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Flashcards in EXAM #2: REVIEW Deck (90)
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1
Q
What is causing the dark urine seen in patients with extravascular hemolysis?
A
Urobilinogen
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
3
Q
What are the five normal components of bile?
A
1) Bile salts
2) Bilirubin
3) Bicarbonate
4) Cholesterol
5) Lecithin
4
Q
What defect is seen in PFIC-1?
A
Defect in the canalicular ATPase
5
Q
What defect is seen in PFIC-2?
A
Defect in the Bile Salt Export Pump
6
Q
What defect is seen in PFIC-3?
A
Defect in the MDR-3 transporter, impairing phosphatidylcholine transport
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
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
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
10
Q
What diseases is macronodular cirrhosis associated with?
A
Viral hepatitis
Alpha-1 antitrypsin
Wilson's Disease
11
Q
What are the three general manifestations of portal HTN?
A
1) Ascites
2) Congestive splenomegaly
3) Portosystemic shunts
12
Q
What is acute liver failure?
A
Liver failure that occurs within 4 weeks of initial symptoms of liver dysfunction
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
14
Q
What are the extrahepatic manifestations of Hepatitis C?
A
1) Cryoglobulinemia
2) Thyroiditis
3) Glomerulonephritis
4) Thrombocytopenia
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
16
Q
What are the microscopic features of acute viral hepatitis?
A
- Swelling of hepatocytes
- Cholestasis
- Lobular disarray
17
Q
What are the microscopic features of chronic viral hepatitis?
A
- Piecemeal necrosis
- Bridging fibrosis
- Nodular cirrhosis
18
Q
What microscopic feature is associated with HCV infection?
A
Lymphoid aggregates in portal tracts
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.
20
Q
What is an alternate name for Primary Biliary Cirrhosis?
A
Granulomatous cholangitis
21
Q
What antibody is associated with PBC?
A
Anti-mitochondrial antibody (AMA)
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
23
Q
What are the lab findings associated with PSC?
A
1) Elevated transaminases
2) Elevated ALP, 5'NT, and GGT
24
Q
What antibody is most specific to PSC?
A
p-ANCA
25
Q
What antibodies are specific to autoimmune hepatitis?
A
ANA
ASMA
ALKM
26
Q
What are the features of Type I autoimmune hepatitis?
A
- Most common
- 10 y/o to elderly affected
- SMA and ANA positive
27
Q
What are the features of Type II autoimmune hepatitis?
A
- Less common
- 2-14 y/o effected
- ALKM antibody
- Poor prognosis
28
Q
What is Type III autoimmune hepatitis?
A
- Less common
- Effects 30-50 year olds
- Anti-SLA positive
29
Q
What type of liver injury is seen with Tetracycline?
A
Microsteatosis
30
Q
What type of liver injury is seen with Amiodarone?
A
Fibrosis
31
Q
What type of liver injury is seen with steroids?
A
Cholestasis
32
Q
What are Mallory Bodies?
A
Damaged intermediate filaments or "alcoholic hyaline" seen in alcoholic hepatitis
33
Q
What are the clinical features of Alcoholic Steatohepatitis?
A
1) Fever
2) Leukocytosis
3) Jaundice
4) AST/ elevatopm
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
35
Q
What can cause Nonalcoholic Steatohepatitis?
A
1) Metabolic Syndrome
2) Drug hepatotoxicity
3) Pregnancy
36
Q
What two drugs are notorious for inducing Steatohepatitis?
A
Tamoxifen
Nifedipine
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