Liver 1 (not including viral hepatitis) Flashcards

1
Q

What is jaundice?

A

Retention of bilirubin –> yellow discoloration

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2
Q

What is icterus?

A

Basically jaundice of the sclera

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3
Q

What is cholestasis?

A

Impaired bile secretion

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4
Q

How is bilirubin made?

A

Reticuloendothelial cells convert heme –> bilirubin

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5
Q

Where does the heme that becomes bilirubin come from?

A

85% from breakdown of senescent RBCs

15% from hepatic heme or marrow RBC precursors

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6
Q

What happens to the bilirubin after it is formed from heme?

A

Bilirubin is complexed to albumin and transported to the liver

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7
Q

What form is the bilirubin in when it is being transported to the liver?

A

Unconjugated (Indirect)

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8
Q

What happens to bilirubin in the liver?

A

Bilirubin is conjugated with glucuronic acid in liver cells

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9
Q

What distinguishes unconjugated bilirubin from conjugated bilirubin?

A

Unconjugated: water INsoluble, TOXIC to tissues, NOT secreted in urine

Conjugated: water SOLUBLE, NOT toxic to tissues, SECRETED in urine

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10
Q

Although conjugated bilirubin can be excreted in urine, what is it usually excreted in?

A

Bile…poop

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11
Q

What causes unconjugated hyperbilirubinemia?

A

Increased bilirubin production
Impaired hepatic bilirubin uptake
Impaired bilirubin conjugation

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12
Q

What causes conjugated hyperbilirubinemia?

A
Extrahepatic cholestasis (biliary obstruction)
Intrahepatic cholestasis
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13
Q

What are some potential causes of neonatal unconjugated hyperbilirubinemia?

A

Normal neonatal alterations in bilirubin metabolism (increased production, decreased clearance, increased enterohepatic circulation)

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14
Q

What are some possible conditions that could manifest as neonatal unconjugated hyperbilirubinemia?

A

Immune-mediated hemolysis
Inherited RBC membrane/enzyme defects
Sepsis
Inherited defects in UGT1A1 activity (Crigler-Najjar or Gilbert’s syndrome)

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15
Q

What can be affected by neonatal unconjugated hyperbilirubinemia?

A

Neuro system:

Bilirubin Induced Neurologic Dysfunction (BIND)

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16
Q

What is one method of treating neonatal unconjugated hyperbilirubinemia?

A

Phototherapy…converts bilirubin to water soluble isomers

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17
Q

What is Gilbert’s syndrome?

A

A benign autosomal recessive mutation that decreases glucuronyltransferase activity (UGT1A1 30% of normal)

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18
Q

What is the effect of Gilbert’s syndrome?

A

Increased unconjugated bilirubin (

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19
Q

What is seen with hepatocellular cholestasis?

A

Bile within hepatocytes
Canalicular bile stasis
Feathery degeneration of hepatocytes

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20
Q

What is seen with canalicular cholestasis?

A
Bile within hepatocytes
Canalicular bile stasis
Feathery degeneration of hepatocytes
Bile lakes
Bile within distended bile ducts
Portal tract edema
Bile duct proliferation within portal tracts
21
Q

What is chronic passive congestion?

A

Centrilobular congestion

22
Q

What causes chronic passive congestion?

A

Right or left sided heart failure
Hepatic vein thrombosis
Shock (congestion + hypoperfusion –> centrilobular necrosis)

23
Q

What is centrilobular hemorrhagic necrosis?

A

Centrilobular congestion with centrilobular necrosis

24
Q

What is seen with centrilobular hemorrhagic necrosis?

A

Nutmeg liver

Sparing of periportal areas

25
What causes centrilobular hemorrhagic necrosis?
Right or left sided heart failure Hepatic vein thrombosis Shock (congestion + hypoperfusion --> centrilobular necrosis)
26
What is cardiac sclerosis?
Fibrosing reaction following long standing CPC and/or centrilobular necrosis Centrilobular fibrosis
27
What causes cardiac sclerosis?
Right or left sided heart failure Hepatic vein thrombosis Shock (congestion + hypoperfusion --> centrilobular necrosis)
28
What causes a hepatic infarct?
Double blood supply...seen with arterial occlusion d/t vasculitis, embolism, or tumor
29
What is Budd-Chiari syndrome?
Thrombosis of 2 or more hepatic vein branches
30
What microscopic (smaller) things are seen with Budd-Chiari syndrome?
Centrilobular hemorrhagic necrosis | Cardiac sclerosis
31
What are the gross findings of Budd-Chiari syndrome?
Hepatomegaly Ascites Abdominal pain
32
What causes Budd-Chiari syndrome?
Conditions that make blood clots more common (there are a lot of them)
33
What is sinusoidal obstruction syndrome?
Presence of obstructive, non-thrombotic lesions of the small (central) hepatic veins
34
Who is most likely to have sinusoidal obstruction syndrome?
Patients exposed to radiation and/or hepatotoxins
35
What is seen microscopically (smaller) with sinusoidal obstruction syndrome?
Subendothelial swelling/fibrosis --> narrowing/obliteration of central vein lumens
36
What are some signs of acute sinusoidal obstruction syndrome? What is it associated with?
Hepatomegaly Sudden weight gain Increased serum bilirubin Allogeneic bone marrow transplant or chemo patients
37
What are the signs to chronic sinusoidal obstruction? What is it associated with?
Chronic sinusoidal obstruction presents similar to Budd-Chiari syndrome Chronic sinusoidal obstruction is associated with toxic side effects of pyrrolizidine alkaloids (certain herbal teas)
38
What is seen with portal vein thrombosis?
Portal hypertension | NOT ascites
39
What causes portal vein thrombosis?
Extrahepatic causes: intra-abdominal sepsis, inherited or acquired hypercoagulable disorders, trauma, or pancreatitis/pancreatic cancer Intrahepatic causes: cirrhosis, invasion of portal vein by hepatocellular carcinoma
40
What is peliosis hepatitis?
Primary hepatic sinusoidal dilation with sinusoidal rupture...results in the formation of blood filled spaces
41
What are the symptoms of peliosis hepatitis?
Usually asymptomatic | Rarely, intra-abdominal hemorrhage or hepatic failure
42
What causes peliosis hepatitis?
Anabolic steroids, oral contraceptives, or danazol AIDS associated hepatic infection by bartonella henselae Nearby malignancy or tumor
43
What causes acute massive hepatic necrosis?
``` Acute viral hepatitis Toxin induced hepatitis (tylenol OD) Vascular liver disease Autoimmune hepatitis Wilson's disease ```
44
Why isn't cirrhosis commonly seen with massive hepatic necrosis?
There isn't usually fibrosis, so the liver is able to regenerate
45
What is autoimmune hepatitis?
T-cell mediated autoimmune disease | More common in females
46
What markers are seen in autoimmune hepatitis?
Type 1: ANA, anti-smooth muscle (SMA), anti-actin (AAA), anti-soluble liver antigen/liver-pancreas antibodies (anti-SLA/LP) Type 2: anti-liver/kidney microsome (ALKM-1), antibodies to a liver cytosol antigen (ALC-1 or LC1)
47
What is seen on a biopsy of autoimmune hepatitis?
Increased number of plasma cells in the inflammatory infiltrate
48
What is used to treat autoimmune hepatitis?
Steroids