Disease of Infancy and Childhood 1 Congenital Anomalies

Question 1

what are the four time spans of development and different disorders?

Answer 1

Each stage of development of the infant and child is prey to a somewhat different group of disorders. The data available permit a survey of four time spans: (1) the neonatal period (the first 4 weeks of life), (2) infancy (the first year of life), (3) age 1 to 4 years, and (4) age 5 to 14 years.

Question 2

cause of death related with age

<1

1-4

5-9

10-14

Answer 2

Question 3

what are congenital anomalies?

most common cause of mortality when?

Answer 3

Question 4

definitions

malformations? result of?

disruptions? arise from? ex? heritable?

deformations? fundamental to path of defomations? period of growth? maternal factors? fetal/placental?

Answer 4

Question 5

definitions

a sequence? example?

malformation syndorome? similar to? ex?

Answer 5

Question 6

definitions

agenesis?

aplasia?

Atresia?

Answer 6

Question 7

causes of congenital anomalies in humans

genetic? highest frequency?

environ? highest frequency?

Answer 7

Question 8

causes of anomalies

three major categories?

g- what syndromes associated? ex? underlie major malformations?

ex?

Answer 8

Question 9

causes of anomalies

environmental influences- such as?

multifactorial inheritance? iplies? ex? underscored by?

Answer 9

Question 10

what are the major growth times of organs?

CNS?

heart?

arms?

legs?

eyes?

genetalia?

Answer 10

Question 11

pathogenesis

two general principles of develop path? 1st

intrauterine develop phases 2

susceptible? peak? whats being made?

Answer 11

Question 12

pathogenesis

two general principles of develop path? 2nd

ex 3

c- what is it? cause?

v- what is it? cuases?

va- essentail for? defect? 2

Answer 12

Question 13

prematurity defined as? at risk for?

major risk factors for prematurity include?

4

Answer 13

1. Preterm premature rupture of placental membranes (PPROM):
2. Intrauterine infection
3. Uterine, cervical, and placental structural abnormalities:
4. multiple gestation

Question 14

risk factors of prematurity

PPROM: common? PPROM vs PROM important why? risk factors for it?

Intrauterine infection: major cause of? most common one? key players?

Uterine, cervical, and placental structural abnormal:

multiple gest?

Answer 14

Question 15

prematurity for the newborn may give rise to the following: 4

Answer 15

Question 16

fetal growth restriction

undergrown is under what weight?

SGA means? suffer from? result from what? 3

Answer 16

Question 17

FGR

fetal abnormalities: what are they? such as? show what type of growth restriction?

placental abnormalities: stems from? type of growth restriction?

Answer 17

Question 18

FGR

maternal abnormalities: including?

FGR infant hardships?

Answer 18

Question 19

most common cause of respiratory distress in newborn? AKA?

why the name?

untreated infant presentation? weight? association with? may be necessary at birth? 30 min after? imaging? fail to improve situation? situation improves after?

Answer 19

Question 20

Pathogenesis of RDS

most important substrate it develops upon? RDS to gestational age? fundemental defect in RDS? protein help reduce surface tension?

pressure to breath for first breath? with low what it keeps this? hyaline forms how?

surfactant synthesis and hormones? most important?

synthesis suppressed by?

labor vs c-section?

Answer 20

Question 21

RDS morphology

lungs on exam? look? density?

microscopically? not developed? in dead infants? made up of?

survive after 48 hrs?

Answer 21

Question 22

Question 23

RDS clinical features

prevention via?

how do you estimate surfactant?

ventilator complications? 2

r- phase I /II

b- what do you see?

other complications? most importantly?

Answer 23

Question 24

Necrotizing enterocolitis

most common with? risk vs age? how common? in what demographic? pathogenesis is?

cases associated with?

important mediator? does what? see higher levels where?

breakdown of what? causes?

clinical course?

Answer 24

Question 25

necrotizing enterocolitis morph what parts of intestines affected? these segments look? micro- what do you see? reparative? treatment?

Answer 25

Question 26

perinatal infections acquired via what routes? 2 ascending (aka): type of infections? fetus acquires infection via?

Answer 26

Question 27

perinatal infections hematologic (aka): type of infections? via? ex?

Answer 27

Question 28

perinatal infections sepsis two groups? acquired when? most common cause?

Answer 28

Question 29

Fetal Hydrops what are they? when? 2 types?

Answer 29

Question 30

immune hydrops waht is it? major antigens known? underlying basis? when fetal blood cells? type of antibodies? Rh major antigen?

Answer 30

Question 31

factors influence immune response to Rh+ fetal red cells that reach maternal circ: 2 incidence improved since? pathogenesis in ABO? antibody type? firstborn? protection of ABO?

Answer 31

Question 32

Two consequences of excessive destruction of red cells in neonate? 2 a- mild? severe? lead to? j-develops from? can cause?

Answer 32

Question 33

nonimmune hydrops 3 major causes? rapidly developing as an emerging cause? 10% due to?

Answer 33

Question 34

Hydrops- morph most severe manifestation? 3 outcomes? fetal anemia you would see? and also see what in plasma? most serious threat is? look of this?

Answer 34

Question 35

hydrops clin features minimally affected infants display? gravely ill? treatment?

Answer 35