Nephrotic syndrome

Figure 1. Nephrotic edema.

Figure 2. Nephrotic edema.

Nephrotic syndrome This is characterized by proteinuria (Typically > 3.5g/24h), hypoalbuminemia ( less than 30g/dL ) and edema. Hyperlipidaemia is also present. Primary and secondary causes are summarized in Table 2, 3 In practice, many clinicians refer to nephrotic range proteinuria regardless of whether their patients have the other manifestations of the full syndrome because the latter are consequences of the proteinuria.

Pathophysiology

ProteinuriaProteinuria 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.

HypoalbuminemiaHypoalbuminemia 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 .

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.

Edema

Figure 4.

HyperlipidemiaMost 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)

Pathology patterns and clinical presentations of idiopathic nephrotic syndome

In adults, the nephrotic syndrome is a common condition leading to renal biopsy. In many studies, patients with heavy proteinuria and the nephrotic syndromes have been a group highly likely to benefit from renal biopsy in terms of a change in specific diagnosis, prognosis, and therapy. Selected adult nephrotic patients such as the elderly have a slightly different spectrum of disease, but again the renal biopsy is the best guide to treatment and prognosis (Table 2, 3).

Renal biopsy

PRIMARY NEPHROTIC SYNDROME Minimal Change Disease Focal Segmental Glomerulosclerosis Membranous Nephropathy Membranoproliferative Glomerulonephritis (MPGN)

Treatment1. General treatment2. Symptomatic treatment (e.g.diuresis to relieve edema, treating dyslipidemias, anticoagulate treatment, etc.)3. Immunosupressive treatment

Pemberian prednisolonStandard60mg/m2/hari selama 4 minggu40 mg/m2/ selang seling dari miggu ke 5 8Jangka panjang60mg/m2/hari selama 4 minggu60 mg/m2/ selang seling dari miggu ke 5 650 mg/m2/ selang seling dari miggu ke 7 840 mg/m2/ selang seling dari miggu ke 9 1030 mg/m2/ selang seling dari miggu ke 11 1220 mg/m2/ selang seling dari miggu ke 13 1410 mg/m2/ selang seling dari miggu ke 15 16

RESPON TERHADAP PEMBERIAN PREDNISONKOMPLIT REMISIPROTEIN URIA: TRACE ATAU NEGATIFPROETINURIA 24 JAM

TIDAK RESPON TERHADAP PEMBERIAN PREDNISONPROTEIN URIA > 3 GRAM / HARI SETELAH 16 MINGGU

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