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

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2
Q

UDPGT

A

uridyldiphosphate glucuronyl transferase
-conjugates bilirubin in liver at SER

adds glucuronide x2

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3
Q

heme oxygenase

A

heme > biliverdin

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4
Q

van den berg test

A

measure conjugated and unconjugated bilirubin levels

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5
Q

urobilinogen

A

converted in intestine (terminal ileum and colon)
colorless

20% reabsorbed

collection of sterco, meso, urobilinogen

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6
Q

normal range bilirubin

A

0.2-1

less than 0.2 conjugated

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7
Q

kernicterus

A

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

aka brain encephalopathy

due to polycythemia, Rh incompatibility

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8
Q

pre-hepatic jaundice

A

excess hemolysis

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9
Q

hepatic jaundice

A

infection, drugs, alcohol, cirrhosis, pregnancy

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10
Q

post-hepatic jaundice

A

blockage of duct

cancer of bile duct, pancreas head

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11
Q

excess hemolysis

A

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

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12
Q

biliverdin reductase

A

biliverdin > biliverdin

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13
Q

stercobilin

A

stool pigment

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14
Q

urobilin

A

in urine

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15
Q

causes of jaundice

A

excess bilirubin production
reduced hepatocyte uptake
impaired conjugation
impaired bile flow

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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)

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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

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18
Q

crigler najjar type II

A

autosomal dominant

partial deficiency UDPGT

unconjugated bilirubin elevated 5-20

responds to phenobarbitol

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19
Q

phenobarbitol

A

Tx for crigler najjar type II

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20
Q

dubin johnson syndrome

A

autosomal recessive

elevated conjugated

defective canalicular excretion

liver biopsy - black**

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21
Q

rotor syndrome

A

elevated conjugated

liver biopsy - normal

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22
Q

pepsin

A

stomach

23
Q

trypsin

A

small intestine

24
Q

aminopeptidase

carboxypeptidase

A

small intestine

25
Q

excess AA sources

A

leftover
dietary
body protein breakdown

26
Q

transamination

A

take away amino group (NH2)

27
Q

glutamate

A

carries nitrogen 1

28
Q

glutamine

A

carries nitrogen 2

29
Q

pyridoxal phosphate cofactor

A

PLP

all aminotransferases use this cofactor

30
Q

alpha-ketoglutarate

A

to glutamate
-by aminotransferase

takes NH2 from amino acid

31
Q

L-glutamine

A

storage of NH2 x2

transport in blood to kidney

glutamine liberates NH2 groups in liver

32
Q

glutamate dehydrogenase

A

alpha-ketoglutarate > glutamate

33
Q

glutamine synthetase

A

glutamate > glutamine

34
Q

carbamoyl phosphate synthetase I

A

glutamine > carbamoyl phosphate

deficiency leads to ammonia in blood

35
Q

ornithine transcarbamoylase

A

carbamoyl phosphate + ornithine > cirtulline

deficiency - cant make urea

36
Q

argininsuccinate synthetase

A

cirtulline > argininosuccinate

37
Q

argininosuccinate

A

to fumarate and arginine

fumarate to citric acid cycle (makes ATP)

arginine to urea + ornithine

38
Q

ornithine

A

recycled component of urea cycle

39
Q

urea cycle chemical equation

A

3ATP + HCO3 + NH4 + asp > 2ADP + AMP + 2P + PP + fumarate + urea

40
Q

argininosuccinase deficiency

A

protein depleted diet

supplement high levels of L-arginine

41
Q

brain swelling

A

glutamine and glutamate are osmolites in brain

hepatic encephalopathy

42
Q

PKU

A

deficienct phenylalanine hydroxylase

no phenylalanine to tyrpsine/Epi

phenylalanine to phenylpyruvate (both toxic CNS)

mental retardation occurs
-common ADHD

tested in newborns

43
Q

aspartame

A

nutrasweet

-has phenylalanine

44
Q

phenylalanine conversion

A

> tyrosine > L-dopa > dopamine > NE > E

45
Q

BUN

A

blood urea nitrogen
liver and kidney function

higher - CHF, GI bleed, hypovolemia, kidney disease, shock

lower - low protein diet, malnutrition, over hydration

46
Q

ALT, AST, ALP

A

elevated - liver damage

47
Q

albumin and total protein

A

decreased - liver damage

48
Q

GGT

A

elevated - liver or bile duct damage

49
Q

LDH

A

elevated - liver damage

50
Q

PT

A

increased - liver damage

51
Q

MRP2

A

transport of direct bilirbubin to bile canaliculus

52
Q

phototherapy

A

trans to cis unconjugated bilirubin

cis in yo piss

53
Q

post-hepatic jaundice

A

no urobilinogen in urine (bc conjugated didnt go to intestine to get converted before reabsorbed)

however, conjugated bilirubin will be in urine

54
Q

hepatitis

A

hepatic jaundice
damage of hepatocytes

elevated serum direct and indirect bilirubin

total reaches 5-10 mg/dL