EXAM #2: LIVER PATHOLOGY IV Flashcards Preview

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Flashcards in EXAM #2: LIVER PATHOLOGY IV Deck (60):
1

What is Hereditary Hemochromatosis?

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

2

What gene is implicated in Hereditary Hemochromatosis?

HFE gene

3

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

- Hepcidin blocks Fe++ absorption from the gut
- Decreased Hepcidin causes increased Fe++ absorption from the gut
- HFE membrane product regulates the transcription of Hepcidin

4

What is the pathogenic change that happens in early hemochromatosis?

Fe++ deposits in periportal hepatocytes

5

What is the pathogenic change that happens in advanced hemochromatosis?

Fe++ deposition in:
- Kupffer cells
- Bile duct epithelium
- Fibrous septa

6

What are the lab findings in Hereditary Hemochromatisis?

- High Ferretin
- Low TBIC
- High serum iron
- High % saturation

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

7

What are the complications of Hereditary Hemochromatosis?

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

8

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

THALAS

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

9

In secondary hemochromatosis, where is iron loaded?

Macrophages i.e. Kupffer cells

10

What is the treatment for Hemochromatosis?

Phlebotomy

11

List the roles that copper plays in the body.

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

12

What is the role of ceruloplasmin in the body?

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

13

What is Wilson's Disease?

Herditary disease that causes the body to retain copper
- Decreased ceruloplasmin
- Excessive copper deposition

14

What organs are most affected by Wilson's Disease?

Brain
Kidneys

15

What gene is mutated in Wilson's Disease?

ATP7B copper transporter

16

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

- Impaired biliary copper excretion
- Copper cannot get to the biliary canaliculi

17

What are the clinical manifestations of Wilson's Disease?

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

18

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

Hepatitis

19

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

Neurologic/ psychiatric disease

20

How is Wilson's Disease diagnosed?

- Low serum ceruloplasmin
- Increased urinary copper
- Increased hepatic copper

21

What PE sign is associated with Wilson's Disease?

Kayser-Fleischer rings

22

How is Wilson's Disease treated?

Chelation

23

What is alpha-1 antitryspin deficiency?

- Deficiency in the enzyme that inhibits proteases
- Leads to abnormal breakdown of proteins, especially in lungs

24

What genotype is assocaited with alpha-1 antitrypsin?

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

25

What is the etiology of alpha-1 antitryspin deficiency?

Misfolded/nonfunctional PiZ accumulates in hepatocytes

26

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

Hepatitis/cholestasis

27

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

Chronic hepatitis/cirrhosis

28

How is alpha-1 antitryspin deficiency diagnosed?

- Low serum Alpha-1 AT
- Abnormal Alpha-1 AT electrophoresis
- Liver biopsy

29

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

Early onset emphysema

30

What is Focal Nodular Hyperplasia of the liver?

- Benign tumor of the liver
- More common in women
- Asymptomatic--does NOT require treatment

31

What is the morphological appearance of Focal Nodular Hyperplasia?

Solitary mass on the liver with a central scar

32

What is Nodular Regenerative Hyperplasia?

Diffuse nodular transformation of the liver WITHOUT fibrosis

****Think of regeneration gone awry*****

33

What is Nodular Regenerative Hyperplasia commonly confused with?

Cirrhosis

34

What is the most common complication of Nodular Regenerative Hyperplasia?

Noncirrhotic portal hypertension

35

What is the surgical treatment for Nodular Regenerative Hyperplasia?

Transjugular Intrahepatic Portal Shunt

36

What is Nodular Regenerative Hyperplasia associated with?

Transplantation
Chemotherapy
Vasculitis

37

What is the most common benign hepatic tumor?

Hemangioma

38

What is a Hemangioma?

Blood vessel tumor

39

What patient population are Hemangiomas more common in?

Women on oral contraceptives

40

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

Do NOT biopsy

41

What is a Liver Cell Adenoma?

Glandular tumor of the liver

42

What patient populations are Liver Cell Adenomas more common in?

- Young women on oral contraceptives
- GSD (I and III)

43

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

Rupture during pregnancy

44

What is the most common primary malignant liver tumor?

Hepatocellular Carcinoma

45

Geographically, where is HCC most common?

Asia
Africa

46

What is the biggest risk factor for HCC?

Cirrhosis and the other disorders that lead to cirrhosis

47

List the risk factors for HCC.

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

48

What genetic change is associated with progression to HCC?

p53

Note that Aspergillus/ aflatoxin causes p53 mutations

49

How does HBV lead to HCC?

- HBV is dsDNA virus
- Integrates into host
- Activates proto-oncogenes

50

What are the clinical manifestations of HCC?

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

51

What lab elevation is associated with HCC?

AFP greater than 1,000

52

What subtype of HCC has the best prognosis?

Fibrolamellar HCC

53

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

Cholangiocarcinoma

54

What are the risk factors for Cholangiocarcinoma?

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

55

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

Hepatoblastoma

56

What is a Hepatoblastoma?

asdf

57

What lab is associated with Hepatoblastoma?

Elevated AFP

58

What is an angiosarcoma?

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

*V. poor prognosis

59

What is the most common malignant tumor of the liver?

Metastases

60

Where are the primary tumors of liver metastases commonly located?

- GI
- Breast
- Lung
- Pancreas
- Melanoma