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Test 8 > Path 7 Intrahepatic > Flashcards

Path 7 Intrahepatic Flashcards

1
Q

Bile secreted per day

A

1 liter

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2
Q

Bile pathway

A

Bile canaliculi -> Canals of Hering -> bile ductules -> terminal bile ducts

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3
Q

Biliverdin v Bilirubin

A

water soluble v lipid soluble

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4
Q

Direct bilirubin

A
  • Unconjugated

- Transported on Albumin

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5
Q

Bilirubin in liver

A

Conjugated with Glucaronic acid by

-UGT1A1 (Uridine diphosphate glucuronyl transferase)

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6
Q

Bilirubin in gun

A

Bilirubin diglucuronide

-Hydrolized by bacteria to Urobilinogen

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7
Q

Bile composition

A

Bile salts (90% reabsorbed)
Phospholipids
Cholesterol
Bilirubin

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8
Q

Bile function

A
  • Excretion of endogenous lipids

- Absorption of dietary lipids

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9
Q

Jaudice

A

-Bilirubin above 2.0 mg/dl

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10
Q

Unconjugated bilirubinemia (3 causes)

A
  • Hemolysis
  • Reduced uptake (drug, genetic)
  • Impaired conjugation (genetic, physiologic newborn)
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11
Q

Conjugated bilirubinemia (2 causes)

A
  • Decreased excretion (cirrhosis, hepatitis)

- Impaired bile flow

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12
Q

Physiologic jaundice of newborn (cause, pathology, exacerbation, Tx)

A
  • Transient low UGT1A1 activity
  • Direct bilirubin deposited in basal ganglia (kernicterus)
  • Exacerbated by breast feeding
  • Tx with phototherapy
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13
Q

Dubin-Johnson syndrome

A
  • Recessive conjugated hyperbilirubinemia
  • Pigmented cytoplasmic globules
  • MRP2 mutation (canalicular multidrug protein2)
  • Impaired bilirubin glucuronide excretion
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14
Q

Rotor syndrome

A
  • Recessive conjugated hyperbilirubinemia

- Decreased hepatic uptake and storage

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15
Q

Gilbert syndrome

A
  • Recessive unconjugated hyperbilirubinemia

- Decreased UGTA1

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16
Q

Criglar-Najjar syndrome type 1

A
  • Recessive unconjugated hyperbilirubinemia
  • Absent UGTA1
  • Fatal
17
Q

Crigler-Najjar syndrome type 2

A
  • Recessive unconjugated hyperbilirubinemia
  • Decreased UGTA1
  • Mild course, occasional kernicterus
18
Q

Secondary biliary cirrhosis

A

Due to obstruction of extrahepatic biliary tree

19
Q

Primary biliary cirrhosis clinical

A
  • Obstruction symptoms
  • Xanthomas, skin pigmentation
  • Osteomalacia (due to vit. D malabsorption)
  • Cirrhosis
20
Q

Primary biliary cirrhosis labs

A
  • Antimitochondrial Ab

- Elevated biliarubin, alkaline phosphatase

21
Q

Primary biliary cirrhosis histo

A
  • Granulomas (epithelioid histiocytes)
  • Mixed inflammatory cells around ducts
  • Duct proliferation, native ductopenia
22
Q

Primary sclerosing cholangitis histo

A

-Onion-skin periductal fibrosis and inflammation

23
Q

Primary sclerosing cholangitis

A
  • 80% have Ulcerative Colitis
  • Obstruction symptoms
  • Cholangiocarcinoma risk
24
Q

Primary sclerosing cholangitis labs

A
  • p-ANCA
  • Elevated IgG4 (autoimmune pancreatitis)
  • Elevated serum alkaline phosphatase
25
Q

Hilar Klatskin’s tumor

A

Cholangiocarcinoma (most common)

-Jaundice & pruritis

26
Q

Distal peripheral bile duct tumor

A

Cholangiocarcinoma

  • Hepatomegaly
  • Weight loss, fatigue
  • Abdominal pain
27
Q

Cholangiocarcinoma risks (5)

A
  • Primary sclerosing cholangitis
  • Congenitally dilated bile ducts
  • HCV
  • Thorium dioxide
  • Liver flukes
28
Q

Cholangiocarcinoma histo

A

Big fat duct cells with fibrous stroma

29
Q

Liver protein synthesis (4)

A
  • Albumin
  • Prothrombin
  • Fibrinogen
  • Lipoproteins
30
Q

Liver metabolite storage (3)

A
  • Triglycerides
  • Glycogen
  • Vitamin A
31
Q

Liver metabolic functions (2)

A
  • Gluconeogenesis

- Urea production

32
Q

Liver architecture

A
  • Reticulin

- No basement membrane

33
Q

Liver clean up

A

-Kupffer cells (mononuclear phagocyte)

34
Q

Ito cell

A

Perisinusoidal stellate cell

-Stores and metabolizes vitamin A

35
Q

First cells to die from ischemia and toxicity

A

Centrilobular
(central vein)
(zone 3)

36
Q

Cirrhosis mechanism

A

Lobular fibrosis via stellate (ito) cells in perisinusoidal space of Disse

37
Q

Liver cirrhosis genetic risk

A

a1-antitrypsin deficiency

38
Q

Cirrhosis cancer risk

A

Hepatocellular carcinoma

39
Q

Hemangiosarcoma risks (3)

A
  • Vinyl chloride
  • Thorium dioxide
  • Arsenic

Test 8 (21 decks)

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