Other Hepatic Disorders

Question 1

What are the 3 major patterns of drug induced liver injury?

Answer 1

• Hepatocellular injury
• Cholestatic injury
• Mixed pattern

Question 2

What is the second most common cause of acute drug induced liver injury?

Answer 2

• antibiotics

* Augmentin, INH, Rifampin

Question 3

Drug induced liver injury is a result of what? (pathophysiology)`

Answer 3

• direct effects of drug or metabolites = dose dependent

* intrinsic hepatotoxicity

Question 4

What are the 2 forms of drug induced idiosyncratic hepatotoxicity?

Answer 4

• hypersensitivity (allergy)

- metabolic

Question 5

What is the enzymatic profile of hepatocellular type DILI?

Answer 5

• ALT/Alk Phos >5

Question 6

What is the enzymatic profile of cholestatic type DILI?

Answer 6

• ALT/Alk Phos <2

Question 7

What is the enzymatic profile of mixed type DILI?

Answer 7

• ALT/Alk Phos 2-5

Question 8

Autoimmune hepatitis is characterized by what?

Answer 8

• circulating antibodies = hypergammaglobulinemia

Question 9

What 2 antibodies are seen in Type 1 autoimmune hepatitis?

Answer 9

• ANA (Anti-nuclear antibody)

- ASMA (Anti-smooth muscle antibody)

Question 10

What % of patients with autoimmune hepatitis will present with cirrhosis at time of diagnosis?

Answer 10

25%

Question 11

What is the first line treatment of autoimmune hepatitis?

Answer 11

• prednisone (+ azathioprine)

Question 12

Primary biliary cholangitis is characterized by what?

Answer 12

• granulomatous destruction of interlobular bile ducts

Question 13

What antibodies are seen in primary biliary cholangitis?

Answer 13

• AMA (Anti mitochondrial antibodies)

Question 14

In what population is primary biliary cholangitis most commonly seen?

Answer 14

• caucasian middle aged women

Question 15

Xanthomata and Xanthelasma suggests what?

Answer 15

cholestasis (flow of bile from liver is blocked)

Question 16

What is the treatment of primary biliary cholangitis?

Answer 16

• ursodeoxycholic acid (UDCA)

Question 17

Primary sclerosing cholangitis is characterized by what?

Answer 17

• obliteration of bile ducts (both extra/intrahepatic)

Question 18

“Beads on a string” seen on a cholangiogram is classic for what?

Answer 18

Primary sclerosing cholangitis

Question 19

The majority of patients with Primary sclerosing cholangitis also have THIS additional GI disease.

Answer 19

• Ulcerative colitis (70%)

Question 20

What antibody is seen in 80% of cases of primary sclerosing cholangitis?

Answer 20

• p-ANCA

Question 21

What diagnostic procedure for PCS can also treat obstructions causing acute cholangitis?

Answer 21

• ERCP

Question 22

What type of genetic inheritance is seen in Wilson’s Disease?

Answer 22

• autosomal recessive disorder

Question 23

Wilson’s disease results in what?

Answer 23

• copper overload

Question 24

Kayser-Fleischer rings seen on a slit lamp exam is consistent with disease?

Answer 24

• Wilson’s disease

Question 25

What lab values are low in Wilson's Disease?

Answer 25

- Ceruloplasmin levels

Question 26

What is the most effective copper chelating agent used to treat Wilson's Disease?

Answer 26

- Penicillamine

Question 27

Hereditary Hemochromatosis results in what?

Answer 27

- iron overload | * increased iron absorption with toxic deposition into multiple organs

Question 28

What type of genetic inheritance is seen in Hereditary Hemochromatosis?

Answer 28

- Autosomal recessive

Question 29

What lab findings are consistent with Hemochromatosis?

Answer 29

- Fasting transferrin saturation > 45%

Question 30

What is the most common chromosome mutation resulting in hemochromatosis?

Answer 30

- C282Y homozygous

Question 31

What MRI findings are consistent with hemochromatosis?

Answer 31

- decrease signal intensity of liver compared to skeletal muscle

Question 32

What is the treatment for hemochromatosis?

Answer 32

- Phlebotomy

Question 33

If a patient presents with suspected hemochromatosis who needs to be screened?

Answer 33

- first degree relatives | * check fasting iron sat and transferrin

Question 34

What allele pattern causes the disease of a1 antitrypsin deficiency.

Answer 34

- PiZZ

Question 35

Premature emphysema is a common sign of this disease.

Answer 35

- a1 antitrypsin deficiency

Question 36

Positive diastase resistant globules seen on acid Schiff staining is consistent with this disease.

Answer 36

- a1 antitrypsin deficiency