Liver Metabolism: Urea Cycle Flashcards

1
Q

formation of bilirubin

A

Hg - to heme and globin

heme > biliverdin > bilirubin (direct)

carried to liver on albumin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

UDPGT

A

uridyldiphosphate glucuronyl transferase
-conjugates bilirubin in liver at SER

adds glucuronide x2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

heme oxygenase

A

heme > biliverdin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

van den berg test

A

measure conjugated and unconjugated bilirubin levels

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

urobilinogen

A

converted in intestine (terminal ileum and colon)
colorless

20% reabsorbed

collection of sterco, meso, urobilinogen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

normal range bilirubin

A

0.2-1

less than 0.2 conjugated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

kernicterus

A

jaundice in infant - bilirubin > 15-20
-brain damage

aka brain encephalopathy

due to polycythemia, Rh incompatibility

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

pre-hepatic jaundice

A

excess hemolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

hepatic jaundice

A

infection, drugs, alcohol, cirrhosis, pregnancy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

post-hepatic jaundice

A

blockage of duct

cancer of bile duct, pancreas head

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

excess hemolysis

A

increased unconjugated and urinary urobilinogen
dark urine
total usually not > 5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

biliverdin reductase

A

biliverdin > biliverdin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

stercobilin

A

stool pigment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

urobilin

A

in urine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

causes of jaundice

A

excess bilirubin production
reduced hepatocyte uptake
impaired conjugation
impaired bile flow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

gilberts syndrome

A

mild unconjugated hyperbilirubinemia
1.5-3

worse with stress
often misdiagnosed - chronic hepatitis

low activity of UDPGT (UGT 1A1 mutation)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

crigler-najjar type I

A

absence of UDPGT
-very high unconjugated bilirubin

autosomal recessive

most patients die of kernicterus
Tx: early liver transplant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

crigler najjar type II

A

autosomal dominant

partial deficiency UDPGT

unconjugated bilirubin elevated 5-20

responds to phenobarbitol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

phenobarbitol

A

Tx for crigler najjar type II

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

dubin johnson syndrome

A

autosomal recessive

elevated conjugated

defective canalicular excretion

liver biopsy - black**

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

rotor syndrome

A

elevated conjugated

liver biopsy - normal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

pepsin

23
Q

trypsin

A

small intestine

24
Q

aminopeptidase

carboxypeptidase

A

small intestine

25
excess AA sources
leftover dietary body protein breakdown
26
transamination
take away amino group (NH2)
27
glutamate
carries nitrogen 1
28
glutamine
carries nitrogen 2
29
pyridoxal phosphate cofactor
PLP | all aminotransferases use this cofactor
30
alpha-ketoglutarate
to glutamate -by aminotransferase takes NH2 from amino acid
31
L-glutamine
storage of NH2 x2 transport in blood to kidney glutamine liberates NH2 groups in liver
32
glutamate dehydrogenase
alpha-ketoglutarate > glutamate
33
glutamine synthetase
glutamate > glutamine
34
carbamoyl phosphate synthetase I
glutamine > carbamoyl phosphate deficiency leads to ammonia in blood
35
ornithine transcarbamoylase
carbamoyl phosphate + ornithine > cirtulline deficiency - cant make urea
36
argininsuccinate synthetase
cirtulline > argininosuccinate
37
argininosuccinate
to fumarate and arginine fumarate to citric acid cycle (makes ATP) arginine to urea + ornithine
38
ornithine
recycled component of urea cycle
39
urea cycle chemical equation
3ATP + HCO3 + NH4 + asp > 2ADP + AMP + 2P + PP + fumarate + urea
40
argininosuccinase deficiency
protein depleted diet | supplement high levels of L-arginine
41
brain swelling
glutamine and glutamate are osmolites in brain hepatic encephalopathy
42
PKU
deficienct phenylalanine hydroxylase no phenylalanine to tyrpsine/Epi phenylalanine to phenylpyruvate (both toxic CNS) mental retardation occurs -common ADHD tested in newborns
43
aspartame
nutrasweet | -has phenylalanine
44
phenylalanine conversion
> tyrosine > L-dopa > dopamine > NE > E
45
BUN
blood urea nitrogen liver and kidney function higher - CHF, GI bleed, hypovolemia, kidney disease, shock lower - low protein diet, malnutrition, over hydration
46
ALT, AST, ALP
elevated - liver damage
47
albumin and total protein
decreased - liver damage
48
GGT
elevated - liver or bile duct damage
49
LDH
elevated - liver damage
50
PT
increased - liver damage
51
MRP2
transport of direct bilirbubin to bile canaliculus
52
phototherapy
trans to cis unconjugated bilirubin cis in yo piss
53
post-hepatic jaundice
no urobilinogen in urine (bc conjugated didnt go to intestine to get converted before reabsorbed) however, conjugated bilirubin will be in urine
54
hepatitis
hepatic jaundice damage of hepatocytes elevated serum direct and indirect bilirubin total reaches 5-10 mg/dL