Tumors of Liver and metabolic Dx Flashcards Preview

GI > Tumors of Liver and metabolic Dx > Flashcards

Flashcards in Tumors of Liver and metabolic Dx Deck (43):
1

most common form of hereditary hemochromatosis

AR dx of adult onset caused by mutations in the HFE gene produing hepcidin

2

ways to get secondary iron overload

multiple transfusions, ineffective erythropeosis, increased iron intake

3

major other diseases in hemochromatosis

cirrohosis, diabetes, and skin pigmentation

4

pathogenesis of hereditary hemochromatosis

defect in regulation of intestinal absorption of dietary iron

5

ways excess iron damages tissue

lipd peroxidation
stimulation of collagen formation
direct interactions of iron with DNA

6

morpholoc changes in hemochromatosis are responses to

deposition of hemosiderin in the liver, pancreas, heart and other organs

7

hepatomegaly, skin pigmentation, deranged glucose homeostatis/Diabetes, atypical arthritis

hemochromatosis

8

treatment for hemochromatosis

phlebotomy

9

genetic cause of wilson's disease

loss of function in ATP7B gene on Chromosome 13

10

patheogenesis of wilson's disease

lasck of ATP7B activity keeps copper from being excreted into bile, so then accumulates in hepatocytes

11

method of cellular damage in Wilson's disease

excecc copper: Promotes free radicals, binds to sulfydryl groups of cellular proteins, and displaces other metals in hepatic metalloenzymes

Free copper damages red cells

12

main sx of Wilsons dx

green-brown rings in eyes

13

gross organ damage in Wilsons disease

fatty liver, hepatitis like injury progessing to cirhoisiss, basal ganglia damage, ele lesions

14

acute or chronic liver dx with psychosis, parkinson-like disease, and eye rings

Wilson disease

15

liver disease plus liver disease equals

a1 antitrypsin deficiency

16

PiZZ genotype

a1 antitrypsin deficiency

17

periodic acid-schiff stain showing magenta cytoplasmic granules

a1 antitrypsin deficiency

18

most common benign lesion of liver

cavernous hemangioma

19

what NOT to do in a cavernous hemangioma

stick a needle in one

20

discreete red-blue, soft nodules just below the capsule

cavernous hemangioma

21

loalized, well-demarceted, poorly encapsulated lesion with a central fibrous scar

focal nodular hyperplasia

22

occurs in women of child bearing age taking OCPs

hepatic adenoma

23

well-demarcated but unencapsulated pale, yellow-tan or bile colored lesion

hepatic adenoma

24

risks of hepatic adenoma

can rupture, particularly in pregnancy, Can possible turn into hepatocellular carcinoma

25

major pathway to hepatocellular carcinoma from chronic liver disease

dysplastic nodules in cirrhotic livers

26

major liver carcinogens

Hep B/C, alcoholic cirrhosis, alfatoxin exposure

27

almost universial feature of hepatocellular carcinoma

structural and numeric chromosomal abnormalities indicitive of genomic instability

28

most important genomic instabilies in live cancer

inflammation and regeneration from chronic hepatitis
aquired mutatins in oncogenes and tumor supressors
aquired defects in DNA repair

29

liver cancer has a strong propensity for

vascular invasion

30

globules of bile within the cytoplasm of cels and pseudocanaliculi

liver cancer

31

rapid increase in liver size, sudden worsening of ascites, fever or pain

liver cancer

32

current test for liver cancer

radiologic screening of pts with cirrhosis every 6 months

33

age and sex of most hemochromatosis

50-70, male

34

most common mutations in hemachromatosis

c282y and H63D on HFR

35

most common type of liver malignancy

hetastases

36

adenocarcinoma of the bile ducts is called

cholangiocarcinoma

37

produces malignnat glands and tubules and does not contain bile

cholangiocarcinoma

38

can cause cholangiocarcinoma

thorotrast, liver flukes, primary scedosing cholangitis

39

dense desmoplastic stroma

cholangicarcinoma

40

cords and nests of malignant hepatocytes

hepatic carcinoma

41

bile and hyaline globules

hepatic carcinoma

42

liver cancer with decent prognosis

fibrolamellar

43

broud fibrous bands separating islands of eosinophillic cells

fobrolameller