00:01
Okay,
now this one's kind of fun.
00:02
I know this is nerd
version of fun,
but knowing what these names
mean are really important.
00:08
So anti glomerular basement
membrane antibody disease
Got that?
So let's break that down.
00:16
We've got anti glomerular
basement membrane
and that is referring
to the basement membrane
in the glomerulus.
00:24
Now the basement
membrane is kind of what
keeps everything intact
you have basement membranes
throughout your body.
00:30
Yeah, but in the glomerulus,
they're really important
to help keep things intact
and we're talking
about the glomerulus
because we're talking about
goodpasture's syndrome
or anti glomerular basement
membrane antibody disease.
00:45
Now so we've got
anti being against
glomerular basement membrane
is in the glomerulus
and antibody disease.
00:53
We've got the antibodies
there for you.
00:56
So we're thinking about
a basement membrane.
00:57
I didn't want to just
skip over this point.
00:59
I wanted to make sure
that you understood
how important these are
because take a look
at our graphic.
01:06
This is a section of
a small blood vessel.
01:09
Now the inner surface of
the vessel is lined by
endothelial cells now they
sit on a really thin membrane
that we call the
basement membrane.
01:19
Okay,
so take a look at our drawing
you can see we have the
round circle on the top
and then we have a cross section
of the blood vessel
on the bottom.
01:27
So are you with me?
Okay, cool.
01:29
Now look at the endothelial cell
see those little shapes there
and they're labeled
endothelial cells.
01:36
Look they are lining
your blood vessels.
01:39
You have endothelial cells
all throughout your body
lining your blood vessels.
01:44
Now, you've got the basement
membrane see it there.
01:47
Yeah.
We've got it labeled for you.
01:49
See what the
basement membrane is.
01:51
So you've got endothelial cells
and they sit on this
really thin membrane,
for really thin but
it's really important.
02:00
So keep an eye on that
we'll talk about what
happens in this disease.
02:04
So in anti GBM disease
antibody start to
attack the collagen
in the basement membranes of
the lungs and the kidneys.
02:14
That's how we end up with
hemorrhaging lungs and kidneys.
02:17
They get all clogged up.
02:19
Now before you go on,
let's let that sink in
for just a little bit.
02:24
Look at the top,
you're looking as if we took a slice
of a blood vessel on the bottom
as if we dissected
It down the middle
and laid it open in half.
02:33
Find the endothelial cells
find the basement membrane.
02:38
Cool. Okay, you've got those
so you can see how
the basement membrane
kind of keeps
everything together.
02:44
Now what's flowing through that,
Look how we have
those labeled for you,
got some other wbc's,
those are the blue ones
you've got red blood cells.
02:53
Remember those are just stacks
that carry oxygen right,
as adults they carry
four hemoglobins
as little tiny infants,
they 6 when you're a newborn,
I think that's super cool.
03:03
And super weird about
babies at the same time
And you've got neutrophil
types of white blood cells.
03:10
So in this bloodstream,
you see two of our five
kinds of white blood cells.
03:14
You see red blood
cells, endothelial cells
and the basement membrane.
03:20
Now I need you to
really slow down here
and make sure this
makes sense to you
because basement membranes
are also throughout your body
just like endothelial cells.
03:30
So this is an important concept
that you're gonna be
able to apply in lots
of other places.
03:35
And we have a
beautiful graphic here
for you to be able
to understand it.
03:40
So in anti GBM disease
antibody start attacking what?
Right.
03:47
The basement membrane.
03:48
Okay, cool.
03:48
You're getting it
now you're with us.
03:50
That's awesome.
03:51
But I want you to do is
draw some notes on here.
03:54
So you can have that
solid in your brain
that in anti GBM disease
antibodies attack the collagen
in the basement membrane.
04:02
So write that in your notes
antibodies attack collagen
in the basement membrane.
04:09
All right, let's keep going.
04:11
So let's do a quick
review of antibodies.
04:13
I know you've probably
looked at them before
but it's kind of helpful sometimes
to have a review of what they do.
04:19
So antibodies are
large y-shaped proteins
and that's why we draw
them like this there
for you to help you remember.
04:26
So whenever you're reading
and you're seeing
things about antibodies,
this is a great visual
for you to remember.
04:32
They're also called immunoglobin
see those things like IG,
that's what we're talking about.
04:38
So antibodies are large
y-shaped proteins.
04:42
So when they clog things up,
they really clog things up.
04:45
They're big they're
y-shaped their proteins.
04:48
They also go by another
name of immunoglobins,
and they're found in our blood
and some of our other body fluids.
04:54
Okay so antibody quick review,
giant proteins shaped like a Y.
05:00
They're also called
immunoglobins
and they're found in our blood
and some other body fluids.
05:06
That is an awesome summary slide
about antibodies so mark that
if you're doing some other
reading about your immune system,
but wait, there's more just
like they say on late-night TV.
05:18
Let's talk a little bit
more about antibodies
because these are
critical players in our
adaptive immune system.
05:24
Now, if you don't mind
go ahead and underline
adaptive immune system.
05:28
This is the bad a
part of antibodies.
05:31
This is so cool
that they can recognize bad
guys and fight them off.
05:36
These antibodies
recognize a unique part
of a foreign thing.
05:41
We call it an antigen.
05:43
So if a foreign
thing is in our body
and its trying to attack us,
the antigens will remember
that's part of the adaptive
part of our immune system.
05:52
So we've got these
antibodies they have a memory
and they recognize
this unique part
of the antigen, right.
05:59
So what they do,
in autoimmune diseases
is instead of recognizing
a foreign thing
autoimmune mistakenly
looks at my own body part
and starts to attack that.
06:12
So in autoimmune disease,
it looks at self instead of a
foreign thing and attacks it
It mistakes me as an antigen
and then I have an
autoimmune problem.
06:25
So we need antibodies
to survive.
06:27
We definitely need an
adaptive immune system.
06:30
But in autoimmune,
it starts to recognize me
as an antigen mistakenly
and attacks my own body.
06:38
That's what happens.
06:39
That's how the basement
membrane is attacked.
06:42
So back to our blood slides
See that we've got the
slice of it for you there.
06:46
If we open it up in half
you got the different,
two different types
of white blood cells.
06:52
You've got the red cells
got the endothelial cells
and the basement membrane.
06:57
All right, were you able
to identify those with me
as I walked through?
Well an anti GBM, anti glomerular
basement membrane antibody disease,
the antibodies bind to the
capillary basement membrane.
07:10
Whoohoo!
Good reading on my part, right?
Well, I wanted to walk you
through that really slowly
because I want you to see
take the chance now
just charter walkthrough
in your brain
what that would look like
pause and read that
statement again
and see if it gives you an
idea of how that disease works.
07:28
Then we'll come back and
I'll show you graphically
how the illustration.
07:35
Okay, ready?
I bet you're pretty
close just from the name
and what you've done
with medical terminology,
but let's look at what happens.
07:43
See those antibodies.
07:45
Now, we've got those
antibodies are those large
y-shaped proteins right there
floating in the bloodstream
similar blood and in
some other tissues.
07:54
Now what's going on
is they're starting
to attach themselves to
that basement membrane
see that there?
So notice how they're starting to
attach to that basement membrane.
08:04
Danger Will Robinson.
08:06
We've got a problem because that
basement membrane is really
thin, pretty fragile.
08:12
So if you start attaching
yourself to this,
this is going to be a problem
because when those antibodies
bind to the capillary
basement membranes
this starts attracting white blood
cells, the neutrophils.
08:25
Okay.
Normally, this is a good idea.
08:27
Because the antibodies like,
Hey guys,
we got a problem come help us
but here it's mistakenly
identifying me.
08:34
It's identifying make
basement membrane.
08:36
So when those antibodies
attach to my basement membrane,
their job is to attract
white blood cells
specifically the neutrophils,
Oh, no,
it is on like Donkey Kong
because the idea is
you're supposed to be
fighting off infection.
08:50
But instead it's attacking me.
08:53
So neutrophils attack
the vessel walls
and cause inflammation.
08:57
That's what leads to
the glomerulonephritis
and in the of
alveolar capillaritis.
09:03
So that's we end up with
bleeding in the lungs
and glomerulonephritis
in the kidneys.
09:10
So see the picture there
walk back through it again.
09:13
What happens is those
antibodies right?
Their job is to attract
the white cells.
09:18
The antibodies attach to the
capillary basement membrane
when they attach to the
capillary basement membrane
that attracts the neutrophils
the neutrophils job,
they cause this major
inflammatory response
and we end up with
bleeding in the lungs,
because I see everything
leaking out through there
and glomerulonephritis
in the kidneys.