metabolic and autoimmune liver disease

Question 1

In the liver, A1AT deficiency results in a

Answer 1

storage disease - misfolded protein, not excreted

Question 2

In the LUNG, A1AT deficiency results in a

Answer 2

unopposed elastase activity and emphysema

Question 3

2 ways to diagnose A1AT deficiency

Answer 3

• serum

- electrophoresis

Question 4

2 staining patterns of A1AT deficiency in the liver

Answer 4

• PAS positive globules

- immunoperoxidase staining for A1AT

Question 5

diagnosis of wilson disease

Answer 5

• low ceruloplasmin- copper deficiency in the ER
• increased urine 24 hr copper
• penicillamine challenge
• biopsy

Question 6

Histological features of wilson disease

Answer 6

• looks like Fatty liver D, exclude NASH, AHEP
• marked copper acumulation
• ductular reaction

Question 7

Mechanism of iron overload in HFE-mutations

Answer 7

• continuous absoption by enterocyte in duodenum

- decreased hepcidin

Question 8

Extra-hepatic manifestations of HFE

Answer 8

• skin
• pancreas
• pituitary
• joints - MCP 2/3
• heart

Question 9

diagnosing HFE

Answer 9

• high transferrin
• high Fe
• High ferritin
• biopsy

Question 10

What is ferroportin

Answer 10

• exports Fe - absorbs

inhibited by Hepcidin normally degrades it

Question 11

During infection and inflammation what happens to ferroportin

Answer 11

• turned off, hepcidin upregulated

Question 12

Histologic features in iron overload in the liver

Answer 12

• brown granules
  PERICANALICULAR pattern of iron distribution
• use a PERLS stain

Question 13

which of the 3 genetic disease do you have a crisis

Answer 13

Wilson’s disease -

extra copper secretion - hemolysis

Question 14

autoimmune hepatitis is predominantly inflammation where?

Answer 14

in the lobules!

• syncitial giant cells
• acidophil bodies

Question 15

In PBC where is the inflammation predominantly?

Answer 15

interlobular bile ducts

granulomas

Question 16

In PSC what is the characertistic lesion

Answer 16

onion-skin appearance around the large bile ducts

Question 17

3 features define cirrhosis pathologically

Answer 17

• loss of small veins
• fibrosis
• regenerative nodules

Question 18

Most common causes of cirrhosis

Answer 18

• viral hepatitis B and C

- Alcoholic and NASH

Question 19

Is cirrhosis symptomatic?

Answer 19

Not until liver failure

Question 20

when your % chance of death is 18% or higher you are now eligible for

Answer 20

a liver transplant

Question 21

Portal hypertension is when pressure differences are

Answer 21

> = 6 mm Hg

Question 22

3 main reasons for developing ascites

Answer 22

• increased hydrostatic pressures with retention of sodium/water
• ## decreased oncotic pressures - low albumin

Question 23

Early cirrhosis
Moderate
Severe ascites

Answer 23

excrete 4-5g Na per day
Need to add diuretics
paracentesis

Question 24

SAAG - serum albumin- serum in ascitic fluid - indicating cirrhosis

Answer 24

> 11g

Question 25

most common cause of HE

Answer 25

SBP

- or electrolyte abnormalities from overdiuresis

Question 26

How do you manage HE

Answer 26

• lactulose -

Question 27

What is the major risk factor for HCC

Answer 27

cirrhosis - hepatomas at ANY stage of cirrhosis

Question 28

Appearance of a hepatoma on CT contrast

Answer 28

• Bright hypervascular in arterial phase

- early wash out in venous phase - dark

Question 29

what is a prognostic indicator of HCC

Answer 29

small vessel invasion

CEA +ve

Question 30

what does the Child pugh score include

Answer 30

• INR
• Alb
• Bili
• Ascites
• HE

Question 31

Difference between CPT and MELD

Answer 31

• Includes creatinine

- removes albumin