Liver Path VI Flashcards
wilson disease
auto recessive - impaired excretion of cupper
accumulates in liver, brain, eyes
age 5-15yo
kayser fleischer rings
wilson disease - copper overload
diagnosis of wilson disease
low serum ceruloplasmin - screening
increased urine copper - specific
liver biopsy
tx of wilson disease
chelation or transplant for cirrhosis
ATP7B
wilson disease
ATP7A
mencke
brittle hair - death - no Cu absorption
menke
no Cu absorption
- brittle hair
- death
steatosis
can occur in wilson disease
rhondanine stain
copper stain
for wilson diseas
diagnosis of wilson disease
kayser-fleischer ring - of eye
copper deposition
basal ganglia lesions
wilson disease
globus pallidus
alpha1 antitrypsin deficiency
auto recessive
protein accumulate in hepatocyte inducing apoptosis
also - neutrophil elastase - emphysema of lung
worse with smoking
diagnosis of alpha-1 antitrypsin
serum protein electrophoresis
low alpha-1 band
PAS stain
alpha-1 antitrypsin deficiency
neonatal cholestasis
alpha-1 antitrypsin deficiency
biliary atresia
idiopathic
intrahepatic biliary tract disease
biliary cirrhosis
sclerosing cholangitis
anomalies of biliary tree
extrahepatic biliary tract disease
large duct obstruction - lead to second biliary cirrhosis if prolonged
primary biliary cirrhosis
progressive cholestatic liver disease
-destruction of small to medium bile ducts
AMA against PCD_E2
AMA against PCD-E2 in small bile duct of hering canal
with primary biliary cirrhosis
primary biliary cirrhosis clinical
middle aged women
pruritis, late jaundice
diagnosis of PBC
positive AMAs
liver biopsy - non-suppurative granulomatous destruction of medium sized bile ducts - florid duct lesion
florid duct lesion
medium sized bile ducts show intense chronic inflammation and damage
in PBC
granulomatous reaction
AMA test
positive in primary biliary cirrhosis
pt serum on normal test tissue
incubated - allowing AMA to bind PDC-E2
washed and immunofluorescent AMA-Ab dropped to tissue
if positive - PBC
primary sclerosing cholangitis
progressive liver disease with cholestasis
obliterative fibrosis of intra and extrahepatic bile ducts
seen in UC and crohn - majority UC
male age 40yo
cholelithiasis formation - stones
diagnosis of PSC
beading on imaging
NOT biopsy
- rarely see the onion skinning fibrotic lesions
- biopsy for staging
beading of bile ducts
PSC
von meyenburg complex
abnormal bile duct proliferation
-benign - but more problem with ciliopathy elsewhere
ciliopathy
cilia often involved in biochemical signaling
-so defective - can cause problems
ex - polycystic kidney disease
caroli syndrome
cysts in bile duct and hepatic fibrosis
ciliopathy
ADPKD
auto dom polycystic kidney disease
hepatic cysts
rupture berry aneurysm - circle of willis
mitral valve prolapse
abdominal or flank pain
meckel syndrome
auto recessive
perinatal death
dysplasion and malformation of multiple organs
ciliopathy
mutated polycystin
Wnt an hedgehog malfuction
loss of cell polarity and cystic expansion occurs
liver segmentation
8 divisions
can be removed individually during surgery
liver will regenerate
as occlude portal vein
flow in hepatic artery increases
space of mall
has defining membrane
chemicals - adenosine and NO can control blood flow to the portal tract
control flow in this shunt - to get more O2 to hepatocytes
portal vein thrombosis
seen with ultrasound
fenestrations of sinusoids
can get bigger/smaller
hypovolemic shock
zone 3 has least O2 supply
hepatocytes here die first
first necrotic changes due to hypovolemia
shock liver
zone 3 necrosis
like acetaminophen toxicity
pink areas - necrosis
CHF
pressure damage and ischemic damage in zone 3
get necrosis around zone 3
acute passive congestion
nutmeg liver
acute passive congestion
with centrilobular necrosis
impaired intrahepatic flow
sickle cell
DIC
tumor
hepatic vein outflow obstruction
- budd-chiari - hepatic vein thrombosis
- sinusoid obstructive syndrome
infarct of zahn
block of intrahepatic radical giving discrete red-blue area
budd chiari syndrome
obstruction of at least TWO hepati veins or IVC
RUQ pain, ascites, hepatomeglay
imaging - doppler to see lack of flow
prognosis - life threatening if acute
tx - anticoagulant, thrombolytic, shunt
tense capsule
with budd-chiari syndrome
budd chiari
zone 3 hemorrhagic necrosis
preeclampsia
HTN and proteinuria - pathologic edema
maternal endothelial dysfunction
eclampsia
seizure in women with preeclampsia
HELLP
hemolysis, elevated liver enzymes, low platelets
-unknown cause
periportal sinusoids accumulate fibrin deposits with hemorrhage to space of disse - necrosis of zone 1**
tx - delivery
acute fatty liver of pregnancy
rare
microvesicular fatty change
3rd trimester - leading to liver failure
unknown cause
