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Flashcards in Wk5 Liver Path pt2 Deck (70):
1

early EtOH abuse

enlarged, lipid filled liver

How does it look microscopically?

Alcoholic steatosis--fatty liver

large lipid vacuoles

**reverses with abstinence

2

Alcoholic hepatitis = steatosis + ?

hepatocyte injury and/or inflammation

"steatohepatitis"

3

Cytokeratin aggregates in hepatocytes:

Associated with?

Mallory bodies

Alcoholic hepatitis

4

Usually seen surrounding Mallory bodies:

neutrophilic inflammation

5

Alcoholic hepatitis occurrence

pre-cursor to cirrhosis

steatofibrosis

**perivenular and pericellular

6

Grossly: fibrosis + nodules

cirrhosis

7

Liver in early stages of alcoholic cirrhosis

enlarged

fatty

micronudular (less 3 mm)

8

Liver in late stages of alcoholic cirrhosis:

shrunken

non-fatty

variable nodule size

cholestasis usually present

9

5 major causes of death from alcoholic liver disease:

1. hepatic encephalopathy and coma

2. Massive GI tract hemorrhage (esophageal varicies)

3. infx

4. hepatorenal syndrome

5. hepatocellular carcinoma

10

NAFLD?

Patient profile?

Non-alcoholic fatty liver disease

-metabolic syndrome
-obesity
-DM2
-dyslipidemia
-insulin resistance

11

autoimmune cholangiopathy

progressive destruction of small and medium sized INTRAhepatic bile ducts

extrahepatic ducts are spared

middle age females

Primary biliary cirrhosis

12

AMA (antimitochondrial antibodies)

associated with other autoimmune disorders

Primary biliary cirrhosis

13

fatigue

anicteric pruritis

xanthomas

steatorrhea

vit D malabsorption

Primary biliary cirrhosis

14

Tx for PBC:

urodeoxycholic acid

liver transplant

15

Dx of PBC:

liver biopsy

16

Damage due to prolonged EXTRAhepatic bile duct obstruction

Secondary biliary cirrhosis

17

progressive

RANDOM

autoimmune

uneven fibroinflammatory

extra/intrahepatic bile ducts

Primary sclerosing cholangitis (PSC)

18

pANCA

IBD association

Primary sclerosing cholangitis

19

"beaded" appearance on cholangiogram

PSC

primary sclerosing cholangitis

20

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

hemochromotosis

21

Inheritance pattern of primary hemochromotosis

autosomal recessive

22

Liver protein decreased in hemochromotosis

hepcidin --> excessive intestinal iron absorption

23

Most common genetic mutation in hereditary hemochromotosis

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

24

Causes of secondary hemochromotosis

parenteral Fe overloads (transfusions)

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

increased oral intake of Fe

Chronic EtOH liver disease

25

Reread slide 42

distribution of excess Fe

26

Early pathological finding of primary hemochromotosis:

periPORTAL iron deposits in hepatocytes

27

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

Fe accumulates in Kupffer cells not hepatocytes

28

Classic triad for hemochromotosis presentation:

1. cirrhosis

2. diabete

3. skin pigmentation

"Bronze diabetes"

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

29

Best screening test for hemochromotosis:

fasting transferrin saturation

if greater than 45% --> serum ferritin

if ferritin elevated --> HFE gene test

30

Reason for liver bx in hemochrmatosis:

concern for cirrhosis

31

Tx for hemochromatosis:

chelating agents / phlebotomy

32

Autosomal recessive

disorder of copper metabolism

Wilson's disease

33

Genetic defect in Wilson's disease

ATP7B gene

chromosome 13

34

ATP7B?

gene for liver copper transporting ATPase protein

impaired secretion as ceruloplasmin and impaired secretion into bile

--> toxic levels of Cu in tissues

35

Dx test for Wilson's

plasma ceruloplasmin level

24 hr urine copper excretion

36

When to consider Wilson's in your Ddx:

liver disease in anyone under 30 yo

37

Presenting sx of Wilson's disease:

neurpsychiatric

KAYSER-FLEISCHER rings
-copper deposits at limbus of cornea

38

What is alpha-1-antitrypsin?

a protease inhibitor (primarily neutrophils elastase)

**without it, tissues get degraded unchecked

39

A1AT gene location

ch 14

40

Pathogenesis of liver disease in A1AT deficiency:

intracellular misfolding --> accumulation --> apoptosis of hepatocytes

41

Most commonly dx hepatic disorder in infants and childrren:

A1AT deficiency

42

PAS stain

A1AT deficiency

43

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

Toxins

44

CPC -- chronic passive congestion

slide 73

45

nutmeg liver

centrilobular hemorrhagic necrosis

46

thrombosis of two or more hepatic vein branches

classic triad:

hepatomegaly

ascites

abd pain

Budd-Chiari syndrome

47

most common causes of Budd-Chiari syn:

hyercoaguable states

48

Dx for Budd-Chiari

Doppler US

MRI/CT

looking for clots

49

obstructive, NONthrombotic lesions of small (central) hepatic veins

radiation/hepatotoxin exposure

BM tx

Sinusoidal obstruction syndrome

50

sudden weight gain

hepatomegaly
increased serum bilirubin

Sinusoidal obstruction syndrome (ACUTE)

51

prolonged conjugated hyperbilirubinemia in the neonate

neonatal cholestasis

52

Two causes of neonatal cholestasis

1. biliary atresia (extrahepatic)

2. neonatal hepatitis

53

Causes of granulomatous hepatitis:

idiopathic 50%

sarcoid 22%

drugs 6%

TB 3%

other 19%

54

HELLP syndrome?

pregnancy

Hemolysis

Elevated Liver enzymes

Low Platelets

55

microvesicular steatosis in pregnancy

Acute fatty-liver of pregnancy

56

mild increase in conjugated bilirubin in pregnancy

Intrahepatic cholestasis of pregnancy

57

portal inflammation

lymphocytic cholangitis

"vanishing bile ducts"

chronic GVHD from BM tx

58

Tests that reflect hepatocellular damage

NOT hepatic function

AST/ALT

59

Reflect injury to bile ducts/canalicular membranes

markers of cholestasis

Alk Phos

GGT

60

Markers of hepatic function

Albumin

PT

clotting factors

61

Most specific test for liver damage

ALT

**AST is found in other tissues

62

Why is AST elevated preferentially in EtOH disease?

found in mitochondria

EtOH is mitochondrial toxin

63

Increased Alk Phos usually due to?

How to help differentiate?

bone or liver

GGT from biliary epithelium

64

Two purposes of Liver biopsy:

1. determine possible cause of disease

2. determine extent of liver damage

65

Liver bx:

ground glass hepatocytes

chronic viral HBV

66

Liver bx:

plasma cells

autoimmune hepatitis

PBC (primary biliary cirrhosis)

67

Liver bx:

lymphocytic/granulomatous cholangitis

PBC (primary biliary cirrhosis)

68

Liver Bx:

Fibrous obliterative cholangitis

PSC (primary sclerosing cholangitis)

69

Liver Bx:

periportal hepatitis

mild steatosis

chronic viral HCV

70

Liver bx:

Globular hepatocyte inclusions

A1AT deficiency