Focal segmental glomerulosclerosis

Published on August 31, 2016   38 min

Other Talks in the Series: The Kidney in Health and Disease

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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.
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.
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.
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.
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.