Pathology of the GI Tract- Liver and Gallbladder (3) Flashcards

1
Q

what is the most common morphologic pattern of drug or toxin induced liver injury?

A

necrosis of perivenular hepatocytes (zone 3) is the typical feature of drug-induced liver injury

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2
Q

what is the most common culprit for idiosyncratic reactions, which are the most common form of drug-induced liver injury ?

A

antimicrobial drugs

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3
Q

what are the three forms of alcohol related liver disease?

A

steatosis, steatohepatitis, and steatofibrosis/cirrhosis

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4
Q

how does steatosis present?

A

hepatomegaly with minimal symptoms, mildly elevated bilirubin and alk phos

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5
Q

how does steatohepatitis present?

A

tender hepatomegaly, +/- cholestasis, hyperbilirubinemia, AST:ALT 2:1 levels, elevated alk phos

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6
Q

how does steatofibrosis/cirrhosis present?

A

labs reveal hepatic dysfunction if enough liver tissue left, hypoproteinemia, coag abnormalities

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7
Q

what is NAFLD strongly associated with?

A

insulin resistance

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8
Q

what is a patient with NASH at risk for developing?

A

NASH cirrhosis or HCC

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9
Q

where does fibrosis of the liver typically develop?

A

around the central vein

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10
Q

how does pediatric NAFLD differ from adult NAFLD?

A

typically children show more diffuse steatosis and portal (rather than central) fibrosis, and ballooned hepatocytes may not be present

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11
Q

how does NAFLD present?

A

it is typically asymptomatic except for signs and symptoms of metabolic syndrome

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12
Q

what is hemochromatosis caused by?

A

excessive iron absorption

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13
Q

what is occurring in hereditary hemochromatosis?

A

regulation of intestinal absorption of dietary iron is abnormal

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14
Q

what is the main regulator of iron absorption?

A

hepcidin

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15
Q

what gene encodes for hepcidin?

A

HAMP gene

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16
Q

where is hepcidin produced?

A

the liver

17
Q

the adult form of hemochromatosis is usually caused by mutations of what gene?

A

HFE gene

18
Q

what is the most common HFE mutation?

A

a cysteine-to-tyrosine substitution at amino acid 282

19
Q

which population of people is the HFE mutation most common?

A

caucasian populations of european origin

20
Q

what are the principal manifestations of hemochromatosis?

A

hepatomegaly, abdominal pain, abnormal skin pigmentation, deranged glucose homeostasis or DM, cardiac dysfunction, and atypical arthritis

21
Q

how is hemochromatosis diagnosed?

A

very high levels of serum iron and ferritin and liver biopsy or genetic testing screening of family members of probands is important

22
Q

what stain shows hemochromatosis on histology slides?

A

prussian-blue stain

23
Q

what could be the final outcome of hemochromatosis?

A

cirrhosis or cardiac disease; hepatocellular carcinoma

24
Q

what is wilson disease?

A

an autosomal recessive disorder caused by mutation of the ATP7B gene resulting in impaired copper excretion into bile and a failure to incorporate copper into ceruloplasmin

25
Q

what are the manifestations of wilson disease?

A

accumulation of toxic levels of copper in many tissues and organs (liver, brain, and eye); hemolytic anemia

26
Q

what is the age of onset of wilson disease?

A

6 to 40 years of age

27
Q

how is the biochemical diagnosis of wilson disease made?

A

decreased serum ceruloplasmin, increased hepatic copper content, and increased urinary excretion of copper

28
Q

how does a patient with wilson disease present?

A

some with acute or chronic liver disease, some may have neurologic involvement (movement disorders or psychiatric symptoms) or hemolytic anemia

29
Q

what happens when copper is deposited into Descemet’s membrane of the cornea?

A

it presents as Kayser-Fleischer rings

30
Q

what is alpha-1-antitrypsin deficiency?

A

an autosomal recessive disorder of protein folding marked by very low levels of circulating alpha-1 antitrypsin

31
Q

what is the function of alpha-1-antitrypsin?

A

it inhibits proteases (particularly neutrophil elastase)

32
Q

what does alpha-1-antitrypsin deficiency lead to?

A

pulmonary emphysema and it causes liver disease as a consequence of hepatocellular accumulation of the misfolded protein

33
Q

what is the most common clinically significant mutation leading to alpha-1-antitrypsin deficiency?

A

PiZ

34
Q

when is alpha-1-antitrypsin deficiency diagnosed?

A

because of it’s early presentation with liver disease, alpha 1 AT deficiency is the most commonly diagnosed inherited hepatic disorder in infants and children

35
Q

what type of mutation leads to alpha-1 AT deficiency?

A

a point mutation (glu342–>Lys342)

36
Q

what are the histologic features associated with alpha-1 AT deficiency?

A

cytoplasmic globular inclusions in hepatocytes

37
Q

what are the 3 clinical presentations of alpha-1 AT deficiency?

A

neonatal hepatitis with cholestatic jaundice; adolescence (hepatitis, cirrhosis, or lung disease); middle to late life (cirrhosis +/- HCC)