00:00
Type I MPGN. Most common type it is.
00:03
Nephrotic syndrome presentation, what does that mean to you?
You begin with protein loss.
00:09
And greater than 3.5 grams of protein per day.
00:11
Hypoalbuminemia and generalized edema.
00:15
You would have loss of antithrombin III,
Anticoagulation, maybe a little bit of hypertension.
00:20
That's your nephrotic presentation, isn't it?
Some cases it will have nephritic.
00:25
I told you earlier that MPGN and DPGN will have a little bit of nephritic, a little bit of nephrotic.
00:33
Now, most of this with MPGN in fact, is nephrotic.
00:36
Associated with hepatitis C, more common or cryoglobulinemia.
00:42
What does that even mean?
That means that you might have conditions
and at some point in time, when you get into cryoglobulinemia,
there are three different types.
00:51
One, two, and three, cryoglobulinemia.
00:52
The big ones.
00:53
Type I associated with multi-myeloma.
00:57
Type II and III, more or less associated with infections and autoimmune diseases.
01:02
What do all of these have in common? Autoimmune diseases.
01:05
Monoclonal gammopathies and hepatitis C could be put in there as well.
01:10
They are associated with antigen-antibody complexes. Okay.
01:19
Two components, circulating through the body.
01:23
All of a sudden cryo.
01:24
What does that mean? Cold exposure.
01:29
The globulins will precipitate resulting in all kinds of issues in your patient.
01:34
Mostly, what do you know this about Waldenstrom?
In Waldenstrom, what then happens when you have your globulins that will precipitate in the blood?
It’s almost like a thrombus, isn't it?
And so therefore it’s hyperviscosity. Hyperviscosity.
01:52
Cryoglobulinemia is a big deal, a big clinical condition or syndrome,
and I've given you a few examples of type I, type II, type III.
02:01
My point at this point though, what kind of MPGN are we dealing with? Type I.
02:07
In the previous discussion what branch of our MPGN would that follow?
Immunecomplex mediated or would it be complement-mediated?
Good, immune complex mediated. Much more common, type I.
02:23
Which type I? On your light microscopy,
can't really tell the difference between type I and type II, that I can tell you for certain.
02:31
However, where you can start seeing differences?
When you hear at this point, it should be reflexive.
02:39
When you say subendothelial immune complexes, what kind of biopsy is this?
Good. Electron microscopy.
02:46
If you see a granular pattern, what kind of biopsy is that?
Good. Immunofluorescence.
02:51
Aren’t you feeling good about this now?
You're reading, as if you’re reading a clinical vignette.
02:55
You're reading as if it was a medical journal.
02:56
Lancet, New England’s Journal Of Medicine. Whatever it may be.
02:59
And these are the kind of information that it has and you should be able to-
These are the kind of questions, information that everyone expects you to know,
at your level of medical education.
03:11
The immune complexes activate classical and alternative complement pathway.
03:16
You know that already.
03:17
Electron microscopy shows tram-track.
03:22
What’s that even mean?
Tram-track means, the membranes being involved, membranoproliferative.
03:28
The membranes been involve, what happens?
Split of the glomerular basement membrane by ingrowth of mesangium.
03:37
Tram-track, is that specific? No. It’s not.
03:41
Anything that weakens that membrane,
do you think that you would then have deposition of mesangium and splitting of it?
Sure. Another good differential, Alport Syndrome.
03:51
Hypertension, 35% of the majority of your patients will have hematuria.
03:56
Remember, MPGN? Nephritic/Nephrotic presentation.
03:59
Majority will progress to chronic renal failure.
04:02
Which was most common, type I or type II? Type I.
04:06
If it’s type I, what kind of association? Immune complexes.
04:10
Examples, autoimmune disease, infections such as Hepatitis C and cryoglobulinemia.
04:17
And we have monoclonal gammopathies, including Waldenstrom.
04:21
With type I, response to corticosteroids not established, let it go.