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P&T Block 3 Pulm & GI > L72 > Flashcards

L72 Flashcards

1
Q

2 normal roles of bile

A
  1. Elim wastes - bilirubin

2. Emulsify dietary fats for absorption

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2
Q

Bilirubin synthesis. Include steps to get it into bile

A
  1. Heme broken down
  2. Biliverdin -> bilirubin
    Occurs in blood
  3. Bilirubin + albumin (otherwise BR would be insoluble in blood) -> uptake into liver
  4. Bilirubin + glucuronic acid via UGT1A1 = water soluble for excretion in bile
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3
Q

What is indirect jaundice?

3 causes

A

Excess unconjugated bilirubin
1. Too much bilirubin made b/c of hemolysis issue
2. ↓hepatic uptake
3. Impaired intrahepatic conj
PROBLEM b/c means can’t be excreted in urine = TOXIC

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4
Q

What is kernicterus?

A

Bilirubin depositing in brain - complication of excess unconjugated BR that can’t get out of the body

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5
Q

What is direct jaundice?

A 
Excess conjugated bilirubin
Due to:
- Impaired transport out of hepatocytes
- Faulty excretion aka bile duct problem
Not a huge issue b/c conj BR can be excreted into urine
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6
Q

What is unconj hereditary hyperbilirubinemia?

A

UGT1A1 defect - aka BR isn’t getting conj to glucuronic acid

Most concerned about autosomal dominant form

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7
Q

Are you worried about conj hereditary hyperbilirubinemia?

A

Not really b/c all forms are benign

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8
Q

If a newborn shows up with jaundice, what 3 things are you thinking of?

A
  1. Normal
  2. Biliary atresia - surgical emergency
  3. Neonatal hepatitis
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9
Q

What is cholestasis? 2 general categories

A

Bile building up in the liver
“Static bile”
Obstructive - something blocking the bile from leaving
Non-obstructive

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10
Q

If the cholestasis is obstructive, what is your next step in determining the etiology?

A

Intra vs extrahepatic block

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11
Q

What are lab findings to make you think an intrahepatic cause of obstructive cholestasis? Name 2 major intrahepatic diseases

A

↑Alk phos + BR
PBC = primary biliary cirrhosis
PSC = primary sclerosing cholangitis

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12
Q

Primary biliary cirrhosis (PBC)

  • Cause
  • Pathophys of cirrhosis
  • Men or women?
  • Unique lab findings
A

PBC - women - AMA
Idiopathic
T cells + granulomas destroy bile ducts inside the liver
AMA + = anti-mitochonridal Ab

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13
Q

What does PBC look like on histo?

A

Inflammatory cells + granuloma gradually compress the bile duct out of existence

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14
Q

Primary sclerosing cholangitis (PSC)

  • Pathophys
  • Associated diseases
  • Male vs women
  • Increased risk of what disease
A 
Cause unknown 
Intra and/or extrahepatic bile duct damage due to 
- Inflam
- Obliterative fibrosis 
**Associated with CHRON's + UC**
↑risk cholangiocarcinoma
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15
Q

PSC histo

A

Onion skin fibrosis around bile duct
Skip pattern
- Might miss on biopsy
- MRI better dx

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16
Q

Secondary biliary cirrhosis

  • Aka cirrhosis is secondary to something else… what?
  • Intra or extra hepatic bile duct block
  • How will entire liver look?
A 
INTRAhepatic
Secondary to:
- Tumors
- Bile stones
- Pancreatitis
Liver is GREEN
17
Q

Secondary biliary cirrhosis on histo

A

Large, irregular islands of fibrosis

  • *Bile ductules
  • Liver is tring to make new ducts but don’t work
  • Non-fxnal but used for dx
18
Q

What test would you order if you suspect:
PBC
PSC
Obstruction as cause for biliary block

A

PBC - AMA

PSC + obstruction = cholangiogram

19
Q

Acute hepatitis histo

A

Lobular disarray
- Liver looks disorganized b/c cells have been lost and regenerated irregularly
Injury markers:
- Ballooning -> apoptosis -> bridging necrosis
IF see inflam cells - makes you think viral cause

20
Q

What is the difference between toxic liver injury due to intrinsic vs idiosyncratic hepatotoxins?

A 
Intrinsic = toxins that if you give a big enough dose will damage anyone's liver (acetaminophen, alcohol)
Idiosyncratic = causes injury only in rare cases
21
Q

Which zone sees necrosis in acetaminophen injury?

A

Zone 3 = closest to central vein (furthest from O2 supply)

22
Q

Disorders in blood flow can damage the liver. 3 classifications of vascular disorders leading to hepatitis - examples of each

A

Where is the impaired flow:

  1. Prehepatic
    - Thrombosis
  2. Intrahepatic
    - Cirrhosis
  3. Post-hepatic aka hepatic venous outflow
    - Budd Chiari
23
Q

Does the liver usually infarct if the inflow is impaired?

A 
NO
B/c dual vasc blood supply 
- Hepatic artery
- Portal vein
EXCEPT transplant pts
24
Q

Remember, what is Budd Chiari?

A

IVC or portal vein thrombosis

25
Q

What is veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS)?
Where does this impair blood flow

A

Post-hepatic

Vascular lumen of hepatic vein clots off -> blood trapped in liver

26
Q

Hemangioma

Dx

A

= benign prolif of BVs
Small - peripheral near capsule
Dx via radiology - DONT biopsy b/c will crazy bleed!

27
Q

Focal nodular hyperplasia
Malignant or benign
Pt pop
Looks like…

A

Benign - cause unclear
YOUNG pts
Central scar + cirrhosis around it

28
Q

Liver cell adenoma
Malignant or benign
Pt pop
Appearance

A

Benign but is true clonal neoplasm
HORMONAL parallel:
- Young women on birth control - will regress if taken off
- Watch for pregnant women

29
Q

Gross + histo appearance of liver cell adenoma

A

Normal appearing liver tissue in sheets - just not the same as the rest of liver tissue
Has BVs but no bile ducts… aka not fxnal liver tisse

30
Q

Is dysplastic liver tissue a direct precursor for cancer?

A

Not always!

↑risk

31
Q

3 main causes of HCC

A

HBV or HCV
Alcohol
Food contaminants - Africa

32
Q

2 treatments for HCC

A

Resect - background liver normal

Transplant if not

33
Q

Fibrolamellar carcinoma

  • Type of cancer
  • Pt pop
  • Why are you worried
A

Type of HCC
Kids + teens (10 - 20)
No known risk factors -> often undx until presents as large lesion

34
Q

Fibrolamellar carcinoma histo

A

Fibrous bands = lamellae
- Pass thru tumor
Large hepatocytes
+/- pale inclusions

35
Q

What is cholangiocarcinoma

A

Tumor from bile ducts - intra or extra hepatic
**Adenocarcinoma - look for glands!!
Poor prognosis b/c asymptomatic until late

36
Q

Hepatoblastoma

  • Pt pop
  • Appearance
  • Treat
A

YOUNG kids - infant - 2/3 yo
Mesenchymal component - aka primitive liver tissue
Resect or transplant

37
Q

What does another cancer met to the liver look like?

A

Multiple round masses near capsule

P&T Block 3 Pulm & GI (31 decks)

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