Glomerular disease Flashcards
Features of glomerular haematuria:
Inflammation of glomerulus –>
- Haematuria: 80% red cells dysmorphic (phase contrast microscope)
- RBC casts (pathognomonic of glomerulonephritis) - light microscope
- Proteinuria
- Reduced renal function
Features of nephritic syndrome:
Inflammation of glomeruli.
Symptoms usually abrupt in onset:
P - Proteinuria (<3g/day) H - Hypertension A - Azotemia R - RBC casts / Haematuria O - Oliguria
Features of nephrotic syndrome:
= Proteinuria ≥3g/1.73m2/24hrs.
Secondary features: - Hypoalbuminaemia - Oedema - Hyperlipidaemia (mixed hypercholesterolaemia / hypertriglyceridaemia)
+/- Hypertension
+/- Haematuria
+/- Renal impairment
LOOKUP - immune effects of minimal change disease ?Tcells
Tcell dysfunction
Nephrotic syndrome
Glomerulopathies with both low c3 and low c4?
Low C3 & C4:
SLE - diffuse
Membranoproliferative Type I
Cryoglobulinaemia
Glomerulopathies with low c3 but normal c4?
Low c3, normal c4:
SLE - diffuse Membranoproliferative Type II PSGN (post-infectious) Chronic infection Shunt nephritis HUS/TTP Cholesterol emboli
Pathological change in minimal change disease?
Fusion of podocyte foot processes, seen on electron microscopy.
Loss of charge selectivity of glomerular BM.
Leads to nephrotic syndrome.
What is the most common cause of idiopathic nephrotic syndrome in children?
Minimal change disease.
Presents as nephrotic syndrome.
HTN, haematuria, renal impairment are uncommon.
May follow URTI.
Can be complicated by thrombosis, AKI or infections.
Treatment with steroids shortens the course of the illness.
Minimal change disease in adults:
- presentation?
- secondary causes?
M:F = 1
Nephrotic syndrome.
Compared with paediatric cases, greater prevalence of microscopic haematuria / HTN / renal impairment.
Majority are primary.
Secondary causes: neoplasia, drugs, infection, atopy, MC superimposed on another renal disease.
Treatment of minimal change disease (adults):
- High dose prednisone for initial treatment or for relapse.
Decrease dose when free of proteinuria (sustained). - Steroid resistant: alkylating agents or cyclosporine.
FSGS vs MCN:
Minimal change nephropathy: fusion of podocyte foot processes.
FSGS: effacement of foot processes.
Both present as nephrotic syndrome.
FSGS more likely to present with mild haematuria, HTN, impaired renal function.
Both can be steroid-responsive, but FSGS often slow / no response (except Tip Variant - overlap with MCN and usually steroid-responsive).
FSGS is progressive.
MCN usually completely resolves.
Primary Causes of FSGS:
Primary causes FSGS:
- Gene defects affecting glomerular apparatus:
slit diaphragm proteins (nephrin, podocin, etc) and cytoskeletal proteins.
Hereditary conditions:
- Mitochondiral cytopathies (kidney disease + DM + hearing loss + muscle weakness or macular pattern dystrophy)
- WT1 mutation
(Wilms Tumour suppressor gene) - regulates podocyte functions
Secondary Causes of FSGS:
Secondary Causes of FSGS:
- Hyperfiltration (shear stress on podocytes)
- Hypertensive Nephrosclerosis
- Viruses: HIV, parvovirus B19
- Drugs: heroin, lithium, pamidronate, interferon, sirolimus, calcineurin inhibitors
- Aging
Causative agents in post-infectious GN?
- Children: majority causes by streptococcal throat or skin infections.
- onset 1-3 weeks post pharyngitis
- 3-6 weeks post skin infection - Adults / elderly:
Strept appox. 30%
Staphylococcal infection more common cause
- latent period short than with strept
- site: skin > resp tract > UTI
- usually immunocompromised: T2DM, malignancy
Immunosuppressive therapy in PIGN?
No role for immunosuprressive therapy or plasmapheresis in PIGN.
Supportive therapy only
- control HTN
- electrolyte and fluid management
- RRT if indicated
