Wk5 Liver Path pt2 Flashcards

1
Q

early EtOH abuse

enlarged, lipid filled liver

How does it look microscopically?

A

Alcoholic steatosis–fatty liver

large lipid vacuoles

**reverses with abstinence

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2
Q

Alcoholic hepatitis = steatosis + ?

A

hepatocyte injury and/or inflammation

“steatohepatitis”

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3
Q

Cytokeratin aggregates in hepatocytes:

Associated with?

A

Mallory bodies

Alcoholic hepatitis

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4
Q

Usually seen surrounding Mallory bodies:

A

neutrophilic inflammation

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5
Q

Alcoholic hepatitis occurrence

pre-cursor to cirrhosis

A

steatofibrosis

**perivenular and pericellular

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6
Q

Grossly: fibrosis + nodules

A

cirrhosis

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7
Q

Liver in early stages of alcoholic cirrhosis

A

enlarged

fatty

micronudular (less 3 mm)

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8
Q

Liver in late stages of alcoholic cirrhosis:

A

shrunken

non-fatty

variable nodule size

cholestasis usually present

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9
Q

5 major causes of death from alcoholic liver disease:

A
  1. hepatic encephalopathy and coma
  2. Massive GI tract hemorrhage (esophageal varicies)
  3. infx
  4. hepatorenal syndrome
  5. hepatocellular carcinoma
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10
Q

NAFLD?

Patient profile?

A

Non-alcoholic fatty liver disease

  • metabolic syndrome
  • obesity
  • DM2
  • dyslipidemia
  • insulin resistance
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11
Q

autoimmune cholangiopathy

progressive destruction of small and medium sized INTRAhepatic bile ducts

extrahepatic ducts are spared

middle age females

A

Primary biliary cirrhosis

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12
Q

AMA (antimitochondrial antibodies)

associated with other autoimmune disorders

A

Primary biliary cirrhosis

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13
Q

fatigue

anicteric pruritis

xanthomas

steatorrhea

vit D malabsorption

A

Primary biliary cirrhosis

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14
Q

Tx for PBC:

A

urodeoxycholic acid

liver transplant

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15
Q

Dx of PBC:

A

liver biopsy

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16
Q

Damage due to prolonged EXTRAhepatic bile duct obstruction

A

Secondary biliary cirrhosis

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17
Q

progressive

RANDOM

autoimmune

uneven fibroinflammatory

extra/intrahepatic bile ducts

A

Primary sclerosing cholangitis (PSC)

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18
Q

pANCA

IBD association

A

Primary sclerosing cholangitis

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19
Q

“beaded” appearance on cholangiogram

A

PSC

primary sclerosing cholangitis

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20
Q

disorders of excessive iron absorption resulting in accumulation of iron in tissues producing organ injury

A

hemochromotosis

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21
Q

Inheritance pattern of primary hemochromotosis

A

autosomal recessive

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22
Q

Liver protein decreased in hemochromotosis

A

hepcidin –> excessive intestinal iron absorption

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23
Q

Most common genetic mutation in hereditary hemochromotosis

A

HFE – chromosome 6

C282Y – 80% of cases in adults

**most common in male of northern european descent

low penetrance so not all homozygotes express disease

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24
Q

Causes of secondary hemochromotosis

A

parenteral Fe overloads (transfusions)

ineffective erythropoesis –> increased Fe absorption
-(B-thalassemis, sideroblastic anemia)

increased oral intake of Fe

Chronic EtOH liver disease

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25
Q

Reread slide 42

A

distribution of excess Fe

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26
Q

Early pathological finding of primary hemochromotosis:

A

periPORTAL iron deposits in hepatocytes

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27
Q

Early pathological finding of secondary hemochromotosis (different from primary):

A

Fe accumulates in Kupffer cells not hepatocytes

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28
Q

Classic triad for hemochromotosis presentation:

A
  1. cirrhosis
  2. diabete
  3. skin pigmentation

“Bronze diabetes”

**also may see sx in other organ systems (heart, joints, etc.)

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29
Q

Best screening test for hemochromotosis:

A

fasting transferrin saturation

if greater than 45% –> serum ferritin

if ferritin elevated –> HFE gene test

30
Q

Reason for liver bx in hemochrmatosis:

A

concern for cirrhosis

31
Q

Tx for hemochromatosis:

A

chelating agents / phlebotomy

32
Q

Autosomal recessive

disorder of copper metabolism

A

Wilson’s disease

33
Q

Genetic defect in Wilson’s disease

A

ATP7B gene

chromosome 13

34
Q

ATP7B?

A

gene for liver copper transporting ATPase protein

impaired secretion as ceruloplasmin and impaired secretion into bile

–> toxic levels of Cu in tissues

35
Q

Dx test for Wilson’s

A

plasma ceruloplasmin level

24 hr urine copper excretion

36
Q

When to consider Wilson’s in your Ddx:

A

liver disease in anyone under 30 yo

37
Q

Presenting sx of Wilson’s disease:

A

neurpsychiatric

KAYSER-FLEISCHER rings
-copper deposits at limbus of cornea

38
Q

What is alpha-1-antitrypsin?

A

a protease inhibitor (primarily neutrophils elastase)

**without it, tissues get degraded unchecked

39
Q

A1AT gene location

A

ch 14

40
Q

Pathogenesis of liver disease in A1AT deficiency:

A

intracellular misfolding –> accumulation –> apoptosis of hepatocytes

41
Q

Most commonly dx hepatic disorder in infants and childrren:

A

A1AT deficiency

42
Q

PAS stain

A

A1AT deficiency

43
Q

Always include what in Ddx of any form of liver disease?

A

Toxins

44
Q

CPC – chronic passive congestion

A

slide 73

45
Q

nutmeg liver

A

centrilobular hemorrhagic necrosis

46
Q

thrombosis of two or more hepatic vein branches

classic triad:

hepatomegaly

ascites

abd pain

A

Budd-Chiari syndrome

47
Q

most common causes of Budd-Chiari syn:

A

hyercoaguable states

48
Q

Dx for Budd-Chiari

A

Doppler US

MRI/CT

looking for clots

49
Q

obstructive, NONthrombotic lesions of small (central) hepatic veins

radiation/hepatotoxin exposure

BM tx

A

Sinusoidal obstruction syndrome

50
Q

sudden weight gain

hepatomegaly
increased serum bilirubin

A

Sinusoidal obstruction syndrome (ACUTE)

51
Q

prolonged conjugated hyperbilirubinemia in the neonate

A

neonatal cholestasis

52
Q

Two causes of neonatal cholestasis

A
  1. biliary atresia (extrahepatic)

2. neonatal hepatitis

53
Q

Causes of granulomatous hepatitis:

A

idiopathic 50%

sarcoid 22%

drugs 6%

TB 3%

other 19%

54
Q

HELLP syndrome?

A

pregnancy

Hemolysis

Elevated Liver enzymes

Low Platelets

55
Q

microvesicular steatosis in pregnancy

A

Acute fatty-liver of pregnancy

56
Q

mild increase in conjugated bilirubin in pregnancy

A

Intrahepatic cholestasis of pregnancy

57
Q

portal inflammation

lymphocytic cholangitis

“vanishing bile ducts”

A

chronic GVHD from BM tx

58
Q

Tests that reflect hepatocellular damage

NOT hepatic function

A

AST/ALT

59
Q

Reflect injury to bile ducts/canalicular membranes

markers of cholestasis

A

Alk Phos

GGT

60
Q

Markers of hepatic function

A

Albumin

PT

clotting factors

61
Q

Most specific test for liver damage

A

ALT

**AST is found in other tissues

62
Q

Why is AST elevated preferentially in EtOH disease?

A

found in mitochondria

EtOH is mitochondrial toxin

63
Q

Increased Alk Phos usually due to?

How to help differentiate?

A

bone or liver

GGT from biliary epithelium

64
Q

Two purposes of Liver biopsy:

A
  1. determine possible cause of disease

2. determine extent of liver damage

65
Q

Liver bx:

ground glass hepatocytes

A

chronic viral HBV

66
Q

Liver bx:

plasma cells

A

autoimmune hepatitis

PBC (primary biliary cirrhosis)

67
Q

Liver bx:

lymphocytic/granulomatous cholangitis

A

PBC (primary biliary cirrhosis)

68
Q

Liver Bx:

Fibrous obliterative cholangitis

A

PSC (primary sclerosing cholangitis)

69
Q

Liver Bx:

periportal hepatitis

mild steatosis

A

chronic viral HCV

70
Q

Liver bx:

Globular hepatocyte inclusions

A

A1AT deficiency