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Gastrointestinal System > EXAM #2: LIVER PATHOLOGY IV > Flashcards

EXAM #2: LIVER PATHOLOGY IV Flashcards

1
Q

What is Hereditary Hemochromatosis?

A

Herediatry disorder that increases the amount of iron absorbed from the gut

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2
Q

What gene is implicated in Hereditary Hemochromatosis?

A

HFE gene

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3
Q

What is the link between transferrin, hepcidin, and iron absorption?

A
  • Hepcidin blocks Fe++ absorption from the gut
  • Decreased Hepcidin causes increased Fe++ absorption from the gut
  • HFE membrane product regulates the transcription of Hepcidin
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4
Q

What is the pathogenic change that happens in early hemochromatosis?

A

Fe++ deposits in periportal hepatocytes

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5
Q

What is the pathogenic change that happens in advanced hemochromatosis?

A

Fe++ deposition in:

  • Kupffer cells
  • Bile duct epithelium
  • Fibrous septa
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6
Q

What are the lab findings in Hereditary Hemochromatisis?

A
  • High Ferretin
  • Low TBIC
  • High serum iron
  • High % saturation

*Classically there is 2x normal transferrin saturation and and drastically elevated serum ferretin

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7
Q

What are the complications of Hereditary Hemochromatosis?

A

1) DM
2) Cardiomyopathy
3) Hypogonadism
4) Skin pigmentation
5) Pseudo-gout

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8
Q

What is the mnemonic to remember the causes of secondary iron overload? What is most common?

A

THALAS

T= Transfusion***** 
H= Hemochromatosis 
A= Alimentary (Bantu siderosis)
L= Liver Disease 
A= Anemia (Thalassemia) 
S= Sideroblastic anemia
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9
Q

In secondary hemochromatosis, where is iron loaded?

A

Macrophages i.e. Kupffer cells

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10
Q

What is the treatment for Hemochromatosis?

A

Phlebotomy

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11
Q

List the roles that copper plays in the body.

A

1) Pigment formation
2) NT production
3) Peptide formation
4) Connective tissue biosynthesis
5) Antioxidant defense

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12
Q

What is the role of ceruloplasmin in the body?

A

Liver protein that binds copper for non-toxic circulation in the body

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13
Q

What is Wilson’s Disease?

A

Herditary disease that causes the body to retain copper

  • Decreased ceruloplasmin
  • Excessive copper deposition
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14
Q

What organs are most affected by Wilson’s Disease?

A

Brain

Kidneys

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15
Q

What gene is mutated in Wilson’s Disease?

A

ATP7B copper transporter

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16
Q

What is the result of the mutation in Wilson’s Disease?

A
  • Impaired biliary copper excretion

- Copper cannot get to the biliary canaliculi

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17
Q

What are the clinical manifestations of Wilson’s Disease?

A

1) Degeneration of the basal ganglia causing a PD like pheotype
2) Hepatitis
3) Cirrhosis
4) Hemolytic anemia
5) Cardiomyopathy

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18
Q

What is the most common initial manifestation of Wilson’s Disease in children?

A

Hepatitis

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19
Q

What is the most common initial manifestation of Wilson’s Disease in adults?

A

Neurologic/ psychiatric disease

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20
Q

How is Wilson’s Disease diagnosed?

A
  • Low serum ceruloplasmin
  • Increased urinary copper
  • Increased hepatic copper
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21
Q

What PE sign is associated with Wilson’s Disease?

A

Kayser-Fleischer rings

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22
Q

How is Wilson’s Disease treated?

A

Chelation

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23
Q

What is alpha-1 antitryspin deficiency?

A
  • Deficiency in the enzyme that inhibits proteases

- Leads to abnormal breakdown of proteins, especially in lungs

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24
Q

What genotype is assocaited with alpha-1 antitrypsin?

A

PiZZ

  • Normal genotype of antitrypsin is PiMM
  • PiZZ variant is the most clinically significant form that leads to alpha-1 antitrypsin deficiency
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25
Q

What is the etiology of alpha-1 antitryspin deficiency?

A

Misfolded/nonfunctional PiZ accumulates in hepatocytes

26
Q

What are the clinical manifestations of alpha-1 antitryspin deficiency in children?

A

Hepatitis/cholestasis

27
Q

What are the clinical manifestations of alpha-1 antitryspin deficiency in adults?

A

Chronic hepatitis/cirrhosis

28
Q

How is alpha-1 antitryspin deficiency diagnosed?

A
  • Low serum Alpha-1 AT
  • Abnormal Alpha-1 AT electrophoresis
  • Liver biopsy
29
Q

Aside from liver disease, what else is highly associated with alpha-1 antitryspin deficiency?

A

Early onset emphysema

30
Q

What is Focal Nodular Hyperplasia of the liver?

A
  • Benign tumor of the liver
  • More common in women
  • Asymptomatic–does NOT require treatment
31
Q

What is the morphological appearance of Focal Nodular Hyperplasia?

A

Solitary mass on the liver with a central scar

32
Q

What is Nodular Regenerative Hyperplasia?

A

Diffuse nodular transformation of the liver WITHOUT fibrosis

Think of regeneration gone awry*

33
Q

What is Nodular Regenerative Hyperplasia commonly confused with?

A

Cirrhosis

34
Q

What is the most common complication of Nodular Regenerative Hyperplasia?

A

Noncirrhotic portal hypertension

35
Q

What is the surgical treatment for Nodular Regenerative Hyperplasia?

A

Transjugular Intrahepatic Portal Shunt

36
Q

What is Nodular Regenerative Hyperplasia associated with?

A

Transplantation
Chemotherapy
Vasculitis

37
Q

What is the most common benign hepatic tumor?

A

Hemangioma

38
Q

What is a Hemangioma?

A

Blood vessel tumor

39
Q

What patient population are Hemangiomas more common in?

A

Women on oral contraceptives

40
Q

What do you need to remember about the diagnosis of a Hemangioma?

A

Do NOT biopsy

41
Q

What is a Liver Cell Adenoma?

A

Glandular tumor of the liver

42
Q

What patient populations are Liver Cell Adenomas more common in?

A
  • Young women on oral contraceptives

- GSD (I and III)

43
Q

What is the most feared complication of a Liver Cell Adenoma?

A

Rupture during pregnancy

44
Q

What is the most common primary malignant liver tumor?

A

Hepatocellular Carcinoma

45
Q

Geographically, where is HCC most common?

A

Asia

Africa

46
Q

What is the biggest risk factor for HCC?

A

Cirrhosis and the other disorders that lead to cirrhosis

47
Q

List the risk factors for HCC.

A

1) Chronic hepatitis (HBV and HCV)
2) Cirrhosis
- Alcohol
- NAFLD
- Hemochromatosis
- Wilson’s
- A1AT
3) Aflatoxin

48
Q

What genetic change is associated with progression to HCC?

A

p53

Note that Aspergillus/ aflatoxin causes p53 mutations

49
Q

How does HBV lead to HCC?

A
  • HBV is dsDNA virus
  • Integrates into host
  • Activates proto-oncogenes
50
Q

What are the clinical manifestations of HCC?

A

1) Painful hepatomegaly
2) Abdominal mass
3) Weight loss
4) Portal/hepatic vein thrombosis
5) Hemorrhagic ascites
6) Hepatic Failure
7) Massive bleeding

51
Q

What lab elevation is associated with HCC?

A

AFP greater than 1,000

52
Q

What subtype of HCC has the best prognosis?

A

Fibrolamellar HCC

53
Q

What is the primary malignant tumor of the bile duct epithelium?

A

Cholangiocarcinoma

54
Q

What are the risk factors for Cholangiocarcinoma?

A

1) Primary Sclerosing Cholangitis
2) Thorotrast
3) Caroli Disease
4) Liver Flukes

55
Q

What is the most common primary malignant hepatic tumor in children?

A

Hepatoblastoma

56
Q

What is a Hepatoblastoma?

A

asdf

57
Q

What lab is associated with Hepatoblastoma?

A

Elevated AFP

58
Q

What is an angiosarcoma?

A

This is a cancer of the inner lining of blood vessels associated with:

  • Thorotrast
  • Steroids

*V. poor prognosis

59
Q

What is the most common malignant tumor of the liver?

A

Metastases

60
Q

Where are the primary tumors of liver metastases commonly located?

A
  • GI
  • Breast
  • Lung
  • Pancreas
  • Melanoma

Gastrointestinal System (42 decks)

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