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Gastrointestinal > Liver Pathology IV > Flashcards

Liver Pathology IV Flashcards

1
Q

What is the underlying cause of hereditary hemochromatosis?

A

HFE gene mutation, causing changes in hepatocidin

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2
Q

What are the ssx of hemachromatosis?

A

psychosis

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3
Q

What is the pathophysiology of hereditary hemochromatosis?

A

Excess deposition of Fe in the liver d/t increased uptake

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4
Q

What is the transport enzymes in the enterocytes that is regulated by heptacidin?

A

Ferroportin

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5
Q

What is the M:F ratio of hereditary hemochromatosis?

A

7:1

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6
Q

What are the two mutant alleles with hemochromatosis?

A

C282Y and H63D

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7
Q

What are the early changes in hemochromatosis?

A

Fe deposits in the periportal hepatocytes and other cells

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8
Q

What are the late changes in hemochromatosis?

A

Fe deposition in Kupffer cells, bile duct epi

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9
Q

When do the severe ssx usually occur with hemochromatosis?

A

40s ish

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10
Q

What level of serum Fe suggests hemochromatosis?

A

> 300 mg/dL

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11
Q

What level of transferrin saturation suggests hemochromatosis?

A

> 2x normal

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12
Q

What level of transferrin serum ferritin suggests hemochromatosis?

A

> 2x

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13
Q

What are the two lab tests the demand thinking about hemochromatosis?

A

Elevated transferrin and ferritin

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14
Q

What are the comorbidities of hemochromatosis?

A

DM
Caridomyopathy
Hypogonadism

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15
Q

What are the skin changes with hemochromatosis?

A

gray pigmentation

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16
Q

What is the cause of pseudogout in hemochromatosis?

A

build up of Fe in joints

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17
Q

What are the pituitary changes with hemochromatosis?

A

Hypopituitarism

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18
Q

What is the stain for Fe?

A

Prussian blue

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19
Q

What CA are pts with hemochromatosis susceptible to?

A

Hepatocellular CA

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20
Q

What are the histological changes in the liver with hemochromatosis?

A

Hepatocellular swelling

Fe stain in Kupffer cells

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21
Q

What cause the DM in hemochromatosis?

A

Fe build up in the pancreas

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22
Q

What are the causes of secondary hemochromatosis?

A 
Transfusions
Hemochromatosis
Alimentary
Liver disease
Anemia
Sideroblastic anemia
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23
Q

What is the cause of sideroblastic anemia?

A

Inability to incorporate Fe into Hb–causes ring cells

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24
Q

What cells store the Fe in secondary hemochromatosis in the liver?

A

Kupffer cells

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25
Q

What is the cause of Wilson’s disease?

A

AR disorder d/t mutation in ATP7B Cu-transporter, causing deposition of Cu in the liver

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26
Q

What is Cu needed for?

A
  • Pigmentation
  • Neurotransmitter production
  • CT synthesis
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27
Q

What is the protein that hold Cu in the circulation?

A

Ceruloplasmin

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28
Q

How is Cu excreted?

A

Biliary system

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29
Q

What are the two organs that accumulate Cu in Wilson’s disease?

A

Brain and liver

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30
Q

What are the ssx of Wilson’s disease?

A

Psychosis

Parkinson’s

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31
Q

What is the function of ATP7B?

A

Takes Cu in hepatocyte into the biliary system

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32
Q

What are the histological changes in Wilson’s disease?

A

Steatosis
Mallory bodies
Nuclear vacuoles

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33
Q

What cause the parkinson’s symptoms in hemochromatosis?

A

Destruction of Dopaminergic neurons

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34
Q

What are the kidney issues with Wilson’s disease?

A

Aminoaciduria

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35
Q

What are the eye findings with Wilson’s disease?

A

Kayser-Fleischer rings

36
Q

What are the cardiac problems with Wilson’s disease?

A

Cardiomyopathy

37
Q

What are the childhood problems with Wilson’s disease?

A

Hepatitis

Acute/fulminant hepatitis

38
Q

What are the diagnostic labs for Wilson’s disease? (3)

A
  • Decreased serum ceruloplasmin
  • Increased hepatic copper
  • Increase urinary Cu
39
Q

What is the stain for Cu?

A

Rhodanine stain

40
Q

What is alpha-1-antitrypsin deficiency?

A

AR disorder in anti- Trypsin (protease), causing unregulated trypsin destruction

41
Q

What is the treatment for Wilson’s disease?

A

Chelating agents

42
Q

What is the treatment for hemochromatosis?

A

Phlebotomy

43
Q

What is the primary liver disease in children?

A

Alpha-1-antitrypsin deficiency

44
Q

What is the protein that is misfolded in alpha-1-antitrypsin deficiency?

A

PiZ

45
Q

What protein accumulates in alpha-1-antitrypsin deficiency? Where?

A

PiZ in hepatocytes

46
Q

What are the ssx of alpha-1-antitrypsin deficiency?

A

Neonatal = hepatitis/cholestasis

Adults = chronic hepatitis and cirrhosis

47
Q

What is the treatment for alpha-1-antitrypsin deficiency?

A

Liver transplant

48
Q

What are the diagnostic labs for alpha-1-antitrypsin deficiency?

A
  • Low serum alpha 1AT

- Abnormal electrophoretic alphaAT

49
Q

What is the stain that is useful for alpha-1-antitrypsin deficiency?

A

PAS

50
Q

What is the cause of COPD with alpha-1-antitrypsin deficiency?

A

degradation of the alveolar sacs

51
Q

What zone of the liver is affected first in alpha-1-antitrypsin deficiency?

A

Zone 1

52
Q

What is nodular regenerative hyperplasia?

A

benign nodular regeneration all over the liver

53
Q

What is focal nodular hyperplasia?

A

Frequent benign liver tumor, often discovered incidentally

54
Q

What is nodular hyperplasia usually confused with?

A

Hepatocellular CA

55
Q

In whom does focal nodular hyperplasia usually occur?

A

Women on birth control

56
Q

What is the treatment for focal nodular hyperplasia?

A

No issues with it.

57
Q

What are the gross findings with focal nodular hyperplasia?

A

Solitary mass

Central scar

58
Q

Are focal nodular hyperplasia usually encapsulated?

A

No, but well demaracated

59
Q

What is the difference between nodular regenerative hyperplasia, and hepatocellular CA?

A

Nodules WITHOUT fibrous septa

60
Q

What does nodular regenerative hyperplasia cause? What is the treatment for this?

A

Non-cirrhotic portal HTN

Shunt/Endoscopic treatment/beta blockers

61
Q

What is the most common complication of nodular regenerative hyperplasia? How do they fix this surgically?

A

Portal HTN

TIPS shunt

62
Q

What is the most common benign hepatic tumor?

A

Hemangioma

63
Q

What drug is correlated with hepatic adenomas?

A

Oral contraceptives or steroids

64
Q

What are cavernous hemangiomas?

A

composed of dilated, variably-sized vascular spaces lined by flattened endothelial cells and supported by connective tissue septa. Thrombi are often present.

65
Q

Who usually have liver cell adenoma?

A

Young women on oral contraceptives, or men taking steroids

66
Q

What glycogen storage diseases cause liver cell adenoma?

A

1 (Pompe’s) and 3

67
Q

Is there a capsule around the hepatic adenomas? Is there vessels within it?

A

Encapsulated without vessels

68
Q

What is the most common primary malignant liver tumor?

A

Hepatocellular CA

69
Q

What can cirrhosis lead to?

A

Hepatocellular CA

70
Q

What are the aflatoxins that cause cause hepatocellular CA?

A

Fungal toxins

71
Q

What is the pathophysiology with hepatocellular CA?

A

Proliferating hepatocytes d/t chronic insult

72
Q

HBV is what type of virus? Genetic material? What are the things it has that causes hepatocellular CA?

A

Hepadnavirus
dsDNA
HBx inhibits p53

73
Q

What is the pre-S mutation?

A

HBV surface antigen mutation that inhibit p26, Rb, other cell cycles.
Found on some, but not all HBVs

74
Q

What are the ssx of hepatocellular CA?

A

Painful hepatomegaly

Massive bleeding

75
Q

What is the protein that is associated (high) with hepatocellular CA?

A

Alpha Fetoprotein >1000

76
Q

Do fibrolamellar hepatocellular CA have a better or worse prognosis that hepatocellular CA? What are these?***

A

Better

Broad thick bands of fibrous tissue that surround the malignant cells

77
Q

What is cholangioCA?

A

Malignant primary tumor of the bile duct epithelium

78
Q

What are the Risk factors for cholangioCA?

A
  • Liver flukes (opisthorchis sinensis)

- Primary sclerosing cholangitis

79
Q

What is a hepatoblastoma? What is the pathway that is thought to be mutated in this disease?

A

Uncommon malignant liver cancer occurring in infants and children and composed of tissue resembling fetal liver cells, mature liver cells, or bile duct cells. They usually present with an abdominal mass. The disease is most commonly diagnosed during a child’s first three years of life. Caused by a WNT pathway mutation

80
Q

What is the most common primary malignant tumor of children?

A

Hepatoblastoma

81
Q

What is the lab that is elevated with hepatoblastomas? What if this lab is not elevated?

A

AFP–if not bad prognosis

82
Q

What are angiosarcomas? In whom does the liver type occur in?

A

Malignant endothelial cells of blood vessels. If in the liver, usually caused by PVC workers

83
Q

What are the predisposing factors to angiosarcomas?

A

Exposure to thorotrast

Vinyl chloride

84
Q

What is the prognosis for angiosarcomas?

A

Very poor

85
Q

What is the most common malignant tumor of the liver (NOT primary tumor)? What are the ssx of this?

A

Mets from the GI tract, or breast.

ssx are usual liver problems

86
Q

What are the histological characteristics of cholangiocarcinoma?

A

Tons of duct and glands

Gastrointestinal (42 decks)

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