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Year 2 Gastrointestinal > Pathology of the liver IV > Flashcards

Pathology of the liver IV Flashcards

1
Q

what is hereditary hemochromatosis?

A

inherited disorder that increases the amount of iron that the body absorbs from the gut

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2
Q

most cases of hereditary hemochromatosis are caused by mutations in what gene?

A

HFE

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3
Q

what does HFE code for?

A

membrane protein regulating interaction of transferrin receptors with transferrin

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4
Q

is hepcidin increased or decreased in hereditary hemochromatosis?

A

decreased

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5
Q

what are the lab values for serum ferritin, transferrin saturation, and hepatic iron index in hereditary hemochromatosis?

A

serum ferritin over 300 (more than double)
transferrin saturation more than double
hepatic iron index greater than double

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6
Q

what warrants genetic testing for hereditary hemochromatosis?

A

high transferrin saturation and ferritin concentration

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7
Q

iron overload due to hereditary hemochromatosis can lead to what complications / conditions?

A 
diabetes 
cardiomyopathy 
hypogonadism 
skin pigmentation 
pseudo-gout
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8
Q

what are the causes of secondary iron overload?

A

THALAS

transfusions 
hemochromatosis, neonatal 
alimentary (Bantu) 
liver disease 
anemia, congenital (thalassemia) 
sideroblastic anemia
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9
Q

where is ceruloplasmin synthesized? what is its role?

A

liver

copper transporter

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10
Q

what are the ceruloplasmin levels in wilson disease?

A

low

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11
Q

what are the main organs affected by wilson disease?

A

liver, brain

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12
Q

what is the inheritance of wilson disease?

A

autosomal recessive

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13
Q

what characterizes wilson disease?

A

impaired copper biliary excretion

copper accumulation

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14
Q

what is the normal role of ATP7B?

A

shuttles copper from TGN to biliary system in hepatocytes

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15
Q

what lab values constitute diagnosis for wilson disease?

A

decreased serum CP
increased hepatic copper
increased urinary copper
kaiser fleischer rings

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16
Q

what is the inheritance of a1AT deficiency?

A

autosomal recessive

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17
Q

what genotype is associated with a1AT deficiency?

A

PiZZ genotype

18
Q

a1AT deficiency is due to misfolding of what protein?

A

PiZ

19
Q

what is the pathogenesis of a1AT deficiency?

A

accumulation of PiZ in hepatocytes

reduced intracellular protein degradation

increased autophagocytosis

20
Q

what lab values constitute diagnosis for a1AT deficiency?

A

low serum a1AT
abnormal electrophoretic a1AT
liver biopsy

21
Q

a1AT patients suffer from what condition?

A

pan-acinar emphysema

22
Q

which type of liver lesion is usually very benign?

A

focal nodular hyperplasia

23
Q

what are the features of focal nodular hyperplasia?

A

solitary mass
central scar
fibrous septa

24
Q

what are the features of nodular regenerative hyperplasia?

A

nodules WITHOUT fibrous septa
portal HTN

regeneration gone awry

25
Q

nodular regenerative hyperplasia can lead to what condition?

A

noncirrhotic portal HTN

26
Q

nodular regenerative hyperplasia is usually treated with what procedure?

A

TIPS

transjugular intrahepatic portal systemic shunt

27
Q

what is the most common benign hepatic tumor?

A

hemangioma

28
Q

hemangiomas are often associated with what medication?

A

oral contraceptives

29
Q

what is the morphology of hemangiomas?

A

solitary mass

30
Q

liver cell adenoma usually affects what population?

A

young women

31
Q

liver cell adenoma is associated with what condition?

A

glycogen storage disease

32
Q

what is the most common primary malignant liver tumor?

A

hepatocellular carcinoma

33
Q

what is the major risk factor hepatocellular carcinoma?

A

cirrhosis

34
Q

what tests are used to image hepatocellular carcinoma?

A

US
angiography
CT
MRI

35
Q

what lab test indicates hepatocellular carcinoma?

A

AFP over 1000

alpha fetoprotein

36
Q

cholangiocarcinoma affects what tissue?

A

bile duct epithelium

37
Q

how does fibrolamellar hepatocellular carcinoma affect prognosis?

A

good prognosis

38
Q

what is the most common primary malignant hepatic tumor in children? what is the diagnostic lab value?

A

hepatoblastoma

very high AFP

39
Q

angiosarcoma usually affects what population? what is the prognosis?

A

men in 5th and 6th decade

poor

40
Q

which primary tumors typically metastasize to the liver?

A 
GI 
breast 
lung 
pancreas 
melanoma

Year 2 Gastrointestinal (38 decks)

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