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liver Flashcards

1
Q

role of fetal liver

A

major site of hematopoiesis in fetus: mesodermal hematopoietic stem cells migrate from mesonephros → to liver at 3 wk GA (source of fetal blood cell until BM at 28 wks GA)

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2
Q

portal triad

A

portal vein
hepatic artery
bile ductule

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3
Q

blood supply to liver

A 
portal vein (from mesenteric veins, gastric vein): toxins + nutrition from GI
hepatic artery: O2 blood
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4
Q

hepatocytes role

A 
make proteins
energy, vitamin, mineral storage
metabolize drugs/toxins
make bile → dump bile into bile canaliculi (opposite flow to sinusoid flow → bile ductule → hepatic duct → common hepatic duct...)
excrete bilirubin
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5
Q

zone affected first by ischemia + hypotension

A

zone 3: pericentral vein zone (farthest from O2 blood source - hepatic artery)

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6
Q

zone affected first by viral hepatitis

A

zone 1: periportal zone (next to portal vein)

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7
Q

zone most sensitive to metabolic toxins (acetaminophen overdose, site of alcoholic hepatitis)

A

zone 3: pericentral vein zone

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8
Q

zone with highest concentration of P450 enzymes

A

zone 3: pericentral vein zone

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9
Q

examples of proteins made by liver

A 
coag factors
complement
albumin
apolipoproteins: lipid transport
transferrin: iron transport
ceruloplasmin: copper transport
others:
cholesterol
phosopholipids
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10
Q

examples of drug + toxin metabolism of liver

A

P450 enzymes
UDP glucoronyl transferase
ALT and AST: transaminases
steroid hormones → inactive metabolites (need for breakdown of estrogen, progesterone, testosterone, vitamin D, cortisol, thyroid hormone)

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11
Q

storage role of liver

A

glucose as glycogen
store cholesterol with TG in liver (esp VLDL)
iron bound to ferritin
B12, ADEK

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12
Q

role of bile

A

breakdown fats

carrier for excretion of bilirubin

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13
Q

complication of excess unconjugated bilirubin for several months in newborn

A 
kernicterus: unconjugated bilirubin in brain
→ 
chorea
cerebral palsy
hearing loss
gaze abnormalities
brain damage → death
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14
Q

neuro effects of excess unconjugated bilirubin in newborn (occur in hrs-days)

A

bilirubin is neurotoxic:
lethargy
hypotonia

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15
Q

prevent kernicterus

A

phototherapy: convert bilirubin to isomer that can excrete

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16
Q

AR mutation in promotor for UDP-glucoronyltransferase gene →↓ amounts of UDP-glucoronyltransferase enzyme
conjugate bilirubin more slowly → slight elevation of INDIRECT bilirubin
benign and asymptomatic unless infection or alcohol (incidental finding)

A

Gilbert syndrome

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17
Q

complete absence of UDP-glucoronyltransferase enzyme
can’t conjugate bilirubin and excrete it into the bile
jaundice + ↑ INDIRECT bilirubin in first few days of life
may have NEURO sx
if no treatment: kernicterous (die in a couple years)

A

type I crigler-najjar syndrome

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18
Q

treatment of type 1 crigler-najjar syndrome

A

phototherapy
plasmaphoresis - remove unconjugated bilribubin bound to albumin
definitive: liver transplant (makes UDP-GT)

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19
Q

mutated UDP-glucoronyltransferase enzyme

jaundice + ↑ INDIRECT bilirubin in first few days of life (but less than type I)

A

type II crigler-najjar syndrome

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20
Q

distinguish type I vs type II crigler-najjar syndrome

A 
give phenobarbital (barbiturate): induces liver enzyme production (even UDP-GT)
type II pr Gilbert: ↓ bilirubin
type I: no change (no UDP-GT in liver!)
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21
Q

problem putting conjugated bilirubin back into bile → conjugated bilirubin trapped in hepatocytes → turns liver BLACK
↑ DIRECT serum bilirubin
benign - no treatment necessary

A

dubin-johnson syndrome

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22
Q

milder form of dubin-johnson syndrome
mild elevation in DIRECT bilirubin
liver DOESN’T turn black

A

rotor syndrome

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23
Q

stages of alcoholic liver disease

A

steatosis (fatty liver)
alcoholic hepatitis
alcoholic cirrhosis

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24
Q

fat droplets in cytosol of hepatocytes seen in HEAVY drinks

REVERSIBLE if stop drinking

A

steatosis: fatty liver

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25
Q

inflammation + steatosis
swollen necrotic hepatocytes
neutrophils in liver parenchyma
MALLORY bodies: intracytoplasmic eosinophilic inclusions of keratin

A

alcoholic hepatitis

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26
Q 
RUQ pain
anorexia
jaundice
low-grade fever
↑AST>ALT (AST >2x the ALT, A Scotch and Tonic = AST)
enlarged, swollen liver
A

alcoholic hepatitis

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27
Q

IRREVERSIBLE
scarring + fibrosis
palpate hard, nodular liver edge
enlarged OR shrunken liver
AST/ALT: may be high, normal, low (trashed liver)
residual nodules of hepatocytse with collagen + sclerosis (blue on trichrome stain) surrounding them (replaced necrotic hepatocytes)
sclerosis located around central vein (zone 3)

A

alcoholic cirrhosis

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28
Q

complications of cirrhosis

A

liver failure: no hepatocytes

portal HTN: blood can’t flow through sinusoids (causes hepatosplenomegaly) → portal vein

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29
Q

coagulopathy: ↑PT, PTT (need vitamin K)
bleeding/bruising
↓osmotic pressure → peripheral edema, ascites
no metabolism of ammonia → hepatic encephalopathy (confusion, delerium, hypersomnia, coma, death, asterixis, fetor hepaticus (musty odor of breath)
↑estradiol levels: testicular atrophy, gynecomastia, spider telangiectasia on chest, palmar erythema
↑ unconjugated bilriubin: jaundice, slceral icterus
↓LDL and HDL (liver not making)

A

liver failure

30
Q

hepatosplenomegaly (blood backs up in these organs, can cause enlarged liver even though cirrhotic)
fluid leaks from liver due to back up of blood → ascites
caput medusa: umbilica veins dilated
anorectal varices: tortuous, dilated vein
esophageal varices (if active bleed → hematemesis, melena)

A

portal hypertension

31
Q

complication of ascites

A

spontaneous bacterial peritonitis (SBP)

32
Q

treatment of active esophogeal variceal bleed

A

↓ splanchnic circulation: octreotide (somatostatin analog)

B blocker: propranolol, nadolol

33
Q

prevent esophageal varicel bleeding

A

endoscopic banding
transjugular intrahepatic portosystemic shunt (TIPS) - create shunt from portal vein → systemic circulation to bypass liver →↓ portal HTN, ↓ variceal blood flow, ↑ hepatic encephalopathy

34
Q

treatment of hepatic encephalopathy

A

lactulose: trap ammonia in gut so excreted in stool →↓ serum ammonia levels

35
Q

cirrhosis (alcohol, hep viral infection, hemachromatosis) is risk factor for

A

hepatocellular carcinoma

36
Q

cocktail of meds for severe cirrhosis

A

diuretics
B blocker: nadolol, propranol
vitamin K: make clotting factors
lactulose: encephalopathy

37
Q

aminotransferase (AST, ALT, liver enzymes) can suggest

A

viral hepatitis: ALT = or > AST

alcoholic hepatitis: AST > ALT (AST >2x)

38
Q

gamma-glutamyl transpeptidase (GGT)

A

↑ in following:
liver + biliary tract diseases
excessive alcohol

39
Q

akaline phosphatase

A

↑ in following:
biliary obstruction: gallstones, cancer
active bone formation: children (normal), Paget disease of bone, bone cancer
HCC

40
Q

R sided heart failure or Budd-Chiari syndrome→ back up of blood into liver → nutmeg liver → if persists, centrilobular congestion + necrosis → cardiac cirrhosis

A

nutmeg liver (speckled)

41
Q

occlusion of IVC or hepatic veins → congested liver
hepatomegaly
ascites
ab pain
portal HTN → esophageal varices, caput medusae
NO JVD

A

budd-chiari syndrome

42
Q

congested liver

JVD

A

right sided heart failure

43
Q 
liver disease associated with:
hypercoagulable states
pregnancy
polycythemia
HCC
A

budd-chiari syndrome

44
Q

children receiving aspirin for viral infection → aspirin metabolites inhibit mitochondrial enzymes →↓ B oxidation →
hepatoencephalopathy:
rash, vomit, headache, confusion → hypoglycemia, stupor, coma, death

A

reye syndrome

45
Q

AR ATP7B enzyme defect:
1) inadequate copper excretion into bile → copper accumulates in:
liver, brain, cornea, kidneys, joints
2) impaired conversion of copper to ceruloplasmin (need for transport in blood) →↓ serum ceruloplasmin

A

wilson disease

46
Q

features of wilson disease

A 
cirrhosis of liver
Kayser-Fleischer rings: corneal deposits
↓ serum ceruloplasmin
basal ganglia degeneration: parkinsonian sx
asterixis, dyskinesia, dysarthria
hemolytic anemia
hepatic encephalopathy → dementia
fanconi syndrome: PT dysfunction in kidney
↑ risk HCC
47
Q

Kayser-Fleischer rings: golden corneal deposits near edge of eye

A

wilson’s disease

48
Q

complication of wilson disease

A

↑ risk HCC

49
Q

treatment of wilson disease

A

penicillamine (copper penny)

50
Q 
excess iron deposition 
triad:
cirrhosis
diabetes mellitus
skin pigmentation
other complications:
congestive heart failure
testicular atrophy
↑ risk HCC
A

hemachromatosis

51
Q

called “bronze diabetes”

A

hematochromatosis

52
Q

causes of hematochromatosis

A

AR disease

secondary: excessive transfusion (chronic anemia states: B thalasemia, SCD)

53
Q

↑ ferritin (complex of iron in apoferritin- primary cellular storage of iron): screening test
↑ total serum iron
↓ total iron binding capacity
↑ transferrin saturation (binds iron and transports it through plasma)

A

hemachromatosis

54
Q

treatment of hematochromatosis

A 
phlebotomy (iron is in RBCs - DOC)
subq:
deFEroxamine (for Fe): chelating agent
oral agents:
deferiprone
deferasirox
55
Q

panacinar emphysema and liver cirrhosis in young person or non-smoker

A

α1 antitrypsin deficiency

56
Q

autosomal codominant disease
↑ elastase activity → break down elastic tissue in lungs →
panacinar emphysema
mutated form of α1 antitrypsin made in liver and polymerizes → accumulates in liver →cirrhosis: jaundice, ↑ LFT

A

α1 antitrypsin deficiency

57
Q

role of α1 antitrypsin

A

inhibit elastase (breaks down elastin)

58
Q

female 20-40 yo
asymptomatic (incidental on imaging) or RUQ pain
associated with OCP use, anabolic steroids, glycogen storage disease type I and III

A

hepatic adenoma

59
Q

complication of hepatic adenoma

A

malignant transformation to HCC in 10%

60
Q

treatment of hepatic adenoma

A

stop OCP
serial imaging
check AFP to ensure not getting HCC
resect if > 5cm

61
Q

malignant endothelial neoplasm of liver

A

hepatic angiosarcoma

62
Q

risk factors of hepatic angiosarcoma

A

vinyl chloride

arsenic

63
Q

liver disease with elevated α fetoprotein

A

HCC

64
Q

risk factors for HCC

A 
hep B/C
wilson disease
hemachromatosis
α1 antitrypsin deficiency
hepatic adenoma
alcoholic cirrhosis
carcinogen exposure (aflatoxin from aspergillus)
65
Q 
jaundice
tender hepatomegaly
ascites
polycythemia due to ↑ epo
hypoglycemia
A

HCC

66
Q

serum marker for HCC

A

AFP

67
Q

risk factors for hepatitis

A

IVDU (Hep B + C)
alcoholism (alcoholic hepatitis)
travel to developing countries/poor sanitation (fecal oral route: Hep A + E)

68
Q 
asymptomatic (but can transfer via sexual contact)
symptoms:
malaise
arthralgias
fatigue
N/V
RUQ pain
jaundice/scleral icterus
tender hepatosplenomegaly
LAD
A

hepatitis

69
Q 
labs:
bilirubinuria 
↑AST + ALT (ALT higher or equal to AST in viral, AST higher in alcohol)
↑ serum bilirubin
↑ serum alk phosphatase
A

hepatitis labs

70
Q

resolves with absitence

A

fatty liver disease

71
Q

due to binge drinking

A

alcoholic hepatitis

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