0:00
Hello.
My name is Moin Saleem,
I'm the Professor
of Paediatric Renal Medicine
at the University of Bristol
in the UK
and the Children's Renal Unit.
I'm gonna talk today about focal
segmental glomerulosclerosis.
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So first to put this into context;
this is about the kidney's filter.
The kidney, I call the world's
most sophisticated filter.
And the filtration unit
of the kidney is the glomerulus.
The glomerulus
is a specialized capillary bed.
And we have about a million
to two million of these each.
0:34
And when the glomerulus breaks down
and the filtration breaks down,
we get what is called,
the idiopathic nephrotic syndrome.
And this results
in heavy proteinuria,
so protein leaks from
the bloodstream into the urine,
low-levels of protein
in the blood particularly happens.
And so you get hypoalbuminaemia
which leads to
low osmotic pressures
in the inter-vascular space.
And which leads to
peripheral tissue oedema,
which you can see here.
Rugby player Jonah Lomu,
is a very famous patient with
nephrotic syndrome, who sadly died.
1:05
I'm a pediatric nephrologist
and I see mainly children,
so clinically in children the size
of oedema are in the lower limbs,
you can see it here.
The abdomen where you get
ascites. And facial oedema,
as you can see here.
1:19
So idiopathic nephrotic syndrome
has various
different classifications
and are usually based on
observational signs.
It can be classified histologically;
so it can be classified
as minimal change
nephrotic syndrome
or as focal
segmental glomerulosclerosis.
I'll expand on that in a bit.
It can also be classified
by steroid response.
So you can have steroid
sensitive nephrotic syndrome,
or steroid resistant
nephrotic syndrome
and various different nuances
of those different responses.
Both of these classifications
have overlaps
and they're not based
on the mechanism
of the underlying disease.
