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Printable Handouts
Navigable Slide Index
- Introduction
- The kidney: the world’s most sophisticated filter
- Idiopathic nephrotic syndrome
- Oedema in children
- Classifications of idiopathic nephrotic syndrome
- Minimal change nephrotic syndrome (MCNS)
- Focal segmental glomerulosclerosis (FSGS)
- Nephrotic syndrome: steroid response classification
- Nephrotic syndrome: disease of filtration barrier
- Nephrotic syndrome: current clinical classification
- Structure of the glomerulus
- The glomerular filtration barrier
- Actin filaments & neighbouring foot processes
- Podocyte slit diaphragm as a ‘nerve centre’
- Podocyte morphology in health and disease
- Slit diaphragm based cytoskeletal arrangement
- The podocyte is a dynamic cell
- Signals to control podocyte behaviour
- Reclassification of idiopathic nephrotic syndrome
- Steroid resistant nephrotic syndrome (SRNS)
- Congenital nephrotic syndrome
- Genes associated with isolated renal disease
- Genes associated with syndromes involving SRNS
- Nephrotic genes expressed in the podocyte
- The UK study of NephroS
- Benefits of the Nephros approach
- Next generation sequencing in NephroS
- Health services guidelines for SRNS
- Nephrotic genes and associated syndromes
- New Genes discovered
- Nuclear pore complex genes
- Circulating factors in nephrotic syndrome
- Integrin activation & podocyte motility changes
- Integrins: important effectors in podocyte motility
- Circulating factor disease
- Podocyte derived biomarkers & circulating factors
- How to search for a circulating factor
- Identifying biomarker for ‘circulating factor disease’
- Direct test of circulating factor on podocytes
- Defining nephrotic syndrome pathogenesis
- Actin remodelling pathway & disease plasma
- VASP phosphorylation & nephrotic plasma
- VASP phosphorylation specificity in podocytes
- Disease plasma & increase in podocyte motility
- Circulating factor and its surface receptor
- FSGS relapse plasma & excess protease activity
- Protease activated receptors (PARs)
- Signalling pathways for enhanced motility
- Podocytes & breakdown of filtration
- Clinical clues for circulating factor disease
- Early steroid sensitivity & circulating factor disease
- Initial steroid sensitivity and risk of recurrence
- Genetic abnormalities and risk of recurrence
- Forest plot odds ratios for recurrence
- Re-classification of idiopathic nephrotic syndrome
- Take home messages
- Acknowledgements
Topics Covered
- The podocyte as the central cell of the glomerular filtration barrier
- Podocyte function through cytoskeleton regulation
- Pathological altering of cytoskeletal dynamics (genetic defects vs. circulating factors)
- Restoring normal balance using novel drugs/compounds
Links
Series:
Categories:
Therapeutic Areas:
Talk Citation
Saleem, M. (2016, August 31). Focal segmental glomerulosclerosis [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved October 8, 2024, from https://doi.org/10.69645/GDHF7852.Export Citation (RIS)
Publication History
Financial Disclosures
- Prof. Moin Saleem has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.
Other Talks in the Series: The Kidney in Health and Disease
Transcript
Please wait while the transcript is being prepared...
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.
0:13
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.