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DISEASES
Glomerular Syndrome
1. Acute nephritic syndrome
2. Rapidly progressive glomerulonephritis
3. Chronic renal falure
4. Asymptomatic hematuria/proteinuria
Nephrotic Syndrome
• Manifestations
– Massive proteinuria
– Hypoalbuminemia
– Generalized edema
– Hyperlipidemia and lipiduria
Minimal Change Disease
• Lipoid nephrosis, Nil disease, Idiopathic
nephrotic syndrome
• Most common cause of NS in children
• Dramatic response to steroids
• Of unknown cause – NSAID, allergic
reactions
• May present with acute renal failure
• Differentiate from focal segmental
glomerulosclerosis
Minimal Change Disease
• LM: few, if any changes
• IF: usually negative; may have mesangial
IgM
• EM: widespread effacement of podocyte
foot processes
– Villous hypertrophy of podocytes
– Absence of deposits
Focal Segmental
Glomerulosclerosis
• Classifications
– In association with other known conditions
• HIV, heroin, sickle cell disease, massive obesity
– As a secondary event reflecting glomerular
scarring
– As an adaptive response
– In certain inherited, congenital forms of NS
– As a primary disease --- idiopathic
•Failure to respond to steroids
•May have microscopic hematuria,
effacement
• accumulation in collapsed loop of matrix-like
material
•
Membranous
Glomerulonephritis
• Can be primary or secondary
• Secondary causes
– Drugs
– Underlying malignant tumors
– SLE
– Infections
– Metabolic disorders
•Some have asymptomatic proteinuria
•Nephrotic syndrome
paramesangial areas
•
•
Pathogenesis: deposition in glomeruli of
•
nephropathy
Poststreptococcal
Glomerulonephritis
• 1-4 weeks after a streptococcal infection
of the pharynx or skin
• 6-10 yo but may affect any age
• Group A beta- hemolytic streptococci
types 12, 4 and 1
• Acute nephritis syndrome: gross
hematuria, edema and hypertension
•A minority --- severe renal insufficiency
•Low C3
Ag-Ab complexes
•LM: proliferative, exudative (PMNs), +/-
crescents
•IF: granular GBM --- IgG +/- C3
•EM: subepithelial humps +/- small
subendothelial/mesangial deposits
•Course: resolves in vast majority of cases
•Dx: ASO up, complement down, nephritic
syndrome, HPN
•Nonstreptococcal acute GN
–Bacterial, viral and parasitic infections
Rapidly Progressive GN
• Crescentic GN
– Rapid and progressive loss of renal function
associated with severe oliguria and (if
untreated) death from renal failure within
weeks to months
–LM: over 50% of glomeruli with crescents
–IF:
•1/3 granular (immune complex GN, e.g. SLE, post-
infectious GN)
•1/3 linear (anti-GBM, e.g. Goodpasture’s disease)
•1/3 no deposits (e.g. PAN, Wegener’s, vasculitis)
–EM: glomerular deposits anywhere
–Course: terrible (progression to ESRD except
post-infectious)
Anti-GBM Disease
• Autoantibodies against the NC1 domain of
the alpha 3 chain of type IV collagen
(Goodpasture Ag)
• Associated with pulmonary hemorrhage
• LM: crescentic
• IF: strong linear staining along the GBM
Hereditary Nephritis
• Alport syndrome
– Nephritis accompanied by nerve deafness
and various eye disorders (lens dislocation,
post. Cataracts and corneal dystrophy)
– Males > females, hematuria
– Defective form of BM due to mutations in
genes that encode components of type IV
collagen
•In 80% x-linked inherritance
•In 15% autosomal recessive