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Nephrotic Syndrome

1. Defenition

The nephrotic syndrome is a clinical complex characterized by a number of renal

and extrarenal features, the most prominent of which are proteinuria of >3.5 g per 1.73

m2 per 24 h (in practice, >3.0 to 3.5 g per 24 h), hypoalbuminemia, edema,

hyperlipidemia, lipiduria, and hypercoagulability.

2. Etiology

1. Primary Kidney Diseases

a. Glomerulonefritis post Streptococcus

b. Idiopatic ( Lipoid, Membranos, Membrano-proliferatif)

2. Metabolic and Collagen Tissues Diseases

a. Diabetic Mellitus

b. Amiloidosis

c. Henoch-Schoenlein Purpura

d. Systemic Lupus Erytematosous

3. Mechanic Circulation Disturbance

a. Right Heart Syndrome

b. Renal Vein Thrombosis

4. Malignancy Diseases

a. Hodgkin

b. Limfosarcom

c. Mieloma Multipel

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5. Infections

Malaria, syphilis, abdominal typhoid, zoster herpes

6. Specific Toxin

Drugs and Metal

7. Congenital

8. Others

Hepar cirrhosis, Obesity, Pregnancy, Renal Transplantation.

3. Pathophysiology

In general, the greater the proteinuria, the lower the serum albumin level.

Hypoalbuminemia is compounded further by increased renal catabolism and inadequate,

albeit usually increased, hepatic synthesis of albumin. The under filling hypothesis

postulates that hypoalbuminemia results in decreased intravascular oncotic pressure,

leading to leakage of extracellular fluid from blood to the interstitium. Intravascular

volume falls, thereby stimulating activation of the renin-angiotensin-aldosterone axis and

the sympathetic nervous system and release of vasopressin (antidiuretic hormone), and

suppressing atrial natriuretic peptide release. These neural and hormonal responses

promote renal salt and water retention, thereby restoring intravascular volume and

triggering further leakage of fluid to the interstitium.

Hyperlipidemia is believed to be a consequence of increased hepatic lipoprotein synthesis

that is triggered by reduced oncotic pressure and may be compounded by increased

urinary loss of proteins that regulate lipid homeostasis. Low-density lipoproteins and

cholesterol are increased in the majority of patients, whereas very low density

lipoproteins and triglycerides tend to rise in patients with severe disease.

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Hypercoagulability is probably multifactorial in origin and is caused, at least in part, by

increased urinary loss of antithrombin III, altered levels and/or activity of proteins C and

S, hyperfibrinogenemia due to increased hepatic synthesis, impaired fibrinolysis, and

increased platelet aggregability.

Nephrotic syndrome can complicate any disease that perturbs the negative electrostatic

charge or architecture of the GBM and the podocytes and their slit diaphragms. Six

entities account for greater than 90% of cases of nephrotic syndrome in adults: minimal

change disease (MCD), focal and segmental glomerulosclerosis (FSGS), membranous

glomerulopathy, MPGN, diabetic nephropathy, and amyloidosis. Diabetic nephropathy

and amyloidosis, being manifestations of systemic diseases, are discussed in. Renal

biopsy is a valuable tool in adults with nephrotic syndrome for establishing a definitive

diagnosis, guiding therapy, and estimating prognosis.

DIFFERENTIAL DIAGNOSIS

Other potential diagnoses include vascular, cystic, and tubulointerstitial diseases;

papillary necrosis; hypercalciuria and hyperuricosuria; benign prostatic hypertrophy; and

renal calculi.

TREATMENT

The treatment principle of sindrom nephrotik based on patophysiology

1.Damage of glomerulus : Immunosupresif, anticoagulan, trombosit anti aggregation

2. lose of protein: Diit low protein

3. hipoalbuminemia and decreased of oncotik pressure : IVFD human albumin

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4. secretion of aldosteron : diuretic spironolakton

5. retention of natrium and water : diuretic furosemid and diit low salt

6. persisten oedem : drainage and ultrafiltrasi