|
Disease |
Most Frequent Clinical Presentation |
Pathogenesis |
Light Microscope |
F.M. (Fluorescence Microscope)
E.M. (Electron Microscope) |
Age Group
Affected |
Treatment and
Outcome |
 |
Minimal Change Disease |
Selective proteinuria
(Albumin)
|
Loss of foot processes
Loss of GBM polyanionic
sites
Appearance of villi on epithelial cells
|
Normal
Lipid in tubules |
F.M. = negative
E.M. = loss of foot processes,
lipid vacuoles |
#1 cause of
Nephrotic
Syndrome in
children, esp.
boys younger
than 6 yrs. old. |
Responds well to
corticosteroids.
No progression into
chronic renal failure |
| Focal Segmental Glomerular Sclerosis
(Lipoid Nephrosis) |
Non-selective
proteinuria
Hypertension
Microscopic
hematuria
|
Idiopathic
Lower renal mass (in
obese)
2 causes: heroin use, HIV
|
Focal and segmental
sclerosis
Hyalinosis
Adhesions to Bowman's Capsule
Hypercellular
mesangium
Thick B.M.
|
F.M. = IgM, C3
E.M. = Loss of foot processes, detachment of epithelium from B.M. |
Majority occur in older
children. Also
occurs in
adults. |
Does not respond to
corticosteroids.
Leads to renal failure. |
| Membranous
Nephropathy
(Glomerulonephritis) |
Persistent proteinuria
|
Idiopathic
2 causes: carcinomas,
SLE, hepatitis, Diabetes
Mellitus, thyroiditis,
drugs.
|
Glomeruli are enlarged
yet normocellular
No cellular proliferation
B.M. Thickening
|
F.M. = "Spike and Dome."
Granular IgG, C3
E.M. = Subepithelial immune deposits in B.M.,
thickened B.M. |
#1 cause of
Nephrotic syndrome in
adults |
Benefit of
corticosteroids is unknown. |
| Diabetic
Nephropathy
(Diabetic
Glomerulosclerosis) |
Proteinuria
No hematuria
|
Diabetic microangiopathy
Thickened B.M.
Massive mesangial growth
"Kimmelstiel Wilson"
nodular glomerulosclerosis
Diffuse glomerulosclerosis
|
Kimmelstiel-Wilson
Nodules are
pathognomonic.
Massive mesangial
hypercellularity.
|
F.M. = negative
E.M. = massive mesangial
growth, thickened B.M. |
Diabetics |
Progresses to renal
failure |
| Renal Amyloidosis |
|
Subendothelial and
mesangial amyloid deposits |
Amyloid deposits are
initially mesangial, producing mesangial widening without
hypercellularity.
Later, the amyloid
obliterates the lumen
PAS(-)
Congo-Red (+)
|
F.M. = negative
E.M. = characteristic criss-cross fibrillary proteins. |
Any age group |
Severe amyloid infiltration leads to renal
failure |
 |
Alport Syndrome
(Hereditary Nephritis) |
Recurrent hematuria
before age 20
Hypertension
Deafness and ocular
problems
|
Structural defect in Collagen
IV leads to leaky basement
membranes. |
Looks normal |
F.M. = negative
E.M. = glomerular B.M.
splitting |
Symptoms
appear before
age 20 |
Progresses to renal
failure |
| Benign Familial
Hematuria
(Thin B.M. Disease) |
Recurrent hematuria
Most frequent cause
of asymptomatic
hematuria.
|
Reduced thickness of glomerular B.M. |
Looks normal |
F.M. = negative
E.M. = reduced glomerular
B.M. thickness |
|
|
| Acute (Post-Streptococcal)
Glomerulonephritis |
Acute nephritis
Abrupt oliguria,
hematuria, facial
edema, hypertension.
|
Immune-complex mediated (Type-III hypersensitivity)
Occurs after Streptococcal
pharyngitis or Hepatitis-B
High ASO-titer, low C3
|
Glomerular
hypercellularity
Increase in endothelial
cells, mesangial cells,
and PMN's.
No increase in epithelial cells.
No B.M. thickening
|
F.M. = "lumpy-bumpy"
granular deposits of IgG and
C3
E.M. = Subepithelial (not
subendothelial) "humps,"
otherwise normal appearing
B.M. |
Common renal
disease in
childhood |
Return to normal in 8
weeks.
Complete recovery
without treatment
(especially in kids)
within 3 years. |
| SLE Nephropathy |
Degree of kidney involvement correlates
with prognosis in SLE. |
Anti ds-DNA antibodies. |
WHO I: Normal
WHO II: Increased
mesangial matrix
WHO III: Focal proliferation
WHO IV: Diffuse proliferation, worst.
WHO V: Identical to
Membranous
Nephropathy
|
F.M. = IgM, IgG + C3
Type-I: Granular appearance
Type-II: Pseudo-linear
appearance
|
|
|
| Focal Segmental
Glomerulonephritis |
IgA Nephropathy
(Berger's Disease):
Most common primary
glomerulonephritis |
Circulating IgA + fibronectin
(due to chronic liver disease) |
Mesangial cell proliferation
|
F.M. = Granular appearance,
IgG + C3
E.M. = Mesangial deposits |
Young men
15-30 |
|
| Henoch-Schonlein
Purpura |
Same as above, plus systemic
disease: purpura of extremities, arthritis, colicky abdominal pain. |
Mesangial cell proliferation, more serious than
above.
|
F.M. = Granular appearance,
IgG + C3
E.M. = Mesangial deposits |
Children |
|
| Endocarditis |
S. Aureus |
Subepithelial immune
deposits
|
F.M. = Granular appearance,
IgG + C3 |
|
Kidney disease resolves when infection
is cured. |
| Rapidly Progressive
Crescentic
Glomerulonephritis |
Wegener's: kidney + upper respiratory
tract.
Anuria
Oliguria
|
Inflamed glomerular capillaries
ANCA (+)
|
Cells accumulate in
Bowman's Capsule
Fibrin trapped in
glomeruli
Epithelial cell proliferation
Macrophage, PMN infiltrates
|
F.M. = Pauci-immune. Irregular
E.M. = wrinkling, discontinuity of B.M. |
|
Must be treated or it
will go to renal failure within weeks. |
| Goodpasture Syndrome
(Anti-BM Antibody
Disease) |
Lung (hemoptysis) +
kidneys (hematuria) |
Anti-B.M. antibodies,
against Type-IV collagen |
Similar to Crescentic
glomerulonephritis, as
above. |
F.M. = Linear pattern, IgG +
C3
E.M. = No immune complex
deposits |
Males 25-30 |
Responds to
immunosuppressive
therapy and
plasmapheresis |
| Membranoproliferative
Glomerulonephritis
(Mesangiocapillary
Glomerulonephritis) |
|
|
B.M. thickening and
cellular proliferation
Mesangial expansion
makes glomerular B.M.
appear as though it
were in two layers
|
E.M. = "Tram-track" appearance, resulting from
double-layer appearance of
glomerular B.M. |
|
|
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