Inflammatory diseases of the liver 2

Question 1

Liver bx: PAS positive globules within hepatocyte that are resistant to digestion with diastase

Test? Worse prognosis if?

Answer 1

A1AT (misfolded protein appears as PAS positive globules within hepatocyte)

A1AT phenotype (NOT level)

PiZZ phenotype

Question 2

Liver bx: PAS positive globules within hepatocyte that are resistant to digestion with diastase

Mechanism of injury?

Answer 2

A1AT

Excess misfolded alpha-1 AT accumulates in the liver, leading to OXIDATIVE damage to hepatocytes.

Question 3

Standard treatment regimens for AIH?

Answer 3

corticosteroids (prednisone or prednisolone) alone

or corticosteroids + azathioprine

Question 4

Antibodies for AIH type 1? Type 2?

Answer 4

ANA, anti-smooth muscle antibodies (ASMA), and anti-SLA (soluble liver antigen).

Type 2 AIH (seen more often in children) is characterized by the presence of anti-LKM (liver-kidney-microsomal) or anti-LC-1 antibodies

Question 5

Second line treatment for AIH if side effect to azathioprine

Answer 5

mycophenolate mofetil and calcineurin inhibitors (such as cyclosporin)

Question 6

Treatment for Cystic fibrosis induced liver disease?

Answer 6

Ursodeoxycholic acid

Question 7

Biopsy finding of ischemic hepatitis?

Answer 7

Zone 3 necrosis

Question 8

Non-transplant related causes of ischemic cholangiopathy?

Answer 8

AIDS cholangiopathy, polyarteritis nodosa, and hereditary hemorrhagic telangiectasia

Question 9

a cirrhotic patient has evidence of PVT, make sure to check for? How?

Answer 9

HCC-related tumor thrombus

endoluminal material enhances during the arterial phase of imaging or if there is evidence of arterial pulsatile flow is seen on Doppler ultrasound

Question 10

Patient with PVT without cirrhosis - most likely etiology?

Answer 10

myeloproliferative disorders present in 30-40 percent of cases.

Question 11

VOD/SOS prophylaxis?

Answer 11

ursodeoxycholic acid or low-dose heparin in the pre-transplant period

Question 12

Liver bx: fibrinogen deposition, vascular congestion, and eventually hepatocyte necrosis in a centrilobular pattern

Answer 12

SOS/VOD

Question 13

If a question asks about liver disease in a patient with a psych illness, think:

Answer 13

Wilson’s disease

Question 14

Diagnosis of Wilson’s:

Serum ceruloplasmin?
24-hour urine copper?
Liver biopsy?

Answer 14

Serum ceruloplasmin less than 20 mg/ dL warrants further workup (less than five mg/dL is highly suggestive)

24-hour urine copper levels of greater than 40 mcg/24 hrs warrant additional testing (over 100 mcg are highly suggestive)

hepatic copper content of greater than 250 mcg/gram highly suggestive

Question 15

Explain the copper levels in Wilson’s Disease?

Answer 15

Serum copper levels are often decreased due to the decrease in circulating ceruloplasmin

non-ceruloplasmin bound copper will be elevated, typically above 250 mcg/L

Question 16

Therapies for Wilson’s disease?

Answer 16

D-penicillamine, trientine, and zinc

Question 17

Wilson’s disease patient on maintenance trientine or D- penicillamine should have urine copper excretion of?

zinc therapy?

Answer 17

200-500 mcg/24 hrs

75 mcg/24 hrs