okay so welcome to another MedCram lecture we're continuing our discussion
about actual diseases in terms of cholestatic liver diseases so let's go
ahead to the table okay so here are three major diseases we've got primary
biliary cirrhosis we've got primary skill Rowson
cholangitis and we've got large bile duct obstruction let's talk about
primary biliary cirrhosis first in this area we've got these these categories
alkaline phosphatase total bilirubin history and diagnosis so primary biliary
cirrhosis is more common in women it causes fatigue and itching so think
about a woman who's itching and has some jaundiced or the alpha is going to be
definitely elevated and that's because what this primary biliary cirrhosis is
what they are is like little portal granulomas so little granulomas
affecting the portal area and you get destruction of the intrahepatic biliary
ducts so this is intra hepatic destruction of the biliary ducts we see
this in middle-aged women and you do get an increase in cholesterol retention it
can be asymptomatic for years or even decades so what you see is a high
alkaline phosphatase you may actually also see a high cholesterol as we
mentioned and the the bilirubin does go up but it doesn't go up until later in
the disease here's the key though for the diagnosis okay and this is what
they're going to test you on is you're going to have positive auntie my toe
con drill antibodies that's the key positive anti mitochondrial antibodies
in more than 95% of the time you can diagnose this
with liver biopsy okay so if you've got lady who fits into this category make
sure you get an anti mitochondrial antibody okay let's talk about primary
sclerosing cholangitis now with this whereas this was just
intro hepatic now we're talking about both intra and extra hepatic ductal
problems it's associated with actually ulcerative colitis okay so you think
about those type of inflammatory diseases what you're seeing here is
fibrosis cholangitis of the biliary ducts and what you'll see is kind of
like a beads on a string type of appearance if you were to do a colon jia
gram so you'll have this fibrosis here's the biliary duct and then you'll have
this fibrosis fibrosis eyebro --ss so it looks like beads on a string if you were
to do a colon Geograph okay so this beaded appearance makes you think of
sclerosing cholangitis and so the diagnosis is actually a k-- Cola NGO
clan geography if you will that means you just shoot dye in there and you can
see it light up it's pretty pretty interesting to see that so the beaded
appearance is almost pathognomonic and it's associated with these diseases and
it looks very similar to primary biliary cirrhosis in terms of labs so you get
the elevated alq foss and you get this total bilirubin elevation okay what
about large bile duct obstruction again because alpha is an inducible enzyme
you'll see that you also see increased T Billy here however because you actually
have a large bile duct obstruction this is going to look like gallbladder
disease or cholecystitis you're gonna get pain you're gonna get fever
okay these type of things and the diagnosis here again is you can do colon
geography you could also do ultrasound and you could do ercp that's endoscopic
retrograde cholangiopancreatography
which is a long word so ii RCP you can do an mr c p that's where you use a
magnetic resonance imaging you can do ultrasound you can do clan geography
okay so that takes care of the cholestatic liver diseases and thanks
for joining us
you