Primer 27 - Liver Pathology Flashcards

1
Q

What is the etiology if AST is greater or equal to ALT?

A

That a virus might be responsible for the liver pathology.

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2
Q

What does GGT stand for and when is it elevated?

A

Gamma Glutamil Transpeptidase. Elevated in liver pathologies, excess alcohol consumption, and disease that affect the biliary tract.

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3
Q

When is alkaline phosphatase elevated?

A

Elevated in biliary obstruction (gallstones, cancer). Relatively nonspecific, because it is elevated in active bone formation (children), cancer of the bone and Paget’s disease.

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4
Q

What is Nutmeg liver?

A

Occurs when there is a backup of blood into the liver commonly due to right sided heart failure. Can also be caused by Budd-Chiary syndrome.

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5
Q

What can long term congestion of the liver lead to?

A

Centralobular congestion and necrosis. Can also lead to fibrosis. This is sometimes called cardiac cirrhosis from the right sided heart failure.

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6
Q

What is Budd-Chiary syndrome?

A

It is a syndrome caused by the obstruction of either Inferior vena cava or hepatic veins. Clinical manifestations are hepatomegaly, ascities, abdominal pain, and eventual liver failure. Good change to develop portal hypertension, leading to esophageal varices and caput medusa.

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7
Q

What is a clinical manifestation difference between Budd-Chiary syndrome and Right sided heart failure?

A

In Budd-Chiari syndrome there is no jugular vein distention (JVD) unlike right-sided heart failure.

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8
Q

What is Reye’s syndrome?

A

Idiopathic but associated with children using aspirin and/or viral infections (varicella or influenza). Can happen without aspirin. The main pathology it causes is hepatoencephalopathy (of the liver and brain). It is thought to be caused by damage to mitochondria and the aspirin metabolites decrease beta-oxidation by reversible inhibition of mitochondrial enzymes.

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9
Q

What is Wilson’s disease?

A

An autosomal recessive (defect in enzyme ATP-7B), causes inadequate copper excretion in the bile, which is the main mechanism of how we excrete excess copper. There is also a defect of copper transportation in the serum in the form of ceruloplasmin. Ceruloplasmin is going to be low, which is one of the diagnostic criteria.

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10
Q

Where organs does Wilson disease affect?

A

There is copper accumulation in liver (Hepatocellular carcinoma), brain (Basal ganglia degeneration and Parkinsonian symtoms), cornea (Kayser-Fleischer ring), kidney (hepatocellular carcinoma) and joints. Can also cause hemolytic anemia.

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11
Q

If AST is greater than ALT, what does it suggest?

A

[A Scotch and Tonic: AST > ALT]

That it might be an alcohol induced liver pathology.

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12
Q

What are the symptoms of Wilson disease?

A

Hepaticencephalopathy, dementia, Fancoi’s syndrome (Proximal tubule dysfunction).

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13
Q

What is the treatment for Wilson disease?

A

Penicillamine. [Pennies are made of copper]

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14
Q

What is hemochromatosis and what is elevated in lab findings?

A

Excess iron deposition in the body. Ferretin levels are increased. Increased total iron, decreased total iron-binding capacity (TIBC), increased transferrin saturation.

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15
Q

What is the classic triad of hemochromatosis?

A

AKA bronze diabetes: it is cirrhosis, diabetes mellitus, and skin pigmentation. It also cause congestive heart failure, testicular atrophy and increase risk of hepatocellular carcinoma.

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16
Q

What are the causes of hemochromatosis?

A
  1. Primary hemochromatosis: Autosomal recessive disorder.

2. Secondary hemochromatosis: Transfusion (chronic).

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17
Q

What is the treatment for hemochromatosis?

A

Phlebotomy and deferoxamine.

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18
Q

What does alpha 1 antitrypsin do?

A

Alpha 1 antitrypsin protects the cells from the proteolytic enzyme elastase.

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19
Q

What is hepatic angiosarcoma?

A

A malignant endothelial neoplasm in liver.

20
Q

What are the risk factors for hepatic angiosarcoma?

A

Vinyl chloride, arsenic.

21
Q

What is the percent of malignant transformation of hepatic adenoma?

A

10% of patients.

22
Q

What are the symptoms of alpha 1 antitrypsin deficiency?

A

Early onset of emphysema (panacinar). Cirrhosis (jaundice and elevated LFTs).

23
Q

What is a buzzword for Alcoholic cirrhosis?

A

AST to ALT greater than 2 to 1.

24
Q

What is a buzzword for biliary tract?

A

Elevated GGT and elevated Alkaline phosphate.

25
Q

What is a buzzword for bone formation?

A

Increased alkaline phosphate.

26
Q

What is a buzzword for Budd-Chiari?

A

No Jugular venous distention, ascities and hepatomegaly.

27
Q

What is a buzzword for Reye syndrome?

A

Aspirin, Peds (Pediatric), viruses.

28
Q

What is a buzzword for Wilson’s disease?

A

Copper, decreased ceruloplasmin, Keyser-Fleischer rings, Penicillamine.

29
Q

What is a buzzword for Hemachromatosis?

A

Bronze diabetes. Increased ferratin, decreased TIBC, Increased Transferrin sat.

30
Q

What is the buzzword for alpha1-antitrypsin deficiency?

A

Liver and lung emphysema (panacinar).

31
Q

What are the risk factors for hepatic adenoma?

A

OCP use, anabolic steroids, glycogen storage disease I and II. Most often in females 20-44 with years with OCP.

32
Q

What are the symptoms of hepatic adenoma?

A

RUQ pain, but usually there are no symptoms because it is often an incidental finding on imaging.

33
Q

What is the treatment for hepatic adenoma?

A

Discontinue OCP, serial imaging and AFP levels, and resection if over 5 cm.

34
Q

What is the most common primary tumor of the liver?

A

Hepatocellular carcinoma.

35
Q

What are some risk factors for hepatocellular carcinoma?

A

Hepatitis B and C, Wilson disease, Hemochromatosis, alpha antitrypsin deficiency, alcoholic cirrhosis, carcinogen exposure.

36
Q

What is the serum marker for hepatocellular carcinoma?

A

Alpha-fetoprotein (AFP).

37
Q

What is seen in the Budd-Chiari syndrome and what conditions are associated with?

A

Occlusion of the IVC or hepatic vein. It is associated with polycythemia vera, pregnancy, and hepatocellular carcinoma.

38
Q

What is the mechanism by which aspirin can cause Reye syndrome?

A

The metabolites of aspirin inhibit mitochondrial enzymes. It causes decreased beta-oxidation.

39
Q

A young man presents with ataxia and tremors. He has brown pigmentation in a ring around the periphery of his cornea. What treatment should he receive?

A

Penicillamine.

40
Q

What is the underlying problem in Wilson disease? What are the characteristics of Wilson disease? What is the treatment for Wilson disease?

A

The underlying problem is the impaired copper excretion; the body does not put copper into bile appropriately. It can cause asterixis, basal ganglia degeneration, low ceruloplasmin, cirrhosis, Kayser-Fleischer rings, copper accumulation, dementia, dyskinesia, and dysarthria. Treatment is penicillamine.

41
Q

RFF: Hepatomegaly, abdominal pain, ascites.

A

Budd-Chiari syndrome.

42
Q

RFF: Green/Yellow corneal deposits.

A

Wilson disease.

43
Q

RFF: Low serum ceruloplasmin.

A

Wilson disease.

44
Q

RFF: Cirrhosis, diabetes, and hyperpigmentation.

A

Hemochromatosis.

45
Q

Why does hemochromatosis cause diabetes?

A

Iron deposition in the pancreas causes dysfunction causing diabetes.