Other Hepatic Disorders Flashcards

1
Q

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

A
  • Hepatocellular injury
  • Cholestatic injury
  • Mixed pattern
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2
Q

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

A
  • antibiotics

* Augmentin, INH, Rifampin

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3
Q

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

A
  • direct effects of drug or metabolites = dose dependent

* intrinsic hepatotoxicity

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4
Q

What are the 2 forms of drug induced idiosyncratic hepatotoxicity?

A
  • hypersensitivity (allergy)

- metabolic

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5
Q

What is the enzymatic profile of hepatocellular type DILI?

A
  • ALT/Alk Phos >5
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6
Q

What is the enzymatic profile of cholestatic type DILI?

A
  • ALT/Alk Phos <2
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7
Q

What is the enzymatic profile of mixed type DILI?

A
  • ALT/Alk Phos 2-5
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8
Q

Autoimmune hepatitis is characterized by what?

A
  • circulating antibodies = hypergammaglobulinemia
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9
Q

What 2 antibodies are seen in Type 1 autoimmune hepatitis?

A
  • ANA (Anti-nuclear antibody)

- ASMA (Anti-smooth muscle antibody)

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10
Q

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

A

25%

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11
Q

What is the first line treatment of autoimmune hepatitis?

A
  • prednisone (+ azathioprine)
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12
Q

Primary biliary cholangitis is characterized by what?

A
  • granulomatous destruction of interlobular bile ducts
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13
Q

What antibodies are seen in primary biliary cholangitis?

A
  • AMA (Anti mitochondrial antibodies)
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14
Q

In what population is primary biliary cholangitis most commonly seen?

A
  • caucasian middle aged women
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15
Q

Xanthomata and Xanthelasma suggests what?

A

cholestasis (flow of bile from liver is blocked)

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16
Q

What is the treatment of primary biliary cholangitis?

A
  • ursodeoxycholic acid (UDCA)
17
Q

Primary sclerosing cholangitis is characterized by what?

A
  • obliteration of bile ducts (both extra/intrahepatic)
18
Q

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

A

Primary sclerosing cholangitis

19
Q

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

A
  • Ulcerative colitis (70%)
20
Q

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

A
  • p-ANCA
21
Q

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

A
  • ERCP
22
Q

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

A
  • autosomal recessive disorder
23
Q

Wilson’s disease results in what?

A
  • copper overload
24
Q

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

A
  • Wilson’s disease
25
Q

What lab values are low in Wilson’s Disease?

A
  • Ceruloplasmin levels
26
Q

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

A
  • Penicillamine
27
Q

Hereditary Hemochromatosis results in what?

A
  • iron overload

* increased iron absorption with toxic deposition into multiple organs

28
Q

What type of genetic inheritance is seen in Hereditary Hemochromatosis?

A
  • Autosomal recessive
29
Q

What lab findings are consistent with Hemochromatosis?

A
  • Fasting transferrin saturation > 45%
30
Q

What is the most common chromosome mutation resulting in hemochromatosis?

A
  • C282Y homozygous
31
Q

What MRI findings are consistent with hemochromatosis?

A
  • decrease signal intensity of liver compared to skeletal muscle
32
Q

What is the treatment for hemochromatosis?

A
  • Phlebotomy
33
Q

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

A
  • first degree relatives

* check fasting iron sat and transferrin

34
Q

What allele pattern causes the disease of a1 antitrypsin deficiency.

A
  • PiZZ
35
Q

Premature emphysema is a common sign of this disease.

A
  • a1 antitrypsin deficiency
36
Q

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

A
  • a1 antitrypsin deficiency