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Liver path I Flashcards

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1
Q

What is the most common cause of chronic liver disease? Second most common?

A
  1. Hep C

2. EtOH

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2
Q

What are the two enzymes that are used in LFTs?

A

AST

ALT

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3
Q

Which is more specific for the liver: AST or ALT

A

ALT

AST is in muscles

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4
Q

What does LDH measure?

A

RBC destruction

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5
Q

What causes increase LFTs?

A

Breakage of hepatocytes

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6
Q

What are the three tests that measure the liver synthesis function?

A

Serum albumin
INR
Serum ammonia

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7
Q

What is the test that you want to order to differentiate if there is an isolated elevated alk phos to determine if it comes from the liver vs from the bone?

A

Gamma-glutamyl-transpeptidase

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8
Q

Isolated elevation in alk phos is suspicious for what disease?

A

Bile duct problems

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9
Q

Primary sclerosing cholangitis vs primary biliary cholangitis***

A
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10
Q

What form of bilirubin is conjugated: direct or indirect?

A

Direct

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11
Q

What is the normal range of direct bilirubin?

A

0.1-1.2 mg/dL

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12
Q

Greater than what level of direct bili causes jaundice?

A

> 2 mg/dL

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13
Q

Why is direct bilirubin named as such?

A

Can be directly measured in the lab since it’s water soluble?

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14
Q

How do they calculate indirect bili?

A

Subtract direct from the total bili

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15
Q

Where does bili concentrate?

A

Hepatocytes

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16
Q

Why is it that there is an increase in direct bili with alcoholic cirrhosis?

A

Edema of the hepatocytes causing obstruction of bile flow

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17
Q

Where are the best places to tell if an AA pt hwat jaundice?

A

Sclera

Mucous membranes

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18
Q

What are the prehepatic causes of increased bili

A

Hemolysis

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19
Q

What are the hepatic causes of increased bili?

A

Cirrhosis

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20
Q

What are the post hepatic causes of jaundice?

A

Obstruction of the bile duct

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21
Q

Heme is converted to what? What are the enzymes at each of these steps?

A

Biliverdin to bilirubin

heme oxygenase and biliverdin reductase respectively

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22
Q

Where does bilirubin formation occur?

A

Hepatocytes

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23
Q

Prehepatic causes of bilirubin cause an increase in what type of bilirubin?

A

Unconjugated

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24
Q

Hepatic causes of hyperbilirubinemia usually has what type of bilirubin?

A

Conjugated or unconjugated

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25
Obstructive causes of hyperbilirubinemia usually has what type of bilirubin?
Conjugated
26
What is stercobilinogen?
Color of bili in the stool
27
What is the bili found in urine?
Urobilinogen
28
How is urobilinogen formed?
made by bacteria
29
What is the enzymes that conjugated heme?
UGT1A1--uridine diphosphate-glucuronyltransferase 1 A1
30
What is the transporter for bilirubin into hepatocyte ER?
Bilirubin-glutathione S tranferas
31
What is the chemical change that occurs with bilirubin metabolism?
add sugar moieties
32
What are the two causes of elevation of unconjugated bili?
Overproduction | Reduce uptake
33
What are the causes of elevated levels of conjugated bili?
Defective conjugation Defective excretion Defective secretion
34
What are the causes of impaired uptake of bili? (3)
Hepatocellular injury Drugs Newborn
35
What is the cause of Gilbert syndrome? How is this inherited?
Impaired UDP glucuronyl transferase activity, causing jaundice with stress AR
36
What is the cause of Crigler-Najjar syndrome type I? How is this inherited? Is this fatal?
Absent UDP glucuronyl transferase activity AD Causes death in infants
37
What is the cause of dubin-Johnson syndrome? How is this inherited?
Impaired secretion of bili AR
38
What is the cause of Rotor syndrome?
AR decreased hepatic uptake and storage
39
What is the liver pathology with Dubin-Johnson syndrome?
Pigmented cytoplasmic globules
40
What is the cause of Crigler-Najjar syndrome type II? How is this inherited? Is this fatal?
Decreased Glucuronyl transferase activity AD with variable penetrance Generally mild
41
What is the underlying genetic cause of Gilbert syndrome?
promoter mutation for UGT1A1
42
What is the treatment for Gilbert syndrome?
None
43
What is the treatment for Crigler-Najjar syndrome type I?
Liver transplant and phototherapy No response to phenobarbital
44
What causes death with Crigler-Najjar syndrome type I?
Kernicterus
45
Which type of Crigler-Najjar syndrome responds to phenobarbital?
Type II
46
What is the underlying defect with Dubin-Johnson syndrome?
MDR protein 2 defect, causing inability to transport
47
What happens to the liver with Dubin-Johnson syndrome? What causes this?
Blackened Build up of epi in lysosomes
48
What is the diagnostic test for Dubin-Johnson syndrome?
Total urinary porphyrins are normal, but increased isomer I (usually III is higher)
49
What happens with total coproporphyrin in Rotor syndrome?
Increased total, but normal isomer I
50
What is the treatment for Rotor syndrome?
None required
51
What is progressive familial intrahepatic cholestasis? What causes it?
A group of familial cholestatic conditions caused by defects in biliary epithelial transporters, that progresses to hepatic failure.
52
What are the normal contents of bile? (4)
Cholic Cholesterol Lecithin Bicarb
53
What is (PFIC-1) Byler's syndrome?
Problems with ATPase (ATP8B1) in liver, so not enough energy for transfer into the canalicular system
54
What is PFIC-2?
ABCb11; bile salt export pump
55
What is PFIC-3?
ABCB4; MDR3 phosphatidylcholine transfer
56
What is the end result of PFIC 1, 2, and 3?
Cirrhosis/liver failure Fat soluble vit deficiency Cholestasis
57
What are the histological characteristics of Progressive familial intrahepatic cholestasis?
Liver cell rosettes Feathery degeneration
58
What are the drugs that cause hepatocellular dysfunction?
Chlorpromazine | Erythromycin
59
What are the pregnancy conditions that can cause hepatocellular dysfunction?
Preeclampsia | Toxemia
60
What are the two most common types of bile obstruction?
Gallstones | CA of the head of the pancreas
61
What are the ssx of cholestasis? (skin x2, stool, urine, bones)
``` Pruritus Jaundice Clay-colored stool Dark urine Osteoporosis ```
62
True or false: xanthomas can occur with cholestasis
True
63
What happens to hepatocytes and kupffer cells with cholestasis?
Packing with bile
64
What are the three outcomes of cholestasis in the liver?
Inflammation Cirrhosis Fibrosis
65
Feathery degeneration of hepatocytes is manifested by what?
hydropic swelling, diffuse impregnation with bile pigment and a reticulated appearance
66
What are bile lakes?
extravasation of a large amount of bile in extrahepatic obstruction
67
What are the histological characteristics of cholestasis?
Neutrophils in the walls of the bile ducts and focally filling the lumen of ducts, often dilating or destroying them
68
What are the three main causes of cirrhosis?
Viral EtOH Obesity
69
What is the pathogenesis of cirrhosis?
Hepatocyte necrosis | Progressive fibrosis
70
What happens when hepatocytes repair themselves from insult that can lead o cirrhosis?
Abnormal vascular connections | Disruption of function
71
What causes the fibrosis of the liver?
Stellate cells convert to fibroblasts
72
What are the factors that are released by macrophages to cause fibrosis?
PDGF TNF TGF-beta
73
What is the space of Disse?
Area between a hepatocyte and a sinusoid. It contains the blood plasma. Microvilli of hepatocytes extend into this space, allowing proteins and other plasma components from the sinusoids to be absorbed by the hepatocytes
74
Micronodular cirrhosis is usually caused by what insult to the liver?
EtOH
75
What is the main cause of macronodular cirrhosis?
Metabolic disorders
76
What is the stain for collagen?
Trichrome
77
What are the two types of cirrhosis?
Compensated | Decompensated

Gastrointestinal (42 decks)

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