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GI 2 > Path slide set 4 > Flashcards

Path slide set 4 Flashcards

1
Q

accumulation of fat

A

steatosis

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2
Q

accumulation of bilirubin

A

cholestasis

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3
Q

when injury is not reversible, hepatocytes die principally by what 2 mechanisms

A
  • apoptosis

- necrosis

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4
Q

This form of hepatocyte injury is the predominate mod of death in ischemic/hypoxic injury and a significant response to oxidative stress

A

necrosis

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5
Q

councilman bodies

A

apoptotic hepatocytes

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6
Q

apoptotic hepatocytes in acute and chronic hepatitis

A

acidophil bodies due to deeply eosinophilic staining characteristics

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7
Q

severe, zonal loss of hepatocytes

A

confuent necrosis from widespread parenchymal loss

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8
Q

Regeneration of lost hepatocytes occurs primarily by what?

A

mitotic replication of hepatocytes adjacent to those that have died

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9
Q

in the most severe forms of acute liver failure, there is activation of the primary intrahepatic stem cell niche, called what

A

canal of Hering

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10
Q

principal cell type involved in scar deposition in liver

A

hepatic stellate cell

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11
Q

a hepatic stellate cell does what in quiescent form

A

lipid (vitamin A) storing cell

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12
Q

in several forms of acute and chronic injury, the stellate cell can become activated and are converted into what?

A

highly fibrogenic myofibroblasts

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13
Q

Proliferation of hepatic stellate cells and activation into myofibroblasts is initiated by increase expression of what

A

PDGFR-B and TNF

-also Kupffer cells and lymphocytes release cytokine and chemokines that modulated expression of TGF-B and MMP-2 and TIMP-1

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14
Q

myofibroblast contraction is stimulated by what

A

endothelin-1 (ET-1)

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15
Q

If injury persists, where does scar deposition often begin?

What diseases is this important

A

space of Disse

alcoholic and NAFLD

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16
Q

if chronic injury ceases, what can break down the scar, potentially reversing the formation

A

metalloproteinases

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17
Q

Adaptive immunitey plays a big role in what

A

viral hepatitis

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18
Q

Antigen specific and CD8+ cells are involved in what

A

eradication of hepatitis B and C

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19
Q

What percent of hepatic functional capacity must be lost before failure

A

80 to 90

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20
Q

definition of Acute liver failure

A

-an acute liver illness associated with ENCEPHALOPATHY and COAGULOPATHY that occurs within 26 weeks of initial injury in absence of pre-existing liver disease

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21
Q

another term for acute liver failure

A

fulminant liver failure

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22
Q

What causes acute liver failure

A

massive hepatic necrosis

-most often by drugs or toxins

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23
Q

What accounts for almost 50% of acute liver failure in US

A

Acetaminophen

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24
Q

what predominantly causes acute liver failure in Asia

A

hepatitis B and E

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25
Q

What does liver look like grossly with acute liver failure

A

small and shrunken

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26
Q

is there scarring in acetaminophen acute liver failure

A

no. . .usually happens within hours to days, too brief a time period for scarring

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27
Q

What viruses can cause acute liver failure in immunocompromised

A
  • CMV
  • Herpes
  • adenovirus
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28
Q

what are elevated in acute liver failure

A
  • at first liver serum transaminases increase

- as parenchyma dies, liver shrinks and transaminases decrease

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29
Q

a characteristic sign of acute liver failure, nonrhythmic, rapid extension-flexion movements of the head and extremities, best seen when the arms are held in extension with dorsiflexed wrists

A

Asterixis

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30
Q

What gets elevated in the blood with acute liver failure that correlates with impaired neuronal function and cerebral edema

A

ammonia

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31
Q

Coagulopathy and acute liver failure

A
  • easy bruising

- fatal intracranial bleeding

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32
Q

form of renal failure occurring in individuals with liver failure in whom there are no intrinsic morphologic or functional causes for kidney dysfunction

A

Hepatorenal syndrome

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33
Q

leading causes of chronic liver failure worldwide

A
  • chronic hep B
  • Chronic hep C
  • Non alcoholic fatty liver disease
  • alcoholic fatty liver disease
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34
Q

Liver failure in chronic liver disease is most often associated with what

A

cirrhosis

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35
Q

diffuse transformation of the entire liver into regerative parenchymal nodules surrounded by fibrous bands and variable degrees of vascular (often portosystemic) shunting

A

cirrhosis

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36
Q

What is the classification of cirrhosis which uses different morphologic features which helps monitor the decline of patients on the path to chronic liver failure

A

The Child-Pugh classification of cirrhosis

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37
Q

cirrhosis when there is no clear cause

A

cryptogenic cirrhosis

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38
Q

in chronic liver disease, what increases with advancing stage of disease and are usually most prominent in cirrhosis

A

ductular reactions

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39
Q

What percent of individuals with cirrhosis are asymptomatic until the most advanced stages of disease

A

40%

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40
Q

The ultimate causes of death in chronic liver failure, whether cirrhotic or not

A
  • those seen in acute liver failure
  • multiorgan system failure
  • hepatic encepholopathy, bleeding from esophageal varices, and bacterial infections
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41
Q

additional grim outcomes associated with chronic liver failure in the context of cirrhosis

A

hepatocellular carcinoma

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42
Q

Additional features of chronic renal failure that are different than acute

A
  • pruritus (intense itching)
  • Hyperestrogenemia
  • Palmar erythema
  • spider angiomas
  • hypogonadism
  • gynecomastia
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43
Q

is portal hypertension more common in acute or chronic liver failure

A

chronic

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44
Q

what are the 3 types of portal HTN

A

-Pre, intra, and post hepatic

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45
Q

the major prehepatic conditions leading to portal HTN

A
  • obstructive thrombosis

- massive spenomegaly

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46
Q

main posthepatic causes of portal HTN

A
  • severe right sided heart failure
  • constrictive pericarditis
  • hepatic vein outflow obstruction
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47
Q

The dominant intrahepatic cause, accounting for most cases of portal HTN

A

cirrhosis

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48
Q

in portal HTN, the increased resistance to portal flow is at the level of what?

A

sinusoids

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49
Q

4 major clinical consequences of portal HTN?

A
  • Ascites
  • formation of portosystemic venous shunts
  • congestive splenomegaly
  • hepatic encephalopathy
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50
Q

how much fluid is needed for ascites to become clinically detectable

A

at lease 500 mL

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51
Q

This appears in about 40% of individuals with advanced cirrhosis of the liver and causes massive hematemesis and death in about half of them

A

esophageal varices

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52
Q

Each episode of bleeding from esophageal varices has what % mortality

A

30%

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53
Q

2 additional syndromes that occur with chronic liver failure

A
  • hepatopulmonary syndrome

- portopulmonary HTN

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54
Q

Hep virus: usually benign, self-limiting disease with an incubation period of 2 to 6 weeks

A

Hep A

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55
Q

Hep virus: does NOT cause chronic hepatitis or a carrier state and only uncommonly causes acute hepatic failure

A

Hep A

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56
Q

the only DNA hep virus

A

Hep B

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57
Q

route of transmission for Hep A

A

fecal-oral

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58
Q

reliable marker of acute infection with HAV

A

IgM antibody against HAV

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59
Q

The Hep virus that can leads to chronic liver disease and is then a precursor for hepatocellular carcinoma even in the absence of cirrhosis

A

Hep B

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60
Q

what is the best predictor of chronicity with HBV

A

age at time of infection

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61
Q

risk factors for HCV infection

A
  • IV drugs
  • multiple sex partners
  • having surgery within last 6 months
  • needle stick injury
  • multiple contacts with an HCV infected person
  • employment in medical or dental fields
  • unknown
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62
Q

elevated titers of what after an active infection of HCV do not confer effective immunitiy

A

anti-HCV IgG

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63
Q

what are the hallmarks of HCV infection

A

persistent infection and chronic hepatitis despite the generally asymptomatic nature of the acute illness

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64
Q

what must be measured to confirm diagnosis of HCV infection

A

HCV RNA

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65
Q

in HCV infection, levels of what waxes and wanes but never become normal

A

serum aminotransferases

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66
Q

which hep virus will cause chronic disease in 80-90%

A

HCV

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67
Q

Which hep virus has an association with metabolic syndrome

A

HCV

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68
Q

Which hep virus is dependent for its life cycle on HBV

A

HDV

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69
Q

which hep virus is cocaine use a risk factor

A

HCV

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70
Q

time frame needed for it to be chronic hepatitis

A

more than 6 months

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71
Q

acute or chronic hepatitis?

  • portal inflammation minimal or absent
  • “spotty necrosis”
  • lobular hepatitis
A

Acute

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72
Q

defining histologic feature of chronic viral hepatitis

A

mononuclear portal infiltration

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73
Q

acute or chronic hepatitis?

-interface hepatitis

A

Chronic

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74
Q

“GROUND GLASS” hepatocytes

A

chronic Hepatitis B

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75
Q

diagnostic hallmark of HBV

A

cells with endosplasmic reticulum swollen by HBsAg

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76
Q

which Hep virus: LYMPHOID FOLLICLES

A

HCV

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77
Q

Chronic, progressive hepatitis

  • autoantibodies
  • therapeutic response to immunosuppression
A

autoimmune hepatitis

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78
Q

gender for autoimmune hepatitis

A

female predominance (78%)

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79
Q

antibodies for type 1 autoimmune hepatitis

A
  • ANA
  • SMA
  • anti-SLA/LP
  • less commonly, AMA
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80
Q

antibodies for type 2 autoimmune hepatitis

A
  • anti-LKM-1 (directed against CYP2D6)

- ACL-1 antibodies

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81
Q

age for type 1 autoimmune hepatitis

type 2?

A

middle aged to older

children and teenagers

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82
Q

hepatitis with PLASMA cell predominance and hepatocyte “ROSETTES” in areas of marked activity

A

autoimmune hepatitis

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83
Q

What should always be included in the differential diagnosis of any form of liver disease

A

exposure to a toxin or therapeutic agent

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84
Q

toxic agent for hepatocellular necrosis

A

Acetaminophen

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85
Q

toxic agent for periportal and pericellular fibrosis

A

alcohol

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86
Q

Toxic agent for Budd-Chiari syndrome

A

-Oral contraceptives

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87
Q

toxic agent that can cause cholangiocarcinoma

A

thorotrast

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88
Q

toxic agents for peliosis hepatis: blood filled cavities, not lined by endothelial cells

A

anabolic steroids and tamoxifen

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89
Q

toxic agents for hepatocellular adenom

A
  • oral contraceptives

- anabolic steroids

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90
Q

Most common cause of acute liver failure necessitating transplantation in the U.S.

A

Acetaminophen

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91
Q

Leading cause of liver disease in most western countries

A

Excessive alcohol consumption

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92
Q

3 distinct forms of alcoholic liver injury

A
  • hepatocellular steatosis
  • alcoholic (or steato-) hepatitis
  • steatofibrosis
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93
Q

This form of alcoholic injury is completely reversible if there is abstention from further intake of alcohol

A

Fatty change or steatosis

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94
Q

What 3 things characterize alcoholic or steato-hepatitis

A
  • hepatocyte swelling and necrosis
  • Mallory-Denk Bodies
  • Neutrophilic reaction
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95
Q

keratins 8 and 18

A

Mallory Denk bodies

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96
Q

chicken wire fence pattern of alcoholic hepatic injury

A

alcoholic steatofibrosis

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97
Q

perisinusoidal scarring leads to a classic micronodular or LAENNEC CIRRHOSIS . . . burned out stage

A

alcoholic steatofibrosis

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98
Q

Mallory-Denk bodies seen in what

A
  • alcoholic hepatitis or steatohepatitis
  • Non-alcoholic fatty liver disease
  • Wilson disease
  • chronic biliary tract disease
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99
Q

What percent of alcoholics develop cirrhosis

A

only 10 to 15%

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100
Q

gender for alcoholic hepatic injury?

WhY?

A

women more susceptible

estrogen increases gut permeability to endotoxins which increase LPS receptor CD14 in Kupffer cells.
This predisposes to increased production of proinflammatory cytokines

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101
Q

Ethnicity for alcoholic hepatic injury?

genes involved

A

AA

ALDH*2

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102
Q

comorbid conditions for alcoholic hepatic injury

A
  • iron overload

- infection from HCV and HBV

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103
Q

what does alcohol that causes injury

A
  • Acetaldehyde
  • ROS
  • decreases glutathione
  • releases bacterial endotoxin
  • contraction of activated myofibroblastic stellate cells (portal HTN)
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104
Q

Hepatic steatosis may cause what?

A

hepatomegaly with mild elevation of serum bilirubin and alkaline phosphatase levels

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105
Q

Alcoholic hepatitis tends to appear acutely following what?

A

a bout of heavy drinking

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106
Q

lab findings for alcoholic hepatitis

A
  • hyperbilirubinemia
  • elevated serum aminotransferase
  • alkaline phosphatase
  • neutrophilic leukocytosis
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107
Q

ALT:AST ratio for alcoholic hepatitis

A

other chronic diseases ALT tends to be higher BUT!!!!

AST:ALT ratio is 2:1

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108
Q

Lab findings for alcoholic cirrhosis

A
  • elevated serum aminotransferases
  • hyperbilirubinemia
  • hypoproteinemia
  • anemia
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109
Q

In the end-stage alcoholic, the proximate causes of death are what

A
  • hepatic coma
  • massive GI hemorrhage
  • intercurrent infection
  • hepatorenal syndrome
  • hepatocellular carcinoma
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110
Q

most common acquired metabolic liver disorder

A

Non-alcoholic fatty liver disease (NAFLD)

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111
Q

Most common cause of chronic liver disease in the US

A

NAFLD

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112
Q

What term is often used to denote overt clinical features if liver injury in NAFLD

A

nonalcoholic steatohepatitis (NASH)

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113
Q

histologic hallmarks of NAFLD are most consistent with what syndrome?

A

metabolic syndrome

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114
Q

what has resulted in increasing rates of NAFLD

A

increased obesity in US

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115
Q

NAFLD increases risk for what?

A

hepatocellular carcinoma in ABSENCE of scarring

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116
Q

2 hit model for NAFLD

A
  • insulin resistance gives risk to hepatic steatosis

- hepatocellular oxidative injury

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117
Q

What are the inherited metabolic liver diseases

A
  • Hemochromatosis
  • Wilson disease
  • alpha 1 antitrypsin deficiency
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118
Q

Criteria for metabolic syndrome:
One the following:

AND

two of the following:

A
  • DM
  • impaired glucose tolerance
  • Impaired fasting glucose
  • insulin resistanct
  • BP: >140/90
  • Dyslipidemia
  • central obesity: waist:hip ratio >.9 for males, .85 for females or BMI >30
  • microalbuminuria
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119
Q

ethnic groups for NAFLD

A

Hispanic>AAs>Caucasians

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120
Q

Pathologic steatosis is defined as involving more than what percent of hepatocytes?

A

more than 5%

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121
Q

Greater than 90% of previously described “cryptogenic cirrhosis” is now thought to represent what?

A

“burned out” NAFLD

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122
Q

stain for NAFLD

A

Masson Trichrome stain

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123
Q

what is the most reliable diagnostic tool for NAFLD and NASH

A

-liver biopsy

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124
Q

excessive iron absorption, most of which is deposited in parenchymal organs such as the liver and pancreas, followed by hearts, joints, and endocrine organs

A

Hemochromatosis

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125
Q

what causes ssecondary hemochromatosis

A

usually transfusion

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126
Q

Fully developed cases of severe iron overload in the body exhibit what?

A
  • Micronodular cirrhosis in all
  • DM in 75-80%
  • abnormal skin pigmentation in 75-80%
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127
Q

When do symptoms of hereditary hemochromatosis usually first appear

A

4th to 5th decade of life and later in women wince menstrual bleeding counterbalances the accumulation until menopause

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128
Q

Gender for heriditary hemochromatosis

A

Males 5 to 7:1

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129
Q

grams of iron accumulation per year in hereditary hemochromatosis

A

.5 to 1 gm/year

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130
Q

how many grams of iron are stored in hereditary hemochromatosis before the disease manifests

A

after 20 grams

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131
Q

Main regulator of iron absorption?

what gene?

A

hepcidin

HAMP

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132
Q

3 genes in hemochromatosis

Adult form almost always caused by which?
-specific mutation

A

HFE, HJV, and TFR2

HFE
-C282Y

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133
Q

C282Y mutation of HFE gene for hemochromatosis is confined to what race

A

caucasian

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134
Q

penetrance of hemochromatosis

A

low

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135
Q

What mutations cause severe juvenile hemochromatosis

A
  • HAMP

- HJV

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136
Q

Which form of hemochromatosis is milder? adult or juvenile

A

adult

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137
Q

what stain for hemochromatosis

A

prussian blue

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138
Q

Classic tetrad of hemochromatosis

A
  • cirrhosis with hepatomegaly
  • abnormal skin pigmentation
  • DM
  • cardiac dysfunction
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139
Q

Slate-gray colored skin

A

hemochromatosis

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140
Q

joints and hemochromastosis

A

acute synovitis . .. psuedogout

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141
Q

Testes and hemochromatosis

A

may be small and atrophic

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142
Q

gender and age for hemochromotosis

A

often males and rarely before 40

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143
Q

Death in hemochromostosis is from what

A

cirrhosis or cardiac disease

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144
Q

Risk of hepatocarcinoma in hemochromatosis

A

200 fold greater . . . . significant cause of death

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145
Q

Treatment by what steadily depletes tissue iron stores in hemochromatosis

A

regular phlebotomy

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146
Q

is neonatal hemochromatosis an inherited disease

A

no . . just congenital

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147
Q

in neonatal hemochromatosis, hemosiderin deposition is detected where and needs to be documented for correct diagnosis

A

buccal biopsy

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148
Q

inheritance pattern of Wilson disease

A

automsomal recessive

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149
Q

Gene for Wilson disease

A

ATP7B

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150
Q

Wilson disease principally involves what organs

A

liver, brain, eyes

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151
Q

deposition of copper

A

Wilson disease

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152
Q

Free copper usually binds to what

A

ceruloplasmin

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153
Q

Accumulation of copper in Wilson disease causes toxic liver injury by what 3 mechanisms

A
  • formation of free radicals by the Fenton reaction
  • binding to sulfhydryl groups of cellular proteins
  • displacing other metals from hepatic metalloenzymes
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154
Q

in wilson disease, what increases in urine

A

Copper

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155
Q

Stains for Wilson Disease

A
  • Rhodamine stain for copper

- Orcein stain for copper-associated protein

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156
Q

Toxic injury to the brain in Wilson disease affects what areas

A

-basal ganglia, particularly the putamen which shows atrophy and even cavitation

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157
Q

Kayser-Fleischer rings in Wilson disease affect what in the eye

A

Descemet membrane in the limbus of the cornea

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158
Q

Age of onset for Wilson disease

A

average 11.4 . . . 6-40

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159
Q

Neurologic symptoms of Wilson disease

A

Parkinsonian symptoms

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160
Q

Most sensitive and accurate test for Wilson?

most specific screening test?

A

increase in hepatic copper content

increased urinary excretion

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161
Q

Inheritance pattern for alpha 1 antitrypsin deficiency

A

autosomal recessive

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162
Q

disorder of protein folding

A

a1-antitrypsin deficiency

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163
Q

Major function of a1-antitrypsin

A

inhibition of proteases, particularly neutrophil elastase, cathepsin G, and proteinase 3

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164
Q

Gene for a1-antitrypsin on what chromosome

A

14

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165
Q

amino acid substitution for PiZ genotype

A

Glu342 to Lys342

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166
Q

pathogenesis of alpha1-antitrypsin deficiency

A

abnormally folded protein creates endoplasmic reticulum stress and triggers a signaling cascade (UPR) that leads to apoptosis

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167
Q

cytoplasmic globular inclusions in hepatocytes

-PAS positive

A

a1-antrypsin deficiency

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168
Q

what is seen in 10 to 20% of newborns with a1-antitrypsin deficiency

A

Neonatal hepatitis with cholestatic jaundice

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169
Q

presenting symptoms of a1-antitrypsin deficiency in adolescence

A

hepatitis, cirrhosis, or pulmonary disease

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170
Q

2 major functions of hepatic bile

A
  • emulsification of dietary fat in lumen of the gut through detergent action of bile salts
  • elimination of bilirubin, excess cholesterol, xenobiotics
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171
Q

The metabolism of bilirubin by the liver consists of four separate but interrelated events: what are they

A
  • uptake from circulation
  • intracellular storage
  • conjugation with glucuronic acid
  • biliary excretion
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172
Q

bilirubin is the end product of what?

A

heme degradation

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173
Q

what converts heme to biliverdin

A

intracellular heme oxygenase

174
Q

biliverdin is immediately reduced to bilirubin by what

A

biliverdin reductase

175
Q

Bilirubin is formed outside of the liver and is released and bound to what

A

serum albumin

176
Q

Hepatic processing of bilirubin involved carrier-mediated uptake where?

A

sinusoidal membrane

177
Q

conjugation of bilirubin is done by waht

A

bilirubin uridine disphosphate (UDP) glucuronyl transferase (UGT1A1)

178
Q

Most bilirubin glucuronides are deconjugated in the gut lumen by what? and degraded to what?

A

bacterial B-glucuronidases

colorless urobilinogens

179
Q

solubility of unconjugated bilirubin

A

insoluble at physiologic pH and exists in tight complexes with serum albumin

180
Q

can unconjugated bilirubin be excreted in urine

A

no . . not soluble

181
Q

What kind of bilirubin can diffuse into tissues, particularly the brain of infants, and produce toxic injury

A

the albumin free unconjugated bilirubin in plasma

182
Q

Hemolytic disease of the newborn (erythroblastosis fetalis) may lead to accumulation of what in the brain . . .kernicterus

A

unconjugated bilirubin

183
Q

This type of bilirubin is water-soluble, nontoxic, and can only loosely bind to albumin . .. excreted in urine

A

conjugated

184
Q

the 2 conditions that result from specific defects in hepatocellular bilirubin metabolism

A
  • neonatal Jaundice

- Hereditary Hyperbilirubinemias

185
Q

Because the hepatic machinery for conjugating and excreting bilirubin does not fully mature until about 2 weeks of age, almost every newborn develops transient and mild unconjugated hyperbilirubinemia termed what?

A

neonatal jaundice or PHYSIOLOGIC JAUNDICE OF THE NEWBORN

186
Q

mutations of UGT1A1 cause hereditary UNCONJUGATED hyperbilirubinemias . . what ones

A
  • Crigler-Najjar

- Gilbert syndrome

187
Q

these result from defects that lead to CONJUGATED hyperbilirubinemias?

inheritence pattern

A
  • Dubin-Johnson
  • Rotor syndrome

autosomal recessive

188
Q

This caused by impaired bile formation and bile flow that gives rise to accumulation of bile pigment in the hepatic parenchyma

A

Cholestatis

189
Q

Patients with cholestatis may have what symptoms

A
  • jaundice
  • pruritis
  • skin xanthomas
  • malabsorption of A,D, K
190
Q

characteristic lab finding in cholestasis

A
  • elevated serum alkaline phosphatase
  • elevated gamma-glutamyl transpeptidase (GGT)

-AST and ALT probably normal

191
Q

“feathery degeneration”

A

Cholestasis . . droplets of bile pigment that accumulate in hepatocytes and take on a fine, foamy appearance

192
Q

a1-antitrypsin deficiency in middle to late life

A

cirrhosis with or without HCC

193
Q

Black liver (pigmented cytoplasmic globules)

A

Dubin-Johnson

194
Q

What hereditary hyperbilirubinemia is fatal

A

Crigler-Najjar syndrome type 1

195
Q

The 2 cholestasis diseases we need to know

A
  • Large Bile duct obstruction (adults)

- Biliary Atresia (neonatal)

196
Q

most common cause of bile duct obstruction in adult

A

extrahepatic cholelithiasis (gallstones)

197
Q

subtotal or intermittent obstruction of the bile duct may promote what

A

ascending cholangitis, a secondary bacterial infection of the biliary tree that aggravates the inflammatory injury

198
Q

Cholangitis usually pressent with what

A
  • fever
  • chills
  • abdominal pain
  • jaundice
199
Q

What is the most severe form of cholangitis

A

suppurative cholangitis in which purulent bile fills and distends the bile ducts

200
Q

What dominates suppurative cholangitis

A

sepsis rather than cholestasis

201
Q

Histologic hallmark of ascending cholangitis

A

influx of periductular neutrophils directly into the bile duct epithelium

202
Q

Which cholestasis is ammenable with surgery?

A

Extrahepatic

intrahepatic will get worse so proper diagnosis is key

203
Q

Charcots triad

What disease

A
  • Jaundice
  • RUQ pain
  • Fever

Acute cholangitis

204
Q

The presence of pus in the biliary ducts may result in Reynold’s pentad: what is this

A

Charcot’s triad plus hypotension and confusion

205
Q

The single most frequent cause of death from liver disease in early childhood

A

Biliary atresia

206
Q

Neonates who have jaundice beyond how many days after birth should be evaluated for neonatal cholestasis

A

14-21 days

207
Q

Kasai procedure

A

surgical intervention of biliary atresia

208
Q

complete or partial obstruction of the lumen of the extrahepatic biliary tree within the first 3 months of life

A

biliary atresia

209
Q

What accounts for 50 - 60% of children referred for liver transplantation

A

biliary atresia

210
Q

2 forms of biliary atresia

A

fetal and perinatal

211
Q

Which type of biliary atresia? associated with other anomalies resulting from ineffective establishment of laterality of thoracic and abdominal organ during development

A

fetal

212
Q

which type of biliary atresia is more common and the normally developed biliary tree is destroyed following birth probably from viral infection or immune reactions

A

perinatal

213
Q

inflammation and fibrosing stricture of the hepatic or common bile ducts

A

biliary atresia

214
Q

multinucleate giant hepatocytes

A

neonatal hepatitis

215
Q

biliary atresia limited to the common duct

A

type 1

216
Q

biliary atresia of right and/or left hepatic bile ducts

A

type 2

217
Q

biliary atresia in which there is obstruction at or above the porta hepatis (90%)?

Why is this a problem?

A

type 3

not surgically correctable by Kasai procedure

218
Q

presentation of biliary atresia

A
  • normal birth weight
  • postnatal weight gain
  • slight female predominance
  • stools change to acholic as disease progresses
219
Q

what remains the primary hope for salvage of biliary atresia patients

A

liver transplant

220
Q

without surgical intervention, when does death occur in biliary atresia

A

within 2 years of birth

221
Q

2 main autoimmune disorders of intrahepatic duct

A
  • Primary biliary cirrhosis (PBC)

- Primary sclerosis cholangitis (PSC)

222
Q

autoimmune disease characterized by nonsuppurative, inflammatory destruction of small and medium sized intrahepatic bile ducts

A

PBC

223
Q

age and gender for PBC

A

middle aged women with female predominance of 9:1 occurring 30-70. ave of 50

224
Q

geography for PBC

A
  • northern European countries (England and Scotland)

- Northern US (Minnesota)

225
Q

most characteristic Lab finding with PBC

A

Antimitochondrial antibodies

-recognize E2 compenent of pyruvate dehydrogenase complex (PDC-E2)

226
Q

Florid duct lesion

A

PBC

227
Q

associated condition with PBC

A

Sjogren

228
Q

Serology with PBC

A

AMA-positive and ANA-positive

229
Q

what is elevated in PBC

A

alkaline phosphatase and GGT

230
Q

PBC is confirmed by what?

A

Liver biopsy which is considered diagnostic if a florid duct lesion is present

231
Q

in PBC, over a period of two or more decades, untreated patients will follow one of two pathways to end-stage disease . .

A
  • hyperbilirubinemia predominates

- prominent portal HTN

232
Q

With progression of PBC, what secondary features may emerge

A
  • skin hyperpigmentation
  • Xanthelasmas
  • Steatorrhea
  • Vitamine-D malabsorption-related osteomalacia and/or osteoporosis
233
Q

Inflammation and obliterative fibrosis of intrahepatic and extrahepatic bile ducts with dilation of preserved segments

A

Primary sclerosis cholangitis (PSC)

234
Q

Beading on radiograph

A

PSC

235
Q

what condition coexists in about 70% of PSC patients

A

-IBD particularly UC

236
Q

age and gender for PSC

A

males age 30

237
Q

What HLA in PSC suppports that it is an inherent immunologic process

A

HLA-B8

238
Q

Autoantibody in 65% of those with PSC

A

perinuclear antineutrophil cytoplasmic antibodies (pANCA)

239
Q

small duct morphology for PSC

A

“onion skin” fibrosis leading to a “tombstone” scar

240
Q

Asymptomatic pts with PSC may come to attention only because of what?

A

persistent elevation of serum alkaline phosphatase, particularly in pts with UC who are being routinely screened

241
Q

Risk for what cancer with PSC

A

cholangiocarcinoma . . 1.5% annual risk and 20% lifetime

242
Q

A distinctive type of sclerosing cholangitis with elevated IgG4 levels in association with what

A

autoimmune pancreatitis

243
Q

congenital dilations of the common bile duct

age

gender

A

choledochal cysts

before 10

female to male 3:1 to 4:1

244
Q

heterogeneous group of lesion in which the primary abnormalities are congenital malformations of the biliary tree

A

fibropolycystic disease of the liver

245
Q

the most severe forms of fibropolycystic disease manifest as what

A

hepatosplenomegaly or portal HTN in absence of hepatic dysfunction, starting in late childhood or adolescence

246
Q

3 pathologic findings that may be seen in fibropolycystic Disease

A
  • Von Meyenburg complexes (small bile duct hamartomas)
  • Single or multiple intrahepatic or extrahepatic biliary cysts
  • congenital hepatic fibrosis
247
Q

when biliary cysts occur along with congenital hepatic fibrosis

A

Caroli SYNDROME

248
Q

When biliary cysts are in isolation and may be asymtomatic due to ascending cholangitis

A

Caroli DISEASE

249
Q

Stain for Congenital hepatic fibrosis

A

Masson Trichrome stain

250
Q

Structural anomalies of the biliary tree are related to abnormal development of what

A

ductal plate

251
Q

Fibropolycystic liver disease often occurs with what other disease?

gene

A

autosomal recessive polycystic renal disease

-polycystin

252
Q

Persons with fibropolycystic liver disease have an increased risk for what?

A

cholangiocarcinoma

253
Q

two conditions where solitary or multiple hyperplastic hepatocellular nodules may develop in the noncirrhotic liver

A
  • focal nodular hyperplasia

- nodular regerative hyperplasia

254
Q

What is the common factor in both focal nodular hyperplasia and nodular regenerative hyperplasia?

A

focal or diffuse alterations in hepatic blood supply, arising from obliteration of portal vein radicles and compensatory augmentation of arterial blood supply

255
Q

spontaneous mass lesion in an otherwise normal liver

  • younger to middle aged adult
  • lighter than surrounding liver
  • STELLATE scar
A

focal nodular hyperplasia

256
Q

liver entirely transformed into nodules but without fibrosis

A

nodular regenerative hyperplasia

257
Q

nodular regenerative hyperplasia can lead to what?

A

portal HTN

258
Q

most common benign liver tumor

A

cavernous hemangioma

259
Q

discrete, red-blue, soft nodule usually less than 2 cm in diameter, located directly beneath the capsule

A

cavernous hemangioma

260
Q

conditions associated with nodular regenerative hyperplasia

A
  • HIV

- rheumatologic diseases/SLE

261
Q

gender for cavernous hemangioma

A

F>M

262
Q

chief clinical significance of cavernous hemangioma

A

might be mistaken radiographically or intraoperatively for metastatic tumors

263
Q

Benign neoplasm developing from hepatocytes

A

hepatocellular adenoma

264
Q

3 subtypes of hepatocellular adenoma

A
  • HNF1-a inactivated hepatocellular adenomas
  • B-catenin Activated hepatocellulr adenoma
  • Inflammatory hepatocellular adenoma
265
Q

What drugs are associated with development of hepatocellular adenoma

A
  • Oral contraceptives (30-40x increased risk)

- anabolic steroids

266
Q

Hepatocellular adenoma that is responsible for autosomal dominant MODY-3 (maturity onset diabetes of the young type 3)

A

HNF1-a inactivated

267
Q

Hepatocellular adenoma that has very high risk for malignant transformation

A

B-Catenin activated

268
Q

Hepatocellular adenoma that is associated with Non-alcoholic fatty liver disease

A

Inflammatory

269
Q

risk of malignant transformation in inflammatory Hepatocellular adenoma

A

small but definite risk

270
Q

mutations in inflammatory Hepatocellular adenoma

A

activating mutations in gp130, a coreceptor for IL-6 that lead to JAK-STAT signaling

271
Q

This hepatocellular adenoma has tumors that are often fatty and devoid of cellular or architectural atypia.

  • Almost no risk for malignant transformation
  • Liver fatty acid binding protein (LFABP) is ABSENT
A

HNF1-a inactivated

272
Q

these hepatocellular adenomas have a high degree of cytoogic of architectural dysplasia

  • nuclear translocation
  • Glutamine synthetase diffusely positive
A

B-catenin Activated

273
Q

These hepatocellular adenomas have fibrotic stroma, mononuclear inflammtion, ductular reactions, dilated sinusoids, and telangiectatic vessels
-express C reactive protein and serum amyloid

A

inflammatory

274
Q

hepatocellular adenoma: DAB (brown) stain and hemotoxylin counterstain

A

inflammatory

275
Q

Most primary liver cancers arise from hepatocytes and are termed what?

A

hepatocellular carcinoma

276
Q

Much less common carcinomas of bile duct origin

A

cholangiocarcinoma

277
Q

Most common liver tumor of early childhood?

Age?

A

Hepatoblastoma

rarely over age 3

278
Q

2 types of hepatoblastoma

A
  • epithelial

- mixed epithelial and mesenchymal

279
Q

Which type of hepatoblastoma: composed of small polygonal fetal cells or smaller embyonal cells forming acini, tubules, or papillary structures vaguely recapitulating liver development

A

epithelial type

280
Q

Which type of hepatoblastoma: contain primitive mesenchyme, osteoid, cartilage, or striated muscle

A

mixed epithelial and mesenchymal type

281
Q

What pathway is activated in hepatoblastomas

A

WNT

282
Q

Patients with what familial disease frequently develop hapatoblastomas

A

Familial adenomatous polyposis

283
Q

FOXG1, a regulator of the TGF-beta pathway, is highly expressed in some of what type of tumor

A

hepatoblastoma

284
Q

Beckwith-Wiedemann syndrome

A

hepatoblastoma

285
Q

another name for HCC

A

hepatoma

286
Q

More than 85% of cases of HCC occur in countries with high rates of what?

A

chronic HBV infection

287
Q

when HCC occures in places with high incidence of chronic HBV what is the age?

Cirrhosis?

Toxin associated?

A

between 20 and 40

50% absence of cirrhosis

Aflatoxin

288
Q

In Western countries, the incidence of HCC is rapidly increasing, largely owing to what?

A

Hep C epidemic

289
Q

age for HCC in western countries?

cirrhosis?

gender?

A

-rarely before age 60

almost 90%

Male predominance: 3:1 in low incidence. 8:1 in high

290
Q

What is the most common setting for emergence of HCC

A

chronic liver disease

291
Q

The most important underlying factors in HCC are what?

A
  • viral infections ( HCV, HBV)

- toxic injury (Aflatoxin, alcohol)

292
Q

what produces aflatoxin

A

Aspergillus

293
Q

Where can you find Aflatoxin metabolites in affected individuals

A
  • urine

- also aflatoxin-albumin adducts in serum

294
Q

What two (maybe 3) things synergize with HBV to increase risk for HCC

A
  • Aflatoxin
  • alcohol
  • possibly cigarette smoke
295
Q

What metabolic diseases markedly increase risk of HCC

A
  • Hereditary hemochromatosis
  • a1AT deficiency

-More importantly, metabolic syndrome associated with obesity, DM, and NAFLD

296
Q

2 most common early mutational events or HCC

A
  • Activation of B-catenin

- inactivation of p53

297
Q

What mutation is strongly associated with HCC that is associted with aflatoxin

A

p53

298
Q

What mutation is more likely associated with HCC that is unrelated to HBV

A

B-catenin

299
Q

signaling pathway implicated in HCC

A
  • IL-6/JAK/STAT

- IL-6 regulates function of HNF-a

300
Q

In chronic liver disease the cellular dysplasias (precursor lesions) are called what?

  • which is directly pre-malignant
  • Which is thought to at least a marker of increased risk of HCC but in HBV they are also directly pre-malignant
A
  • Large cell change
  • small cell change
  • Small cell
  • Large cell
  • MARKERS IN BIOPSY
301
Q

What precursor lesion for HCC is usually detected in cirrhosis

A

dysplastic nodules

302
Q

What are probably the most important primary pathway for emergence of HCC in viral hepatitis and alcoholic liver disease

A

High grade dysplastic nodules

303
Q

HCC Precursor lesion: larger than normal hepatocytes, large often multiple pleomorphic nuclei . . . NORMAL nuclei:cytoplasmic ratio

A

Large cell change

304
Q

HCC precursor lesion: HIGH nuclear:cytoplasmic ratio. mild nuclear hyperchromasia and pleomorphism.
-tiny expansile nodules within single parenchymal lobule

A

small cell change

305
Q

HCC precursor lesion: devoid of cytologic or architectural atypia. Probably neoplastic.

A

Low grade dysplasia

306
Q

HCC precursor lesion: small cell change, occasional psuedoglands, trabecular thickening. Overt HCC may arise within and overgrow it

A

high grade dysplastic nodule

307
Q

3 ways grossly HCC may appear as

A

1: unifocal (usually large) mass
2: multifocal, widely distributed nodules of variable size
3: diffusely infiltrative cancer

-all three may cause liver enlargement

308
Q

What becomes more likely once HCC tumors reach 3 cm in size

A

intrahepatic metastases by either vascular invasion or direct extension

309
Q

Variant of HCC

  • under age 35
  • no gender predilection or predisposing condition
  • single, large, hard “scirrhous” tumor with fibrous bands
  • Well-differentiated cell rich in mitochondria . . ONCOCYTES growing in nests or cords separated by parallel lamellae of dense collegen bundles
  • NO history of cirrhosis
A

Fibrolamellar carcinoma

310
Q

History of liver disease in HCC where aflatoxin is common?

A

No

311
Q

Elevated levels of what in serum are found in 50% of HCC

A

serum alpha-fetoprotein

312
Q

What is most valuable for detection of small HCC tumors

A

imaging studies

313
Q

what forms the basis for diagnostic imaging of HCC

A

increasing arterialization in the process of conversion from high grade dysplastic nodule to early HCC then to fully developed HCC. .. bascially vascular changes

314
Q

Death from HCC usually occurs from what?

A
  • cachexia
  • GI or esophageal variceal bleeding
  • Liver failure with hepatic coma
  • rarely, rupture of the tumor with fatal hemorrhage
315
Q

second most common primary malignant tumor of the liver after HCC

A

chalangiocarcinoma (CCA)

316
Q

malignancy of the biliary tree, arising from bile ducts within and outside of the liver

A

CCA

317
Q

In Areas where infestation with liver flukes is endemic, what is more common than HCC

A

CCA

318
Q

All risk factors for CCA cause what

A
  • chronic inflammation

- cholestasis

319
Q

risk factors for CCA

A
  • infestation by liver flukes (Opisthorchis and Clonorchis)
  • chronic inflammatory disease of large bile ducts
  • Primary sclerosing cholangitis
  • hepatolithiasis
  • fibropolycystic liver disease
320
Q

Extrahepatic forms of CCA include perihilar tumors known as what?

A

Klatskin tumor

321
Q

Where are Klatskin tumors located?

A

at the junction of the right and left hepatic ducts

322
Q

prognosis for CCA regardless of location

A

dismal

323
Q

Most important premalignant lesion for CCA?

Which is highest grade and highest risk for malignant transformation

A

biliary intraepithelial neoplasias (low to high grade, BiIIN-1, -2, -3)

-BiIIN-3

324
Q

CCAs are usually what type of cancer

A

typical adenocarcinomas . . produce mucin

325
Q

What do CCAs usually incite

A

marked desmoplasia

326
Q

whats more common in liver . . metastasis or primary hepatic neoplasia

A

Metastasis is far more comfMmon

327
Q

most common sources for metastasis to liver

A
  • colon
  • breast
  • lung
  • pancreas
328
Q

More than 95% of biliary tract disease is attributable to what?

A

cholelithiasis (gallstones)

329
Q

What is the most common anomaly of the gallbladder?

A

a folded fundus, creating a phrygian cap

330
Q

Agenesis of all or any portion of the hepatic or common bile ducts and hypoplastic narrowing of biliary channel

A

true “biliary atresia”

331
Q

2 general classes of gallstones

A
  • cholesterol stones (crystalline cholesterol monohydrate)

- pigment stones (bilirubin calcium salts)

332
Q

Ethnicity that gall stones are really prevalent

A
  • Native American
  • more prevalent in US and western europe
  • uncommon in developing countries
333
Q

Pigment gallstones are predominent type where?

what settings?

A

Non-western populations

bacterial infections of biliary tree and parasitic infestations

334
Q

Major risk factors for gallstones

A
  • Age and Sex (middle to older age, females, caucasian women twice as likely) . . .also metabolic syndrome and obesity
  • Environmental factors: Estrogen (oral contraceptive use during pregnancy)
  • acquired disorders: Gallbladder stasis
  • Hereditary: ABC . . ATP-binding cassette transporters
335
Q

ABCG8 gene

A

Cholesterol Gallstones

336
Q

Four conditions that contribute to formation of cholesterol gallstones

A
  • supersaturation of bile with cholesterol
  • hypomotility of the gallbladder
  • accelerated cholesterol crystal nucleation
  • hypersecretion of mucus in gallbladder
337
Q

Disorders that are associated with elevated levels of unconjugated bilirubin and increase risk of developing pigment stones

A
  • chonic hemolytic anemia
  • Severe ileal dysfunction or bypass
  • bacterial contamination of biliary tree
338
Q

What organisms increase likelihood of pigment stone formation

A
  • E. coli
  • Ascaris lumbricoides
  • Liver fluke C. sinensis
339
Q

This gallstone is pale yellow, round to ovoid and exclusively in gallbladder

A

Cholesterol stone

340
Q

Black pigment stones are found where

A

sterile gallbladder bile

341
Q

Brown pigment stones are found where

A

infected large bile ducts

342
Q

What kind of gallstone: laminated and soft and may have soaplike or greasy consistency

A

Brown pigment stones

343
Q

what is a prominent symptom of gallstones that may be excruciating

A

biliary colic

344
Q
  • constant pain
  • follows fatty mean which forces stone against the gall bladder outlet increasing pressure
  • Pain in right upper quadrant or epigastrium that may radiate to the right shoulder or back
A

biliary colic

345
Q

More severe complictions of gallstones

A
  • empyema
  • perforation
  • fistulas
  • inflammation of biliary tree (cholangitis)
  • obstructive cholestasis
  • pancreatitis
346
Q

What size stones are more dangers

A

very small or “gravel” stones

347
Q

When a large stone erode directly into an adjacent loop of small bowel, generating intestinal obstruction

A

“gallstone Ileus” or “Bouveret syndrome”

348
Q

Gallstones increase risk for what

A

Gallbladder carcinoma

349
Q

Cholecystitis (inflammation of gallbladder) occurs almost always in association with what

A

gallstones

350
Q

Acute calculous cholecystitis is precipitated in 90% of cases by what

A

obstructin of the nuck or the cystic duct by a stone

351
Q

What is the primary complication of gallstones and the most common reason for emergency cholecystectomy

A

Acute cholecystitis

352
Q

What percent of Cholecystitis cases are without (acalculous) gallstones

A

10%

353
Q

Acute calculous cholecystitis results from what?

A

chemical irritation and inflammation of a gallbladder obstructed by stones

354
Q

Acute calculous cholecystitis frequently develops in who?

A

diabetic patients who have symptomatic gallstones

355
Q

Acute acalculous cholecystitis without stone involvement is thought to result from what?

A

ischemia

356
Q

Risk factors for acute Acalculous cholecystitis

A
  • sepsis with hypotension and multisystem organ failure
  • Immunosuppresion
  • major trauma and burns
  • DM
  • infections
357
Q

gallbladder is enlarged and tense. may assume a bright red or blotchy, violaceous to green-black discoloration

A

Acute cholecystitis

358
Q

An attack of acute cholecystitis begins with progressive right upper quadrant or epigastic pain that lasts for how long?

A

more than 6 hours

359
Q

Which cholecystitis has higher incidence of gangrene and perforations

A

Acalculous

360
Q

What infections may cause acute acalculous cholecystitis

A
  • Salmonella typhi

- Staph

361
Q

Rokitansky-Aschoff sinuses (Outpouchings of the mucosal epithelium through wall)

A

Chronic Cholecystitis

362
Q

Porcelain gallbladder?

why is this notable

A

chronic cholecystitis

increased incidence of cancer

363
Q

gallbladder has a massively thickened wall and is shrunken, nodular . .triggered by rupture of Rokitansky-Aschoff sinuses

A

Xanthogranulomatous cholecystitis (a type of chronic)

364
Q

an atrophic, chronically obstructed, often dilated gallbladder that may contain only clear secretions

A

hydrops of the gallbladder (chronic cholecystitis)

365
Q

most common malignancy of the extrahepatic biliary tract

A

Carcinoma of the gallbladder

366
Q

Areas with higher risk for carcinoma of the gallbladder

A

In the US areas with large numbers of Native American or Hispanic populations such as the southwest

367
Q

Gender for Gallbladder cancer

A

at least twice as common in women than men

368
Q

5 year survivial for gallbladder cancer

A

less than 10%

369
Q

What is the most important risk factor for gallbladder cancer (besides gender and ethnicity)?

A

gallstones

370
Q

common thread that ties gallstones or chronic infections together with gallbladder cancer

A

chronic inflammation

371
Q

overexpression of ERBB2 (Her-2/neu)

A

Gallbladder cancer

372
Q

mutations of chromatin remodeling genes such as PBRM1 and MLL3

A

Gallbladder cancer

373
Q

2 patterns of growth for carcinomas of the gallbladder?

which more common

A
  • infiltrating
  • exophytic

Infiltrating more common

374
Q

Most carcinomas of the gallbladder are what

A

adenocarcinomas

375
Q

What is the only effective treatment of gallbladder cancer

A

surgical resection, often including adjacent liver

376
Q

Murphy’s sign

A

cholecystitis

377
Q

Most common congenital anomaly of the pancreas

A

Pancreas divisum

378
Q

Failure of fusion of the fetal duct systems of the dorsal and ventral pancreatic primordia

A

Pancreas divisum

379
Q

What is the result of pancreas divisum

A

bulk of the pancreas drains into the duodenum through the small-caliber minor papilla instead of the duct of Wirsung through pailla of Vater

380
Q

This congenital anomaly is a band-like ring of normal pancreatic tissue that completely encircles the second portion of the duodenum . . . can produce obstruction

A

Annular Pancreas

381
Q

Favored sites for extopia of the pancreas

A
  • stomach and duodenum

- followed by jejunum, Meckel diverticula, and Ileum

382
Q

What may ectopic pancreas cause

A

may cause pain from localized inflammation or rarely incite mucosal bleeding

383
Q

gene for pancreatic agenesis

A

PDX1

384
Q

Cullen’s and Grey Turner’s signs

A

Acute Pancreatitis

385
Q

Both acute and chronic pancreatitis are initiated by what

A

injuries that lead to autodigestion of the pancreas by its own enzymes

386
Q

Acute pancreatitis: reversible or not

A

reversible

387
Q

What 2 things account for 80% of cases of acute pancreatitis in Western countries

A
  • Biliary tract disease
  • alcoholism
  • gallstones also
388
Q

Gender and age predominance of acute pancreatitis based on etiology

A
  • Alcoholism: Males 6:1 and younger

- Gallstones: Females 3:1 and older

389
Q

3 major initiating events that cause activation of pancreatic enzymes in sporadic forms of acute pancreatitis

A
  • Pancreatic duct obsruction
  • Primary acinar cell injury
  • Defective intracellular transport of proenzymes within acinar cells
390
Q

3 genes implicated in hereditary pancreatitis

A
  • CFTR
  • PRRS1
  • SPINK1
391
Q

Alcohol increases contraction of what leading to pancreatitis

A

sphincter of Oddi . .the muscle at the Papilla of Vater

392
Q

recurrent attack of severe acute pancreatitis often beginning in childhood and ultimately leading to chronic pancreatitis

A

Hereditary pancreatitis

393
Q

The shared feature of most forms of hereditary pancreatitis

A

a defect that increases or sustains the activity of Trypsin

394
Q

Which gene associated with hereditary pancreatitis is Loss of function and autosomal recessive

A

SPINK1

395
Q

Hereditary pancreatitis risk for pancreatic cancer

A

40% lifetime risk

396
Q

Fat necrosis in pancreatitis is due to what

A

activity of lipase

397
Q

Pancreatic substance that is red-black with interspersed foci of yellow-white chalky fat necrosis

A

Acute necrotized pancreatitis

398
Q

cardinal manifestation of acute pancreatitis

A

abdominal pain

399
Q

referred pain for acute pancreatitis

A

upper back and LEFT shoulder

400
Q

elevated levels of what support at diagnosis of acute pancreatitis

A

amylase and lipase

401
Q

time frame for elevated serum amylase in acute pancreatitis?

lipase?

A

first 24 hours

72-96 hours

402
Q

What is the key to managing acute pancreatitis

A

“resting” the pancreas by total restriction of oral intake and by supportive therapy

403
Q

What may be seen with acute pancreatitis

A
  • Glycosuria in 10%

- Hypocalcemia

404
Q

5% of patients with severe acute pancreatitis may die withing the first week of illness due to what?

A
  • acute respiratory distress syndrome

- acute renal failure

405
Q

possible sequalae of acute pancreatitis

A
  • sterile pancreatic abscess

- pancreatic psuedocyst

406
Q

is Chronic pancreatitis reversible?

A

NO

407
Q

prolonged inflammation of pancreas associated with destruction of exocrine parenchyma, fibrosis, and in late stages, destruction of endocrine parenchyma

A

chronic pancreatitis

408
Q

age and gender most common for chronic pancreatitis

A

middle age males

409
Q

most common cause of chronic pancreatitis

A

by far long term alcohol abuse

410
Q

What cytokines tend to predominate in chronic pancreatitis?

These induce activation and proliferation of what?

A

fibrogenic factors like TGF-b and PDGR

periacinar myofibroblasts resulting in deposition of collagen and fibrosis

411
Q

Pathogenically distinct form of chronic pancreatitis that is associated with presence of IgG4-secreting plasma cells

A

Autoimmune pancreatitis

412
Q

Autoimmune pancreatitis may mimic what?

how do you differentiate?

A

pancreatic carcinoma

responds to steroid therapy

413
Q

Dropout of pancreatic acinar cells

A

chronic pancreatitis

414
Q

visualization of calcifications within the pancreas by CT and Ultrasound

A

chronic pancreatitis

415
Q

long term outlook for chronic pancreatitis

A

poor

416
Q

What are the things that lead to significant morbidity and contribute to mortality in chronic pancreatitis

A
  • Pancreatic exocrine insufficiency
  • chronic malabsorption
  • DM
417
Q

localized collections of necrotic and hemorrhagic material that are rich in pancreatic enzymes and lack an epithelial lining

A

Psuedocyst

418
Q

When do psuedocysts usually arise?

A

following a bout of acute pancreatitis, particularly one superimposed on chronic alcoholic pancreatitis

can also arise from trauma

419
Q

Where may a psuedocyste be located

A
  • may be within the pancreas
  • more commonly in lesser omental sac or in retroperitoneum b/t stomach and transverse colon
  • or between liver and stomach
420
Q

invasive pancreatic cancers are believed to arise from what?

A

well-defined noninvasive precursor lesion in small ducts referred to as pancreatic intraepithelial neoplasia (PanIN)

421
Q

epithelial cells in PanIN show what

A

dramatic telomere shortening

422
Q

most frquently altered ONCOGENE in pancreatic cancer?

Most common Tumor suppressor gene?

Tumor suppressor gene most specific to pancreatic cancer?

A

KRAS

CDKN2A –>p16/INK4a

SMAD4

423
Q

age and ethnicity for pancreatic cancer

A
  • older adults . . 60-80
  • Black>whites
  • Ashkenazi jews slightly more common
424
Q

strongest environmental influence for pancreatic cancer?

other risk factors

A

smoking

  • diets rich in fats
  • chronic pancreatitis
  • DM
425
Q

Germline mutations in CDKN2A are associated with pancreatic cancer and are almost always observed in individuals from families with increased incidence of what?

A

Melanoma

426
Q

Most pancreatic cancers arise where

A

head

427
Q

Inherited diseases that predispose to pancreatic cancer

A
  • Peutz-Jeghers

- HNPCC

428
Q

Pancreatic carcinomas that remain silent for a long time and may be quite large and most are widely disseminated by the time they are discovered?

Why?

A

body and tail

don’t impinge on biliary tract

429
Q

characteristic microscopic feature of pancreatic cancer

A

intense desmoplastic reaction with dense stromal fibrosis

-also has perineural invasion

430
Q

what is usually first symptom of pancreatic cancer

A

pain . . . by the time they present, usually beyond cure

431
Q

Migratory thrombophlebitis and Trousseau’s sign

A

pancreatic cancer

432
Q

serum levels that are often elevated in pancreatic cancer

A
  • carcinoembyonic antigen - CEA

- CA19-9

GI 2 (13 decks)

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