EXAM #2: REVIEW Flashcards

1
Q

What is causing the dark urine seen in patients with extravascular hemolysis?

A

Urobilinogen

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2
Q

What labs differentiate Dubin-Johnson Syndrome and Rotor Syndrome?

A

Dubin-Johnson=

  • Normal urine coproporphyrinogen
  • Elevated isomer I

Rotor Syndrome=

  • Elevated urine coproporphyrinogen
  • Normal isomer I
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3
Q

What are the five normal components of bile?

A

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

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4
Q

What defect is seen in PFIC-1?

A

Defect in the canalicular ATPase

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5
Q

What defect is seen in PFIC-2?

A

Defect in the Bile Salt Export Pump

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6
Q

What defect is seen in PFIC-3?

A

Defect in the MDR-3 transporter, impairing phosphatidylcholine transport

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7
Q

List five clinical features of the PFICs.

A

1) Cholestasis
2) Fat malabsorption
3) Fat soluble vitamin deficiency
4) Osteopenia
5) Liver failure

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8
Q

List seven clinical manifestations of bile duct obstruction.

A

1) Jaundice
2) Steatorrhea
3) Dark urine
4) Xanthomas
5) Pruritis
6) Osteoporosis
7) Bleeding

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9
Q

List the lab findings in bile duct obstruction.

A

1) Elevated conjugated bilirubin
2) Decreased urine urobilinogen
3) Elevated: ALP, 5’-NT, and GGT
4) Hyperlipidemia

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10
Q

What diseases is macronodular cirrhosis associated with?

A

Viral hepatitis
Alpha-1 antitrypsin
Wilson’s Disease

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11
Q

What are the three general manifestations of portal HTN?

A

1) Ascites
2) Congestive splenomegaly
3) Portosystemic shunts

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12
Q

What is acute liver failure?

A

Liver failure that occurs within 4 weeks of initial symptoms of liver dysfunction

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13
Q

List four clinical signs of hepatorenal syndrome.

A

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

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14
Q

What are the extrahepatic manifestations of Hepatitis C?

A

1) Cryoglobulinemia
2) Thyroiditis
3) Glomerulonephritis
4) Thrombocytopenia

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15
Q

What four factors will increase the likelihood of HCV infection leading to HCC?

A

1) Alcoholism
2) Cirrhosis
3) Male
4) Old age

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16
Q

What are the microscopic features of acute viral hepatitis?

A
  • Swelling of hepatocytes
  • Cholestasis
  • Lobular disarray
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17
Q

What are the microscopic features of chronic viral hepatitis?

A
  • Piecemeal necrosis
  • Bridging fibrosis
  • Nodular cirrhosis
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18
Q

What microscopic feature is associated with HCV infection?

A

Lymphoid aggregates in portal tracts

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19
Q

List the clinical manifestations of acute hepatitis.

A
  • Mixed jaundice
  • Dark urine
  • Fever
  • Malaise
  • Nausea
  • Elevated liver enzymes

I.e. flu-like illness with mixed jaundice, dark urine, and elevated liver enzymes.

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20
Q

What is an alternate name for Primary Biliary Cirrhosis?

A

Granulomatous cholangitis

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21
Q

What antibody is associated with PBC?

A

Anti-mitochondrial antibody (AMA)

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22
Q

List all of the lab findings associated with PBC.

A

1) Elevated antibodies: AMA, anti-M2, and anti PDH-E2
2) Elevated IgM
3) Elevated ALP/ 5’-NT, and GGT
4) Elevated cholesterol

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23
Q

What are the lab findings associated with PSC?

A

1) Elevated transaminases

2) Elevated ALP, 5’NT, and GGT

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24
Q

What antibody is most specific to PSC?

A

p-ANCA

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25
Q

What antibodies are specific to autoimmune hepatitis?

A

ANA
ASMA
ALKM

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26
Q

What are the features of Type I autoimmune hepatitis?

A
  • Most common
  • 10 y/o to elderly affected
  • SMA and ANA positive
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27
Q

What are the features of Type II autoimmune hepatitis?

A
  • Less common
  • 2-14 y/o effected
  • ALKM antibody
  • Poor prognosis
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28
Q

What is Type III autoimmune hepatitis?

A
  • Less common
  • Effects 30-50 year olds
  • Anti-SLA positive
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29
Q

What type of liver injury is seen with Tetracycline?

A

Microsteatosis

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30
Q

What type of liver injury is seen with Amiodarone?

A

Fibrosis

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31
Q

What type of liver injury is seen with steroids?

A

Cholestasis

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32
Q

What are Mallory Bodies?

A

Damaged intermediate filaments or “alcoholic hyaline” seen in alcoholic hepatitis

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33
Q

What are the clinical features of Alcoholic Steatohepatitis?

A

1) Fever
2) Leukocytosis
3) Jaundice
4) AST/ elevatopm

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34
Q

What are the four most common causes of death in chronic alcoholism?

A

1) Hepatic coma
2) Massive GI hemorrhage (esophageal varice)
3) Infection
4) Hepatorenal syndrome

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35
Q

What can cause Nonalcoholic Steatohepatitis?

A

1) Metabolic Syndrome
2) Drug hepatotoxicity
3) Pregnancy

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36
Q

What two drugs are notorious for inducing Steatohepatitis?

A

Tamoxifen

Nifedipine

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37
Q

Name four causes of a hepatic infarct. How does a liver infarct present?

A

1) Liver transplant
2) Chemoembolism–chemo delivery into the liver
3) Polyarteritis nodosium
4) Surgery

Presents with RUQ pain, fever, and jaundice.

38
Q

What causes ischemic hepatitis/ shock liver/ hypoxic hepatitis?

A

This is when there is occlusion of the HEPATIC ARTERY (branch of the celiac trunk)

1) Left sided heart failure
2) Heart surgery
3) Shock

39
Q

What are the clinical manifestations of ischemic hepatitis?

A
  • Centrilobular coagulative necrosis
  • AST/ALT elevation
  • Jaundice

Not enough oxygenated blood is feeding the liver via the hepatic artery

40
Q

What are the extra-hepatic etiologies of Portal Vein Thrombosis?

A

This is an issue with the PORTAL VEIN OUTSIDE OF THE LIVER:

1) Abdominal infection
- Appendicitis
- Diverticulitis
- Pancreatitis
2) Hypercoaguability
3) Surgery

41
Q

What are the intrahepatic etiologies of Portal Vein Thrombosis?

A

These are all problems with the BRANCHES OF THE PORTAL VEIN, in the liver i.e. intra-hepatic:

1) Cirrhosis
2) Malignancy
3) Stasis
4) Pregnancy
5) Idiopathic

Note that this will result in INFARCTS OF ZAHN

42
Q

What are the clinical manifestations of portal vein thrombosis?

A

1) Abdominal pain
2) Portal HTN causing:
- Esophageal varices
- Splenomegaly
- Ascites
3) Bowel Infarction

43
Q

What causes Hepatic Vein Obstruction?

A

1) Right heart failure causing backflow of blood into the liver via the HEPATIC VEIN
2) Budd-Chiari Syndrome

44
Q

What causes Hepatic Passive Congestion?

A

1) Right heart failure
2) Constrictive pericarditis
3) Congenital heart disease

45
Q

What is Budd-Chiari Syndrome?

A

Thrombosis of the hepatic vein or IVC–prevents the liver from draining

46
Q

What are the etiologies of Budd-Chiari Syndrome?

A

1) HCC
2) Abdominal cancer
3) Myeloproliferative disorder
4) PNH
5) Hypercoagulability
6) Pregnancy
7) Contraceptives

47
Q

What are the clinical features of acute Budd-Chiari Syndrome?

A
  • Abdominal pain
  • Hepatomegaly
  • Ascites
  • Jaundice
  • Liver failure
48
Q

What are the clinical features of chronic Budd-Chiari Syndrome?

A
  • Portal HTN
  • Cirrhosis
  • Obliterative Hepatocavopathy
49
Q

What is an Obliterative Hepatocavopathy?

A

IVC obstruction at its hepatic portion caused by the development of fibromembranous webs causing:

  • Edema of the abdominal wall
  • Tortuous abdominal venous pattern
  • Edema of the legs

*Note that this is endemic in Nepal and is suspected to have an infectious etiology

50
Q

What is Veno-Occlusive Disease of the liver?

A

Complication following bone marrow transplant or ingestion of plant alkaloids (Jamaican)
- Vasculitis causes sinusoid obstruction

51
Q

What is the link between transferrin, hepcidin, and iron absorption?

A
  • HFE gene codes for a membrane product that regulates Hepcidin
  • Hepcidin normally prevents iron absorption from the gut by breaking down ferroportin
  • HFE mutation= decreased hepcidin and increased iron absorption

The membrane product coded for by HFE alters the relationship between transferrin receptors and transferrin; the liver thinks the body needs more iron; consequently Hepcidin goes down

52
Q

What pathologic change is seen in early Hemochromatosis? Late?

A

Early= Fe++ deposition in periportal hepatocytes

Late= Fe++ deposition in kupffer cells, bile duct epithelium, and fibrous septa

Really, iron deposition occurs all over the body

53
Q

List six complications of Hemochromatosis.

A

1) DM
2) Cardiomyopathy
3) Hypogonadism
4) Skin pigmentation changes (i.e. gray color)
5) Pseudo-gout
6) Cirrhosis

*Note that these complications are seen at roughly 40 years-old.

54
Q

In secondary hemochromatosis, where is iron loaded?

A

Kupffer cells i.e. liver macrophages

55
Q

List five roles that copper plays in the body.

A

1) Pigment formation
2) Neurotransmitter production
3) Peptide formation
4) Connective tissue biosynthesis
5) Antioxidant defense

56
Q

What organs are most affected in Wilson’s Disease?

A

Brain, liver, and kidneys

57
Q

What gene is mutated in Wilson’s Disease? What is the result?

A

ATP7B copper transporter

*Copper cannot be excreted into bile canaliculi

58
Q

List the clinical manifestations of Wilson’s Disease.

A

1) Degeneration of the basal ganglia–> PD-like syndrome–adults
2) Hepatitis–kids
3) Cirrhosis

59
Q

What is the normal function of alpha-1 antitrypsin?

A

This is an enzyme that prevents action of trypsin/proteases

60
Q

What causes the hepatic symptoms of alpha-1 antitrypsin disease?

A

Misfolded/nonfunctional PiZ accumulates in hepatocytes and can’t get out of the liver to do its job

*PiZZ genotype

61
Q

How does alpha-1 antitrypsin deficiency present in kids?Adults?

A
Kids= Hepatitis/ cholestasis 
Adults= Chronic hepatitis/ cirrhosis
62
Q

What is Focal Nodular Hyperplasia of the liver?

A
  • Benign tumor of the liver
  • More common in women
  • Asymptomatic–does NOT require treatment
63
Q

What is the morphological appearance of Focal Nodular Hyperplasia?

A

Solitary mass on the liver with a central scar

64
Q

What is Nodular Regenerative Hyperplasia?

A

Diffuse nodular transformation of the liver WITHOUT fibrosis

Think of regeneration gone awry*

65
Q

What is Nodular Regenerative Hyperplasia commonly confused with?

A

Cirrhosis

Note that on Trichrome stain, you don’t see the outline of the nodules like you do with cirrhosis*

66
Q

What is the most common complication of Nodular Regenerative Hyperplasia?

A

Noncirrhotic portal hypertension

67
Q

What is the surgical treatment for Nodular Regenerative Hyperplasia?

A

Transjugular Intrahepatic Portal Shunt i.e. TIPS

68
Q

What is Nodular Regenerative Hyperplasia associated with?

A

Transplantation
Chemotherapy
Vasculitis

69
Q

What is the most common benign hepatic tumor?

A

Hemangioma

70
Q

What patient population are Hemangiomas more common in?

A

Women on oral contraceptives

71
Q

What do you need to remember about the diagnosis of a Hemangioma?

A

Do NOT biopsy; it will hemorrhage

72
Q

What is a Liver Cell Adenoma?

A

Glandular tumor of the liver

73
Q

What patient populations are Liver Cell Adenomas more common in?

A
  • Young women on oral contraceptives

- GSD (I and III)

74
Q

What is the most feared complication of a Liver Cell Adenoma?

A

Rupture during pregnancy

75
Q

What is the most common primary malignant liver tumor?

A

Hepatocellular Carcinoma

76
Q

Geographically, where is HCC most common?

A

Asia

Africa

77
Q

What is the biggest risk factor for HCC?

A

Cirrhosis and the other disorders that lead to cirrhosis

78
Q

List the risk factors for HCC.

A

1) Chronic hepatitis (HBV and HCV)
2) Cirrhosis
- Alcohol
- NAFLD
- Hemochromatosis
- Wilson’s
- A1AT
3) Aflatoxin and Tyrosinemia

79
Q

What genetic change is associated with progression to HCC?

A

p53

Note that Aspergillus/ aflatoxin causes p53 mutations

80
Q

How does HBV lead to HCC?

A
  • HBV is dsDNA virus
  • Integrates into host
  • Activates proto-oncogenes
81
Q

What are the clinical manifestations of HCC?

A

1) Painful hepatomegaly
2) Abdominal mass
3) Weight loss
4) Portal/hepatic vein thrombosis
5) Hemorrhagic ascites
6) Hepatic Failure
7) Massive bleeding

82
Q

What lab elevation is associated with HCC?

A

AFP greater than 1,000

83
Q

What subtype of HCC has the best prognosis?

A

Fibrolamellar HCC –thick fibrous bands within the tumors

84
Q

What is the primary malignant tumor of the bile duct epithelium?

A

Cholangiocarcinoma

85
Q

What are the risk factors for Cholangiocarcinoma?

A

1) Primary Sclerosing Cholangitis
2) Thorotrast
3) Caroli Disease
4) Liver Flukes

86
Q

What is the most common primary malignant hepatic tumor in children?

A

Hepatoblastoma

87
Q

What lab is associated with Hepatoblastoma?

A

Very elevated AFP

88
Q

What is an angiosarcoma?

A

This is a cancer of the inner lining of blood vessels associated with:

  • Thorotrast
  • Steroids

*V. poor prognosis

89
Q

What is the most common malignant tumor of the liver?

A

Metastases

90
Q

Where are the primary tumors of liver metastases commonly located?

A
  • GI
  • Breast
  • Lung
  • Pancreas
  • Melanoma