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Gastrointestinal System > EXAM #2: LIVER PATHOLOGY > Flashcards

EXAM #2: LIVER PATHOLOGY Flashcards

1
Q

What are the four most common causes of chronic liver disease in US? (List in order of prevalence)

A

1) Hepatitis C
2) Alcoholic liver disease
3) Non-alcoholic fatty liver disease
4) Hepatitis B

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2
Q

What is meant by hepatocyte integrity?

A

Membrane integrity of hepatocytes

When damaged, hepatocytes are “leaky”

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3
Q

What are the two classic liver lab tests?

A

AST
ALT

Finding these enzymes in the serum indicates that there has been damage to the hepatocytes that have made them “leaky”

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4
Q

What three lab tests test hepatocyte function? What happens to these tests with impaired liver function?

A

1) Serum albumin–decreased
2) Prothrombin time–increased
3) Serum ammonia–increased

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5
Q

What lab tests are associated with obstructed bile flow?

A

1) ALP
2) 5’-Nucleotidase (5’-NT)
3) GGT

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6
Q

What are the two types of bilirubin? What is the difference between direct and indirect bilirubin?

A
  • Conjugated= Direct, has sugar moieties added and can be excreted
  • Unconjugated= Indirect, no sugar moieties

Unconjugated is “indirect” b/c it is an indirect measurement (Total- conjugated= unconjugated)

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7
Q

What is the normal serum total bilirubin level?

A

0.1-1.2 mg/dL

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8
Q

What are the clinical manifestations of hyperbilirubinemia?

A
  • Jaundice

- Cholestasis= slowing of the flow of bile

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9
Q

How high does the serum bilirubin need to be for there to be clinical symptoms?

A

Carvenale= greater than 2 mg/dL

Pathoma= greater than 2.5 mg/dL

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10
Q

Outline normal bilirubin metabolism.

A

1) RBCs are consumed by splenic macrophages
2) Protoporphyrin (from heme) is converted into UNCONJUGATED bilirubin (UCB)
- Heme oxygenase= Biliverdin
- Biliverdin reductase= Unconjugated Bilirubin
3) UCB binds albumin and is carried to the liver
4) In the hepatocyte ER, UCB is CONJUGATED to CB, via Uridine Glucuronyl Transferase (UGT)
5) CB is transferred to bili canaliculi via MDR-2 and MDR-3 transporters to form bile
6) Bile is stored in the gallbladder
7) Bile is released into the duodenum
8) Intestinal flora convert CB to UROBILINOGEN in the bowel
9) Urobilinogen is oxidized to STERCOBILIN and UROBILIN

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11
Q

What enzyme conjugates UCB in the hepatocyte?

A

UGT, Uridine Glucuronyl Transferase

  • Adds one sugar to bilirubin i.e. monoglucuronides
  • OR adds 2x sugars to make diglucuronides

(UGT1A1)

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12
Q

What heme degradation product gives stool its color?

A

Sterocobilin

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13
Q

What heme degradation product gives urine its color?

A

Urobilin

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14
Q

What is the etiology of hyperbilirubinemia seen in extravascular hemolysis or ineffective erythropoiesis?

A
  • Increased UCB b/c of heme degradation

- UCB overwhelms liver’s ability to conjugate

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15
Q

What is the lab finding in extravascular hemolysis/ ineffective erythropoiesis?

A

Elevated UCB

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16
Q

What are the clinical features of extravascular hemolysis/ ineffective erythropoiesis?

A

1) Dark urine due to increased urine UROBILINOGEN
2) Increased risk for pigmented bilirubin gallstones (excess UCB must eventually be converted to CB–hyperaccumulation of which can cause gallstones)

Note that UCB is NOT water soluble and will NOT be in urine

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17
Q

What is the etiology of physiologic jaundice of the newborn?

A

Newborns have transiently low UGT (UCB–>CB)

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18
Q

What is the lab manifestation in physiologic jaundice of the newborn?

A

Elevated UCB

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19
Q

What is the clinical manifestation of physiologic jaundice in the newborn?

A

Possible kernicterus

  • UCB is fat soluble
  • Deposits in the basal ganglia and causes neurologic deficits/death
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20
Q

How is physiologic jaundice of the newborn treated?

A

Phototherapy, which makes UCB more water souble

Note that phototherapy does NOT conjugate UCB*

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21
Q

What is the etiology of Gilbert Syndrome?

A

Mildly low UGT activity due to a promoter mutation

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22
Q

What is the inheritance pattern of Gilbert Syndrome?

A

Autosomal recessive

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23
Q

What lab finding is associated with Gilbert Syndrome?

A

Elevated UCB

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24
Q

What are the clinical features associated with Gilbert Syndrome?

A

1) Predominantly asymptomatic

2) STRESS e.g. severe infection causes hyperbilirubinemia

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25
Q

What is the etiology of Crigler-Najjar Syndrome Type I?

A

Autosomal recessive absence of UGT

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26
Q

What is the lab finding in Crigler-Najjar Syndrome?

A

Extremely elevated UCB

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27
Q

What are the clinical manifestations of Crigler-Najjar Syndrome?

A

Kernicterius that is typically fatal

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28
Q

How does Crigler-Najjar Syndrome Type 2 differ from Type 1?

A

Type 2=

  • AUTOSOMAL DOMINANT with variable penetrance
  • Moderate decrease in UGT activity
  • Only occasional kernicterus

NOTE that this is TREATED WITH PHENOBARBITAL*

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29
Q

What is the etiology of Dubin-Johnson Syndrome?

A

Autosomal recessive mutation in MDR-2 transporter that impairs CB transfer to bile canaliculi

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30
Q

What lab finding is associated with Dubin-Johnson Syndrome?

A

Elevated CB

31
Q

What are the clinical manifestations of Dubin-Johnson Syndrome?

A

Dark liver otherwise innocuous

32
Q

What is Rotor Syndrome?

A

Autosomal recessive disorder similar to Dubin-Johnson Syndrome, but NO BLACK LIVER

33
Q

How do you tell the difference between Dubin Johnson Syndrome and Rotor Sydrome?

A

1) +/- black liver

2) Labs

34
Q

What labs are characteristic of Dubin-Johnson’s Sydrome?

A
  • Normal urine coproprophyrins (heme proteins)

- Increased isomer I

35
Q

What labs are characteristic for Rotor Syndrome?

A
  • Increased urine coproprophyrins

- Normal isomer I

36
Q

What is the etiology of Progressive Familial Intrahepatic Cholestasis (PFIC)?

A
  • Defects in various biliary epithelial transports (transporters that secrete bile components into bile canaliculi)
  • Causes cholestasis and failure to thrive
37
Q

What are the normal components of bile?

A

1) Bile salts that emulsify fat
2) Cholesterol
3) Lecithin
4) Bilirubin
5) Bicarbonate

38
Q

What is the specific mutation in PFIC-1?

A

Defect in the canalicular ATPase

- Cannot shunt bile into canaliculi without ATP

39
Q

What is the specific mutation in PFIC-2?

A

Defect in a Bile Salt Export Pump

40
Q

What is the specific mutation seen in PFIC-3?

A

Defect in MDR-3 and impairs phosphatidylcholine transport

41
Q

What is the inheritance pattern for the PFICs?

A

Autosomal recessive

42
Q

What are the clinical manifestations of the PFICs?

A

1) Cholestasis (has a toxic effect on the liver and can cause fibrosis/cirrhosis)
2) Fat malabsorption
3) Fat soluble vitamin deficiency
4) Osteopenia
5) Liver failure

Note that symptoms begin in infancy and progress to cirrhosis within the first decade of life

43
Q

What are the two most common bile duct obstructions?

A

1) Gallstones

2) Pancreatic carcinoma

44
Q

What are the clinical manifestations of bile tract obstruction i.e. obstructive jaundice?

A

1) Jaundice
2) Steatorrhea w/ malabsorption (no bile for fat emulsification)
3) Dark urine due to BILIRUBINURIA
4) Xanthomas from hypercholesterolemia
5) Pruritis due to increased plasma bile acids
6) Osteoporosis (not absorbing Vitamin D)
7) Bleeding–impaired Vitamin K production

45
Q

What are the lab findings biliary tract obstruction?

A

1) Elevated CB
2) Decreased urine urobilinogen
3) Elevated
- ALP
- GGT
- 5-NT
4) Hyperlipidemia

46
Q

What are the most common causes of cirrhosis?

A

1) Alcoholic liver disease
2) Viral hepatitis
3) Obesity

47
Q

Outline the pathogenic progression of cirrhosis. What are the hallmarks of cirrhosis?

A
  • Hepatocyte necrosis leads to:
    1) Progressive fibrosis
    2) Regenerative hepatocyte nodules

Bands of fibrosis and regenerative nodules are the hallmarks for cirrhosis

48
Q

What is the mechanism of liver fibrosis?

A

1) Kupffer Cells secrete factors that cause stellate cells to convert to myofibroblasts
2) Stellate cells/ myofibroblasts secrete TGF-B
3) TGF-B causes fibrosis

49
Q

What is the definition of micronodular cirrhosis? What type of cirrhosis is this associated with?

A

Cirrhosis with regenerative nodules less than 3mm

Alcoholic

50
Q

What is the definition of macronodular cirrhosis? What is macronodular cirrhosis associated with?

A

Cirrhosis with regenerative nodules greater than 3mm

  • Viral hepatitis
  • Alpha-1 antitrypsin disease
  • Wilson’s Disease
51
Q

What are the four manifestations of decompensated cirrhosis?

A

1) Portal HTN
2) Hepatorenal Syndrome
3) Liver Failure
4) Hepatic Encephalopathy

52
Q

What are the clinical manifestations of portal hypertension?

A

1) Ascites
2) Congestive splenomegaly/hypersplenism (consumption of RBCs and platelets)
3) Porotsystemic shunts
- Gastro-esophageal varices
- Rectal varices
- Caput medusae
- Ascites
- Hypersplenism

53
Q

What are the clinical manifestations that result from decreased detoxification in cirrhosis?

A

1) Hyperammonemia causing:
- Mental status change
- Asterixis
- Coma
2) Reduced liver removal of estrogen/hyperestrinism causing:
- Gynecomastia
- Spider angiomata
- Palmar erythema
- Testicular atrophy
3) Jaundice

54
Q

What does decreased protein synthesis in cirrhosis lead to?

A

1) Hypoalbuminemia and edema
2) Coagulopathy b/c of
- Decreased synthesis of clotting factors
- Impaired activation of epoxide reductase (Vitamin K)

55
Q

What is ascites?

A

Excess fluid in the peritoneal cavity

56
Q

What causes ascites?

A

1) Portal hypertension i.e. liver disease
2) Non-portal hypertension
- TB
- Pancreatitis
- Nephrotic Syndrome

57
Q

What does SAAG stand for?

A

Serum-ascites albumin gradient

58
Q

What does the SAAG help you differentiate?

A

Portal HTN= SAAG greater than 1.1 g/dL

Non-portal HTN= less than 1.1 g/dL

59
Q

How much liver function must be lost for a patient to have clinical manifestations of liver failure?

A

Greater than 80%

60
Q

What are the major clinical manifestations of hepatic failure?

A

1) Encephalopathy
2) Coagulopathy
3) Jaundice
4) Multiple organ failure

61
Q

What is the definition of hyperacute liver failure?

A

Failure occurs within 7 days of first symptoms of liver dysfunction (jaundice)

62
Q

What is the definition of acute liver failure?

A

Failure occurs within 4 weeks of first symptoms of liver dysfunction

63
Q

What is the definition of subacute liver failure?

A

Failure occurs within 5-12 weeks of first symptoms of liver dysfunction

64
Q

What is the definition of chronic liver failure?

A

Liver failure in the context of decompensated cirrhosis

65
Q

What is Hepatorenal Syndrome?

A

Renal failure that occurs in the setting of cirrhosis or fulminant liver failure

66
Q

What causes renal failure in Hepatorenal Syndrome?

A

Alteration in blood flow/ tone to the kidney in the face of liver failure

  • Vasodilation in periphery
  • Vasoconstriction to the kidney
67
Q

What are the manifestations of Hepatorenal Syndrome?

A

1) Oliguria/anuria
2) Increased BUN/ Creatinine
3) Low urinary Na+
4) Normal urinary sediment

68
Q

What is the difference between Type I and Type II Hepatorenal Syndrome?

A

Type 1= rapidly progressive

Type 2= moderate progression

Strict definitions are based on serum creatinine*

69
Q

What is Hepatopulmonary Syndrome?

A

Triad of:

1) Chronic liver disease
2) Hypoexmia
3) Intra-pulmonary vascular dilation

This results in V/Q mismatch–less diffusion of oxygen as blood is shunted rapidly through dilated pulmonary vessels

70
Q

What mediates Hepatopulmonary Syndrome?

A

Nitric Oxide

71
Q

What is Hepatic Encephalopathy?

A

Hyperammonemia that results in

1) cerebral edema
2) changes in mental status

Mercaptans are also part of the pathophysiology, but their role is poorly understood*

72
Q

What are the clinical manifestations of Hepatic Encephalopathy?

A

1) Mental status changes
2) Asterixis (flapping tremor)
3) Coma

73
Q

What is fulminant Hepatic Failure?

A

Acute liver failure with massive liver necrosis leading to:

  • Hepatic encephalopathy
  • Increased PT/INR

Gastrointestinal System (42 decks)

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