Bilirubin Pathways Flashcards

1
Q

bilirubin is an open chain called?

A

a tetrapyrrole rings

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2
Q

bilirubin breakdown of blood usually where?

A

spleen

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3
Q

Macrophages break form RBCs, then what?

A

into haem>biliverdin>free bilirubin

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4
Q

colour of bilirubin?

A

yellow

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5
Q

colour of biliverdin?

A

green

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6
Q

some double bonds in bilirubin isomerize when what? what does that do to bilirubin?

A

exposed to light = more soluble

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7
Q

When use phototherapy for bilirubin?

A

jaundiced newborns

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8
Q

2 forms of bilirubin?

A

conjugated and unconjugated

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9
Q

bilirubin usually excreted as?

A

conjugated (glucuronide) in bile

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10
Q

high bilirubin in newborns?

A

neurotoxic

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11
Q

RBCs stimulated by?

A

EPO

erythropoietin

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12
Q

how many RBCs made a die per second?

A

2 million

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13
Q

future RBCs leave the bone marrow as what? how long to mature to RBCs?

A

reticulocytes mature in a day

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14
Q

how long to create RBCs from stems?

A

7 days

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15
Q

RBCs last?

A

~120 days

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16
Q

what is met-haemoglobin?

A

Fe3+ that doesn’t bind O2 well

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17
Q

What is in white pulp and red pulp in spleen?

A

White pulp: T/B cells

Red pulp: blood vessels to remove old RBCs via macrophages

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18
Q

Mononuclear phagocyte is precursor to?

A

macrophage

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19
Q

Are there Mononuclear phagocytes in the liver?

A

yes. the Kupffer cells help out

20
Q

what does the white pulp in spleen do?

A

opsonizes old RBCs to help with recognition by macrophages

21
Q

aged RBCs lose what on their surface?

A

neuraminic/sialic acid from glycophorin

22
Q

3 big factors for haemolysis?

A

strepto/enterococcus
parasitic: Malaria
congenital/genetic/autoimmune

23
Q

What is Haptoglobin?

A

plasma protein that binds haemoglobin of RBCs that die away from spleen, allows macrophages to remove

24
Q

Haptoglobin looks like?

A

trypsin

25
Q

what carried haem (not Haemoglobin) to liver?

A

hemopexin recycles the iron

26
Q

4 consequences of haemolysis

A

unconjugated bilirubin = jaundice
increased stercobilin
gall stones
reticulocytosis

27
Q

Fe2+ carried by?

A

transferrin

28
Q

Haem oxygenase produces?

A

biliverdin

29
Q

How is biliverdin produces from Haem?

A

via haem oxygenase

30
Q
Bruises: what happens at?
1-2 days
5-10 days
10-14 days
>14 days
A

1-2 days: blue, purple black: deoxy/met-Hb
5-10 days: green/yellow = biliverdin
10-14 days: yellow/brown = bilirubin
>14 days : fades away

31
Q

serum albumin binds what?

A

HIGHLY hydrophobic bilirubin

32
Q

what converts bilirubin to conjugated bilirubin?

A

bilirubin UDP glucuronyltransferase

33
Q

whats the difference between conjugated and unconjugated bilirubin?

A

conjugated is soluble

34
Q

defect in bilirubin UDP glucuronyltransferase leads to what disease?

A

Crigler-Najjar disease

35
Q

what actively pumps bilirubin diglucuronide into canaliculi?

A

cMOAT or MRP2

36
Q

what is Crigler-Najjar disease?

A

defect in bilirubin UDP glucuronyltransferase

37
Q

What is Dubin Johnson syndrome? prognosis?

A

defect in cMOAT

asymptomatic, no Rx necessary

38
Q

Crigler-Najjar disease symptoms?

A

persistent jaundice

kernicterus

39
Q

What is kernicterus?

A

bilirubin in brain

40
Q

Crigler-Najjar disease initial and subsequent treatment?

A

initially: phototherapy 10-12 hr/day
subsequently: liver transplant

41
Q

How much bile is recycled?

A

95% in terminal ileum

42
Q

what are bile pigments?

A

bilirubin diglucuronide

43
Q

what are bile salts?what do they do?

A

detergent derivatives of cholesterol

emulsify fats

44
Q

90% of urobilinogen in faeces, 10% goes where?

A

10% back into portal vein
9% back to biliary system
1% into kidney and into urine = yellow pee

45
Q

Why does old pee look more yellow?

A

+++ oxidized urobilinogen

46
Q

What converts bilirubin diglucoruronide to sterco and urobilinogen?

A

microbial enzymes