Hepatobiliary Worksheet- Paulson (Exam 3)

Question 1

_________ _________ _________ is a progressive disease of the liver and gallbladder characterized by inflammation and scarring/fibrosis of intrahepatic and/or extrahepatic bile ducts.

Answer 1

Primary sclerosing cholangitis

Question 2

Pt’s with Primary sclerosing cholangitis may be asymptomatic, or complain of _______, __________, _________, and/or ______ pain. Hepatomegaly and splenomegaly may also be seen.

Answer 2

jaundice, pruritis, fatigue, RUQ

Question 3

In Primary sclerosing cholangitis, a cholestatic pattern is seen, with ___ usually predominantly elevated.
These pt’s may also have a positive ________, as well as elevated ________ and ___/_____.

Answer 3

ALP (predominantly)

P-ANCA

bilirubin, ALT/ AST

Question 4

Multifocal stricturing and dilation of intrahepatic and/or extrahepatic bile ducts on MRCP or ERCP would be indicative of __________ ___________ _________.

Answer 4

Primary sclerosing cholangitis

Question 5

Primary sclerosing cholangitis is strongly associated with what 3 other conditions?
These pt’s are also at higher risk for developing ___________ (type of cancer).

Answer 5

inflammatory bowel dz, ulcerative colitis (90%), and +/- Crohns Dz.
Cholangiocarcinoma

Question 6

What is the best definitive treatment for Primary sclerosing cholangitis?

Answer 6

Liver transplant

Question 7

True or false?

No meds have been shown to slow the disease process in Primary sclerosing cholangitis.

Answer 7

TRUE.

UDCA is a med that is often used, but none have been proven to slow the Dz.

Question 8

_________ _________ __________ is an autoimmune disease of intrahepatic small bile ducts, caused by T-cells attacking them causing gradual destruction/disappearance, which leads to cholestasis, and eventually cirrhosis/liver failure.

Answer 8

Primary biliary cirrhosis

Question 9

Primary biliary cirrhosis is usually seen in what patient population?

Answer 9

middle aged women

Question 10

Pt’s with primary biliary cirrhosis will have what symptoms?

Answer 10

Fatigue*
Pruritus*
RUQ discomfort
jaundice
hepatomegaly

Question 11

Primary biliary cirrhosis will cause a significant elevation in _____ and ______. Almost all pt’s will have positive _________ antibodies.

Answer 11

ALP and GGT

Mitochondrial

Question 12

What is the 1st line treatment for primary biliary cirrhosis?

Answer 12

UDCA (Ursodeoxycholic acid or Ursadiol)

Question 13

__________ ___________ is a chronic disease with continuing hepatocellular inflammation and necrosis that tends to progress to cirrhosis.

Answer 13

Autoimmune hepatitis

Question 14

Pts with autoimmune hepatitis may be asymptomatic or have symptoms like ____________, stigmata of chronic liver dz, ________, or _______ _______ _________.

Answer 14

hepatosplenomegaly
jaundice
acute liver failure

Question 15

In autoimmune hepatitis, what 2 labs would you expect to be elevated?
These pt’s may also have _____ _______ antibodies or a positive ____.

Answer 15

Transaminases and bilirubin
smooth muscle antibodies
positive ANA

Question 16

How would you make the diagnosis of autoimmune hepatitis?

Answer 16

Liver biopsy

Question 17

What is the treatment for autoimmune hepatitis?

Answer 17

Prednisone and Azathioprine

then taper pred down

Question 18

______ ______ _________ is the development of severe acute liver injury with encephalopathy and elevated INR (>1.5) in a pt without cirrhosis or pre-existing liver disease.

Answer 18

Acute liver failure

Question 19

_____________ _________ is the most common cause of acute liver failure, but other common causes include _____ ________ and ________ _________.

Answer 19

Acetaminophen toxicity

drug reactions and viral hepatitis

Question 20

Clinical manifestations of acute liver failure include? (There are 6)

Answer 20

encephalopathy (by definition)
jaundice
hepatomegaly 
RUQ tenderness
renal failure
thrombocytopenia

Question 21

_______ __________ and ________ _______ often complicate acute liver failure.

Answer 21

Adrenal insufficiency and cardiac injury

Question 22

In acute liver failure, you would expect to see (on labs) a PT/INR greater than ______, and elevated ______ and _____’s.

Answer 22

1.5

ammonia and LFT’s

Question 23

What med is used in the tx of Tylenol overdose? What are you trying to prevent?

Answer 23

Acetylcysteine

Development of cerebral edema

Question 24

What is the definitive treatment for acute liver failure?

Answer 24

Liver transplant

Question 25

NAFLD is subdivided into what 2 categories?

What is the difference between these conditions?

Answer 25

NAFL (non-alcoholic fatty liver)
NASH (Non-alcoholic steatohepatitis)
Difference: in NAFL, hepatic steatosis is present without any significant inflammation, but in NASH hepatic steatosis is associated with inflammation. NASH may be histologically indistinguishable from alcoholic steatohepatitis.

Question 26

True or false? NAFLD cannot progress into cirrhosis.

Answer 26

FALSE, NAFLD can progress into cirrhosis.

Question 27

Name 4 major risk factors for NAFLD.

Answer 27

obesity
DM2
dyslipidemia
metabolic syndrome

Question 28

True or false? Most pt’s with NAFLD are asymptomatic.

Answer 28

True.

Question 29

If pts with NAFLD are symptomatic, what would you expect?

Answer 29

fatigue, malaise, vague RUQ discomfort

Question 30

Labs on a pt with NAFLD would show mild to moderate elevations of what?

Answer 30

AST/ ALT

Question 31

How would you definitively diagnose NAFLD?

Answer 31

liver biopsy

Question 32

What is the treatment for NAFLD? What should these pt’s avoid?

Answer 32

WEIGHT LOSS is associated with histologic improvement.

Avoid: alcohol consumption

Question 33

_______________ is the condition of excess iron absorption and deposition in parenchymal cells of the heart, liver, pancreas, adrenals, testes, pituitary, and kidneys.

Answer 33

Hemachromatosis

Question 34

Hemochromatosis is most common in which population?

Answer 34

Men. (autosomal recessive)

Question 35

Clinical manifestations of hemochromatosis usually begin after age ___ to _____ and include what? (there are 6).

Answer 35

age 40-50 yrs

weakness/lethargy, skin hyperpigmentation, arthralgia, cardiac enlargement +/- heart failure, diabetes, ED/impotence

Question 36

Labs for hemochromatosis would show elevated serum ______ with increased _____ _________ and ______.

Answer 36

iron

transferrin saturation and ferritin

Question 37

If you are trying to rule out or diagnose hemochromatosis, _________ testing can eliminate the need for a liver biopsy.

Answer 37

genetic

Question 38

What is the treatment for hemochromatosis?

What should these pts avoid in their diet?

Answer 38

Therapeutic phlebotomy until iron stores are depleted, then begin maintenance (less frequently). If pt cannot tolerate phlebotomy, chelation therapy is recommended.
Pts should avoid Iron, Vit C, and alcohol