TOPNOTCH

Question 1

APGAR: Order of disappearance in a sick baby

Answer 1

APPEARANCE
RESPIRATION
ACTIVITY
GRIMACE
PULSE

Question 2

APGAR: Order of appearance in a resuscitated baby

Answer 2

PULSE
APPEARANCE
RESPIRATION
GRIMACE
ACTIVITY

Question 3

EINC Core Steps

Answer 3

Immediate and thorough drying
Early skin to skin contact
Properly timed cord clamping
Non separation of mother and baby

Question 4

Benefits of early skin to skin contact

Answer 4

Breastfeeding success
Lymphoid tissue system stimulation
Exposure to maternal skin flora
Sugar (protection from hypoglycemia)
Thermoregulation

Question 5

Newborn screening test

Answer 5

Done at 48HOL

Congenital Hypothyroidism
Congenital Adrenal Hyperplasia
Galactosemia
Glucose 6 Phosphate Dehydrogenase Deficiency
Phenylketonuria
Maple Syrup Urine Disease

Question 6

Prolonged physiologic jaundice (earliest possible physical sign)

Answer 6

Congenital Hypothyroidism

Question 7

21 hydroxylase enzyme deficiency (cortisol deficiency)
Low serum Na, Cl, cortisol, high K
Increased serum 17 OHP

FEMALES: pseudohermaphrodism

Answer 7

Congenital Adrenal Hyperplasia

Question 8

Distinct enzyme deficiencies in galactosemia

Answer 8

Galactose 1 phosphate uridyltransferase deficiency  (GALT)
Galactokinase deficiency (GALK)
Galactose 4 epimerase deficiency (GALE)

Question 9

Increased risk for E. coli neonatal sepsis

Answer 9

Galactosemia

Question 10

Deficient enzyme in phenylketonuria

Answer 10

Phenylalanine hydroxylase

Question 11

Genetic metabolic disorder resulting from the defective activity of the enzyme branched chain alpha keto acid dehydrogenase complex

Answer 11

Maple Syrup Urine Disease

Question 12

Treatment of MSUD

Answer 12

Removal of sources of essential amino acids (leucine, isoleucine, valine) in diet