Nephrology Flashcards
Differential diagnosis of Haematuria?
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A genetic susceptibility and an exaggerated immune response to an URTI is the cause of a primary IgA nephropathy, but what are the causes of a secondary IgA nephropathy.
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Differential diagnosis for micro/macro scopic Haematuria in an otherwise asymptomatic patient who has had Urological malignancy ruled out?
Genetic disorders
Thin membrane disease (relatively benign - painless haematuria)
Alports disease (Rare genetically inherited disorder that causes hearing loss, eye problems and an abnormal Glomerular Basement Membrane) - can cause renal failure - most patients end up on dialysis.
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How do you diagnose IgA nephropathy?
Kidney biopsy
Only indicated if:
- Haematuria and proteinuria
- Haematuria w/ decling kidney fnc
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Kidney biopsy goes through 3 phases, what are they?
- Light Microscopy (first image shows classic crescent shape of IgA nephropathy)
- Immunofluorescence (IHC)
- Transmission electron microscopy (TEM)
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Which scoring system can be used to classify prognosis in IgA nepropathy
Oxford Classification (basically characterizes the the mesangium)
MEST-C
Mesangial hypercellularity
Endothelial Hypercellularity
Segmental Glomeruloscelrosis
Tubular atrophy / Interstitial Fibrosis
Crescent formation
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The hallmark of treatment in IgA nephropathy is ____.
ACE-i
*Not steroids unless acute decline in renal function*
Most common cause of Glomerulonephritis?
IgA nephropathy
- especially prevalent in Caucasians and East Asians
- Men 2:1 Women
- Most common in 2nd and 3rd decade of life
*Remember can present with loin pain and thus may mimic renal calculi*
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Pathophysiology of IgA nephropathy?
Genetic Susceptibilty - URTI - Exaggerated Immune Response - Antibody Complex form in Mesangium - Endothelial/Tubular damage
(i.e Endothelium/GBM/Podocyte interface is damaged)
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Prognostic markers for glomerulonephritis other than the Oxford histology score (MEST-C)?
BP
Proteinuria (Remember proteinuria is significant in nephrology if >1g / 24hrs)
Renal function
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Glomerular Disease:
Nephrotic vs Nephritic Syndrome
The key with nephrotic syndrome is an excess amount of protein in the urine, whereas nephritic syndrome is where there is an excess amount of blood in the urine.
Nephrotic Syndrome:
Injury to Podocytes (Proteinuria)
Changed mesangial architecture
(ex. Minimal change disease/ Diabetic nephropathy/ Amyloid)
Nephritic Syndrome:
Inflammation
Reactive cell proliferation
GBM disruption (slit membrane) - blood leaks into urine
Crescent formation
(Ex. Post-Streptococcal infection/Anti-GBM aka goodpasture’s disease - affects lung BM aswell/ANCA small vessel vasculitis)
As outlines above nephrotic syndrome is characterised by very heavy proteinuria (> 3.5 g/24 hrs), hypoalbuminaemia and oedema.
The Nephritic syndrome is characterised by the presence of haematuria frequently in association with hypertension, oliguria, fluid retention and usually (but not always) reduced / declining renal function. Patients with nephritic syndrome may also exhibit varying degrees of proteinuria, including nephrotic-range proteinuria; the prominence of haematuria on
dipstick should, however, alert the physician to the possibility of a glomerulonephritis.
Indeed, it is important to recognise that the characteristic features of nephritic syndrome and nephrotic syndrome do not always present in isolation, but should be considered to be the extreme phenotypes at either end of a spectrum of presentations.
Students should learn that damage to the podocytes causes (heavy)
proteinuria (see graph above) whilst the glomerular injury caused by Autoantibodies or Immune complex deposition usually causes haematuria +/- mild to moderate proteinuria.
At the other end of the spectrum, inflammation leads to cell damage and proliferation, breaks form in the glomerular basement membrane (GBM) and blood leaks into urine. In its extreme form, with acute sodium retention and hypertension, such disease is labelled nephritic syndrome.
The histology to the right shows a **glomerulus with many extra nuclei from proliferating intrinsic cells**, and influx of inflammatory cells leading to crescent formation (arrows) in response to severe post-infectious glomerulonephritis.
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A blood test can be used to screen for post-infectious (usually streptococcus) glomerulonephritis, which circulating proteins are looked for?
Anti-streptolysin O titre (ASOT)
C3, C4 (complement proteins)
Draw out the RAAS system.
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____ and ___ are the two most common causes of CKD.
Diabetic Nephropathy
Hypertensive Nephropathy
*Both lead to nephrosclerosis*
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What are the 3 ways in which multiple myeloma causes renal disease?
Light chain cast nephropathy (intralubular collection of light chain-protein complexes) - most common
Light chain deposition disease (deposits in the glomerulus)
AL Amyloidosis
Hypercalcaemia and Hyperuricaemia (Tumour lysis syndrome causes tubular crystallization of uric acid) also possible causes of MM induced nephropathy.
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