Fichas
Glomerular disease
Fichas del conjunto (59)
- 1what are the general consequences of GN?inflammation of the glomeruli and nephrons:
1) glomerular damage -> restricted blood flow -> compensatory HTN
2) filtration mechanism damaged -> protein + blood in the urine
3) loss of filtration capacity -> AKI - 2what is the general spectrum of disease encountered with GN?depends on degree of inflammation and cause but:
1) BP - normal to malignant
2) urine dipstick - proteinuria (mild to nephrotic) and haematuria (mild to macroscopic)
3) renal function - normal to severe impairment - 3what syndromes might someone with a GN present with?nephrotic
nephritic - 4what is the gist of nephrotic syndrome?normal BP to mild HTN
proteinuria >3.5g/day
normal GFR to mild impairment - 5describe the gist of the nephritic syndrome?moderate to severe HTN
mild - macro haematuria
moderate to severe GFR impairment - 6what are the 1y causes of nephrotic syndrome?membranous GN
minimal change dz
FSGS
mesangiocapillary GN - 7what are the 2y causes of nephrotic syndrome?DM
SLE (class V nephritis)
amyloid
hep B/C - 8what are the 1y causes of nephritic syndrome?IgA nephropathy
mesangiocapillary GN - 9what are the 2y causes of nephritic syndrome?post strep GN
vasculitis
SLE
anti -GBM dz (goodpastures)
cryoglobulinaemia - 10what investigation categories are appropriate for the GN?bloods
Urine
Imaging
renal bx - 11bloods in GN?FBC
U+E
ESR
CRP
Ig
electrophoresis
complement (C3,4)
autoab: ANA, ANCA, anti-dsDNA, anti-GBM
blood culture
ASOT
HBsAg, anti-HCV - 12urine tests in GN?RBC casts
MC+S
Bence jones protein
ACR - 13what imaging is appropriate in GN?CXR
renal USS - 14why renal bx in GN?gives most information
can classify the GN
1) what? (mesangial cells, capillaries, BM, endothelium)
2) extent? (focal vs diffuse)
3) glomerular extent? (segmental vs global)
4) IF (Ig deposition, complement, immune complexes, pauci immune)
5) EM - 15general mangt in GN?refer to nephrologist
keep BP <130/80 or <125/75 if proteinuria >1g/d
ACEI or ARB - no benefit from dual therapy - 16what is IgA nephropathy?commonest GN in developed world
macro/micro haematuria
sometimes nephritic syndrome - 17who is the typical IgA nephropathy patient?young male
episodic macroscopic haematuria
elevated IgA possibly from infection which forms immune complexes and deposits in mesangial cells - 18renal bx in IgA nephropathy?mesangial proliferation
IF - deposits of IgA + C3 - 19Tx of IgA nephropathy?BP control w/ ACEi
if nephritic then IS can slow decline of renal fxn
px worse if HTN, male, proteinuria or renal failure at presentation, 20% develop ESRF over 20 yrs - 20what is henoch schonlein purpura?systemic variant of IgA nephropathy
small vessel vasculitis - 21what are the features of HSP?purpuric rash on extensor surfaces esp legs
flitting polyarthritis
abdo pain (GI bleeding)
nephritis - 22dx HSP?clinical
confirm w/ IF +ve for IgA + C3 in skin or renal bx (same as IgA nephropathy) - 23tx for HSP?same as for IgA nephropathy
15% nephritic go to ESRF
if nephritic + nephrotic 50% go to ESRF - 24what is anti-GBM dz?anti glomerular basement membrane
also called goodpastures
auto ab to type 4 collagen
pulmonary haemorrhage can also occur as type 4 collagen also in BM in lung - 25presentation and tx for anti-GBM dz?haematuria/nephritic syndrome
AKI
tx - plasma exchange, steroids +/- cytotoxics- start early and can have full recovery w/ relapses rare
px poor if dialysis dependent at presentation - 26what is post strep GN?diffuse, proliferative
1-12 wks post sore throat/skin infxn
strep ag deposited on glomerulus - host reaction + immune complex formation - 27how does post strep GN present?nephritic syndrome
- 28how is post strep GN dx?clinical - history
bx usually not done unless atypical presentation - inflammation of mesangial and endothelial cells, IF: IgG + C3
serology - ASOT elevated + elevated C3 - 29tx post strep GN?supportive - 95% regain fxn
- 30what is rapidly progressive GN?most aggressive
can cause ESRF in days
3 categories - all have crescents affecting the glomeruli on bx - 31what are the categories of RPGN?immune complex dz- 45%
pauci-immune dz - 50%, most ANCA +ve
anti-GBM dz - 3% - 32what immune complex dz cause RPGN?post infectious
SLE
IgA/HSP - 33what pauci immune dz cause RPGN?granulomatosis w/ polyangiitis (wegener's)
microscopic polyangitis
churg strauss - 34what are the clinical features of RPGN?AKI +/- systemic features (fever, myalgia, wt loss, haemoptysis)
pulm haemorrhage commonest COD in anca+ve - 35tx for RPGN?high dose IV steroids + cyclophosphamide +/- plasma exchange
5 yrs @80% - 36what is the nephrotic syndrome?proteinuria >3.5g/d
hypoalbuminaemia (<25g/L)
oedema - always dipstick when oedema so don't miss renal dz
also often severe hyperlipidaemia - total chol >10 - 37what is the pathophysiology of the nephrotic syndrome?podocyte injury - usually wrap around glomerular capillaries and maintain filtration barrier - breakdown = heavy protein loss
- 38what kind of oedema do nephrotic syndrome patients have?pitting
- 39what is the ddx for nephrotic syndrome?CCF - check JVP, pulm oedema, usually mild proteinuria
liver dx - would be low albumin - 40what to suspect in children w/ nephrotic syndrome?minimal change dz
trial of steroids will resolve most - avoid bx unless no steroid response or if likely another cause EG: <1yr, family hx, extrarenal dz eg arthritis, rash, anaemia; renal failure, haematuria. - 41assessment of nephrotic syndrome in adults?renal bx + full Gn assessment
- 42why might renal bx be difficult in pt w/ nephrotic syndrome?hypercoagulable state
gross oedema - 43what are the complications of nephrotic syndrome?infection - cellulitis, strep, SBP - due to low serum IgG, low complement and low T cell fxn (IS tx)
thromboembolism - DVT/PE, renal vein thrombosis. hypercoagulable - increased clotting factors + plt abnormalities
hyperlipidaemia - high chol + triglycerides - from hepatic lipoprotein synthesis in response to low oncotic pressure - 44how is nephrotic syndrome tx-ed?1) reduce oedema - loop diuretics (IV as gut oedema can prevent oral absorption) Fluid and salt restrict, monitor wt.
2) reduce proteinuria - ACEi/ARB
3) reduce complications - anticoagulate, start statin, tx infections and vax (pneumovax II, flu)
4) tx underlying cause - infxn, malignancy, systemic dz, stop drugs. - 45what can cause MCD in adults?idiopathic
NSAIDs
hodgkin's lymphoma - 46what is MCD on bx?normal on LM
EM - effaced podocyte foot processes - 47tx for MCD?most remiss w/ steroids but ALSO relapse
frequent relapses - cyclophophamide/tacromilus/ciclosporin
px - <1% go to ESRF - 48what is membranous nephropathy?most idiopathic
malignancy/ hep B, gold/penicillamine/NSAIDs
AI - thyroid, SLE
dx - diffusely thick GBM + IgG + C3 deposits on IF - 49tx for membranous nephropathy?tx underlying cause
idiopathic - ACEi/ARB, diuretics
complete spontaneous remission 30%
partial spontaneous remission 24-40% @ 5yrs
future - IS tx as phospholipase A2 receptor ab in 80% - 50what is mesangiocapillary GN?two types
IC mediated
complement mediated - 51what is IC mediated MCGN?immune complexes deposit in kidney + activate complement
usually underlying cause eg hep C, SLE, monoclonal gammopathy - 52what is complement mediated MCGN?less common type
persistent activation of alternative complement pathway (unlike IC - classical)
can have extra-renal probs eg drusen - 53bx in MCGN?mesangial and endocapillary proliferation
thick capillary BM
double countouring (tramline) capillary walls
IF - Ig staining, complement or light chains
EM - electron dense deposits - 54what is the tx for MCGN?underlying cause
ACEi/ARB for all
IS if rapid progression - steroids/cyclophos
px - poor where no underlying cause, ESRF can recur in transplants - 55what is FSGS?focal segmental glomerular sclerosis
idiopathic/secondary
presents as nephrotic syndrome or proteinuria - 56what are the 2y causes of FSGS?vesicoureteric reflux
IgA nephropathy
Alport's syndrome
vasculitis
sickle cell
heroin
HIV - associated with the collapsing subtype - poor px - 57bx findings in FSGS?segmental scarring of some glomeruli
IF - IgM + C3 deposits - 58what is the tx for FSGS?corticosteroids in 30%
cyclophosphamide/ciclosporin - 59what is the px for FSGS?untx - ESRF
spontaneous remission <10%
if present w/ abnormal renal fxn - poorer px - 30-50% to ESRF
recurs in 20% transplanted kidneys - may respond to plasma exchange
Información
- Descripción
- University nephrology Fichas sobre Glomerular disease, creado por roatf1 el 27/10/2014.
- Creado por
- roatf1
- Visitas
- 15
- Valoración