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NEPHROTIC
SYNDROME
02/03/2024 Abel W(MD)
Outline
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Introduction
Causes
Idiopathic Nephrotic Syndrome
1. Causes of proteinuria
Abnormal permeability of the
glomerular capillary BM to plasma
protein.
Protein loss exceeds 40mg/m²/hr in
children and >3.5 gm/24hr in adults
It is primarily composed of albumin.
Edema appears when serum albumin
falls below 2.5g/dl.
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Cont’d
2. Mechanism of edema
Initiated by hypoalbuminemia.
Hypoalbuminemia decreased plasma oncotic
pressure transudation of fluid from Intravascular
compartment to interstitial space.
Decreased IV volume dec. renal perfusion
pressure activation of RAAS increase in
distal tubular reabsorption of sodium.
Dec. IV volume stimulate the release of ADH
increase reabsorption of water in02/03/2024
collecting duct.
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3. Mechanism of hyperlipidemia
a. The hypoproteinemia stimulate
generalized protein synthesis in the liver
including lipoproteins.
b. Reduced lipid catabolism due to reduced
level of lipoprotein lipase (loss in urine)
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Introduction
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Mesangial proliferation
is xrized by a diffuse increase in mesangial cells
and matrix on light microscopy.
Immunofluorescence microscopy might reveal
trace to 1+ mesangial IgM and/or IgA staining.
Electron microscopy reveals increased numbers
of mesangial cells and matrix as well as
effacement of the epithelial cell foot processes.
Approximately 50% of patients with this
histologic lesion respond to corticosteroid
therapy. 02/03/2024
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In FSGS, glomeruli show lesions that are both focal (present
only in a proportion of glomeruli) and segmental (localized
to ≥1 intraglomerular tufts).
The lesions consist of mesangial cell proliferation segmental
scarring on light microscopy.
Immunofluorescence microscopy is positive for IgM and C3
staining in the areas of segmental sclerosis.
E/M demonstrates segmental scarring of the glomerular tuft with
obliteration of the glomerular capillary lumen.
Similar lesions may be seen 2O to HIV infection, vesicoureteral
reflux, and intravenous use of heroin and other drugs of abuse.
Only 20% of patients with FSGS respond to prednisone. The
disease is often progressive, ultimately involving all glomeruli,
and ultimately leads to end-stage renal disease in most patients.
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Idiopathic Nephrotic Syndrome
Clinical Manifestations:
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elevated.
Serum complement levels are normal.
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Idiopathic Nephrotic Syndrome
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Complications:
Infection is a major complication of
nephrotic syndrome.
Factors for recurrent infection
Urinary losses of Igs and properdin factor B
that opsonize certain bacteria.
Defective cell-mediated immunity
Immunosuppressive therapy
Malnutrition, and edema or ascites acting as a
potential culture medium. 02/03/2024
Idiopathic Nephrotic Syndrome
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prednisone.
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Thank you
02/03/2024