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
What is seen on liver biopsy of Hemochromatosis?
Periportal iron deposits | Micronodular cirrhosis
26
How is Hemochromatosis diagnosed?
Liver biopsy...perform quantitative iron in paraffin block (I don't actually know what this means...)
27
How are people screened for Hemochromatosis?
Fasting transferrin saturation -(if +)-> repeat transferrin saturate with serum ferritin -(if +)-> HFE gene test
28
How is Hemochromatosis treated?
Phlebotomy | Iron chelating agents
29
What is Wilson's disease?
Autosomal recessive disorder of copper metabolism (ATP7B in liver) Impaired secretion of copper into bile
30
How is Wilson's disease diagnosed?
Serum ceruloplasmin (low...unless also liver disease, then normal) 24hr urine copper is elevated Serum copper levels are low
31
When should Wilson's disease be considered?
Liver disease
32
What is seen on liver biopsy of Wilson's disease?
Increased hepatic copper If negative, do a quantitative liver copper
33
What can be seen in the eyes of a patient with Wilson's disease?
Kayser-Fleischer rings
34
What is Alpha-1-Antitrypsin (A1AT) deficiency?
Co-dominant autosomal disorder characterized by abnormally low levels of A1AT A1AT is produced by the liver
35
What is the effect of A1AT deficiency in the lungs? Liver?
Lungs: empysema (neutrophil elastase >> A1AT) Liver: Accumulation of A1AT in hepatocytes
36
How is A1AT deficiency screened for?
A1AT level and genotype
37
What is seen on a liver biopsy of A1AT deficiency?
Cytoplasmic globular inclusions
38
There are two groups of DRUG induced liver injury: what are the two groups?
Direct (all who are exposed are effected...tylenol) Indirect (not all who are exposed are affected)
39
What is Reye's syndrome?
Liver injury (microvesicular steatosis) and encephalopathy post-viral illness...usually after aspirin treatment
40
How can Reye's syndrome be avoided?
Don't give aspirin to febrile kids
41
What is seen on liver biopsy with Reye's syndrome?
Microvesicular steatosis with small lipid vacuoles within the hepatocytes
42
What is neonatal cholestasis? What are two examples?
Group of disorders characterized by prolonged conjugated hyperbilirubinemia in neonates Biliary atresia and neonatal hepatitis
43
What is biliary atresia? (besides a cause of neonatal cholestasis)
Exactly what it sounds like... complete/partial obstruction of lumen of the extra hepatic biliary tree Noticed within first 3mos. of life
44
What is neonatal hepatitis? (besides a cause of neonatal cholestasis)
Hepatitis occurring in early infancy (1-2mos.)
45
What 4 things can possibly cause neonatal hepatitis?
Biliary atresia Inherited metabolic disorders (A1AT d, tyrosinemia, CF) Infectious agents Drugs
46
What are the 4 main causes of granulomatous hepatitis?
Idiopathic Sarcoidosis Drugs TB
47
Why are AST and ALT measured?
Elevations reflect hepatocellular damage NOT specific to liver
48
What do ALP and GGT assess?
Reflect injury to the bile duct epithelium/canalicular membrane Marker for cholestasis
49
What do albumin, PT, and certain clotting factors indicate about the liver?
General hepatic synthesis (function)
50
How are quantitative liver function tests done?
Measure hepatic metabolism of a drug to determine liver function
51
What are the key findings of chronic liver diseases?
Elevation of AST and ALT is greater than the bilirubin elevation
52
What are the key findings of biliary tract diseases?
Elevation of bilirubin is greater than elevation of AST and ALT