Choudhury Review Material

Question 1

enzymes in mouth

Answer 1

for taste

Question 2

lipase

Answer 2

only 1 from pancreas **

many amylase and peptidase

Question 3

colipase

Answer 3

removes bile salts to allow action of lipase

Question 4

monosaccharide transport

Answer 4

glucose and galactose - need ATP

fructose - no ATP

Question 5

plica circulares

Answer 5

large folds in small intestine

Question 6

enterokinase

Answer 6

trypsinogen to trypsin

Question 7

sucrose

Answer 7

glucose and fructose

Question 8

lactose

Answer 8

glucose and galactose

Question 9

brush border oligosaccharidases

Answer 9

lactase
maltase
sucrase
isomaltase

Question 10

PepT1

Answer 10

H/oligopeptide cotransporter

Question 11

hartnup disease

Answer 11

altered neutral AA transport

-high protein diet can overcome

Question 12

enterohepatic bile absorption

Answer 12

proximal - diffusion

distal - active transport

Question 13

intracellular binding of bilirubin

Answer 13

non-substrate ligand for glutathione S transferases

Question 14

mutation of MRP2

Answer 14

dubin johnson syndrome

Question 15

G6PD deficiency

Answer 15

pre-hepatic jaundice

-RBC breakdown bc they can’t survive with no NADPH

Question 16

enlarged lymph node

Answer 16

may cause biliary obsturction

Question 17

physiological neonatal jaundice

Answer 17

massive fetal Hg lysis

rapid rise bilirubin risk for kernicterus

Tx phototherapy, phenobarbitone, albumin infusion, exchange infusion

Question 18

maternal fetal blood group incompatability

Answer 18

Rh, ABO
mother fetus blood type incompatible

breakdown of fetus RBC

Question 19

breast milk jaundice

Answer 19

jaundice in breastfeeding newborns

chemicals in breast milk inhibit UDPGT

Question 20

occult blood in stool

Answer 20

suggest cancer of ampulla of vater with post-hepatic jaundice

Question 21

triggers for acute hemolytic anemia

Answer 21

drugs
malaria
fava bean

Question 22

kernicterus

Answer 22

increased unconjugated bilirubin

Question 23

glutaminase

Answer 23

glutamine > glutamate
liberates NH4
in kidney

Question 24

urea formation

Answer 24

citosol

Question 25

ornithine transcarbamoylase

Answer 25

carbamoyl P + ornithine > citrulline
-in matrix

moves to cytosol

X-linked inheritance

Question 26

argininsuccinate synthetase

Answer 26

cirtulline > argininosuccinate
-addition of second amino group

disorder - citrullinemia - autosomal recessive

Question 27

formation of urea

Answer 27

arginine > urea + ornithine

ornithine recycled

Question 28

five enzymes for breakdown of ammonia to urea

Answer 28

carbamoyl phosphate synthetase I
ornithine transcarbamylase
argininosuccinatte synthetase
arginosuccinate lyase
arginase

Question 29

carbamoyl phosphate synthase I

Answer 29

pacemaker
determined by N-acetyl glutamate**
-positive effector

Question 30

starvation

Answer 30

urea cycle enzyme level increases

Question 31

liver cirrhosis

Answer 31

or alcohol

-alters carbamoyl phosphate synthetase activity

Question 32

high glutamine and uracil

Answer 32

hyperammonia

defective ornithine transcarbamoylase
-bc high uracil

Question 33

high uracil

Answer 33

ornithine transcarbamoylase

Question 34

ornithine transcarbamoylase deficiency

Answer 34

X-linked

females affected

high uracil

give less protein in diet

Question 35

low BUN

Answer 35

liver disease

Question 36

high BUN

Answer 36

kidney disease

Question 37

CPS I defective

Answer 37

buildup of carbamoylphosphate

-rare X-linked

Question 38

citrulline levels

Answer 38

product of proximal enzymes OTC and CPS1

substrate for distal enzymes ASL and ARG

Question 39

orotic acid

Answer 39

between CPS1 and OTC

Question 40

comatose baby

Answer 40

mito ornithine transcarbamoylase