Pathology - liver Flashcards
1
Q
describe the histopathos in cirrhosis
A
diffuse bridging fibrosis and nodular regeneration via stella celsl that dursrupts the normal architecture of the liver.
2
Q
what does cirrhosis increase the risk for
A
hepatocellular carcinoma
3
Q
what cells cause the fibrosis
A
stellate cells
4
Q
etiologies for cirrhoses
A
OH HepC HepB biliary disease metabolic disorders
5
Q
describe the pathophys effects of cirrhosis
A
compresses sinusoids and central bile canals
- increased intrasinusoidal hypertension
- decreases number of functional sinuisoids
- increases hydrostatic pressure in the portal circulation
6
Q
what is the result of a portosystemic shunt
A
partially alleviates portal hypertension
7
Q
how do portosystemic shunts manifest in increased portal hypertension
A
A) esophageal varices (left gastri with esophageal)
b) caput medusa ( paraumbilica and small epigastrics)
c) anal varices (Superior rectal and middle/inferior rectals)
8
Q
result of portal hypertension please
A
esophageal varices - hematemesis and melena peptic ulcer - melena congestive splenomegaly caput medusa ascites portal hypertensive gastrophaty anorectal varices
9
Q
effects of liver cell failure
A
hepatic encephalopathy scleral icterus fetor hepaticus spider nevi gynecomastia jaundice testicular atropy liver flapasterixis bleeding tendency with increased PT anaemia dependeing pitting oedema/ankle oedema
10
Q
how does cirrhosis show up on a CT
A
splenomegaly with also nodularity of the liver counter due to regeneration macronodules
cirrhosis: diffuse bridging fibrosis and nodular regeneration via stellate cells that disrupts liver architecture.
11
Q
what does disruption of the urea cycle show up as in cirrhoses
A
decreased BUN and increased serum ammonium
12
Q
what does disruption to gluconeogenesis and glycogenolysis present as in cirrhosis?
A
hypoglycemia
13
Q
A-B balance in cirrhosis please
A
chornic respiratory alkalosis: tpxic prodcuts stimulate the respiratory centre to hyperventilated
metabolic acidosis - lactic acid build up bc cant be converted to pyruvate after the cori cycle brings lactate from other tissues
14
Q
what happens to salt balance in cirrhosis
A
get hyponatremia due to secondary aldosteronism
15
Q
hypoalbuminemia in cirrhosis results in
A
hypocalcemia - less carriage, no effect on free ca, just total
dependent pitting oedema
16
Q
why do you see hypocalcemia in cirrhosis
A
decreased Vit D decreased albumin (carries in blood. decrease total but no effect on free Ca...confuses my brain?)
17
Q
list dzs where you see increased alkaline phosphatase
A
cholestatic hepatobiliary disease obstructive hepatobiliary disease hepatocellular carcinoma infiltrative disorders bone disease
18
Q
list diseases wher eyou see increased aminotransferase levels please
A
viral hepaitits ALT > HST
OH hepatitis AST > ALT
19
Q
list dzs where you see increased amylase
A
actue pancreatitis
mumps
20
Q
list dzs where you see changes in ceruloplasmin
A
decreased in Wilson disease
21
Q
list dz where you see altered gamma-glutamyl transpeptidase
A
increased in liver and biliary disease - like AL[
NOT increased in bone disease
associated with alcohol use
22
Q
list dzs where you see increased lipase
A
acute pancreatitis most specific marker
23
Q
child
afebrile, quiet lethargic, sleepy
hepatomegaly
liver dysfucntion
A
reyes syndrome
24
Q
what is reyes syndrome
A
rare
often fatal
childfood enchaphalopathy
25
discuss findins in reyes syndrome
```
mitochondrial abnormalities
microvesicular fatty change in liver
hypoglycemia
vomiting
hematpomegaly coma
```
26
what is reyes syndrome associated with
aspirin to treat viral infection especially VZV and influenza
27
what is the MOA of reyes syndrome
aspirin metabolies decreased beta oxidation by reversible inhibition fo mitochondrial enzymes
28
what is except to avoidance of aspirin in children
Kawasaki disease
29
```
fatty liver
hypoglyecemia
vomiting
hepatomegaly
coma
child
recent VZV or influenza
```
aspirin
| reyes syndrome
30
what is hepatic steatosis
macrovesicular fatty change that may reversible with OH cessation
31
what causes OHic hepatitis
sustained, ong term consumption of OH
32
describe the histopathos of OHic hepatitis
swollen and necrotic hepaticyes with PMN infilriation
| Mallory bodies - intracytoplasmic eosinophilc inlcuisons of damaged keratin filmaets
33
labs in OHic hepatitis
AST> ALT usually 1/5
34
what is OHic cirrhosis
final and irreversible form
35
histoatphos on OHic cirrhosis
micronodular, irregularly shrunken lvier with ''hobnail'' apperances
sclerosis around central vein aka zone III
36
microvesicular fatty change
reye syndrome
37
Mallory odies
OHic hepatitis
38
hobnail apperance
OHic cirrhosis
39
macrovesicular fatty chane
hepatit steatosis with OH
40
swollen and necrotic hepatocytes with lymphocytic infiltration
OHic hepatitis
41
drugs that can cause fatty change in the liver
amiodarone
| methotrexate
42
drugs that can cause fibrosis in the liver
amiodarone
methotrexate
retinoic acid
43
what is non OHic fatty liver disease associated wtih
metabolic syndrome/insulin resistance
44
how does metabolic syndrome/insulin resistance cause non OHic fatty liver disease
increased fatty infiltration of hepatocytes -- cellular ballooinign and evnetal necrosis
45
what can nonOHic fatty liver disease end up as?
cirrhosis and HCC
46
labs in nonOHic fatty liver dsiease
ALT > AST
47
cellular ballonign and necrosis
non OHic fatty liver disease
48
swollen and necrotic hepatocytes with PMN infiltration
OHic hepatitis
49
how does hepatic encephalopathy come about? pathophys time
cirrhosis -- portosystemic shunts -- decreased HN3 metabolsi -- neuropsychiatric dysfuction
50
describe cxpx of hepatic encephalopathy
spectrum: disorientation/asterixis to difficulr arousal or coma
51
```
altered mental status
somnolence
disordered sleep rhythma
asterixa
coma or death
```
hepatic encephalopathy
52
treatment of hepatic encephalopathy
lactulose - increased NH4 generation
| rifaxamin
53
what causes decreased NH3 removal
renal failure
diuretics -- cause alkalosis (loops and thiazides) -- keep NH3 as is not excreted in H buffferint to NH4, hepatic blood flow post TPIS is bypassed
54
what causes increased HN3 production and absorption
dietary protein
gi bleed
constipation
infection
55
what to avoid in pts with hepatic encephaolpaty
```
dietary protein
gi bleeds
constipation
infection
renal failure
diuretics
portosystemic shunts man made.
```
56
what is most common primary malignant tumor in adults
hepatocellular carcinoma
57
what is hepatocellular carcinoma associated with
cirrhosis: including HepB, HepC, Oh and non OH fatty liver disease, autoimmune diseases, hemochromatisos, alpha1 antitrypsin def and wilsons and aflatoxin
58
what syndrome can hepatocellular carcinoma lead to
budd chiari syndrome
59
describe budd chiari syndrome presentation
congestive liver disease: hepatomegaly, varices, abdominal pain, eventual liver failure without JVD
60
```
jaundice
tender hepatomegaly
ascites
polycythemia
anorexia
```
renal cell carcinoma
61
how does HCC spread
hematogensouly
62
fever
pain
increased liver size
blood in ascistes fluid
hepatocellular carcinoma
63
how to diagnosis hepatocellular carcinoma
increased alpha fetoprotein
u/s
contrast CT/MRI, biopsy
64
what are paraneoplastic syndromes with hepatocellular carcinoma
EPO
PTHrP - hypercalcemia
insulin like factor - hypoglycemia
65
serum markers in hepatocellular carcinoma
increased alpha fetoprotein
increased ALP
increased GGT
NO AMINOTRANSFERASE DIFFS
66
what is the most common benign liver tumor
cavrernous hemangioma
67
what is typical tumor in 30-50 years old lvier
cavernous hemangioma
| benign
68
histopathos of cavernous hemangioma please
biopsy is contraindicated due to risk of hemorrhage bc made up of a collection fo dilated blood vessels
69
what liver tumors have a risk of hemorrhage
caveronous hemangioma and hepatic adenoma
70
what is a hepatic adenoma
rare
| benign liver tumor that can spontaneously regress or rupture - hemorrhage
71
who is like to get a hepatic adenoma
OCP and steroid useers
| von gierke glycogen disease
72
hepatic tumor associated with von gierkes?
hepatic adenoma
73
abdominal pain and shock
rupture of a hepatic adenoma
74
what is an angiosarcoma
maligi tumor of epithelial origin
75
rf for angiosarcoma
vinyl chlorid
arsenic
torium dioxide in radiocontrast
76
vinyl chloride
arsenic
thorium dioxide/radiocontrast
angiosarcoma of liver
77
what mets show up in the liver
git, breas, lung
78
what is most common place to find mets
lvier
git
breast
lugn
79
```
hepatomegaly
varices
abdominal pain
eventual liver failure
no JVD
```
budd chiari syndrome
80
what causes budd chiari cyndrome
thrombosis or compression of the hepatic veins with centrilobular congestion and necrosis ie congestive liver disease
81
what is associated with budd chiari syndrome
```
NO JVD REMEMBE OR ELSE RIGHT HEART FAILURE
hypercoaguable states
polycythemia vera
postpartum state
HCC
```
82
what are these risk factors for: hypercoagbaule state, polycythemia vera, postpartum state, HCC
budd chiari
83
gross appearance of lvier in congestive liver disease
nutmeg: centrilobular congestion and necrosis of liver
| mottled apperance
84
what is most common cause of budd chiari?
polycythemia veraq
85
enlarged painful liver
portal hypertension
splenomegaly
ascites
congestive liver disease
RHF if JVD
budd chiari if not
86
pathophys of alpha1 AT def
misfolded gene product protein aggregates in hepatocellular ER - red cytogranules
87
what is inheritance pattern of alpha 1 anti trypsin def
codominant AD
88
what is the normal allele for A1AT?
MM
89
what is worst allele for A1AT?
ZZ
90
how does alpha1 antitrypsin manifest?
at lungs - panacinar emphysema
| at liver - cirrhosis
91
what is the most common cause of cirrhosis in children
alpha1 anti trypsin deficieincy
92
what causes jaundice
tis abnormal yellowing of skin and or sclera due to bilirubin deposition
93
when does jaundice occur
in the face of increased production ofdefective metabolism of bilirubin -- when reach levels of > 2.5 mg/dL
94
what causes unconjugated/indirect hyperbilirubinemia
physiologic neonatal jaundice
gilbert
crigler-najjar
hemolytic anaemia
95
what causes conjugated/direct hyperbilirubinemia
biliary tract obstruction: gall stones, cholangiocarcionma, pancreatic or liver cancer, liver fluke
bilary tract disease: primary sclerosing cholangitis, primary biliary cholangitis
excretion defects: dubin Johnson and rotor syndrome
96
what are biliary tract obstructive causes that lead to conjugated/direct hyperbilirubinema
```
gall stones
cholangiocarcionma
pancreatic cancer
lvier cancer
liver cluke (chlonoarchis sinusis)
```
97
what are bilary tract diseases that can cause conjugated/direct hyperbilirubinema
primary sclerosing cholangitis
| primary biliary cholangitis
98
what causes mixed indirect/direct hyperbilirubinemia?
hepatitis
| cirrhosis
99
pathophys of physiologic neonatal jaundice please
immaute UDP glucuronosyltransferase -- unconjugated hyperbilirubinemia -- jaundlse and kernicterus
100
what is kernicterus
deposition of water insoluble unconjugated bilirubin the the brain particularly at the basal ganglia
101
basal ganglia deposition of liver messing up
Wilson - copper
| kernicterus - unconjugated bilirubin
102
how to treat physiologic neonatal jaundice
phototherapy
103
asymptomatic or mild jaundice with increased unconjugated bilirubin without overt hemolysis
gilbert syndrome
104
clincali consequences of gilbert syndrome
none hurrah
105
pathophys of gilbert syndrome
mildly decreased UDP-glucuronosyltransferase conjucation
AND
impaired bilirubin uptake
106
what makes bilirubin increased in gilberts
fasting and stress
107
what is the pathophys of crigler najjar type I?
absent UDY-glucuronosyltransferase
108
jaundice
kernicterus
increased unconjugaster bilirubin
early in life
crigler najjar syndrome type I
109
prognosis of crigler najjar type I
fatal in a few years of life
110
how to treat crigler najjar type Ii
less severe
| tx with phenobarbital to increase liver enzyme synthesis
111
how dose phenobarbital work
increase enzyme synthesis for treatment of crigler najjar type II
112
grossly black liver
| benign
dubin Johnson syndrome
113
pathophsy of dubin johnson
defective excretion
| hyperconjugated bilirubinemia
114
what is roto syndrome
similar to dubin Johnson
hyper conjugated bilirubinemia
no black liver
115
what syndrome involved impaired uptake of bilirubin
gilberts
| NOT crigler najjar
116
what is Wilson disease aka?
hepatolenticular degeneration
117
what causes Wilson disease
inadequate hepatic copper excretion and failure of coppuer to enter circulation and cerloplasmin
118
where do levels of coppu increased in Wilson diseas at first
liver
119
where do levels of copper increase in Wilson disease later on
```
after liver spews it out
blood
eyes
kidney
brain
red blood cells
joints
```
120
type of inheritance wilsons
chromosome 13
AR
ATP7B
121
how is copper normally dealt with in the liver?
excreted into bile via a hepatcoyte copper transporting ATPase that gets messed with an ATP7B mutation
122
blood levels in wilsons
low cu at fist
| low cerylplasmin
123
describe effects of cu in organs
eyes - deposition in cornea - Kayser-Fleishcher rings
liver: cirrhosis - HCC
kidneys - fanconi syndrome
joints : dysarthria
brain: putamen - parkinsonism, subthalamic nuclei - hemiballismus and dementia
blood: hemolytic anaemia
124
```
asterixis
dementia
hemiballismus
parkinsonism
HCC
fanconi syndrome
hemolytic aneamia
dysarthria
KF rings
```
Wilson disease
125
how do you treat Wilson disease
penicillamine
trientine
oral zinc - inhibits absorption in intestine
126
what is hemochromatosis
the inherited cause of hemochromatosis - deposition of hemosiderine in tissues
127
what is the classic presenting triad of hemochromatosis
cirrhosis
DM
skin pigmentation
128
what are consequences of hemachromatosis
heart failure (restrictive)
testicular atrophy
increased risk of HCC
129
what are causes of hemochromatosis
primary - AR of HFE, C282Y, H63D in HFE gene with HLA-A3
| secondary - chronic transfusion therapy ie beta thalassemia major
130
blood work for hemochromatosis please
increased ferritin
increased fe
decreased TIBC
increased transferrin saturation
131
how do you id iron on a biopsy
Prussian blue stain
132
how much body iron is enough to set off detectors at airport
50 g - can happen in hemochromatosis
133
HLA association with hemochromatosis
HLA-A3
134
HLA-A3
hemochromatosis
135
why is onset of hemochromatosis slower in women than men
meennsseesss
136
how do you treat hemochromatosis
phlebotomy
| chelation with deferasirox, deferoxamine and deferiprone (oral)
137
typical nationality with hemochromatosis
northern European descent
138
list the organs affected in hemochromatosis
```
liver
pancreas
heart
joints
skin
brain - pituitary
```
139
list the organs affected in wilsons
```
liver
eyes
joints
blood
brain - bg
kidney
```
140
list the effects on organs by hemochromatosis
liver - cirrhosis - HCC
heart - restrictive cardiomyoatphy
brain - pit - infertility (decreased levels of Lh and FSH)
pancrease - DM I
skin - bronze - melanine production increased
joints - degenerative arthritis
141
list organs in Wilson vrs hemochromatosis and both
both: liver, joints, brain,
Wilson: eyes, kidney, rbc
hemochromatosis: heart, pancreas, skin
