Nephrotic Syndrome

Glemerular permeaiblity Difinition of Nephrotic Syndrome Causal Diseases Minimal change nephrotic syndrome Focal segmental glomerular sclerosis Membranous Glomerulonephritis

A. Glomerular Permeability

　

B. Difinition of Nephrotic Syndrome

1

1. Massive persistent proteinuria:

>3.5 g/day (adult),
>100 mg/kg/day or 300 mg/dl (child)

2

2. Hypoproteinemia:
or Hypoalbuminemia:

<6.0 g/dl (adult), <5.5 g/dl (infant)
<3.0 g/dl (adult), <2.5 g/dl (infant)

3

Hypercholesterolemia:

>250 mg/dl (adult), >200 mg/dl (infant)

4

4. Edema

C. Causal Diseases

Minimal change nephrotic syndrome (MCNS), Minimal change disease,
Minor glomerular abnormalities, Minimal change abnormalities

Focal segmental glomerulosclerosis (FSGS)

Membranous nephropathy, Membranous glomerulonephritis (MGN)

Mesangial proliferative glomerulonephritis (including IgA nephritis)

Membranoproliferative glomerulonephritis (MPGN), Mesangiocapillary glomerulonephritis (MCGN)

Hereditary nephrotic syndrome

Others

Age-associated Prevalence of Various Glomerular Diseases
in Nephrotic Syndrome

D. Minimal change nephrotic syndrome

Normal podocytes

Podocytes in minimal change NS

E. Focal segmental glomerulosclerosis (FSGS)

Classification

Primary
Classic
Tip lesion
Collapsing

Secondary

Human immunodeficiency virus-associated
Heroin-abuse
Vesicourethelic reflux nephropathy
Oligomeganephronia
Obesity
Analgesic nephropathy
Hypertensive nephrosclerosis
Sickle cell disease
Transplantation
Vasculitis
IgA nephropathy

LM: Focal (some, but not all glomeruli), Segmental (only a portion of the capillary tuft is involved) scars with a collapse of GBM, increase in extracellular matrix, and deposition of hyaline masses (hyalinosis), often with lipoid droplets and foam cells. Crescents may be formed.

IF: IgM and C3 are often present within the hyaline masses in the sclerotic areas

FSGS: two of the glomeruli are normal and one has a segmental scar (arrow), distant from the glomerular hilum (PAM).

F. Membranous Nephropathy (Glomerulonephritis) 　

Classification

Primary (Idiopathic)
Secondary

Neoplasia (carcinoma, lymphoma)
Autoimmune diseases (SLE, thyroiditis)
Infectious diseases (hepatitis B, hepatitis C, schistosomiasis)
Drugs (gold, mercury, nonsteroidal anti-inflammatory drugs, probenecid, captoril)
Other causes (kidney transplantation, sickle cell disease, sarcoidosis)

"Spike formation"

Change of doposition

Immunofluorescence microscopy

Electron-dense deposits

Exracellular matrix

Mesangial matrix
+Mesangial Cell

Glpmerular basement menbrane
(GBM)

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1	1. Massive persistent proteinuria:	>3.5 g/day (adult), >100 mg/kg/day or 300 mg/dl (child)
2	2. Hypoproteinemia: or Hypoalbuminemia:	<6.0 g/dl (adult), <5.5 g/dl (infant) <3.0 g/dl (adult), <2.5 g/dl (infant)
3	Hypercholesterolemia:	>250 mg/dl (adult), >200 mg/dl (infant)
4	4. Edema

	LM: Focal (some, but not all glomeruli), Segmental (only a portion of the capillary tuft is involved) scars with a collapse of GBM, increase in extracellular matrix, and deposition of hyaline masses (hyalinosis), often with lipoid droplets and foam cells. Crescents may be formed. IF: IgM and C3 are often present within the hyaline masses in the sclerotic areas
FSGS: two of the glomeruli are normal and one has a segmental scar (arrow), distant from the glomerular hilum (PAM).

"Spike formation"	Change of doposition

Immunofluorescence microscopy	Electron-dense deposits