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GI II Exam 2 > Chapter 18: Diseases, Disorders, and Neoplasms of the Liver and Gallbladder > Flashcards

Chapter 18: Diseases, Disorders, and Neoplasms of the Liver and Gallbladder Flashcards

1
Q

What tests can we measure for:

  1. Hepatocyte integrity (3)
  2. Biliary excretory function (3)
  3. Damage to bile canaliculus (2)
  4. Hepatocyte synthetic function (3)
A
  1. AST, ALT, LDH
  2. serum bilirubin, urine bilirubin, serum bile salts
  3. serum ALP, serum GGT
  4. serum albumin, coagulation factors, serum ammonia
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2
Q

What is the difference between Hepatocyte Necrosis vs Hepatocyte Apoptosis?

A

N: cells swell and rupture due to fluid when osmotic regulation is interrupted

  • MACROPHAGES at site of injury (Acute inflamm.)
  • death due to ischemia/hypoxic injury

A: cell shrinkage, pyknosis, karyorrhexis, acidophilic bodies (stain eosinophilically)
- NO acute inflammation

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3
Q

What is the difference between Confluent and Bridging necrosis in the liver?

A

C: widespread parenchymal loss; severe zonal loss

  • many begin as dropout around CENTRAL VEIN
  • space filled w/debris, macrophages

B: zone links central veins to portal tracts or bridges portal tracts

  • large areas of contiguous hepatocyte death
  • cirrhosis may result
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4
Q

How does scar formation occur in the Liver?

A
  • principal cell type is fat-containing, myofibroblastic HEPATIC STELLATE CELLs (normally store lipid/Vit A)
  • converts to highly fibrogenic myofibroblast upon injury (inc. PDGFRbeta)
  • Kupffer cells and lymphocytes release TGFb and MMP-2; contraction by endothelin-1

reversible if injurious agent is eliminated

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5
Q

Acute Liver Failure

What is it, what two problems is it associated with, what does it look like, and how does it present clinically?

A
  • occurs within 26 weeks of initial injury (80-90% loss of functional capacity of liver is lost)

A: encephalopathy and coagulopathy

M: massive hepatic necrosis (broad parenchymal loss around regenerating hepatocytes), diffuse microvesicular steatosis

C: nausea, vomiting, jaundice, itching w/slight inc. in liver transaminases, hepatomegaly

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6
Q

What are the A (3), B, C, D (2), E (2), and F (3) causes of Acute Liver Failure?

A

A: ACETAMINOPHEN (MC), Hepatitis A, autoimmune
B: Hepatitis B
C: Hepatitis C
D: Hepatitis D, drugs/toxins (alcohol)
E: Hepatitis E, esoteric (Wilsons/Budd-Chiari)
F: Fatty change (Fatty Liver Pregnancy, tetracycline)

acetaminophen, Hep A/B are most likely causes

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7
Q

Acute Liver Failure

What 7 things it can lead to? (J/C/HE/C/DIC/PH/HS)

A
  • Jaundice/Icterus
  • Cholestasis: retention of bile eliminated things
  • Hepatic Encephalopathy: inc. serum ammonium
    • Asterixis - rapid extension/flexion of hand
  • Coagulopathy: lack of Factors VII, IX, X, II (1972)
  • Disseminated Intravascular Coagulation
  • Portal Hypertension: ascites, encephalopathy
  • Hepatorenal Syndrome: renal failure
    • kidneys normal otherwise
    • hypouria, inc. serum BUN/creatinine
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8
Q

What 4 things commonly cause Chronic Liver Failure, what condition is it associated with, and what does that condition look like on biopsy?

A

C: Chronic Hepatitis B/C, Alcoholic Fatty Liver Disease, Non-Alcoholic Fatty Liver Disease (NAFLD)

  • associated with CIRRHOSIS (diffuse transformation into regenerative nodules surrounded by fibrous bands and degrees of vascular shunting)
    • see blue fibrous tissue streaks around nodules
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9
Q

What are the 3 classes of the Child-Pugh classification of cirrhosis?

A
  • helps monitor decline of pt. on the path to chronic liver disease

Class A: well compensated, less points/more life
Class B: partially decompensated
Class C: decompensated, more points/less life

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10
Q

What are 4 conditions seen in males due to hyper-estrogenemia during cirrhosis? (PE/SA/H/G)

A
  • due to impaired metabolism

- palmar erythema, spider angiomata, hypogonadism, gynecomastia

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11
Q

What are causes of Prehepatic (2), Posthepatic (3), and Intrahepatic (4) Portal Hypertension?

A
  • all are complications of Chronic Liver Failure

Pre: Obstructive thrombosis, massive splenomegaly
Post: right heart failure, constrictive pericarditis, hepatic vein outflow obstruction

Intra: CIRRHOSIS, shistomiasis, fatty change, sarcoidosis
- caused by inc. resistance to flow in SINUSOIDS

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12
Q

What are 4 clinical consequences of Portal Hypertension? (A/PS/CS/HE)

A

ascites, portosystemic shunt formation, congestive splenomegaly, hepatic encephalopathy

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13
Q

What is Ascites, what is it composed of, and how does it develop?

A
  • excess fluid in the peritoneal cavity typically caused by cirrhosis (85%); usually see hydro-thorax on RIGHT
  • fluid is serous (< 3g/dL of albumin)
  • hepatic sinusoidal HTN drives fluid into Space of Disse which gets drained by lymphatics; thoracic duct is unable to keep up with amount of fluid, so it leaks out causing peripheral interstitial edema
  • also inc. splanchnic vasodilation
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14
Q

What are portosystemic shunts and what are 3 examples of it? (EV/CM/H)

A
  • reversed flow through portal into systemic circulation where there are shared capillary beds

Ex: esophageal varices (40% w/cirrhosis, 30% mortality), caput medusa (umbilicus to rib margin dilations), hemorrhoids (rectum)

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15
Q

Hepatitis A Virus

What is it, how is it spread, and how is it seen clinically?

A
  • ssRNA virus (picornovirus) that is benign and does not cause chronic hepatitis or carrier state
  • spread fecal-orally (water) in endemic areas or by raw shellfish in developed countries

C: anti-HAV IgM seen in serum w/symptoms; IgG appears as IgM declines; rash/arthralgia/immune complex

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16
Q

Hepatitis B Virus

What is it, how is it spread, how does it present clinically, and what a diagnostic hallmark found on liver biopsy?

A
  • partial dsDNA virus with a high prevalence of Africa/Asia, usually transmitted in childbirth, but also by horizontal transmission
  • age at time of infection is BEST predictor chronicity (younger age = inc. probability; precursor to HCC)
    • CD8 cells attack infected hepatocytes
    • goal of chronic inf. = slow progression (5-10%)

DH: finely granular, “ground-glass” hepatocytes packed with HbsAg (swollen endoplasmic reticulum)

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17
Q

Hepatitis B Virus

What do the serum markers tell us about infection?

HBs-Ag, HBs-Ab, HBe-Ag, HBe-Ab, HBc-Ab

A

HBs-Ag (surface): seen BEFORE symptoms, last for 12 weeks (donated blood gets screened for this)

anti-HBs-Ab: no rise till disease over (IgG confers immunity); rises when HBs-Ag goes away

HBe-Ag (envelope): HBV-DNA, DNA polymerase; indicates active viral infection, appears AFTER HBs-Ag
- persistent = possible chronic infection

HBe-Ab: acute infection has peaked and is waning

HBc-Ab: core protein just before symptom onset and shows w/inc. aminotransferase levels

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18
Q

Hepatitis C Virus

What is it, how does it present clinically, and what does chronic infection lead to?

A
  • ssRNA virus (HCV IgG Abs do NOT confer immunity); milder than HBV but 80-90% develop chronic infection/20% cirrhosis (MCC of chronic hepatitis)

C: repeated hepatic damage (rarely causes acute hepatitis), “waxing/waning” aminotransferase lvls, cryoglobulinemia; diagnosis w/HCV-RNA in blood
- associated with Metabolic Syndrome

  • chronically leads to Lymphoid Aggregates or fully-formed lymphoid follicles; causes 1/3 of liver cancer
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19
Q

Hepatitis D Virus

What is it, how does it infect (2), who is it seen in in Western Countries, and what is the most reliable indicator of exposure?

A
  • RNA virus dependent on HBV infection (uses HBsAg coat Ag surrounding delta antigen); HBV vaccine also treats HDV
  • either Co-infects (HBV must be established first; acute hepatitis; inc. risk of failure in IV drug users) OR Superinfection (chronic HBV w/new HDV infection; disease in 30-50 days later, severe acute hepatitis)

WC: IV drug users or multiple blood transfusions

RI: IgM anti-HDV most reliable indicator of exposure

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20
Q

Hepatitis E Virus

How is it transmitted, what population does it cause sporadic acute hepatitis in, and what population has the highest mortality rate due to infection?

A
  • enterically transmitted, water-borne infection in young adults (inc. risk w/monkey, cat, pig, dog exposure)
  • 30% sporadic acute hepatitis in INDIA
  • highest mortality among PREGNANT WOMEN
    • no chronic stage; virions are shed in stool
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21
Q

What Hepatitis Viruses:

  1. Do NOT cause chronic hepatitis (2)
  2. Cause FULMINANT (ALF) hepatitis (3)
  3. Responsible for MOST chronic hepatitis (2)
A
  1. HAV and HEV
  2. HAV. HBV, HDV
  3. HBV and HVC (notorious for chronic infection)
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22
Q

What is the difference between Acute, Severe Acute, and Chronic Viral Hepatitis morphology?

A

A: mononuclear infiltrate, spotty necrosis scattered throughout lobule, lack of portal inflammation

SA: confluent necrosis of hepatocytes around central veins; central-portal bridging necrosis = parenchymal collapse

C: mononuclear portal infiltration w/fibrosis; scarring is hallmark of progressive disease (fibrous septum formation); “ground glass” w/HBV (brown stain) or lymphoid aggregates w/HCV

Chronic hepatitis = Peacemeal Necrosis/Interface Hepatitis

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23
Q

What is the 2nd most common cause of liver cirrhosis and why do you NOT biopsy liver abscesses caused by Echinococcus?

A
  • 2nd most common cause = Shistosomiasis

- do NOT biopsy because you risk possibility of infection of the peritoneal cavity if Echinococcus gets out of abscess

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24
Q

Autoimmune Hepatitis

What are the 3 alleles seen in pts., what is the difference between Type 1 and Type 2, and what is seen on biopsy?

A

A: South American (DRB1), Caucasian (DR3 - white F MC), Japanese (DR4)

  • early: severe parenchymal destruction; scarring
  • indolent: leads to liver failure; little scarring

Type 1: middle aged; ANA, ASMA, anti-SLA/LP, AMA
Type 2: child/teens; anti-LKM1 against CYP2D6, ACL1

Biopsy: plasma cells are characteristic component of inflammatory infiltrate

both Type 1 and Type 2 are likely to lead to liver failure if untreated; prognosis better in ADULTS

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25
Q

Liver Drug/Toxin Damage

How do acetaminophen, chlorpromazine, and halothane cause damage?

What is the most common hepatotoxin causing acute liver failure and chronic liver disease?

A

Acetaminophen: MC cause of Acute Liver Failure

  • metabolite from CYP450 breakdown in Zone 3
  • AHF when injury gets to Zone 1

C: cholestasis in pts. slow to metabolize it
H: fatal immune-mediated hepatitis - multiple exposure

Alcohol = most common cause of chronic disease

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26
Q

What are the 3 forms of Alcoholic Liver Disease? (HS/AH/AS)

A
  1. Hepatic Steatosis (fatty liver)
  2. Alcoholic Steato-hepatitis
  3. Alcoholic Steatofibrosis
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27
Q

What is Hepatic Steatosis (Fatty Liver)?

A
  • microvesicular lipid droplets in hepatocytes that accumulate over time, causing a large/greasy/yellow liver (change is REVERSIBLE if pt. abstains from EtOH)
  • due to impaired lipoprotein assembly/secretion
  • causes hepatomegaly, mild inc. in bilirubin and ALP lvls; severe dysfunction rare
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28
Q

What is Alcoholic Steato-hepatitis?

What is a finding that is really only found in this condition?

A
  • hepatocyte swelling and necrosis w/Mallory-Dank bodies (damaged intermediate filaments that are eosinophilic on staining); see neutrophilic rxn
  • due to acetaldehyde-induce lipid peroxidation and protein adduct formation; induced CYP450 inc. conversion of agents –> toxic metabolites
    • dec. hepatic sinusoid perfusion
  • AST:ALT = 2:1 lvls; inc. bilirubin, ALP; neutrophilic leukocytosis
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29
Q

What is Alcoholic Steatofibrosis?

A
  • activation of stellate cells and portal fibroblasts leading to fibrosis (start with sclerosis of Zone 3); scars in a “chicken-wire” pattern (Laennec cirrhosis)
  • due to chronic disorder of steatosis, hepatitis, fibrosis, deranged perfusion
  • causes hepatic dysfunction, anemia, usually irreversible; large, brown, nonfatty liver
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30
Q

What are 5 possible causes of death associated with end-stage alcoholic liver disease? (HC/GIH/I/HRS/HCC)

A
  • hepatic coma
  • GI hemorrhage (esophageal varices)
  • infection
  • hepatorenal syndrome
  • hepatocellular carcinoma
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31
Q

What are the 4 types of Metabolic Liver Disease? (N/H/W/A)

A
  1. Nonalcoholic Fatty Liver Disease
  2. Hemochromatosis
  3. Wilson Disease
  4. alpha-1 Antitrypsin Deficiency

2-4 are all INHERITED disorders

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32
Q

Nonalcoholic Fatty Liver Disease

What is it the most common cause of, what are risk factors of disease, what is its “Two-Hit” model, and what condition can it lead to?

A
  • MOST COMMON cause of chronic liver disease in US (seen in pts. that consume < 20g alcohol/week)

RF: inc. obesity, metabolic syndrome, Hispanic
- inc. risk of hepatocellular carcinoma

TH: insulin resistance = hepatic steatosis, oxidative injury leads to cell necrosis and inflammation

  • can lead to Nonalcoholic Steatohepatitis (involvement of > 5% of hepatocytes); symptoms overlap with alcoholic steatosis (Chicken-Wire pattern around central vein; see on TRICHROME stain); Mallory-Dank LESS common
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33
Q

Hemochromatosis

What is it and what is the difference between Hereditary and Secondary forms?

A
  • excessive iron absorption deposited in parenchymal organs (liver/pancreas/heart)

H: slow progression (lifelong accumulation); 4-5th decade males (symptoms when 20g accumulated)

  • caused by mutation in HFE gene
  • intestinal absorption is ABNORMAL

S: usually due to ineffective erythropoiesis; also transfusions or chronic liver disease (dec. hepcidin)

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34
Q

Hemochromatosis

What is the importance of Hepcidin and what is the most common form of adult hemochromatosis?

A

Hepcidin = main regulator of iron absorption (lowers plasma lvls); binds ferroportin and prevents iron from leaving intestinal cells

Adult Form: due to HFE mutation (C282Y) = inactivation of HFE = inactivation of hepcidin

  • usually caucasians, low penetrance (40 yo M)
  • HJV/HAMP = juvenile hemochromatosis (severe)
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35
Q

Hemochromatosis

What is used to visualize iron accumulations and how does disease affect the Liver, Pancreas, Heart, and Skin?

A
  • use Prussin Blue Stain to see hemosiderin deposits

L: golden-yellow hemosiderin, septae slowly develop
- small, micronodular cirrhosis
- late stages = dark brown-black
P: intense pigmentation, hemosiderin in islet/acinar
- deranged glucose homeostasis or DM
H: enlarged hemosiderin granules
- BROWN COLOR
S: inc. melanin production = gray-slate color

BRONZE DIABETES

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36
Q

Hemochromatosis

What is the common tetrad it presents with, what is it at inc. risk of developing, how is it diagnosed (3), and how can it be treated?

A

T: hepatomegaly, abnormal skin pigment, DM, deranged glucose homeostasis, cardiac dysfunction

  • hypogonadism in men can be presenting symptom
  • also atypical arthritis

Risk: 200x risk of hepatocellular carcinoma

Dx: Prussian Blue stain for iron, screen family members, look for high serum iron/ferritin

Tx: regular phlebotomy (blood-letting) depletes tissue stores = normal life expectancy

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37
Q

What is Neonatal Hemochromatosis?

A
  • sever liver disease and extrahepatic hemosiderin deposition of unknown origin (NOT inherited)
  • liver injury in-utero; may be due to maternal alloimmune injury to fetal liver
  • only supportive care and possible transplant
38
Q

Wilson Disease

What is it, what is it caused by, and how does it look like morphologically (MDB/BGA/KFR)?

What 3 locations is toxic accumulation most likely to occur?

A
  • AR disorder due to ATP7B mutation causing impaired copper excretion into bile and failure to incorporate copper into ceruloplasmin
  • copper is absorbed/delivered to liver normally but cannot be excreted/used = inc. free radical formation = toxic liver damage, hemolytic anemia, systemic pathology

M: acute/chronic hepatitis w/Mallory-Dank bodies, Basal Ganglia atrophy, Kayser-Fleischer rings in Descemet’s membrane

accumulates in LIVER, BRAIN, and EYE

39
Q

Wilson Disease

How does it present clinically (4) and how is it diagnosed?

A

C: elevated urine copper, low plasma ceruloplasmin, acute/chronic liver disease between 6-40 yrs

  • Parkinsonian movements (basal ganglia)
  • hemolytic anemia = elevated INDIRECT bilirubin

Dx: dec. serum ceruloplasmin, inc. hepatic copper (MOST SENSITIVE - > 250 ug), inc. urinary copper (MOST SPECIFIC TEST)

  • use long-term chelation/zinc-based therapy, liver transplant is curative
40
Q

a1-Antitrypsin Deficiency

What is it, what two things does it lead to, what does it look like, and how does it present clinically?

A
  • AR disorder of protein folding; PiZZ homo has 10% of circulating a1-antitrypsin lvls (early liver disease; accumulation of a1AT-z)
  • typically inhibits proteases; leads to pulmonary emphysema (destructive proteases) and hepatic disease (accumulation of misfolded proteins)

M: round/oval globular inclusions in hepatocytes that are acidophilic and PAS (+)

C: neonatal hepatitis and cholestatic jaundice; hepatitis/cirrhosis/pulmonary disease in adolescence
- 2-3% chance of HCC in PiZZ adults

41
Q

a-1 Antitrypsin Deficiency

What are 3 proteases that antitrypsin typically inhibits (N/C/P), what is the point mutation involved, and what is it the most common disorder of?

A

P: neutrophil elastase, cathepsin G, proteinase 3

PM: Glu 342 –> Lys 342

  • most commonly diagnosed inherited hepatic disorder in INFANTS and CHILDREN
42
Q

What are the two main function of bile, what is the most common type of bilirubin in the serum, and what is Neonatal Jaundice?

A

F: emulsify dietary fat in lumen of gut and elimination of bilirubin, cholesterol, xenobiotics, waste that are NOT water soluble (urine)

MC: UNCONJUGATED (complexed to albumin)
- bilirubin is toxic end-product of heme degradation

NJ: conjugate/excrete bilirubin occurs around 2 weeks of age, so it is normal for newborns to have transient phases of jaundice early in life

43
Q

What is the difference between:

Crigler-Najjar Syndrome Type 1
Crigler-Najjar Syndrome Type 2
Gilbert Syndrome

Dubin-Johnson Syndrome
Rotor Syndrome

What type of hyperbilirubinemia is seen in each?

A

CN1: AR, no UGT1A1 activity, FATAL w/kernicterus
CN2: AD, dec. UGT1A1 activity, occasional kernicterus
GS: AR, dec. UGT1A1 but works well enough not to see
- all 3 have UNCONJUGATED HYPERBILIRUBINEMIA

DJS: AR mut. MDR protein 2, black liver/pigment globules
RS: AR, NO black liver
- both have CONJUGATED HYPERBILIRUBINEMIA

44
Q

Cholestasis

What is it, what does it look like, what labs are associated with it, and how is it treated?

A
  • impaired bile formation/flow causing accumulation of bile pigment in hepatic parenchyma (HALLMARK)

M: green-brown plugs (feathery degeneration of periportal hepatocytes); periportal Mallory-Dank body

L: elevated GGT/ALP, AST/ALT normal

T: surgery

45
Q

Large Bile Duct Obstruction

What are 3 common causes in adults (S/M/S) and 4 common causes in kids (BA/CF/CC/S)

A

A: extrahepatic cholelithiasis (stones), biliary tree/pancreatic head malignancy, strictures (surgery)

K: biliary atresia, cystic fibrosis, choledochal cysts, syndrome of insufficient intrahepatic bile ducts

46
Q

What is Ascending Cholangitis?

What two bugs is it normally caused by, what triad is associated with it, and what is its histological hallmark?

A
  • 2nd bacterial infection of biliary tree that aggravates inflammatory injury

C: coliforms/enterococci (enteric bugs)

T: Charcot’s Triad = fever, RUQ pain, jaundice

HH: periductular neutrophils directly into bile duct epithelium and lumen

can lead to suppurative cholangitis

47
Q

What is Suppurative Cholangitis?

A
  • caused by Ascending Cholangitis
  • purulent bile fills/distends bile ducts
  • frequently complicated by SEPSIS
48
Q

What is Cholestasis of Sepsis?

What is its most common form?

A
  • circulating microbial products cause infection of biliary tree when obstructed (abscesses and bacterial cholangitis)

MC: canalicular cholestasis = bile plugs in centrilobular canaliculi and is associated w/Kupffer cells/portal inflammation (no hepatocyte necrosis)

49
Q

What is Primary Hepatolithiasis?

Where is it frequently seen in and what is it at inc. risk of developing?

A
  • intrahepatic gallstone formation leading to repeated bouts of ascending cholangitis, progressive destruction of parenchyma, and inc. risk of biliary neoplasia (seen frequently in EAST ASIA)
  • pigmented calcium bilirubinate stones in distended intrahepatic bile ducts
  • inc. risk of cholangiocarcinoma
50
Q

What is Neonatal Hepatitis?

What is the most common cause?

A
  • neonatal cholestasis = prolonged conjugated hyperbilirubinemia in neonate AFTER 2 weeks
  • liver injury, dark urine, light/acholic stool, hepatomegaly

M: panlobular giant-cell transformation of hepatocytes, cholestasis, extramedullary hematopoiesis

MCC is Biliary Atresia

51
Q

Biliary Atresia

What is it, what is the difference between Fetal and Perinatal forms, how does it present clinically, and how can it be treated (Type 1/2/3)?

A
  • complete/partial obstruction of extrahepatic biliary tree lumen in the first 3 months of life; see inflammatory/fibrosing structure of hepatic/common bile duct

F: aberrant intrauterine development of extrahepatic biliary tree
P: MOST COMMON, destruction of tree after birth

C: neonatal cholestasis, acholic stool change, jaundice after 2 weeks (cirrhosis in 3-6 mo, then death by 2 yo)

T: Kasai procedure (Type 1 and 2), liver transplant (Type 3 = above porta hepatis; no patent bile ducts available)

52
Q

What are 2 forms of Autoimmune Cholangiopathies? (PBC/PSC)

A
  1. Primary Biliary Cirrhosis

2. Primary Sclerosing Cholangitis

53
Q

Primary Biliary Cirrhosis (PBC)

What is it, who is at risk, what does it look like morphologically, and how does it present clinically (AG/HP/X/S/O)

A
  • non-suppurative inflammatory destruction of small/medium-sized intrahepatic bile ducts (most do NOT go to cirrhosis); white middle-aged FEMALES

M: Abs to AMA and ANA (90% and 50%); Florid duct lesions (diagnose w/biopsy), feathery degeneration and ballooned, bile-stained hepatocytes w/Mallory-Dank bodies

C: inc. ALP/GGT; hyperpigmentation, xanthelasmas, steatorrhea, osteomalacia/osteoporosis (dec. Vit D)

**inc. risk of hepatocellular carcinoma

54
Q

Primary Sclerosing Cholangitis (PSC)

What is it, who is at risk, what does it look like morphologically, and how does it present clinically?

A
  • inflamm/fibrosis of intrahepatic AND extrahepatic bile ducts w/dilation of normal segments (BEADING); 20-40 yo MALES with IBD (typically UC)

M: ANCAs Abs in 65% of pts, “onion-skinning” fibrosis of small ducts that is obliterated by tombstone scar

  • can become primary biliary cirrhosis
  • diagnosis by radiography

C: persistent ALP elevation, acute bouts of ascending cholangitis; some fatigue, pruritus, jaundice

55
Q

What are Choledochal Cysts and Fibropolycystic Disease?

What are the pathological findings of Fibropolycystic Disease (VMC/CD/CHF)

A

CC: congenital dilations of common bile duct

  • present before 10 yo; 3-4x more in FEMALES
  • predispose: strictures, stones, stenosis

FPD: congenital malformations of the biliary tree (ductal plate malformations) OF LIVER

  • Von Meyenburg complexes
  • Caroli disease, congenital hepatic fibrosis
  • inc. risk of cholangiocarcinoma
56
Q

Fibropolycystic Disease

What are Von Meyenburg complexes, Caroli Disease, and Congenital Hepatic Fibrosis?

A

VMC: small bile duct hamartomas (indicate FPD if diffuse)
- misshaped bile duct

CD: multifocal cystic dilations of large intrahepatic bile ducts; occur with congenital hepatic fibrosis

CHF: portal tracts enlarged by irregular broad bands of collagenous tissue forming septae that divide liver into irregular islands (may have portal hypertension)

57
Q

What are two common causes of:

Impaired Blood INFLOW to the Liver (PVO/IET)
Impaired INTRAHEPATIC Blood Flow (C/SO)
Hepatic Vein OUTFLOW Obstruction (BCS/SOS)

A
  1. portal vein obstruction and intra/extrahepatic thrombosis
  2. CIRRHOSIS and sinusoidal occlusion
  3. Budd-Chiari Syndrome (Hepatic Vein Thrombosis) and Sinusoidal Obstructive Syndrome
58
Q

What are the most common causes of intrahepatic blood flow obstruction and small portal vein branch obstructions?

A

CIRRHOSIS - MCC of intrahepatic blood flow obstruction

SCHISTOSOMIASIS - MCC of small portal vein branch obstructions

59
Q

What is Peliosis hepatis?

A
  • sinusoidal dilation that occurs in any condition in which efflux of hepatic blood is impeded
  • creates blood-filled cystic spaces either unlined or lined with sinusoidal endothelial cells
60
Q

Budd-Chiari Syndrome

What is it, what triad does it present with (H/P/A), and what is its morphology (CC/N/SL)

A
  • obstruction of two+ major hepatic veins that lead to hepatomegaly, pain, ascites (HIGH MORTALITY)

M: swollen red-purple liver w/tense capsule, severe centrilobular congestion and necrosis, fibrosis if slow developing

T: surgery

61
Q

Sinusoidal Obstruction Syndrome (Veno-occlusive Disease)

What is it, what is its morphology (CC/HN/H/OL), and how does it present clinically (H/A/WG/J)?

What drink is it associated with?

A
  • obliteration of the terminal hepatic venules by subendothelial swelling and collagen deposition
    • caused by toxic injury, JAMACIAN BUSH TEA
    • HIGH MORTALITY

M: centrilobular congestion, hepatocellular necrosis, inc. hemosiderin macrophages; obliteration of lumen of the venule

C: tender hepatomegaly, ascites, weight gain, jaundice
- HISTOLOGY is GOLD STANDARD

62
Q

When is damage to the liver by GVH and HVG disease most commonly seen?

A

GVH - commonly after BONE MARROW transplant

HVG - commonly after liver transplant

63
Q

What is the difference between Acute and Chronic Graft vs Host Disease?

A

A: 10-50 days

  • donor lymphocytes to epithelial cells of liver
  • hepatitis w/necrosis, portal tract inflammation

C: 100+ days

  • portal tract inflammation, selective bile duct destruction
  • eventual fibrosis
64
Q

What is the difference between Acute and Chronic Transplant Rejection?

A

A: cellular

  • infiltration of mixed portal inflammatory infiltrate
  • bile duct injury and endothelitis

C: vascular (“vanishing bile duct sydrome”)

  • obliterative arteriopathy of small/large arteries
  • ischemic changes to parenchyma
65
Q

What is the most common cause of jaundice in pregnancy?

A

VIRAL HEPATITIS

66
Q

What is Preeclampsia and Eclampsia?

What is HELLP Syndrome?

A

PE: maternal HTN, proteinuria, peripheral edema

  • becomes eclampsia with hyperreflexia/convulsions
  • acute fatty liver = FATAL
  • eclampsia has subcapsular hemorrhage (DARK)

HELLP = subclinical manifestation of preeclampsia
- hemolysis, elevated liver enzymes, low platelets

  • periportal sinusoids have fibrin deposits associated w/hemorrhage into the Space of Disse w/necrosis
    • modest/severe inc. in AST/ALT
    • mild serum bilirubin elevation
67
Q

What is the difference between Acute Fatty Liver of Pregnancy and Intrahepatic Cholestasis of Pregnancy?

A

AFLP: present in 3rd trimester (24-26 wks)

  • inc. ALT/AST lvls; hepatic dysfunction
  • diffuse microvesicular steatosis of hepatocytes
  • dangerous; treat with termination of pregnancy

ICP: pruritus followed by dark urine, acholic stool, jaundice in 3rd trimester

  • slight bilirubin/ALP inc; bile salts GREATLY inc.
  • generally benign condition to mother
  • stillbirth, fetal distress, prematurity increased
68
Q

What is the difference between Focal Nodular Hyperplasia (FNH) and Nodular Regenerative Hyperplasia (NRH)?

A

FNH: single, well-demarcated lesion w/central scar

  • gray-white central scar (biopsy to rule out cancer)
  • young-middle age, liver is normal
  • scar with fibrous septa that radiate outwards

NRH: multiple nodules, look like cirrhosis but no septa

  • entire liver is transformed into nodules
  • can lead to PORTAL HYPERTENSION
  • plump hepatocytes with atrophic hepatocytes
  • usually found at autopsy, asymptomatic
69
Q

What are the two types of benign liver neoplasms? (CH/HA)

A
  1. Cavernous Hemangiomas

2. Hepatocellular Adenomas

70
Q

What is a Cavernous Hemangioma?

A
  • MOST COMMON benign liver tumor (blood vessels)
    • red/blue nodules directly under capsule
  • more common in FEMALES and are found incidentally or after hemorrhage
  • can be mistaken for metastatic tumors
71
Q

What is a Hepatocellular Adenoma?

What are its 3 types? (HA/BA/IA)

A
  • benign neoplasm from hepatocytes that can lead to rupture (bleeding that is surgical emergency)
  • more common in FEMALES (males w/anabolic steroid use) and is strongly associated with oral contraceptives (unknown before advent of OCs)
  • HNF1-a inactivated adenomas, B-Catenin activated adenomas, Inflammatory adenomas
72
Q

What is the difference between:

HNF1-a inactivated Hepatocellular Adenoma
B-Catenin activated Hepatocellular Adenoma
Inflammatory Adenoma

A

H: fatty with NO atypia, no risk of malignancy (F > M)

  • MOST COMMON form; MODY-3
  • NO staining with LFA binding protein

B: use of oral contraceptives/anabolic steroids (M > F)

  • VERY HIGH risk of malignant transformation
  • high degree of dysplasia
  • look for glutamine synthase and B-catenin

I: associated with NAFLD (F > M)

  • 2nd MOST COMMON form
  • small but definite risk of malignant transformation
  • overexpress CRP and serum Amyloid A
73
Q

What are the 3 main types of Malignant Liver Tumors? (HB/HCC/CCA)

A
  1. Hepatoblastoma
  2. Hepatocellular Carcinoma
  3. Cholangiocarcinoma
74
Q

Hepatoblastoma

What is it, what is the difference between Epithelial and Mixed Types, and what pathway does it frequently activate?

A
  • MOST COMMON liver tumor of early childhood (< 3 yo);

E: small polygonal fetal cells/small embryonal cells (vaguely look like liver development)

M: foci of mesenchymal differentiation of osteoid, cartilage, or striated muscle

  • frequently activates WNT pathway (APC mutation)
    • pts. w/FAP, Beckwith-Wiedeman Syndrome
75
Q

Hepatocellular Carcinoma

What is it the most common primary malignancy of, what are the two most common mutation events and what are they associated with (AT), and what are the two precursor lesions?

A
  • MOST COMMON primary malignancy of hepatocytes

ME: B-catenin activation/p53 inactivation
- AFLATOXIN (aspergillus flavus and parasiticus)

PL: low-grade nodules (no atypia, high risk of becoming high-grade) and HIGH GRADE nodules (most important pathway for emergence in viral hepatitis and alcoholic liver disease)

76
Q

Hepatocellular Carcinoma

What is the difference between Large and Small cell change, what is its pattern of metastasis, and how does it present clinically?

A

Large: hepatocytes larger than normal w/pleomorphic nuclei around portal tracts; large septa
- nuclear-cytoplasmic ratio normal
Small: high nuclear-cytoplasmic ratio, form tiny nodules, directly pre-malignant

M: vascular route most likely route for extrahepatic metastasis; lung metastasis occurs late

C: liver dysfunction, inc. AFP, diagnostic imaging

  • death via cachexia, GI/variceal bleed, liver failure
  • death usually in first 2 years
77
Q

What is Fibrolamellar Carcinoma?

A
  • variant of HCC that occurs < 35 yo; no gender predilection or pre-disposing condition
  • presents as large, single scirrhous tumor w/fibrous bands coursing through it
  • composed of well-differentiated cells rich in mitochondria growing in nests/cords separated by lamellae of dense collagen fibers
78
Q

Cholangiocarcinoma

What is it, what are 2 common risk factors of it (LF/HCV), where does it most commonly occur (KT), and what is the difference between extrahepatic and intrahepatic morphology?

A
  • second most common primary malignant tumor after HCC; adenocarcinoma of the biliary tree

RF: LIVER FLUKES (opisthorchis, clonorchis) in SE Asia, and HCV

EH: perihilar tumors (KLASKIN) located at junction of hepatic ducts (most common), common bile duct
- firm gray nodules (small at diagnosis)

IH: track along intrahepatic portal tract and create branching tumors (only 10% of tumors)

79
Q

Cholangiocarcinoma

How does it present clinically and what is its prognosis?

A
  • median time to death is 6 mo for intrahepatic, but overall 15% 2 yr survival

IH: obstruction to bile flow or symptomatic liver mass
EH: biliary obstruction, cholangitis, RUQ pain

80
Q

What 3 things is Angiosarcoma historically associated with (VC/A/T) and what are the two most common forms of hepatic lymphomas?

A

A: vinyl chloride, arsenic, THOROTRAST

HL: diffuse large B Cell lymphoma and MALTomas
- middle-aged men, with HBV/HVC/HIV/PBC

81
Q

What 4 cancers commonly metastasize to the liver? (C/B/L/P)

A

Colon, Breast, Lung, Pancreatic cancer

metastases to the liver are MORE common than primary liver cancer

  • commonly clinically silent till late stage
82
Q

What is the most common congenital gallbladder anomaly?

A

Phrygian Cap (fundus that is folded inwards)

83
Q

What is Cholelithiasis and what are its risk factors?

A

GALLSTONES: more than 95% of biliary tract disease is attributable to cholelithiasis

  • either cholesterol or pigment stones

RF: family history, female, fat, fertile, forty, and fair (caucasian)
- metabolic exposure, native american, estrogen exposure

84
Q

Cholesterol Stones

What are they and how are they made, and what is their morphology?

A
  • collect when concentration inc. above capacity of the bile (precipitate out of bile); more common in the West
  • found ONLY in the gallbladder; pure stones look pale-yellow, finely granular, hard, w/crystalline pallisades
  • most are radiolucent due to large cholesterol content, but 10-20% are radiopaque due to Ca. carbonate

become more black and are more lamellated when mixed with other substances

85
Q

Pigment Stones

What are they and how are they made, and what is their morphology?

What 3 organisms can cause development of Pigment Stones? (E/AL/CS)

A
  • stones of unconjugated bilirubin and inorganic calcium salts (happens when elevated levels of unconjugated bilirubin in the bile)
  • black stones are found in sterile bile ducts (bilirubin/salts/mucin) w/50-75% radiopaque due to Ca salts; BROWN STONES are found in infected large bile ducts (soap-like, greasy, soft, lamellated)

Organisms: E. coli, Ascaris lumbricoides, liver fluke C. sinensis

86
Q

What are the clinical implications of Gallstones?

What is Bouveret Syndrome?

A
  • most patients are asymptomatic, but can have excruciating constant pain after fatty meals in RUQ (rule out MI)
  • large stones are less likely to enter ducts or cause obstruction, but smaller “gravel” stones are more dangerous
  • cause gallstone ileus/Bouveret Syndrome: large stone erodes directly into adjacent loop of SB and generates intestinal obstruction

**primary complication is acute cholecystitis –> inc. risk of gallbladder carcinoma)

87
Q

Acute Cholecysitis

What is the difference between Calculous and Acalculous, what is its morphology, and how does it present clinically?

A
  • RUQ/epigastric pain for 6 hours precipitated by obstruction of neck of gallbladder or cystic duct by stone

C: chemical irritation/inflammation by stone (90%)
A: in diabetic pts with NO stones
- results from Cystic Artery ischemia
- also from Primary Bacterial Infection (Salmon/Staph)

M: enlarged/tense gallbladder, may be green-black, bright red, or blotchy (fibrous serosal exudate)

C: 25% of pts. will need surgery, rest will recover in 10 days; inc. risk of gangrene and perforation w/acalculous cholecystitis

88
Q

What is Gallbladder Empyema and Acute Emphysematous Cholecystitis?

A

GE: when exudate is virtually pure puss

  • mild: thick wall, edematous, hyperemic
  • severe: wall green-black with gangrene

AEC: when gallbladder is invaded by gas-forming organisms (clostridia and coliforms)

89
Q

Chronic Cholecystitis

What is it, what are 4 morphological findings of disease (RAS/PG/XC/HG), and how does it present clinically?

A
  • chronic inflammation of the gallbladder that is due to stones 90% of time

Rokitansky-Aschoff sinuses: gallbladder diverticulum
Porcelain Gallbladder: calcifications of wall
Xanthogranulomatous Cholecystitis: very thick walls
- triggered by rupture of RA sinuses (foam cells)
Gallbladder Hydrops: dilated gallbladder with clear secretions

C: reoccurring acute symptoms with intolerance for fatty foods

90
Q

Carcinoma of the Gallbladder

What is it, who is at risk and what is the biggest risk factor, what does it look like, and what does it cause clinically?

A
  • MOST COMMON malignancy of extrahepatic biliary tract (adenocarcinoma; F 2x > M)
  • biggest RF is having gallstones (fundus cancer); 95% of cancer arises from gallstones, but only 1-2% develop

M: infiltrating (poorly defined of mural thickening, MORE COMMON) OR exophytic (grows into lumen, better prognosis); commonly SEED AREA around it

C: presenting symptoms are those of cholelithiasis, most cases found at surgery for gallbladder with stones

  • by now, most patients are advanced disease
  • 5 yr survival < 10% (VERY POOR PROGNOSIS)

GI II Exam 2 (8 decks)

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