Nephrotic syndrome

Figure 1.

Nephrotic edema.

Figure 2. Nephrotic edema.

NEPHROTIC SYNDROME
Pathophysiology
-

Proteinuria
Hypoalbuminemia
Edema
Hyperlipidemia

Cause (diagnosis and differential diagnosis)

-

Systemic renal disease

hepatitis B associated glomerulonephritis, Henoch-Schonlein purpura,
systemic lupus erythematosus, diatetes mellitus, amyloidosis

Idiopathic nephrotic syndrome

Complications
-

Infection
Coagulation disorders
Protein malnutrition and dyslipidemia
Acute renal failure

Pathophysiology

Proteinuria

Proteinuria can be caused by systemic

overproduction, tubular dysfunction, or
glomerular dysfunction. It is important to
identify patients in whom the proteinuria is
a manifestation of substantial glomerular
disease as opposed to those patients who
have benign transient or postural
(orthostatic) proteinuria.

Heavy proteinuria (albuminuria)

Figure 3.

Hypoalbuminemia

Hypoalbuminemia is in part a consequences of

urinary protein loss. It is also due to the
catabolism of filtered albumin by the proximal
tubule as well as to redistribution of albumin
within the body. This in part accounts for the
inexact relationship between urinary protein
loss, the level of the serum albumin, and other
secondary consequences of heavy albuminuria .

Edema

The salt and volume retention in the NS may occur through at

least two different major mechanisms.
In the classic theory, proteinuria leads to hypoalbuminemia, a
low plasma oncotic pressure, and intravascular volume
depletion. Subequent underperfusion of the kidney stimulates
the priming of sodium-retentive hormonal systems such as the
RAS axis, causing increased renal sodium and volume
retention, In the peripheral capillaries with normal hydrostatic
pressures and decreased oncotic pressure, the Starling forces
lead to transcapillary fluid leakage and edema .

Edema

In some patients, however, the intravascular volume

has been measured and found to be increased along
with suppression of the RAS axis. An animal model of
unilateral proteinuria shows evidence of primary renal
sodium retention at a distal nephron site, perhaps due
to altered responsiveness to hormones such as atrial
natriuretic factor. Here only the proteinuric kidney
retains sodium and volume and at a time when the
animal is not yet hypoalbuminemic. Thus, local factors
within the kidney may account for the volume retention
of the nephrotic patient as well.

Figure 4.

Hyperlipidemia

Most nephrotic patients have elevated levels of total and

low-density lipoprotein (LDL) cholesterol with low or
normal high-density lipoprotein (HDL) cholesterol .
Lipoprotein (a) [Lp(a)] levels are elevated as well and
return to normal with remission of the nephrotic
syndrome. Nephrotic patients often have a
hypercoagulable state and are predisposed to deep vein
thrombophlebitis, pulmonary emboli, and renal vein
thrombosis.

Cause

Table 2 CAUSES OF THE NEPHROTIC SYNDROME

Table 3a
NEPHROTIC SYNDROME ASSOCIATED WITH
SPECIFIC CAUSES (SECONDARY NEPHROTIC SYNDROME)

Table 3b
NEPHROTIC SYNDROME ASSOCIATED WITH
SPECIFIC CAUSES (SECONDARY NEPHROTIC SYNDROME)

Diagnosis and Differential diagnosis

Initial evaluation of the nephrotic patient

includes laboratory tests to define whether
the patient has primary, idiopathic
nephrotic syndrome or a secondary cause
related to a systemic disease.

Common screening tests include the fasting blood sugar

and glycosylated hemoglobin tests for diabetes, and
antinuclear antibody test for rheumatoid disease, and
the serum complement, which screen for many immune
complex-mediated disease (Table 3), In selected
patients, cryoglobulins, hepatitis B and C serology, antineutrophil cytoplasmic antibodies (ANCAS), anti GBM
antibodies, and other tests may be useful. Once
secondary causes have been excluded, treating the
adult nephrotic patient often requires a renal biopsy to
define the pattern of glomerular involvement.

Complications

Infection
Coagulation disorders
Protein malnutrition and
dyslipidemia
Acute renal failure

It leads to a multitude of other consequences ,

such as predisposition to infection and
hypercoagulability. In general, the diseases
associated with NS cause chronic kidney
dysfunction, but rarely they can cause ARF. ARE
may be seen with minimal change disease, and
bilateral renal vein thrombosis.

Treatment
1. General treatment
2. Symptomatic treatment
(e.g.diuresis to relieve
edema, treating
dyslipidemias,
anticoagulate treatment,
etc.)
3. Immunosupressive
treatment

Thank you