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My Decks > Path- Liver > Flashcards

Path- Liver Flashcards

1
Q

Acinar zone with highest O2 content

A

Zone 1, closest to the portal triad

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2
Q

Cholestasis

A

disruption of bile flow

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3
Q

Important histologic structures of liver

A

Hepatocytes
Sinusoidal endothelial cells
Kupffer cells (attached macrophages, intrasinusoidal)
Stellate (Ito) cells (perisinusoidal, Space of Disse)

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4
Q

Stellate (Ito) cells

A

Located in Space of Disse.

Function in fat/vitamin/fibrous tissue metabolism and fat storage (Vitamin A).

If injured, can become activated and convert into highly fibrogenic myofibroblasts

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5
Q

Metabolic Functions of Liver

A
  1. Formation and excretion of bile during bilirubin metabolism
  2. Regulation of carbohydrate homeostasis
  3. Lipid synthesis and secretion of plasma lipoproteins
  4. Control of cholesterol metabolism
  5. Formation of urea, serum albumin, clotting factors, enzymes, etc
  6. Metabolism/detoxification of drugs and other foreign substances
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6
Q

Broad Etiologies of Liver Injury

A 
Infectious 
Immune-Mediated 
Drug & Toxin 
Metabolic 
Genetic 
Autoimmune cholangiopathy
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7
Q

Defining characteristics of acute hepatic failure

A 
coagulation abnormality (increased prothrombin time)
encephalopathy (mental alteration)
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8
Q

MELD Score

A

Model for End-stage Liver disease. <15 not a candidate for transplantation

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9
Q

Manifestations of Acute Liver Failure

A 
jaundice 
neurologic symptoms
encephalopathy 
portal HTN 
hepatorenal syndrome (and hepatopulmonary syndrome)
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10
Q

Acute vs Chronic Hepatitis

A

Based on time course

Acute = <26wks

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11
Q

Steatosis

A

Accumulation of fat within hepatocytes, usually reversible

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12
Q

Causes of steatosis

A

Non-alcoholic fatty liver
Alcohol
Drugs
Viruses

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13
Q

Microvesicular steatosis

A

disruption of fat metabolism, usually irreversible

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14
Q

Causes of microvesicular steatosis

A

Reye syndrome
Tetracycline toxicity
Fatty change in pregnancy

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15
Q

Ballooning degeneration

A

Loss of water control, sign of early injury. Often seen with Mallory hyaline. Often associated with alcohol abuse.

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16
Q

Mallory hyaline

A

clumped/precipitated intermediate keratin filaments (complexed with proteins like ubiquitin). Often seen with ballooning degeneration. Often associated with alcohol abuse

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17
Q

Pathognomonic histologic finding of acute hepatitis

A

lobular disarray with mononuclear cell infiltrates

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18
Q

Histologic finding of cirrhosis

A

bridging fibrosis

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19
Q

Parenchymal extinction

A

feature of cirrhosis characterized by a loss of liver parenchyma d/t microscopic areas of ischemia

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20
Q

Characteristic finding of cirrhosis

A

Diffuse nodular regeneration…
surrounded by dense fibrotic septa…
with subsequent parenchymal extinction…
and collapse of liver structures…
causing pronounced distortion of hepatic vasculature…
which leads to increased resistance to portal blood flow…
and subsequent portal HTN

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21
Q

MCC of death in compensated cirrhosis

A

cardiovascular disease followed by stroke, malignancy, and renal disease

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22
Q

MCC of death in decompensated cirrhosis

A

Complications of portal HTN, hepatocellular carcinoma, sepsis

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23
Q

Pre-hepatic causes of portal HTN

A

portal vein thrombosis

narrowing of the portal vein

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24
Q

Intra-hepatic causes of portal HTN

A

cirrhosis (MCC)

sinusoidal obstruction

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25
Q

Post-hepatic causes of portal HTN

A

severe R CHF
constrictive pericarditis
hepatic vein outflow obstruction

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26
Q

Clinical consequences of portal HTN

A
  1. portosystemic venous shunts/portacaval anastomoses (esophageal varices, hemorrhoids, caput medusa)
  2. congestive splenomegaly
  3. ascites
  4. hepatic encephalopathy
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27
Q

Pathophysiology of ascites

A

sinusoidal HTN –> splanchnic vasodilation and hyperdynamic circulation –> decreased plasma oncotic pressure –> increased aldosterone

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28
Q

Hepatopulmonary syndrome

A

a complication of cirrhosis
intrapulmonary vascular dilation –> rapid blood flow –> poor Hb oxygenation –> hypoxia
unknown pathogenesis

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29
Q

Portopulmonary HTN

A

Related to portal HTN, possibly caused by excessive pulmonary vasoconstriction. Causes DOE and clubbing of digits

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30
Q

IgM as a test for hepatitis

A

measures initial response to acute infection

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31
Q

IgG as a test for hepatitis

A

long term response. signifies either ongoing chronic infection or past infection

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32
Q

Lobular disarray

A

histology of acute hepatitis

  • ballooned hepatocytes and acidophilic bodies
  • individual or confluent hepatocyte dropout
  • zonal, bridging, or panlobular necrosis
  • sinusoidal inflammatory cells
  • prominent Kupffer cells
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33
Q

HAV transmission

A

fecal-oral route
person to person
contaminated water and foods
blood-borne is RARE

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34
Q

Risk factors for HAV

A 
48% unknown 
14% sexual or household contact 
10% men who have sex with men
8% day-care
6% IVDA 
5% international travel 
4% food or waterborne outbreak 
8% other
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35
Q

Pathobiology of HAV

A

HAV virus binds to an integral glycoprotein receptor on host cell
Virus serves as mRNA that is translated within the cytoplasm into a polyprotein
Polyprotein is later cleaved to mature viral proteins
Replication of membrane-bound complex generates new viral genomes that get exported out of cell and into bile

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36
Q

Pathophysiology of HAV

A

ingestion –> replication in GI tract –>transported to liver (major site of replication; immune response launched) –> shed in bile, transported to intestines –> shed in feces –> brief viremia –> cellular immune response: clinical disease and control

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37
Q

Hepatitis viruses that only cause acute self-limited disease

A

HAV

HEV

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38
Q

hepatitis viruses- RNA or DNA?

A 
HAV = RNA (picoRNAvirus)
HBV = DNA (hepaDNAviridae)
HCV = RNA (flaviviradae, unstable genome)
HDV = RNA (no classification, a virion)
HEV = RNA (hepEviridae)
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39
Q

HEV transmission

A

enteric, water-borne infection

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40
Q

Population at great risk with HEV

A

pregnant women; mortality rate ~20%

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41
Q

Histology of HEV

A

lobular disarray

*Canalicular cholestasis and gland-like transformation of hepatocytes

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42
Q

HBV transmission

A

blood-borne, unprotected sex and IVDA

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43
Q

Clinical features of HBV

A 
fatigue 
anorexia 
nausea 
jaundice/scleral icterus 
abdominal pain 
arthralgias
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44
Q

Dane Particle

A

intact HBV virion. Has an outer envelope and inner core

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45
Q

HBsAg

A

HBV infection (acute or chronic)

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46
Q

HBeAg

A

High viral load/infectivity

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47
Q

HBV DNA

A

High viral load/infectivity

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48
Q

Anti-HBs

A

immunity

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49
Q

Anti-HBc IgM

A

acute infection

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50
Q

Anti-HBc IgG

A

past or chronic infection

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51
Q

Anti-HBe

A

past or low infectivity chronic infection

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52
Q

Anti-HBc IgG and HBsAg

A

chronic infection

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53
Q

Anti-HBc and anti-HBs

A

resolved (past) infection

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54
Q

Distinctive histology of HBV

A

ground-glass hepatocyte containing abundant HBsAg

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55
Q

HDV transmission

A

follows that of HBV, primarily through parenteral exposure. Can either 1) be acquired at the same time as a primary HBV infection; or 2) later, superimposed on a pre-existent chronic HBV (worse prognosis)

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56
Q

Hepatitis viruses that may cause both acute and chronic disease

A

HBV
HBV + HDV
HCV

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57
Q

HDV Superinfection

A

HDV superimposed on a pre-existent chronic HBV. Most causes (50-70%) develop severe acute hepatitis, and 90% of them become chronic carriers

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58
Q

Histology of HDV

A

sanded nucleus d/t HDAg accumulation

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59
Q

MCC of chronic hepatitis worldwide

A

HCV

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60
Q

MCC of chronic hepatitis in the USA

A

HCV

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61
Q

Characteristic clinical feature of HCV

A

persistent elevation in serum aminotransferases

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62
Q

anti-HCV antibodies

A

In acute symptomatic HCV, Ab’s are only detected initially in 50-70% of pt’s. The remaining pt’s don’t have Ab’s for another 3-6wks

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63
Q

HCV transmission

A

parenteral, sex

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64
Q

Risk factors for HCV

A

54% IVDA
36% multiple sex partners
16% having had surgery w/i 6mths
10% needle stick injury

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65
Q

HCV IgG

A

past resolved or chronic infection
appears weeks after onset of new infection
occasional false positive

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66
Q

HCV RNA detected by PCR means…

A

virus is present in liver/blood

found in acute or chronic HCV

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67
Q

Histology of HCV

A

lobular disarray
steatosis
bile duct damage

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68
Q

Treatable hepatitis

A

HCV
$1000/pill
$100,000/course

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69
Q

Hepatitis viruses causing acute asymptomatic hepatitis

A

all hepatotropic viruses

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70
Q

Hepatitis viruses causing acute symptomatic hepatitis

A

all hepatotropic viruses

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71
Q

etiology of acute liver failure

A

viral hepatitis is responsible for ~10% of cases of acute liver failure
HAV, HEV and HBV, HDV

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72
Q

common causes of chronic viral hepatitis

A

HCV > 80%

HBV < 10% in adults (perinatal >90%)

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73
Q

Carrier state hepatitis

A

small number of HBV

No HCV

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74
Q

HIV and hepatitis

A

10% of HIV pt’s are co-infected with HBV

25% of HIV pt’s are co-infected with HCV

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75
Q

AIDS and hepatitis

A

liver disease is the 2nd MCC of death

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76
Q

When is HAV life-threatening?

A

in the presence of chronic HCV or chronic HBV

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77
Q

Autoimmune hepatitis

A

injury to normal hepatocytes by infiltrating T cells and plasma cells, leading to fibrosis/cirrhosis

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78
Q

Lab tests to detect AIH

A

Anti-nuclear Ab (ANA)
Anti-smooth (actin) muscle Ab (ASMA)
IgG

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79
Q

Outcome and prognosis of AIH

A

Can either develop with a rapidly progressive acute disease, or follow a more indolent path; if untreated, both are likely to lead to liver failure. Although a chronic disease, responds very well to immunosuppression with prednisone and azathioprine

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80
Q

Epidemiology of AIH

A

Most common in young women
Genetic predisposition (HLA-DR in Caucasians)
~50% of pt’s with AIH will have a concurrent autoimmune disorder
~20% of chronic hepatitis in Western Europe and North America

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81
Q

Type 1 AIH

A

middle-aged women

ANA, ASMA, pANCA

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82
Q

Type 2 AIH

A

children or teenagers
mostly female
associated with anti-liver kidney microsomal Ab (anti-LKM1)

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83
Q

Histology of AIH

A

extensive interface hepatitis
plasma cell predominance in the mononuclear inflammatory infiltrates

(*plasma cell = eccentric nucleus and Golgi huff)

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84
Q

Histologic patterns of drug-induced liver injury

A

bile duct injury
steatosis and steatohepatitis
vascular injury/veno-occlusive disease
neoplasms

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85
Q

steatosis vs steatohepatitis

A

steatosis: fat in cytoplasm of hepatocytes
steatohepatitis: neutrophils and fibrosis

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86
Q

Patterns of drug-/toxin-induced hepatic injury

A

Periportal region: gluconeogenesis, cholesterol and urea synthesis; high O2
Pericentral region: glycolysis, bile acid and glutamine synthesis, drug metabolism, p450-dependent bioactivation

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87
Q

Examples of drugs causing

  • hepatocellular injury
  • autoimmune hepatocellular injury
  • cholestatic liver injury
A
  • hepatocellular injury –> acetaminophen
  • autoimmune hepatocellular injury –> halothane hepatitis
  • cholestatic liver injury –> estrogen
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88
Q

MCC of acute liver failure necessitating transplantation in the USA

A

acetaminophen toxicity

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89
Q

acetaminophen toxicity

A

d/t metabolic by-product (NAPQI)
zone 3 necrosis (pericentral)
toxicity is enhanced by ETOH (up regulates CYP450

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90
Q

acetaminophen toxicity antidote

A

N-acetyl cysteine; must give w/i 8-12hrs; restores glutathione

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91
Q

drugs that induce CYP2E1 (increase acetaminophen toxicity)

A

ETOH
Isoniazid (INH)
Phenobarbital

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92
Q

Reye syndrome

A

associated with ASA
Seen in children
Mitochondrial dysfunction, mainly in liver and brain –> microvesicular steatosis (fat droplet accumulation)
Acute liver failure without extensive necrosis

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93
Q

Fatty liver

A

develops in all drinkers after moderate intake
completely reversible until there is fibrosis
have mild elevation of LFTs

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94
Q

Alcoholic hepatitis

A

ballooning (swelling) and necrosis of hepatocytes with formation of Mallory bodies
acute inflammation, especially around degenerating cells
centrilobular fibrosis
10% mortality acute phase, 70% develop cirrhosis

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95
Q

NAFLD

A

Non-alcoholic fatty liver disease
Hepatic steatosis (fatty liver) in pt’s who do not consumer alcohol or do so in very small quantities
Associated with metabolic syndrome (obesity, insulin resistance or DM, hyperlipidemia, and HTN)

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96
Q

NAFLD Presentation

A

Asx with elevated AST/ALT’s (<250IU/L)
Metabolic risk factors
Fatty infiltration on liver imaging

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97
Q

Patterns of NAFLD

A
  1. Fatty liver (NAFLD): >5% fatty change but no necroinflammatory change
  2. Non-alcoholic steatohepatitis (NASH): ballooning degeneration, necrosis, lobular inflammation, +/- fibrosis
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98
Q

Risk factors for alcoholic liver disease

A
  • amount and duration of ETOH consumption
  • F > M
  • genetic factors
  • protein-calorie malnutrition
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99
Q

Risk factors for non-alcoholic liver disease

A
  • obesity
  • DMII
  • dyslipidemia
  • metabolic syndrome
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100
Q

MCC’s of cirrhosis in the USA (3)

A
  1. chronic alcoholism
  2. chronic HCV
  3. Non-alcoholic steatohepatitis
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101
Q

Hepcidin regulation molecules

A

HFE (high iron gene)
HJV (hemojuvelin)
TfR2 (transferrin receptor)

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102
Q

Hepcidin synthesis is activated by

A

increased iron stores

infection or inflammation (IL-6)

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103
Q

Hepcidin synthesis is inhibited by

A

hypoxia

increased EPO

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104
Q

Pathogenesis of hemochromatosis

A

decreased hepcidin synthesis, caused by mutations in HFE protein plays a central role

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105
Q

Treatment of hemochromatosis

A

phlebotomy

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106
Q

Epidemiology of hemochromatosis

A

one of the most common genetic disorders in humans

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107
Q

complications of hemochromatosis

A

cirrhosis

cardiac disease

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108
Q

Classic tetrad of Hemochromatosis

A

hepatomegaly
skin pigmentation
destruction of pancreatic islets
cardiomyopathy

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109
Q

Diagnosing hemochromatosis

A

transferrin saturation
MRI of liver
Iron biopsy (not as useful)
HFE mutation (90% of pt’s are homozygous for C282Y mutations)

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110
Q

bronze diabetes

A

A complication of hemochromatosis; pigmentation d/t increased epidermal melanin.

111
Q

Causes of secondary hemochromatosis

A 
blood transfusions (for hereditary or acquired anemias) 
iron supplements or excess dietary iron (uncommon)
112
Q

Etiology of Wilson’s disease

A

autosomal recessive disorder caused by mutation in copper transporting ATPase, leading to toxic levels of copper accumulation in liver, brain, and eye

113
Q

Epidemiology of Wilson’s disease

A

5-15y/o

or neuropsychiatric pt’s in 20’s

114
Q

treatment of Wilson’s disease

A

chelation with Pencillamine (or zinc, to competitively inhibit copper)

liver transplant for cirrhosis

no treatment can cure neurologic symptoms (Parkinsonian-like)

115
Q

Where is dietary copper absorbed?

A

duodenum

116
Q

ceruloplasmin

A

transporter protein for copper; usually low in Wilson’s disease, but not always

117
Q

Diagnosing Wilson’s Disease

A

Biopsy to assess liver copper content (most sensitive, highest positive predictive value)
low serum ceruloplasmin (screening)
increased urine copper (specific)

118
Q

Histologic preparation for Wilson’s disease

A

Copper (rhodamine) stain

119
Q

Clinical Features of Wilson’s Disease

A

Kayser-Fleischer ring: Brown ring around iris of eye indicating copper accumulation; diagnostic of Wilson’s disease

Parkinson-like features

120
Q

Alpha 1 antitrypsin deficiency

A

autosomal recessive disorder of protein folding marked by very low levels of circulation a1-antitrypsin

121
Q

function of Alpha 1 antitrypsin

A

inhibition of proteases

122
Q

Most common clinically significant mutation of Alpha 1 antitrypsin deficiency

A

PiZZ (a1AT levels are only 10% of normal)

123
Q

Clinical features of Alpha 1 antitrypsin deficiency

A

neonatal hepatitis with cholestatic jaundice in 10-20%
HCC in 2-3% of PiZZ adults
Panlobular emphysema

124
Q

Histologic findings of Alpha 1 antitrypsin deficiency

A

non-specific
globules
fibrosis
variable portal inflammation

125
Q

Primary biliary cirrhosis

A

autoimmune cholangiopathy disease of unknown etiology which selectively affects the small intrahepatic bile ducts with progressive bile duct damage, chronic cholestasis, biliary fibrosis/cirrhosis leading to hepatic failure

126
Q

Epidemiology of Primary biliary cirrhosis

A

middle-aged women 40-50y/o

F > M 9:1

127
Q

Laboratory findings in Primary biliary cirrhosis

A

anti-mitochondrial Ab’s (AMA)

disproportionate elevation of serum Alk Phos

128
Q

Histology of Primary biliary cirrhosis

A

Ductopenia (early ductal inflammation)
Epithelioid granulomas
Necroinflammatory and cholestatic process with fibrosis
portal-portal bridging septa

129
Q

Gross findings of Primary biliary cirrhosis

A

yellow-green, cirrhotic liver

130
Q

Primary Sclerosing Cholangitis

A

a progressive disease of the liver characterized by cholestasis with obliterative fibrosis of intra- and extra hepatic bile ducts with dilation of preserved segments

131
Q

Common association with Primary Sclerosing Cholangitis

A

IBD in 70% of pt’s (90% Crohn’s, 10% ulcerative colitis)

132
Q

Epidemiology of Primary Sclerosing Cholangitis

A

M > F 2:1

~40y/o

133
Q

Prognosis of Primary Sclerosing Cholangitis

A

variable
some pt’s have severe recurrent cholangitis
others progress to biliary cirrhosis

134
Q

Pathogenesis of Primary Sclerosing Cholangitis

A

unknown, features suggest T cell involvement

135
Q

Treatment of Primary Sclerosing Cholangitis

A

no cure
cholestyramine for pruritis
liver transplant for liver failure

136
Q

Diagnosing Primary Sclerosing Cholangitis

A

Pathologic and radiographic findings!
Elevated Alk phos in 90% of pt’s
pANCA in 90% of pt’s (non-specific finding)
strictures, beading, and dilation of large ducts

137
Q

Liver Biopsy in Primary Sclerosing Cholangitis

A

obliterative cholangitis with inflammation
periductular onion ring fibrosis
ductopenia
secondary biliary cirrhosis

138
Q

Histology of hepatic complications in GVHD

A

bile duct damage (lymphocytic infiltration of bile duct epithelium), modest mononuclear portal inflammatory infiltrate

139
Q

Laboratory findings with GVHD

A

cholestatic liver biochemistry

mild elevations in serum transaminases

140
Q

Diagnostic Triad of Cellular Liver Allograft Rejection

A

portal inflammation
bile duct damage
ventral endothelial inflammation

141
Q

Humoral liver allograft rejection

A

endothelial cells in the graft are the main targets for humoral-mediated damage

142
Q

Mechanisms of cholestasis

A

intrahepatic cholestasis
-decreased bile formation (sepsis, estrogen)
-destruction/compression of intrahepatic bile ducts/ductules (primary biliary cirrhosis, infiltration of liver with tumor/granuloma)
-any severe liver disease (viral hepatitis)
Extrahepatic/large bile duct obstruction (tumor, gallstone, duct strictures, primary sclerosing cholangitis)

143
Q

Isolated disorders of uncongugated hyperbilirubinemia

A

Increased bilirubin production i.e. Hemolysis
Decreased hepatocellular uptake i.e. drugs
decreased conjugation:
-Gilbert’s syndrome
-Crigler-Najjar
-Neonatal jaundice
-Diffuse hepatocellular disease (virus, drugs, cirrhosis)

144
Q

Isolated disorders of conjugated hyperbilirubinemia

A

decreased canalicular transport

  • Dubin Johnson syndrome
  • Rotor syndrome
  • Autoimmune cholangiopathies
145
Q

Sepsis-associated cholestasis

A

usually caused by gram (-) bacteria, leading to canalicular cholestasis with activated Kupffer cells, fatty change, and portal inflammation; typically conjugated

146
Q

Lab findings with sepsis-associated cholestasis

A

increase in serum bilirubin out of proportion to Alk phase elevation

147
Q

Prognosis of sepsis-associated cholestasis

A

poor, 60-90% mortality

148
Q

causes of large duct obstruction in adults

A

gallstones (most common)
malignant neoplasms of biliary tree/head of pancreas
PSC

149
Q

causes of large duct obstruction in peds

A

biliary atresia
choledochal cysts
CF

150
Q

Complications of large bile duct obstruction

A

if prolonged –> secondary biliary cirrhosis

if intermittent –> ascending cholangitis

151
Q

hepatolithiasis

A

intrahepatic gallstone formation, leading to progressive inflammatory destruction of parenchyma –> risk of cholangiocarcinoma

152
Q

epidemiology of hepatolithiasis

A

East Asia

153
Q

hepatolithiasis is frequently associated with

A

recurrent ascending cholangitis

154
Q

etiology of ascending (suppurative) cholangitis

A

typically caused by bacteria within small bowel traveling through the Sphincter of Oddi to the liver and biliary tract

155
Q

Clinical features of ascending cholangitis

A

fever
jaundice
abdominal pain

156
Q

laboratory findings with ascending cholangitis

A

increased blood neutrophils, Alk phos, and bilirubin

157
Q

Major causes of neonatal cholestasis

A

Extra-hepatic biliary atresia
infectious hepatitis
alpha 1 antitrypsin deficiency
idiopathic neonatal hepatitis (dx of exclusion)

158
Q

Perinatal biliary atresia

A

absence of all or a portion of the extrahepatic bile ducts. most frequent cause of liver disease death in early childhood; congenital infections have been implicated in initiating autoimmune reaction

159
Q

Hereditary fibropolycystic liver disease

A

group of genetic disorders with segmental dilatations of the intrahepatic bile ducts and associated fibrosis (CYSTS AND/OR FIBROSIS)

160
Q

Pathogenesis of Hereditary fibropolycystic liver disease

A

primary cilia protein gene mutations leading to disruptions of portal tract embryogenesis

161
Q

polycystic liver disease

A

most commonly associated with autosomal dominant polycystic kidney disease, also associated with autosomal recessive PCKD, and present with no renal cysts

162
Q

Von Meyenburg Complexes

A

peripheral bile duct malformations (bile duct hamartoma)

Multiple complexes = polycystic liver disease

163
Q

Congenital hepatic fibrosis is often associated with…

A

autosomal recessive polycystic kidney disease

164
Q

Caroli syndrome

A

Congenitally dilated intrahepatic bile ducts often involving the entire liver + congenital hepatic fibrosis

165
Q

Caroli disease is associated with…

A

recurrent bacterial cholangitis

166
Q

Complication of Caroli disease

A

risk of cholangiocarcinoma

167
Q

Choledochal cysts

A

congenital cystic dilation of extrahepatic and intrahepatic bile ducts; commonly leads to complete inflammatory obstruction of the terminal portion of the bile duct

Type V = Caroli disease

168
Q

complications of choledochal cysts

A

stone formation
stenosis
pancreatitis
cholangiocarcinoma (age related, older = increased risk)

169
Q

Intrahepatic cholestasis of pregnancy

A

mild cholestatic disease occurring in <2% of pregnancies
also seen with oral contraception use
may cause intrauterine fetal death
strong genetic component, 10-15% of first degree female relatives are affected

170
Q

Clinical features of Intrahepatic cholestasis of pregnancy

A

onset in 3rd trimester
Pruritis!
occasional jaundice
resolves within a few days of delivery

171
Q

Acute fatty liver of pregnancy

A 
usually in 3rd trimester
may present with acute failure with modest AST/ALT increase 
may occur in subsequent pregnancies 
maternal mortality 5-26% 
intrauterine fetal death 9-32%
172
Q

Initial symptoms of acute fatty liver of pregnancy

A 
N/V
epigastric abdominal pain 
preeclampsia 
jaundice 
anorexia
173
Q

lab tests for acute fatty liver of pregnancy

A

mildly elevated AST/ALT (<500)

hypoglycemia, abnormal coag studies

174
Q

Characteristics of preeclampsia

A

HTN
proteinuria
peripheral edema
coagulation abnormalities

175
Q

HELLP syndrome

A

hemolysis, elevated LFTs, low platelets

most common form of preeclampsia-related liver disease

176
Q

Pathology of HEELP

A

periportal fibrin and hepatoceullular coagulative necrosis

177
Q

Budd Chiari syndrome

A

hepatic vein thrombosis (2+ veins). Caused by

  • polycythemia vera
  • pregnancy
  • postpartum state
178
Q

Sinusoidal obstruction syndrome

A

obliteration of the terminal hepatic venules seen with chemotherapy and immunosuppressive agents

179
Q

Causes of impaired blood flow through the liver

A 
cirrhosis (MCC)
sinusoid occlusion
-sickle cell disease 
-R CHF 
-eclampsia 
-stellate cells
180
Q

function of gallbladder

A

store and concentrate bile (~50mL)

181
Q

Phyrigian Cap

A

inwardly folded fundus of gallbladder; mimics gallstones

182
Q

congenital anomalies of gallbladder

A

shape (angulations/Pyrigian cap), separation

number (agenesis, duplication)

position (intrahepatic, falciform ligament location)

183
Q

risk factors for cholelithiasis

A

obesity
female sex (2:1)
estrogens, oral contraception, pregnancy
age (middle age and older)

5 F's 
Female
Fair 
Fat 
Flatulent 
Fertile
184
Q

classes of gallstones

A 
cholesterol stones (75%) 
pigment stones (25%)
185
Q

pigment stones

A

calcium salts and unconjugated bilirubin

186
Q

Complications of cholelithiasis

A

75% are asx

biliary pain (obstruction) 
cholecystitis 
pancreatitis 
ascending cholangitis 
fistula 
gallbladder carcinoma
187
Q

indications for treatment of asymptomatic cholecystitis

A

surgery is generally discouraged, but may be indicated in pt’s

1) with gallstones >2cm in diameter
2) with nonfunctional of calcified (porcelain) gallbladder –> high risk of gallbladder carcinoma
3) with sickle sickle cell anemia in which the distinction between painful crisis and cholecystitis is difficult

188
Q

Patients with risk factors for complications of gallstones

A 
cirrhosis 
portal HTN 
children 
transplant candidates 
DM with minor symptoms
189
Q

Outcomes of acute cholecystitis

A

Can present as…

  • acute surgical emergency
  • mildly symptomatic with spontaneous resolution
  • gangrenous cholecystitis, can perforation (acalculous > calculous)
190
Q

histologic hallmark of acute cholecystitis

A

neutrophils in the gallbladder mucosa and wall

mucosa may be ulcerated

191
Q

signal void bubbles

A

MRI finding of acute cholecystitis (emphysematous)

192
Q

Presentation of chronic cholecystitis

A

recurrent attacks of either steady epigastric or RUQ pain (INTERMITTENT PAIN!)
usually 50y/o
N/V, intolerance to fatty foods

193
Q

Etiology of chronic cholecystitis

A

can be a sequel to acute cholecystitis, but often presents without a history of earlier attacks

194
Q

Rokitansky-ashoff sinuses

A

outpouching of the mucosal epithelium into the gallbladder wall, seen with chronic cholecystitis

195
Q

pathology of chronic cholecystitis

A

subserosal fibrosis –> fibrous adhesions –> chronic inflammation –> fibrosis –> metaplasia of gallbladder mucosa –> dystrophic calcification (porcelain gallbladder; associated with increased risk of carcinoma)

196
Q

choledocholithiasis

A

common bile duct stones that may remain asx for years

197
Q

Complications of choledocholithiasis

A

obstructive jaundice
gallstone pancreatitis
acute cholangitis

198
Q

Charcot’s triad

A

fever
jaundice
abdominal pain

classic presentation of choledocholithiasis and/or ascending cholangitis

199
Q

Carcinoma of the gallbladder

A

typically an adenocarcinoma (can also be SqCC, carcinoid, carcinosarcoma)

Associated with gallstones.

Presents with abdominal pain and elevated serum Alk Phos.

Infrequently diagnosed, usually pre-op for something else

200
Q

Px of gallbladder carcinomas

A

terrible, 5-10% 5yr survival rate

papillary variant = best overall px

201
Q

Congenital anomalies of the pancreas

A

pancreas divisor (2 duct system); most common anomaly
annular pancreas (wraps around duodenum, can cause obstruction)
ectopic pancreas
agenesis (rare)

202
Q

acute pancreatitis

A

autodigestion of pancreas by its own enzymes (inappropriate activation of digestive proenzymes–> TRYPSIN)

203
Q

causes of acute pancreatitis

A 
ETOH 
pancreatic duct obstruction (calculi) 
drugs 
vascular injury 
infection 
hereditary factors 
hypercalcemia
204
Q

etiology of acute pancreatitis

A

premature activation of trypsin is a likely cause; primarily a combination of genetic, environmental, and metabolic factors

205
Q

markers of acute pancreatitis

A

serum amylase (elevates in 6-12hrs) and *lipase (elevates 4-8hrs)

206
Q

Types of acute pancreatitis (based on histopathology)

A

Acute interstitial pancreatitis (interstitial edema, focal areas of fat necrosis in peri-pancreatic fat)

Acute necrotizing pancreatitis (necrosis of acinar and ductal tissues and islets of Langerhans)

Hemorrhagic pancreatitis (extensive parenchymal necrosis and hemorrhage within the pancreas)

207
Q

Gross findings of acute pancreatitis

A

yellows specks

chalky white with calcium

208
Q

Clinical findings of acute pancreatitis

A

flank ecchymoses

209
Q

pathophysiology of chronic pancreatitis

A

can be initiated by recurrent acute pancreatitis –> myofibroblast stellate cell activation

irreversible destruction of exocrine parenchyma with fibrosis –> destruction of endocrine parenchyma –> IDDM

210
Q

causes of chronic pancreatitis

A

ETOH
repeat episodes of acute pancreatitis
obstruction (calculi, neoplasm)
metabolic (primary hyperparathyroidism, hyperlipidemia, renal transplantation, CF)

211
Q

diagnosing chronic pancreatitis

A

Histology (gold standard)
Reduction in bicarb in duodenal aspirate after secretin stimulation
ERCP
Intrapancreatic calcifications on plain films (rare)

212
Q

histology of chronic pancreatitis

A

dense interlobular fibrosis with preserved (or atrophic) zones of acinar cells between septa

213
Q

Autoimmune pancreatitis variant

A

Unique mass-forming inflammatory form of chronic pancreatitis
Elevated IgG4
Radiographs mimic pancreatic adenocarcinoma
Responds to corticosteroid therapy

214
Q

Pancreatic pseudocyst

A

non-neoplastic
most common pancreatic cyst
associated with acute or chronic pancreatitis
usually does not require tx
can be associated with infection, bleeding, or rupture

215
Q

serous cystadenoma

A 
most common cystic neoplasm of pancreas 
most are benign 
F > M 2:1
~66y/o
lining: flat to cuboidal cells with clear cytoplasm and hyperchromatic nuclei
216
Q

mucinous cystadenoma

A 
>95% Females 
~50y/o 
can be precursors to invasive carcinoma 
almost always in pancreas TAIL 
lining: tall columnar cells with abundant apical mucin
217
Q

intraductal papillary mucinous neoplasm (IPMN)

A

Involves large ducts of pancreas, usually in the HEAD of the pancreas
M>F
Benign, borderline, and malignant variants
Mucinous cells with various degrees of dysplasia and papillary architecture line mystically dilated ducts
Tall, columnar mucinous epithelial cells

218
Q

invasive ductal carcinoma of the pancreas

A

most common pancreatic neoplasm
5yr survival rate = 5%
9mths = medial survival
most are sporadic, 10% familial

219
Q

Trousseau syndrome

A

migratory thrombophlebitis secondary to release of platelet activating factors and procoagulant factors
associated with 10% of invasive ductal carcinomas of the pancreas.

220
Q

pathology of invasive ductal carcinoma of the pancreas

A

precursor lesion: pancreatic intraepithelial neoplasia (PanIN)
Tends to invade peri-pancreatic tissues and elicit desmoplastic tissue reaction

221
Q

oncogene associated with invasive ductal carcinoma of the pancreas

A

KRAS

222
Q

acinar cell carcinoma of the pancreas

A

uncommon (<2% of pancreatic CA)
>60y/o
15% develop lipase hypersecretion syndrome
metastasizes early
Px: poor, slightly better than invasive ductal carcinoma of the pancreas
Histology: trypsin in acinar cells

223
Q

pancreaticoblastoma

A

uncommon, but most frequent pancreatic tumor in childhood ~4y/o
epithelial tumor
Histology: squamous nests, acinar pattern
Px: curable by surgery if detected early

224
Q

Pancreatic neuroendocrine tumor (PanNET)

A

Most common in adults ~55y/o
most produce a peptide hormone (i.e. glucagon) but these are typically non-functional, so pt’s don’t have sx

preferentially metastasize to regional lymph nodes and liver

225
Q

epidemiology of focal nodular hyperplasia

A

women 20-30y/o

226
Q

gross findings of focal nodular hyperplasia

A

FOCAL! central scar, typically <5cm in diameter, but can be large; no true bile ducts or connection to the biliary outflow tract

227
Q

composition of focal nodular hyperplasia

A

hyperplastic nodules of hepatocytes, separated by fibrous septa which form typical stellate scars

228
Q

gross findings of nodular regenerative hyperplasia

A

liver is entirely transformed into nodules grossly similar to micro nodular cirrhosis, but without fibrosis

229
Q

clinical findings of nodular regenerative hyperplasia

A

portal HTN (may be associated with disease development), most pt’s are asx

230
Q

etiology of nodular regenerative hyperplasia

A

thought to be a regenerative response to vascular injury (small vessel vasculitis)

231
Q

histology of nodular regenerative hyperplasia

A

sinusoidal dilation
no inflammatory infiltrates
no fibrosis
no necrosis

232
Q

nodular regenerative hyperplasia also known as…

A

non-cirrhotic portal HTN

233
Q

etiology of hepatic adenoma

A

oral contraceptives in females

anabolic steroids in males

234
Q

gross findings of hepatic adenoma

A

subcapsular mass, prone to rupture, especially in pregnancy. no central scar, rounded, smooth borders.

235
Q

Treatment for hepatic adenoma

A

resect if >5cm or symptomatic

cessation of sex hormones can lead to full regression

236
Q

subtypes of hepatic adenoma

A

HNF1-alpha inactivated hepatocellullar adenoma
B-Catenin activated hepatocellular adenoma
inflammatory hepatocellular adenoma

237
Q

epidemiology of HNF1-alpha inactivated hepatocellullar adenoma

A

most commonly found in women (low rate of malignancy)

238
Q

B-Catenin activated hepatocellular adenoma

A

minority of adenomas
very high risk for malignant transformation
often have a high degree of cytologic or architectural dysplasia

239
Q

inflammatory hepatocellular adenoma

A

found in men and women
associated with NAFLD
small but definite risk of malignant transformation
JAK/STAT (lots of inflammation)

240
Q

complications of hepatocellular adenoma

A

can lead to fatal hemorrhage

241
Q

epidemiology of cavernous hemangioma

A

most common benign liver tumor

242
Q

diagnosing cavernous hemangioma

A

any imaging

243
Q

epidemiology of hepatoblastoma

A

most common liver tumor of young childhood (90% before age of 5y/o)

244
Q

prognosis of hepatoblastoma

A

fatal within the first few years if not treated

245
Q

pathologic variants of hepatoblastoma

A

epithelial type - composed of polygonal epithelial cells or embryonal cells growing in patterns recapitulating liver development

mixed epithelial-mesenchymal - contains mesenchymal elements i.e. osteoid, cartilage, or striated muscle

246
Q

epidemiology of HCC

A

most common primary hepatic malignancy of adults worldwide

M > F

247
Q

etiology of HCC

A

hepatocellular carcinogenesis secondary to viral infections (HBV, HCV) and toxic injury.

Majority of cases in the world are d/t HBV

Number of HCV-associated cases are increasing in western world

toxins: aflatoxin (aspergillus mycotoxin) - moldy peanuts and grains

activation of B-catenin and inactivation of p53 are most common early mutational events

248
Q

clinical features of HCC

A

clinically silent, most pt’s present with advanced dz –> upper abdominal pain, weight loss, signs of decompensated liver dz i.e jaundice, ascites

249
Q

laboratory findings of HCC

A

elevated serum alpha-fetoprotein in 50% of pt’s

250
Q

precursor lesions of HCC

A
  1. hepatocellular adenoma
  2. chronic liver disease (hepatocellular dysplasias - small cell change [more common] and large cell change)
  3. dysplastic nodules associated with cirrhosis
251
Q

epidemiology of HCC- fibrolamellar variant

A

85% occur under 35y/o
M = F
no association with HBV, HCV, cirrhosis

252
Q

prognosis of HCC- fibrolamellar variant

A

5yr survival rate: 30-75%

slow growing and surgically resectable frequently

253
Q

histology of HCC- fibrolamellar variant

A

fibrous stroma
tumor cells larger than normal liver cells
deeply eosinophilic coarsely granular cytoplasm
lots of mitochondria in cytoplasm

254
Q

cholangiocarcinomas arise from…

A

bile duct epithelium (a type of adenocarcinoma)

255
Q

Klatskin tumor

A

a perihilar cholangiocarcinoma i.e. at the bifurcation (makes up 60% of all cholangiocarcinomas)

5yr survival: 15%

256
Q

Risk factors for cholangiocarcinomas

A

primary sclerosing cholangitis
congenital biliary cystic diseases
thorotrast (radiographic contrast from 1930’s)
parasites (liver fluke- Opisthorchis and Clonorchis)
NOT CIRRHOSIS!

257
Q

prognosis of cholangiocarcinomas

A
  • undergoes hematogenous metastasis (~50%)
  • often undergoes lymphovascular invasion and perineurial invasion
  • lethal, median survival ~6mths
258
Q

cholangiocarcinomas can look like…

A

metastatic carcinoma from breast of pancreas

259
Q

premalignant lesions for cholangiocarcinomas

A

biliary intraepithelial neoplasia (low to high grade)

260
Q

cholangiocarcinomas often produce…

A

mucin

261
Q

epidemiology of angiosarcomas of the liver

A

older pt’s 60-70y/o
rare (10-20 cases/yr in US)
most common primary sarcoma of the liver

262
Q

origin of angiosarcomas

A

vasculature

263
Q

Angiosarcomas can be associated with

A
  • vinyl chloride monomer
  • thorotrast (radiographic contrast from 1930’s)
  • arsenic
  • anabolic steroids
264
Q

metastatic tumors

A

most common tumors in the non-cirrhotic liver

uncommon in the cirrhotic liver

265
Q

most common sites of metastatic tumors to the liver

A

lung
breast
colon
pancreas

266
Q

markers for colon CA

A

CK7, CK20

CK = cytokeratin

267
Q

clinical features of congenital hepatic fibrosis

A

complications of portal HTN i.e. splenomegaly and esophageal varices

(rarely see hepatic cystic lesions)

268
Q

histology of congenital hepatic fibrosis

A

numerous residual biliary channels with widely patent lumens arranged around the periphery of the portal tract

269
Q

Reynold pentad

A

Charcot’s triad (RUQ pain, jaundice, fever)
Hypotension
Sensorium/altered mentation
Sign of severe ascending cholangitis

270
Q

markers for cholangiocarcinoma

A

CEA and/or CA 19-9

271
Q

CCK EF <35% is diagnostic of

A

acalculous chronic cholecystitis

272
Q

Serum-ascitic albumin gradient (SAAG) and Ascites total protein

  • Cirrhosis
  • Malignancy
  • Cardiac disease
A
  • Cirrhosis: high SAAG, low ascites total protein
  • Malignancy: low SAAG, high ascites total protein
  • Cardiac disease: high SAAG, high ascites total protein
273
Q

Porcelain gallbladder

A

calcified gallbladder d/t chronic cholecystitis, usually found incidentally on imaging.

Tx: prophylactic cholecystectomy d/t high rates of gallbladder carcinoma

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