Metabolic Liver Diseases

Question 1

what do you find on liver bx for NASH?

Answer 1

NASH requires histology for dx

• hepatic steatosis
• lobular inflammation
• ballooned hepatocytes

Question 2

what does vitamin E do for NASH?

Answer 2

improves inflammation and steatosis on histology, but not fibrosis

Question 3

Dubin Johnson syndrome.

• LAE pattern
• risk of HCC

Answer 3

normal LAE
no liver failure or risk of HCC

caused by defect in canalicular transport (tf, black liver) of conjugated bilirubin into bile. Causes conjugated hyperbilirubinemia. at birth.

Question 4

for pts suspected of having hemochromatosis, what is the significance of ferritin >1000?

Answer 4

ferritin >1000 is a/w increased risk for having advanced hepatic fibrosis or cirrhosis, and therefore warrants liver bx

Question 5

what is the role of hepcidin and ferroportin in hemochromatosis?

Answer 5

normally, hepcidin is secreted by liver and down regulates iron absorption from intestine and iron release from macrophages.

when there’s iron excess, hepcidin usually increases

ferroportin is an iron transport protein on basolateral membrane of enterocytes to transfer iron from cell cytoplasm into extracellular space. Hepcidin causes ferroportin to be degraded.

Question 6

true or false - pts w/ PBC are at risk of developing varices in the absence of cirrhosis

Answer 6

true - PBC can be associated w/ pre-sinusoidal portal hypertension in the absence of cirrhosis, resulting in risk of developing varices

Question 7

what is the best prognostic marker for pts w/ PBC?

Answer 7

total bilirubin level is the best predictor of survival in PBC

Question 8

what are the benefits of ursodeoxycholic acid in management of PBC?

Answer 8

• decrease LDL
• reduce risk of variceal bleed
• slow progression of PBC
• reduce risk of needing liver transplant

Question 9

histology associations with…

• PBC
• PSC
• autoimmune hepatitis
• acute cellular rejection after liver transplant

Answer 9

• PBC (florid duct lesion)
• PSC (periductal concentric fibrosis (“onion-skinning”)
• autoimmune hep (hepatocyte rosettes w/ plasma cell infiltrate)
• acute cellular rejection (endotheliitis)

Question 10

what kind of cancer screening is recommended for pts w/ PSC?

Answer 10

• cholangiocarcinoma (all adults >=20yo)
• gallbladder cancer (all adults >=20yo)
• colon cancer (OC q1-2yr for pts w/ concomitant IBD. OC q3-5yr if w/o IBD)
• HCC

Question 11

tx for IgG4-related sclerosing cholangitis?

Answer 11

-corticosteroids

must distinguish from PSC since they have diff tx and prognoses

Question 12

what are features of early hepatic artery thrombosis after liver transplant?

tx?

Answer 12

• worsening clinical status
• severe elevation in AST, ALT
• rising INR
• acidosis

tx: urgent liver transplant. a surgical emergency. status 1A for transplant.

Question 13

what are the free hepatic venous pressure (FHVP), wedge hepatic venous pressure (WHVP) and hepatic venous pressure gradient (HVPG) for the following?

• schistosomiasis
• cirrhosis
• R heart failure

Answer 13

WHVP / FHVP / HVPG
schisto (pre-sinusoidal)
n n n
cirrhosis (sinusoidal)
H n H
R heart failure (post-sinusoidal)
H H n

Question 14

What radiographic findings do you see in Budd Chiari syndrome?

Answer 14

Budd Chiari syndrome - hepatic vein problem

caudate lobe hypertrophy - since caudate lobe has separate venous drainage directly into IVC (bypassing the hepatic veins), it gets larger.

venous collaterals

Question 15

which liver disease is Mallory Denk bodies associated w/?

Answer 15

alcoholic hepatitis

though mallory denk bodies are not exclusive to alc hep.

Question 16

what cutoff of Maddrey discriminant function do you use to determine tx of alc hep? why?

how do you assess tx?

Answer 16

MDF >32, predicts 20-50% 30-day mortality if >32

tx w/ prednisolone

assess tx response w/ Lille score on day 7 to see if you should continue for total 28 days of prednisolone

Question 17

what is a safe med to use for ethos abstinence for ethos cirrhotics?

Answer 17

baclofen

acamprosate and naltrexone have possible hepatotoxicity

Question 18

what is the gold standard for dx of alpha 1 antitrypsin deficiency?

Answer 18

a1at phenotyping - tells you if the folded protein is M, S, or Z.

measuring serum a1at is tricky, since a1at is an acute phase reactant and may be falsely elevated during inflammation

Question 19

if azathioprine is a no go for autoimmune hep, what are second line?

Answer 19

mycophenolate mofetil and calcineurin inhibitors (such as cyclosporin)

Question 20

you diagnose portal vein thrombosis in a cirrhotic. What should you check for?

Answer 20

when a cirrhotic pt has PVT, must consider possibility of HCC-related tumor thrombus

Question 21

for suspicion of HCC-associated PVT, what should you check? how do you treat PVT?

Answer 21

check AFP and get further imaging

suspect HCC-related tumor thrombus if endoluminal material enhances during the arterial phase of imaging

tx w/ anticoagulation (coumadin or LMWH) at least 6 months and reimagine w/ ultrasound to check for recanalization

Question 22

what is the goal of tx for acute PVT?

Answer 22

we tx acute PVT w/ anticoagulation, with goal of preventing extension of clot into the mesenteric veins and reanalyzing the portal system to prevent portal HTN

Question 23

what is sinusoidal obstruction syndrome? when does it happen?

Answer 23

sinusoidal obstruction syndrome
injury to the sinusoidal vascular epithelium, leading to fibrinogen deposition, vascular congestion, and eventually hepatocyte necrosis

most often seen w/n 3 weeks of hematopoietic stem cell transplant and also associated w/ specific myeloablative regimens (containing cyclophosphamide), or high dose XRT.

Question 24

how does sinusoidal obstruction syndrome present?

Answer 24

rapid onset ascites, edema, weight gain

tender hepatomegaly, elevated LAE are common

Question 25

what are the diagnostic criteria for sinusoidal obstruction syndrome?

Answer 25

Seattle criteria
-Tbili >2
-hepatomegaly/RUQ Pain
wt gain > 2% body weight

Baltimore criteria:
-Tbili >2
and 2 of the following: hepatomegaly, ascites, wt gain >5%`

Question 26

how do you reduce risk of and treatment sinusoidal obstruction syndrome?

Answer 26

use less toxic myeloablative regimen

give ursodeoxycholic acid or low dose heparin pre-transplant