Term
what is the most common cause of respiratory distress syndrome in the newborn
Definition
prematurity
Term
what is the pathology of respiratory distress syndrome
Definition
immature lung type II pheumocytes are not developed enough and cannot produce surfactant
Term
what are other causes of respiratory distress syndrome other than prematurity (3)
Definition
maternal diabetes
C-section before labor initiates
multiple gestation
Term
why does diabetes cause respiratory distress syndrome
Definition
insulin inhibits surfactant production
Term
what does a C section before labor initiates increase respiratory distress syndrome, what can we do to fix this
Definition
cortisol from labor stress increases surfactant
inject cortisol into mom when thought to be premature
Term
why does the plasma leak in respiratory distress syndrome
Definition
decreased surfactant causes underperfusion and hypoventilation, hypoxemia causes acidosis and pulmonary vasoconstruction and hypoperfusion which causes endothelial and epithelial damage and allows for leaking
Term
what has the incidence of respiratory distress syndrome went down
Definition
exogenous surfactant was produced and can now be administered
Term
when a baby has respiratory distress synrome and exogenous surfactant isnt good enough, then what, what are the complications (2)
Definition
put on ventilator

causes retinopathy of prematurity and bronchopulmonary displasia
Term
retinopathy of prematureity: aka, cause
Definition
retrolental fibroplasia

hyperoxic phase of therapy decreases VEGF that stops vessel growth but then when removed from ventilation it causes rebount of VEGF and abnormal growth of the vessels in the retina
Term
what are the signs of retinopathy of prematurity (4)
Definition
abnormal vessels are fragile, bleed, cause scaring, cause detachment
Term
what qualfieies that someone has bronchipulmonary dysplasia
Definition
neonate <32 wks needing 28d+ of oxygen therapy
Term
what is the cause of bronchopulmonary displasia
Definition
increased alveolar number and decrease in alveolar cells (hypoplasia) due to proinflammatory cytokines released in infants (TNF, IL-1)
Term
what treats/reduces bronchopulmonary displasia (3)
Definition
gentiler ventilation
prophylactic surfactant
glucocorticoid therapy
Term
who gets necrotizing enterocolitis (2)
Definition
premature and very low birth weight babies (<1500g)
Term
what are the causes of necrotizing enterocolitis (3)
Definition
intestinal ischemia
bacterial colonization of the gut
formula feeds
Term
what part of the gut is involved in necrotizing enterocolitis (3), what is seen there (3)
Definition
terminal ileum, cecum, right colon

distension, congestion, gangrenous, submucosal gas bubbles (see on x-ray), ulceration, bacterial colonization
Term
necrotizing enterocolitis: when does it show up, what are the signs (2)
Definition
10-12 days after birth

rapid progression of bloody stool and distension of abdomen
Term
why does the abdomen distend in necrotizing enterocolitis
Definition
bacteria in intestine wall make gas and inflammatory products
Term
what lab signs can be seen for necrotizing enterocolitis, what are the clinical complications of this sign
Definition
pneumatosis intestinalsis (gas within intestine wall)

can perforate intestinal wall and cause death
Term
what is the complication in infants who survive necrotizing enterocolitis
Definition
post-necrotizing enterocolitis from fibrosis from healing
Term
what qualifies sudent infant death syndrome
Definition
sudden death of an infant <1yo unexplained after case investigation, autopsy, examination of death scene, and review of clinical history
Term
what are the three venues of cause fo SIDS
Definition
vulnerable infant
homeostatic mechanisms not completely developed
exogenous stressor
Term
why is the infant vulnerable in SIDS
Definition
have delayed development or arousal and cardiorespiratory control
Term
what part of the brain is not developed enough stopping homeostatic regulation and causig SIDS
Definition
arcuate nucleus
Term
what exogenous stressors can cause SIDS (3)
Definition
hypercarbia, hypoxia, thermal stress
Term
what are some common findings seen in kids with SIDS (4)
Definition
petechiae on thymus, epicardium visceral, parietal pleura
congested lungs with or without pulmonary edema
Term
what are 7 risk factors for SIDS
Definition
young maternal age <20yo
drug abuse in either parent
short intergestational intervals
brain stem abnormalities
defective aroursl and cardiorespiratory control
prematurity/low birth weight
prone sleep position
Term
define fetal hydrops
Definition
fetal accumulation of fluid during intrauterine growth
Term
what are the levels of severity of fetal hydrops (3), define each
Definition
hydrops fetalis: progressive generalized edema, usually fatal

cystic hygroma: postnuchal fluid

localized edema: pleural, peritoneal, etc
Term
what are the two causes of feral hydrops
Definition
immune and non-immune
Term
what are the 3 major causes of non-immune fetal hydrops, give examples
Definition
cardiovascular defects
chromosomal anomalies- turner syndrome
fetal anemia: homozygois a-thalassemia, infection with parvovirus B10
Term
what is a common cases of immune hydrops
Definition
antibody induced hemolytic disease of the newborn
Term
what is the cause of antibody induced hemolytic disease of the newborn (2)
Definition
mother and fetal blood groups are incompatiable

Rh- mom and Rh+ fetus ("D antigen")

type O mom with type A/B fetus (mild)
Term
how does hemolytic disease of the newborn work
Definition
maternal antibodies cross placenta and destory fetal RBC promoting IgM production which cannot cross placenta
baby is born and mom makes IgG
second pregnacy baby is Rh+ and IgG crosses placenta
Term
why is hemolytic disease of the newborn not an issue, how does it work
Definition
RhoGam: give mom antibodies to Rh to prevent sensitization. RBC from baby are coated with antibody and mom will never been sensitized
Term
what can happen if hemolytic disease of the newborn goes untreated (6)
Definition
congestive heart failure

erythroblastosis fetalis: many immature RBC in circulation

hepatosplenomeagly: liver makes more RBC to replace destoried ones by mom's IgG, spleen cleans up destoried RBC

jaundice due to hyperbilirubinemia second to hemolysis increasing bilirubin

kinicterus: bilirubin accumulates in brain parenchyme
Term
what are the 4 main areas of the body CF affects, name two others
Definition
sweat glands, intestine, pancrease, airway

vas deferens, epididmis, other
Term
how do we find out a lot of babies have CF
Definition
mecenium ileus (first baby poo) is very thick and sticky and baby cannot get it out

98% of meconium ileus is caused by CF (not all CF mas meconium ileus issues)
Term
CF: inheritence, severity determination
Definition
autosomal recessive even though it is structural

varies in severity on severity of mutation given from each parent
Term
what is the cause of CF
Definition
mutation in the gene that makes protein for Cl transport
Term
what happens in the sweat cells normally and in someone with CF
Definition
normally NaCl goes out of the lumen into the sweat cell

in CF: Cl cannot get back into the sweat cell so Na hangs out in the lumen with it increasing Na excretion
Term
what happens in the intestines, pancreas, and airway normally and in someone with CF
Definition
normally: Cl enters the airway and Na and water enter the cell

in CF: Cl cannot enter the airway so water and Na super leave the airway and enter the cell making the secretions thick and stopping cillia movement
Term
what is the clinical complication of CF intestine issues
Definition
decreased absorption of nutrients and failure to thrive
Term
what is the clinical complication of increased lung viscosity in CF
Definition
increased infection
Term
what is the clinical complications of the problems in the pacrease in CF (3)
Definition
fibrosis of the pancreas
loss of islets causes diabetes
Term
what is the most common cause of death in someone with CF
Definition
cardiopulmonary complications
Term
what is the initial test for CF diagnosis
Definition
sweat Cl analysis
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