Liver part two...cirrhosis Flashcards

1
Q

What is cirrhosis? What is the typical cause of cirrhosis?

A

Cirrhosis is widely diffuse interconnecting fibrosis scars with nodular parenchymal regeneration

Cirrhosis is usually caused by alcohol

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2
Q

How does cirrhosis cause death?

A

Progressive liver failure
Complications of portal hypertension
Hepatocellular carcinoma

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3
Q

What are three causes of hepatic failure?

A

Chronic liver disease (like cirrhosis)

Acute liver failure w/massive liver necrosis (viral, drug, toxin)

Hepatic dysfunction w/o overt necrosis (Reye’s syndrome, acute fatty liver of pregnancy)

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4
Q

What is the most common cause of portal hypertension?

A

Cirrhosis

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5
Q

What are the 4 main complications of portal hypertension?

A

Ascites
Portosystemic shunts
Splenomegaly
Hepatic encephalopathy

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6
Q

What is seen on an alcoholic steatosis biopsy?

A

Accumulation of lipid

Macrovesicular steatosis (microscopically)

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7
Q

What is seen on an alcoholic hepatitis biopsy?

A

Steatosis

Hepatocyte injury and/or inflammation

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8
Q

What are mallory bodies? What are they seen with?

A

Mallory bodies are cytokeratin

Mallory bodies are seen with alcoholic liver disease, NAFLD, and PBC

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9
Q

What are the gross findings of alcoholic cirrhosis?

A

Bridging fibrosis

Intervening hepatocytes regenerate –> nodules

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10
Q

What are the 5 major causes of death with alcoholic cirrhosis?

A
Hepatic encephalopathy --> coma
Massive GI tract hemorrhage
Infection
Hepatorenal syndrome
Hepatocellular carcinoma
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11
Q

Who is at risk for Non-Alcoholic Fatty Liver Disease (NAFLD)?

A

Patients with obesity, dyslipidemia, hyperinsulinemia, and/or insulin resistance (DMII)

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12
Q

What is Primary Biliary Cirrhosis?

A

PBC is progressive inflammatory destruction of intrahepatic bile ducts

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13
Q

Who usually develops PBC?

A

40-50yo females

May have an autoimmune disorder

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14
Q

What are the primary symptoms of PBC?

A

Insidious onset of fatigue

Anicteric pruritis

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15
Q

What are some key diagnostic labs for PBC?

A

Elevated ALP and cholesterol

AMA+

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16
Q

What is seen on liver biopsy of PBC?

A

Periportal hepatitis and periportal fibrosis –> bridging necrosis with bridging fibrosis –> cirrhosis

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17
Q

What is Secondary Biliary Cirrhosis?

A

Cirrhosis secondary to any disorder causing extra hepatic bile duct obstruction

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18
Q

What are 5 causes of Secondary Biliary Cirrhosis?

A
Stones
Tumors
Biliary atresia
CF
Choledochal cysts
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19
Q

What is Primary Sclerosing Cholangitis?

A

Progressive, random, uneven fibroinflammatory obliteration of extra hepatic and intrahepatic bile ducts

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20
Q

What other GI disorder is usually seen with PSC?

A

80% of PSC cases are associated with IBD…usually ulcerative colitis (pANCA)

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21
Q

How can PSC be diagnosed?

A

pANCA (which is why it is with ulcerative colitis)
Elevated ALP
Biopsy

22
Q

What is seen on a biopsy of PSC?

A

Fibrosing cholangitis –> beads on a string

Obliterated bile duct

23
Q

What is Hemochromatosis?

A

Iron overload

24
Q

What are the manifestations of Hemochromatosis?

A

Iron accumulates in the liver

Hemosiderin deposits in pancreas, heart, skin, endo, joints

25
Q

What is seen on liver biopsy of Hemochromatosis?

A

Periportal iron deposits

Micronodular cirrhosis

26
Q

How is Hemochromatosis diagnosed?

A

Liver biopsy…perform quantitative iron in paraffin block (I don’t actually know what this means…)

27
Q

How are people screened for Hemochromatosis?

A

Fasting transferrin saturation -(if +)-> repeat transferrin saturate with serum ferritin -(if +)-> HFE gene test

28
Q

How is Hemochromatosis treated?

A

Phlebotomy

Iron chelating agents

29
Q

What is Wilson’s disease?

A

Autosomal recessive disorder of copper metabolism (ATP7B in liver)

Impaired secretion of copper into bile

30
Q

How is Wilson’s disease diagnosed?

A

Serum ceruloplasmin (low…unless also liver disease, then normal)

24hr urine copper is elevated

Serum copper levels are low

31
Q

When should Wilson’s disease be considered?

A

Liver disease < 30yo

32
Q

What is seen on liver biopsy of Wilson’s disease?

A

Increased hepatic copper

If negative, do a quantitative liver copper

33
Q

What can be seen in the eyes of a patient with Wilson’s disease?

A

Kayser-Fleischer rings

34
Q

What is Alpha-1-Antitrypsin (A1AT) deficiency?

A

Co-dominant autosomal disorder characterized by abnormally low levels of A1AT

A1AT is produced by the liver

35
Q

What is the effect of A1AT deficiency in the lungs? Liver?

A

Lungs: empysema (neutrophil elastase&raquo_space; A1AT)

Liver: Accumulation of A1AT in hepatocytes

36
Q

How is A1AT deficiency screened for?

A

A1AT level and genotype

37
Q

What is seen on a liver biopsy of A1AT deficiency?

A

Cytoplasmic globular inclusions

38
Q

There are two groups of DRUG induced liver injury: what are the two groups?

A

Direct (all who are exposed are effected…tylenol)

Indirect (not all who are exposed are affected)

39
Q

What is Reye’s syndrome?

A

Liver injury (microvesicular steatosis) and encephalopathy post-viral illness…usually after aspirin treatment

40
Q

How can Reye’s syndrome be avoided?

A

Don’t give aspirin to febrile kids

41
Q

What is seen on liver biopsy with Reye’s syndrome?

A

Microvesicular steatosis with small lipid vacuoles within the hepatocytes

42
Q

What is neonatal cholestasis? What are two examples?

A

Group of disorders characterized by prolonged conjugated hyperbilirubinemia in neonates

Biliary atresia and neonatal hepatitis

43
Q

What is biliary atresia? (besides a cause of neonatal cholestasis)

A

Exactly what it sounds like… complete/partial obstruction of lumen of the extra hepatic biliary tree

Noticed within first 3mos. of life

44
Q

What is neonatal hepatitis? (besides a cause of neonatal cholestasis)

A

Hepatitis occurring in early infancy (1-2mos.)

45
Q

What 4 things can possibly cause neonatal hepatitis?

A

Biliary atresia
Inherited metabolic disorders (A1AT d, tyrosinemia, CF)
Infectious agents
Drugs

46
Q

What are the 4 main causes of granulomatous hepatitis?

A

Idiopathic
Sarcoidosis
Drugs
TB

47
Q

Why are AST and ALT measured?

A

Elevations reflect hepatocellular damage

NOT specific to liver

48
Q

What do ALP and GGT assess?

A

Reflect injury to the bile duct epithelium/canalicular membrane

Marker for cholestasis

49
Q

What do albumin, PT, and certain clotting factors indicate about the liver?

A

General hepatic synthesis (function)

50
Q

How are quantitative liver function tests done?

A

Measure hepatic metabolism of a drug to determine liver function

51
Q

What are the key findings of chronic liver diseases?

A

Elevation of AST and ALT is greater than the bilirubin elevation

52
Q

What are the key findings of biliary tract diseases?

A

Elevation of bilirubin is greater than elevation of AST and ALT