Hepatobiliary Tract Flashcards

1
Q

Hepatic failure

A

80-90% function is lost
- most common cause is consequence of successive waves of injury or progressive chronic damage
Sx: jaundice, hypoalbuminemia, hyperammonemia, fetor hepaticus, hyperestrogenemia due to impaired estrogen metabolism
Complications: coagulopathy, multiple organ failure, hepatic encephalopathy which is lifethreatening disorder of CNS and neuromuscular transmission; porto-systemic shunting, loss of function, brain edema, consciousness, limb rigidity, hyperreflexia, asterixis; hepatorenal syndrome renal failure with decreased renal perfusion pressure and vasoconstriction; hepatopulmonary syndrome presenting with hypoxia caused with intrapulmonary vascular dilation

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2
Q

Acute liver failure

A

Liver illness associated with encephalopathy within 6 months of initial diagnosis
- caused by hepatic necrosis attributable to drug or toxin injury, viral hepatitis, autoimmune damage

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3
Q

Chronic liver disease

A

Most common cause of failure

- chronic hepatitis ending in cirrhosis

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4
Q

Budd-Chiari Syndrome

A

Acute thrombosis of major hepatic veins or inferior vena cava at the level of hepatic veins
Associated with: polycythemia vera, pregnancy, post-partum state, use of oral contraceptives, paroxysmal nocturnal hemoglobinuria, intra-abdominal cancers, including hepatic cancer
Sx: Acute abdominal pain (capsule can’t change fast enough with it), hepatomegaly, ascites
Path: acute and chronic passive congestion

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5
Q

Viral hepatitis causes

A

EBV, CMV and yellow fever or more commonly hep A, B, C, D, E
Morph: ground glass appearance for Hep B and C
- councilman body with acidic apoptosis acidophilic body
- damage around portal triad
- bridging necrosis with bridging fibrosis

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6
Q

Hep A virus

A

Single stranded RNA, benign self limited disease, only fulminant in pregnancy
Spread: fecal oral route
Path: antiHAV IgM in serum, IgG appears as IgM declines and persists for years

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7
Q

Hep B virus

A

Virus: circular partially double stranded DNA virus
- Dane particle with outer surface protein and lipid envelope encasing electron dense core
Disease: Subclinical nonprogressive chronic heptatitis, acute self limited hepatitis, progressive chronic disease culminating in cirrhosis, or fulminant hepatitis with massive liver necrosis, asymptomatic carrier state
Path: HBsAG appears before sx
Sx: anorexia, fever, jaundice

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8
Q

Hep C virus

A

Virus: single stranded RNA enveloped virus
Disease: cirrhosis common 20%, chronic disease in 80-85%
- carcinoma
- potentially curable

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9
Q

Hep D virus

A

Virus: defective RNA virus that can replicate and cause infection only with Hep B
- Dane like particle with HBV envelope
- delta antigen small and circular
Disease: mild to fulminant hepatitis, not chronic

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10
Q

Hep E virus

A

Spread: Fecal-oral
Virus: non-enveloped single stranded RNA virus
Disease: high rate of fulminant disease in pregnant women

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11
Q

Hep G virus

A

Nonpathogenic RNA virus

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12
Q

Hereditary hyperbilirubinemia - unconjugated hyperbilirubinemia (3)

A

Crigler-Najjar Syndrome Type I, Type II, and gilbert syndrome

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13
Q

Hereditary hyperbilirubinemia - conjugated hyperbilirubinemia (2)

A

Dubin-johnson syndrome and Rotor syndrome

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14
Q

Crigler-Najjar Type I

A

Inheritance: auto recessive
- absence of UGTIA1 causes jaundice leading to high unconjugated bilirubin
Morph: histologically normal liver
Sx: death and fatal neurologic damage without liver transplant

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15
Q

Crigler-Najjar Type II

A

Inheritance: auto dominant
- dysfunction of UGTIA1
Sx: not lethal

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16
Q

Gilbert Syndrome

A

Inheritance: auto rec
Path: 30% reduction in UGTIA1 with fluctuating unconjugated hyperbilirubinemia
- hyperbilirubinemia and jaundice may be exacerbated by infection, strenuous exercise or fasting

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17
Q

Dubin-Johnson Syndrome

A

Inheritance: auto rec
Path: defective hepatocyte secretion of bilirubin conjugates due to absent multidrug resistance protein 2 (MDR2)
- responsible for bilirubin gluronide transport
Morph: brown liver with pigment granules
Sx: jaundice but normal lifespan

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18
Q

Rotor syndrome

A

Inheritance: auto rec
Morph: defective hepatocellular bilirubin uptake or excretion
Morph: liver not pigmented
Sx: jaundice but normal lifespan

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19
Q

Hepatocellular necrosis causes

A

Mushroom poisoning, aflatoxin, phosphorus, carbon tetrachloride, chloroform, benzene, cytotoxic drugs, acetaminophen, salicylates, vit A, methyldopa, phenytoin, halothane, isoniazid
Morph: framework in place

20
Q

cholestasis

A

contraceptive and anabolic steroids; estrogen replacement therapy

21
Q

cholestatic hepatitis

A

numerous antibiotics; phenothiazines

22
Q

steatosis

A

ethanol, methotrexate, corticosteroids, total parenteral nutrition

23
Q

steatohepatitis

A

amiodarone, ethanol

24
Q

fibrosis and cirrhosis

A

methotrexate, isoniazid, enalapril

25
Granulomas
sulfonamides, phenbutyzone, etc
26
Vascular lesions
high dose chemotherapy, bush teas, oral contraceptives, numerous other agents, anabolic steroids, tamoxifen
27
Neoplasms
oral contraceptives, anabolic steroids, thorotrast, vinyl chloride
28
Alcoholic liver disease
Leading cause of liver pathology Morph: hepatic steatosis (fatty liver): microvesicular lipid droplets within hepatocytes with moderate or chronic alcohol intake --> chronic turns to macrovesicular lipid droplets displacing nucleus, liver enlarged, soft, greasy, yellow --> sx: hepatomegaly - alcoholic hepatitis: ballooning degeneration and hepatocyte necrosis with mallory body formation neutrophilic reaction --> sx: malaise, anorexia, tender hepatomegaly --> injury from acetaldehyde, induction of CYP450, impaired metabolism of methionine, alcohol caloric food source, alcohol mediated release of bacterial endotoxin - alcoholic cirrhosis: irreversible, fatty and enlarged to brown, shrunken, and nonfatty Path: women, AA
29
Cirrhosis
Diffuse process of fibrosis and nodular regeneration with variable degree of disorganization of the lobular architecture Cause: alcohol abuse, viral hepatitis, non-alcoholic steatohepatitis with biliary disease and hemochromatosis less frequent Morph: bridging fibrosis linking portal tracts, parenchymal nodules, disruption of hepatic parenchymal architecture Path: hepatocyte death, extracellular matrix deposition and vascular reorganization - collagen in space of disse - proliferation and activation of hepatic stellate cells - proinflammatory cytokines, cytokines, disruption of ECM, direct toxin stimulation Sx: anorexia, weight loss, weakness, debilitation - progressive liver failure, portal HTN, hepatocellular CA
30
Primary biliary cirrhosis
Strong evidence for autoimmune pathogenesis Assoc: ulcerative colitis, systemic sclerosis, RA Path: antibodies of anti-mito antibodies, anti-nuclear antibodies (ANA), anti-smooth muscle antibodies (SMA) Morph: dense portal tract inflammation with focal noncaseating granulomas with bile duct destruction and generalized cholestasis - obstruction leads to progressive secondary upstream damage, with ductular proliferation - at end stage, PBC is indistinguishable from other forms of cirrhosis Sx: pruritus, hepatomegaly, jaundice, xanthomas --> progression to cirrhosis, variceal bleeding and ecephalopathy
31
Primary sclerosing cholangitis
Chronic cholestatic disease distinguished by inflammation and obliterative fibrosis of extrahepatic and intrahepatic biliary tree - beading of contrast material - autoimmune Assoc: ulcerative colitis Morph: periductular inflammation and onion skin fibrosis with progressive atrophy Sx: weight loss, ascites, variceal bleeding, encephalopathy - increased incidence of chronic pancreatitis and hepatocellular carcinoma
32
Extrahepatic cholestasis
Bile duct obstruction by stone, stricture, carcinoma - congenital biliary atresia - acute and chronic pancreatitis Morph: centrilobular cholestasis, cytoplasmic and canalicular - edema and inflammation in portal triads - minimal hepatocellular alteration except for feathery degeneration adjacent to severe cholestasis - dilation and proliferation of bile ducts - bile thrombi in dilated ducts - extravasation of bile, bile lakes, bile infarcts - bridging portal fibrosis and cirrhosis - suppurative cholangitis and cholangiolitis
33
Portal hypertension
Splenomegaly with or without hypersplenism - development of porto-systemic venous anastamoses - ascites typically aggravated by hypoalbuminemia
34
Wilson disease
Inheritance: auto rec mutations in ATP7B gene coding canalicular copper transporting ATPase; copper absorption and delivery to liver is normal, copper excretion into bile reduced - copper accumulates in liver which results in hepatic injury through ROS generation Morph: liver damage mild to severe, fatty change, acute and chronic hepatitis with mallory bodies, cirrhosis, and massive necrosis - eye lesions called kayser fleischer rings involved (green-brown copper at corneal limbus) Sx: acute and chronic liver disease before age 40 - parkinsonism Dx: increased hepatic copper content and increased urinary excretion
35
Alpha1-antitrypsin deficiency
Inheritance: auto rec with low levels of protease inhibitor, emphysema and hepatic disease Path: synthesized by hepatocytes, most common genotype is PiMM, most common disease is PiZZ - glutamate to lysine - triggers autophagy, mito dysfunction and proinflammatory NF-kB causing damage Morph: PAS positive cytoplasmic globules in periportal hepatocytes - cholestasis to hepatitis to cirrhosis Sx: jaundice, acute hepatitis or cirrhosis - hepatocellular CA develops in PiZZ adults - smoking worsens lung damage
36
Focal nodular hyperplasia
- one or more nodules - central stellate fibrous scar - scattered bile ducts - normal hepatocytes - bleeding unusual Age: middle aged adults
37
Nodular regenerative hyperplasia
- nodular transformation of liver without fibrosis - consequence of intrahepatic blood flow - occurs in transplants or vasculitis
38
Cavernous hemangiomas
Most common benign liver tumors | - blood vessel tumor
39
Hepatic adenoma
Benign hepatocyte neoplasms - common in young women associatedw tih oral contraceptives - beta-catenin and HNFIalpha associated - sheets of hepatocytes containing arteries and veins - portal tracts with bile ducts are absent - remove because could hemorrhage or be carcinomatous - tendency to bleed
40
Hepatocellular CA
Most common primary liver cancer - associated with HBV infection Path: background of chronic liver disease - viral, chronic alcholism, NASH and food - less common: hemochromatosis, tyrosinemia, alpha1 antitrypsin deficiency Morph: solitary mass, multifocal nodules, diffusely infiltrative cancer with liver enlargement in cirrhosis - fibrolamellar CA single cirrhous hard tumor in 20-40 year olds without chronic liver disease Sx: RUQ pain, weight loss, elevated alpha-fetoprotein
41
Hepatoblastoma
Most common liver tumor of early childhood Morph: activation of Wnt/beta catenin pathway - associated with familial polyposis - epithelial type immitates liver - mixed has foci of mesenchymal differentiation including osteoid, cartilage, striated muscle
42
Cholangiocarcinoma
intra and extrahepatic biliary tree - majority perihilar Path: most without risk antecedent - associated with PSC, fibropoycystic lesions, HCV infection - opisthorchis sinensis Morph: single large mass or multifocal nodules - pale
43
Cholelithiasis
Substance: majority cholesterol stones, some calcium bilirubinate, some calcium carbonate Risk: increased hepatic cholesterol uptake or synthesis - native americans, industrialized countries, female, age, estrogen, obesity, hypercholesterolemia, rapid weight loss, GB stasis Path: cholesterol - bile supersaturated with cholesterol pigment - unconjugated bilirubin from hemolysis conditions --> also with opisthorchis sinensis, E coli, ascaris lumbricoides Morph: chole stones from GB --> hard pale and yellow bilistones --> black color or brown Sx: asymptomatic - spasmodic colicky pain from passing stones in bile ducts - infl leads to cholecystitis or cholangitis or gallstone ileus
44
Strawberry GB
fat, fertile, forty, female, flatulent
45
Cholecystitis
Path: chemical irritation by bile and calculi, bacterial infection, pancreatic reflux - could be from ischemia from sepsis with hypotension Morph: enlarged tense bright red green-black gallbladder Age: 5th - 6th decade, female Sx: RUQ pain, fever, anorexia, taycardia, diaphoresis, N/V
46
Chronic cholecystitis
repeated bouts of low grade cholecystitis - gallstones can be present morph: fibrosis or enlarged Sx: repated colicky epigastric or RUQ pain Complications: bacterial superinfection, GB perforation, abscess formation or peritonitis
47
CA of GB
Gender: women Age: older than 70 Assoc: gallstones, chronic GB inflammation is a risk Morph: infiltrating with GB thickening and induration or exophytic growing into lumen as irregular cauliflower like mass - adenocarcinoma most common Sx: same as cholelithiasis