Accessory GI Flashcards

1
Q

Describe the difference between the classic hepatic lobule and the hepatic acinus

A
  • Hepatic lobule
    • Hexagonal
    • Central vein in center
    • 6 portal tracts at periphery
  • Hepatic acinus
    • Triangle
    • 2 portal tracts at periphery extending towards central vein
    • Describes zones of blood supply
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2
Q

Describe the zones of blood supply in the hepatic acinus version of organization

A
  • Zone 1: Periportal hepatocytes
    • closest to blood supply
    • doing most of the work
    • First to be damaged by toxin in the blood
  • Zone 2: Midzoneal
  • Zone 3: Centrilobular hepatocytes
    • around central vein
    • Farthest from blood supply
    • Recruited when needed
    • First to be damaged by ischemia or lack of blood flow
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3
Q

What is the role of the following cells:

  • Kupfer cells
  • Stellate cells
A
  • Kupfer cells
    • phagocyte
  • Stellate cells
    • Myofibroblasts
      • role in liver fibrosis
    • Store Vit A
    • Located in Space of Disse
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4
Q

Ballooning degeneration of hepatocytes is found in what types of liver damage?

A

Ischemic / hypoxic injury

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5
Q

Feathery degeneration of hepatocytes is found in what types of liver damage?

A

Cells have foamy cytoplasm

  • cholestatic injury
  • Bile accumulation
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6
Q

What macromolecule is accumulated in hepatocytes with steatosis?

A

Triglyceride accumulation

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7
Q

Microvesicular steatosis is found in what types of liver damage?

A

Fatty liver of pregnancy

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8
Q

Macrovesicular steatosis is found in what types of liver damage?

A
  • Obesity
  • Diabetes
  • Hep C
  • Found with microvesicles in Alcohol fatty liver
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9
Q

Mallory bodies are most likely associated with which liver disease?

A

Alcoholic hepatitis

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10
Q

How much liver function must be lost to have Liver Failure?

A

80-90%

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11
Q

What are the features of Hepatic Failure?

A
  • Portal HTN
    • ascites
    • Congestive splenomegaly / hypersplenism
      • ingests more RBCs and platelets
    • Portosystemic shunts
      • Esophageal varicies, hemorrhoids, caput medusae
    • Secondary renal failure
  • Decreased detox
    • Increased ammonia
      • Mental status changes
    • Hyperestrinism
      • gynecomastia, spider angiomata
    • Jaundice
  • Decreased protein synthesis
    • Hypoalbuminemia
      • edema
    • Decreased clotting factors
      • Increased PT
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12
Q

What is the size difference between micronodular and macronodular cirrhosis?

A
  • Micro: <3mm
  • Macro: >3mm
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13
Q

What are the histologic changes that occur in Cirrhosis?

A
  • Deposition of collagen I and III in space of Disse
    • Normally only small amout of type II and IV
  • Loss of fenestrations in sinusoid endothelial cells
    • obliterates biliary channels
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14
Q

What is the main cell responsible for cellular architecture found in cirrosis?

A

Cell of ido (forms a myofibroblast when activated

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15
Q

Where are the regenerative cells of the liver located?

A

Bile ductules

May cause regeneration of cells and regain function following cirrhosis

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16
Q

What outcomes are associated with acute HBV infxn?

A
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17
Q

HBV Ag and significance

A
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18
Q

What is the first serologic marker to rise in HBV?

A

HBsAg (surface Ag)

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19
Q

Which HBV markers indicate acute stage?

A
  • HBsAg = surface Ag
    • first to rise
  • HBeAg / HBV DNA
  • HBcAb = Core Ab
    • IgM
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20
Q

Which HBV markers indicate chronic stage?

A
  • HBsAg (surface Ag)
    • Presence > 6 months
  • HBeAg and HBV DNA
    • may or may not be present
  • HBcAb (Core Ab)
    • IgG
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21
Q

What markers indicate infectivity in HBV?

A
  • HBV DNA
  • HBeAg (Envelope)
    • Need an envelope to mail a letter
    • (mail = transfer to another person)
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22
Q

Which HBV marker indicates immunization or resolution of disease?

A

HBsAb IgG

(Surface Ab)

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23
Q

What is the most common cause of chronic liver disease?

A

HCV

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24
Q

What is the most common indication for liver transplant?

A

HCV

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25
Whic HCV is associated with metabolic syndrome?
Genotype 3
26
What is the serology confirmation of coinfection of HBV and HDV?
* IgM anti-HDAg * new infxn HDV * IgM anti-HBcAg * new infxn HBV
27
What is the serology confirmation of HBV/HDV superinfection?
* IgG or IgM anti-HDV * BHsAg present
28
Outcomes of HDV/HBV Coinfection
Nearly all: Recovery with immunity
29
Outcomes of HDV/HBV Superinfection
Most become chronic =\> cirrhosis
30
Characteristics Hepatitis viruses | (HOV's chart)
31
Hepatitis diagnosis chart | (HOV's chart)
32
What are the characteristics of Acute Asymptomatic infxn with Recovery?
* Minimally elevated serum transaminases * Anti-HAV or anti-HBV Abs
33
What are in characteristics of each stage of Acute Symptomatic Infxn with Recovery (Hepatitis)? * Incubation * Preicteric * Icteric * Convalescence
* Incubation * Infectivity in last days * Preicteric * non-specific symptoms * Serum sickness-like syndrome * immune complex-mediated * Urticaria, arthritis, glomerulonephritis * Icteric * Conjugated Hyperbilirubinemia * Nearly all HAV * 1/2 HBV * Absent in HCV * Convalescence * Strong immune response
34
Acute Hepatitis Morph. Changes
Chronic Hepatitis
35
Ground glass cytoplasm is most characteristic of which type of Hepatitis?
HBV
36
What is the most common cause of Chronic Hepatitis?
1. HCV 2. HBV * determined by age of exposure
37
Steatosis is most characteristic of which type of hepatitis?
HCV
38
What would be found in a serumtest of an inactive Hepatitis carrier?
* (+) HBsAg * (-) HBeAg * b/c can't transmit * (+) anti-HBe * Normal transaminase
39
What are the most common causes of Fulminant Hepatitis globally?
* HAV * HEV
40
Fulminant Hepatitis * Histo * Symptoms
* Histo * Loss of hepatocytes * Collapsed reticulin framework * Symptoms * Jaundice * Coagulopathy * Hepatic encephalopathy * Sepsis
41
The rosettes pictured below are characteristically associated with which form of hepatitis?
Autoimmune Hepatitis * Should also have prominent plasma cells
42
The eosinophilic inclusions in hepatocytes are characteristically associated with which type of hepatitis? What are they called?
Mallory Bodies Alcoholic Hepatitis
43
What comorbidities are associated with progression of Alcoholic Hepatitis to cirrhosis (or more severe forms)?
1. Iron overload 2. HBV infxn 3. HCV infxn
44
What is the physiopathology associated with hepatic steatosis and the formation of vesicular inclusions?
* Hepatocytes shunt normal substrates to lipid biosynthesis * (increased lipid) * Impaired production of lipoproteins * (can't get rid of them) * Increased peripheral fat catabolism * Increased FAs * Go to liver * Increased lipid
45
What is the most common presentation of Hepatic steatosis?
* Hepatomegaly * Mild elevation of * bilirubin * alkaline phosphatase
46
What are the physiological changesthat lead to Hepatic Steatosis?
1. Shunting of normal stubstrates to lipid biosynthesis in hepatocytes 2. Impaired assembly of lipoproteins 3. Increased peripheral fat catabolism * Releases FFA
47
What is the mechanism of hepatic injury in Alcoholic Hepatitis?
* Acetaldehyde disrupts cytoskeleton * Generation of P450 * Decreased glutathione * more susceptible to oxidative injury * Release of bacterial endotoxins * activates NF-kß * Release of endothelins * contraction of stellate cells
48
What is the limit of alcohol consumption for Nonalcoholic Fatty Liver Disease / Steatohepatitis?
less than 20gm/wk
49
What is the two hit model for NAFLD / NASH?
* Insulin resistance causes accumulation of fat in liver * Formation of lipid peroxides and ROS * Oxidative damage
50
What is the difference in Morphology between NAFLD and NASH?
* Nonalcoholic Fatty Liver Disease * Macro / micro vesicular steatosis * No inflammation * NA Steatohepatitis * Macro / micro vesicular steatosis * Inflammation * Mallory bodies * Fibrosis of: * portal tract * central vein * sinusoids
51
What disease process is pictured below?
Nonalcoholic Steatohepatitis
52
What disease process is pictured below?
Nonalcoholic Fatty Liver Disease
53
What gene is mutated in Hereditary Hemochromatosis? What is the most common mutation in the US?
* Gene * HFE * Regulates hepcidin =\> reg. iron absorption * Mutation * C282 more common in US (european origin) * Poorer outcome * H63D * Mild accumulation
54
What disease process is pictured below?
Secondary hemochromatosis * Iron primarily in Kupfer cells * Accumulation not as great as in primary disease
55
What disease process is pictured below?
Primary Hemochromatosis * Greater accumulation of iron * Accumulates toward the center of hepatocytes
56
Where is copper normally absorbed? How is it stored? What happens to the excess?
* Absorbed in the duodenum * Stored in ceruloplasmin * Excess is secreted into bile \*\*\*ceruloplasmin and secretion into bile is decreased in Wilson disease
57
What accumulates in cells in Wilson's disease? What stain is used to visualize this accumulation in cells?
Copper Rhodamine Stain
58
What enzyme conjugates bilirubin? Where does this occur?
* Enzyme: * UDP-glucuronosyl transferase * adds glucuronic acid * Location: * Liver ER
59
Is unconjugated bilirubin soluble? Conjugated bilirubin?
* Unconjugated: NO * bound to albumin before arriving to the liver * Conjugated: Yes * excreted in bile into the gut
60
What does bilirubin become once it is metabolized in the gut? What is responsible for metabolism?
* Metabolism * Gut bacteria glucuronidases deconjugate bilirubin * Becomes Urobilinogen * 80% to feces * 20% reabsorbed by bloodstream and enters urine
61
From what molecule is bilirubin derived originally?
Heme
62
What is the role of bile acids? What are they composed of?
* function * detergents: solubilize wate-insoluble lipids * composition * cholesterol * taurine or glycine
63
Which form of bilirubin is referred to as Delta or Indirect Bilirubin?
Unconjugated | (More abundant than conjugated)
64
Which form of bilirubin is referred to as Direct Bilirubin?
conjugated bilirubin
65
In intrahepatic and extrahepatic obstruction of bilirubin release: * How do enzyme levels change?
* How do enzyme levels change? * Increased **Alkaline phosphatase** * greater for _extrahepatic_ * Increased **ALT** * Increased **GGT** * Increased **AST** * greater for _intrahepatic_
66
In intrahepatic and extrahepatic obstruction of bilirubin release: * How is digestion affected?
* Decreased bile * Malabsorption of fat-soluble vitamins * Decreased Vit K: * Increased PT and PTT * Decreased urobilinogen * Acholic (light) stools
67
What disease process is pictured below?
Cholestasis Bile lake formation from cell degeneration
68
What disease process is pictured below?
Cholestasis * Front: bile plugs * Back: ductular proliferation * tries to accomodate bile
69
What are the causes and symptoms of cholestasis?
* Causes: * Intrahepatic obstruction * Extrahepatic obst. * Symptoms * Jaundice * Pruritis * Xanthomas * Dark urine possible * elevated conjugated bilirubin * water soluble
70
Neonatal Cholestasis * Unique histologic features * Symptoms
* Histo * Lobular disarray * focal hepatocyte apoptosis * Rosettes * multinuclear giant cells * Extramedullary hematopoiesis * Symptoms * Jaundice * Dark Urine * Light stools * Hepatomegaly
71
What histologic changes are associated with primary biliary cirrhosis?
* Lymphocytic infiltrate in portal tracts * Noncaseating granulomatous destruction of intrahepatic bile ducts * Fibrosis * Cirrhosis * Increased hepatocellular cancer
72
What disease process is pictured below?
Primary Sclerosing Cholangitis * Front: onion-skin around bile duct * Back: beading sign on radiography
73
In secondary biliary cirrhosis, what important findings occur before cirrhosis?
* Bile stasis * Ductular proliferation * neutrophil infiltrate * Portal Tract Edema
74
In primary biliary cirrhosis, what important findings occur before cirrhosis?
* Dense lymphocytic PT infiltrate * Granulomatous destruction of bile ducts
75
In primary sclerosing cholangitis, what important findings occur before cirrhosis?
* Periductal PT onion-skin fibrosis * Pattern of stenosis then beading of intra- and extra-hepatic ducts
76
What are von Meyenburg Complexes? What symptoms occur?
* Small clusters of dilated bile ducts * detached from biliary tree * located in portal tract * Symptoms: * clinically insignificant
77
What causes Polycystic Liver Disease? With what other disease is it associated?
* Cause * Inherited * AD * Disease * Strong association with polycystic kidney disease
78
Congenital Hepatic Fibrosis * Appearance * Cause * Associated with what other disease?
* Appearance * PT enlargement * abnormal bile ducts in CT * continuous with biliary tree * Develops portal HTN * Cause * Inherited * AR * Associated with what other disease? * Polycystic Kidney Disease
79
Caroli Syndrome * Appearance * Cause * Associated with what other disease? * What complications can occur?
* Appearance * Dilated bile ducts * Cause * Inherited * AR * Associated with what other disease? * Risk of cholangiocarcinoma
80
What complications arise from cysts in the liver?
* Intrahepatic cholelithiasis * Cholangitis * Hepatic abscess * Portal HTN
81
Manifestations of problems in Hepatic Blood Flow
82
What complication can occur with acute portal vein obstruction?
Congestion of liver and bowel infarction
83
What is the most common intrahepatic obstruction to blood flow?
Cirrhosis
84
Peliosis Hepatis * Histology * Associated conditions * Severe outcomes
* Histology * Dilation of Sinusoids * Due to apoptosis * Associated conditions * Cancer * TB * HIV * Anabolic Steroids * Severe outcomes * Intra-abdominal hemorrhage * Hepatic Failure
85
What symptoms are associated with Hepatic Vein Outflow Obstruction syndromes?
* Tender hepatomegaly * Ascites * Jaundice
86
What type of liver damage is caused by Right sided vs Left sided Heart Failure?
* Right sided * Sinusoidal congestion * Blood backs up due to lack of pumping * Left sided * Ischemic necrosis * Lack of pumping causes reduced systemic blood pressure
87
What effect can chronic CHF have on the liver?
Centrilobular fibrosis
88
What potentially life-threatening pathology of the liver can occur during eclampsia?
Hepatic hematoma Can rupture if under the Glisson capsule
89
Intrahepatic cholestasis of pregnancy * Symptoms * Complications
* Symptoms * Jaundice * conjugated bilirubin * Dark urine * Light stools * elevated transaminases * Complications * Gallstones * Small risk of fetal distress
90
What disease process is pictured below?
Acute Fatty Liver of Pregnancy Microsteatosis
91
What symptoms of acute bone marrow transplant graft vs host disease occur in the liver?
* Donor lymphocytes attack liver * Hepatitis
92
What symptoms of chronic bone marrow transplant graft vs host disease occur in the liver?
Selective bile duct destruction
93
What symptoms occur in chronic liver allograft rejection?
Vanishing Bile Duct Syndrome Obliterative arteritis =\> loss of blood flow to bile duct (w/ immune response) =\> disappears
94
Nodular Regenerative Hyperplasia of the Liver * Definition * Common cause of what hepatic condition?
* Definition * cirrhosis like nodules * no fibrosis present * Cause * decreased intrahepatic blood flow * Condition * non-cirrhotic portal HTN
95
Focal Nodular Hyperplasia * Population * Cause * Appearance
* Population * Women * 20-40 * Cause * Exogenous estrogens * Appearance * White, central, stellate scar
96
What is the most common benign liver tumor?
Cavernous Hemangioma
97
What genes are associated with Hepatocellular Carcinoma?
* p53 * Inactivation * WNT/beta catenin pathway * Activation * IL6/JAK/STAT pathway
98
What is the difference in prognosis between large cell and small cell dysplastic change of the liver?
* Large cell * Marker forincreased surveillance * Small cell * Directly premalignant
99
What are the differences between a low grade and high grade dysplastic nodules?
* Low * No atypia * PT present * High * Small cell change * Atypia * Few PT * Subnodules w/i nodules
100
What disease process is pictured below?
Fibrolamellar variant of SCC of liver
101
What congenital anomaly is pictured below?
Phrygian cap Folded fundus
102
What causes Acute Acalculous Cholecystitis?
Ischemia most common Cystic artery
103
What causes Chronic Cholecystitis? What bugs may initiate an attack?
* Cause * Unknown * Important: Supersaturation of bile * Bugs * E. coli * Enterococci
104
Rokitansky-Aschoff sinuses occur in what pathology of the gallbladder?
Chronic Cholecystitis (due to reactie proliferation of buried crypts)
105
What disease process is pictured below?
Porcelain gallbladder Dystrophic calcification (increased risk cancer) occurs w/ Chronic Cholecystitis
106
What disease process is pictured below?
Xanthogranulomatous cholecystitis Due to Chronic Cholecystitis
107
What disease process is pictured below?
Hydropic Gallbladder Due to Chronic cholecystitis
108
What are the clinical features of chronic Cholecystitis?
Recurrent attacks Nausea / vomiting \*\*\*Intolerance to fatty food\*\*\*
109
What is the most common form of Carcinoma of the Gallbladder?
Adenocarcinoma
110
Carcinoma of the Gallbladder is more common in which population?
* Females * Countries * Chili * Bolivia * N India
111
Which form of CA of the Gallbladder has the best prognosis?
Papillary
112
What mutation can result in Pancreas agenesis?
PDX1 | (homozygous loss of function)
113
What is the pathology and treatment of autoimmune pancreatitis?
Lymphoplasmacytic sclerosing pancreatitis * Path: * IgG4 secreting plasma cells in the pancreas * Treatment: * responds to steroid therapy
114
What genes are important in the progression to Pancreatic Carcinoma?
* K-RAS * p16 * p53 * SMAD4
115