Diseases of Infancy and Childhood I Flashcards Preview

PBD II Exam 1 > Diseases of Infancy and Childhood I > Flashcards

Flashcards in Diseases of Infancy and Childhood I Deck (64):
1

exam

8 brandau
rest is 1/3 each from everything else

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neonatal period

first 4 weeks

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infancy

first year of life

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four time spans of development

neonatal - first 4 weeks
infant - first 1 year
age 1-4
age 5-14

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leading causes of death in first 1 year of life

congenital anomalies
prematurity
SIDS

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leading cause of death 1 - 14 years

accidents

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leading cause of childhood deaths

malnutrition
infections
emerginy disease

rates higher in africa than europe and US

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10ug/mL of Pb in children

development toxicity
-decreased IQ
-decreased hearing
-decreased grwoth


10x this causes death

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microcytic anemia

iron

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macrocytic anemia

B and folate
pernicious anemia

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basophilic stippling

lead intoxication

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lead intoxication in adults

-basophilic stipling in RBCs (blue dotted cells)
-peripheral demyelination neuropathy
-microcytic hypochromic anemia

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evolution and diet

chronic inflammation - sugars

recommend - high fat and high protein diet

gluten is bad - sugar

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congenital anomaly

morph defect present at birth

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malformation

error of morphogenesis - intrinsic abnormal development

usually multifactorial cause

ex/ cleft palate, cleft lip, polydactylyl, syndactylyl

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disruption

secondary destruction of organ or body region that was previously normal in development

ex/ amniotic band

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amniotic band

disruption

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deformation

extrinsic disturbance in development
-localized or generalized compression of fetus by abnormal biomechanical forces

ex/ uterine constraint

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sequence

cascade of anomalies trigered by initiating aberration

ex. potter sequence

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potter sequence

oligohydroamnios

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perinate

around time of birth

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neonate

first 4 weeks after delivery

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infant mortality

has declined in US
-but not in all ethnic groups

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SIDS diagnosis

need to be under 1 year old

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rubella

early infection

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cytomegalovirus

second trimester
-later infection

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common neoplasm in children

B cell ALL

leukemia

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maternal factors

first pregnancy
small uterus
bicornuate uterus
leiomyoma

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fetal or placental factors

oligohydramnios
multiple fetuses
abnormal fetal presentation

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causes of oligohydramnios

leak of amniotic fluid
maternal HTN
renal agenesis

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presentation of potters

flattened facies
position abnormal
dislocated hips
pulmonary hypoplasia
amnion nodusum

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syndrome

constellation of congenital anomalies that cannot be explained by single initiating defecdt

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agenesis

complete absence of organ with associated absence of its primordium

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aplasia

absence of organ due to failure of development of primordium

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atresia

absence of an opening

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most common development defect of forebrain and midface in humans

holoprosencephaly

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single gene mutations leading to congenital anomalies

autosomal dominant or recessive 90%

remainder X-linked

many are loss of function mutations

ex. holoprosencephaly - loss of function in hedgehog pathway

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achondroplasia

short limb dwarfism

gain of function
FGFR3

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diabetic mothers

large babies

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rubella

risk period is conception to 16th week (greater in first 8 weeks)

tetrad - cataracts, heart defects, deafness, retardation

congenital rubella syndrome**

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cytomegalovirus

infection during second trimester

-mental retardation, microcephaly, deafness, hepatosplenomegaly

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organogenesis

during first 9 weeks

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drugs causing congenital malformations

thalidomide, folate antagonists, androgen hormones, alcohol, anticonvulsants, warfarin, 13-cis-retinoic acid

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thalidomide

tranqulizer that causes congenital abnormalities

-limb abnormalities
-downregualtion WNT signaling by increasing WNT repressors

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FAS

fetal alcohol syndrome

growth retardation
microcephaly
atrial septal defect
short palpebral fissures
maxillary hypoplasia

disrupts retinoic acid and hedgehog

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maternal diabetes

leads to fetal hyperinsulinemia
-increased fat, muscle mass, and organomegaly (fetal macrosomia)
-cardiac anomalies, neural tube defects, CNS malformations

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fetal macrosomia

organomegaly from maternal diabetes

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embryonic period

first 9 weeks

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fetal period

9 weeks to birth

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high susceptiblity to teratogenesis

week 3-9

peak sensitivity - week 4-5

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cyclopamine

inhibit hedgehog pathway

holoprosencephaly

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valproic acid

anti-epileptic
-teratogen

-disrupts HOX genes
-abnormal limbs, vertebrae, craniofacial structures

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vit A deficiency during pregnancy

malformed eyes, GU, cardiovascular, diaphragm, lung

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vit A excess during pregnancy

CNS, cardiac, craniofacial defects
-cleft lip
-cleft palate

TGF-beta signaling defect

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preterm

before 37 weeks

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normal pregnancy

38-40 weeks
-266-280 days

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appropriate for gestational age

AGA
-10th to 90th percentile

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post term

born after 42nd week

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prematurity

before 37 weeks
-second most common cause of neonatal mortality

first - congenital anomalies

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PPROM

preterm premature rupture of placental membranes
-occurs before 37 weeks

risk factor of prematurity

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major cause of preterm labor

infections**

chorioamnionitis and funisitis

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funisitis

inflamed umbilical cord

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chorioamnionitis

inflamed placental membrane

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common intrauterine infections

ureaplasma urealyticum, mycoplasma hominis, gardnerella vaginalis, trichomonas, gonorrhea, chlamydia