Nephrotic syndrome

Figure 1. Nephrotic edema.

Figure 2. Nephrotic edema.

Clinical Syndrome 肾脏及泌尿系疾病经常会引起一些临床症

状、 体征和实验室表现相似的综合征。识别患者属于哪一种综合征对诊断很有帮助，因为导致每个综合征的病因较之其包含的个别临床症状和体征的致病原因要少，故识别患者属于哪一种综合征对诊断有帮助。

The most common syndrome of kidney disease

Nephrotic syndrome

Nephritic syndrome

Asymptomatic urinary abnormal

ities

Acute renal failure or Rapidly pr

ogressive renal failure

Chronic kidney disease(Table

1)

(一 )肾病综合征(二 )肾炎综合征(三 )无症状性尿检异常(四 )急性及急进性肾衰竭综合征(五 )慢性肾脏病 (表 1)

肾脏疾病常见综合征

Table 1. STAGES OF CHRONIC KIDNEY DISEASE*

STAGE DESCRIPTION GFR (mL/min/1.73m2)

1 Kidney damage with normal or ↑ GFR ≥90

2 Kidney damage with mild or ↓ GFR 60-89

3 Moderate ↓ GFR 30-59

4 Severe ↓ GFR 15-29

5 Kidney failure <15 (or dialysis)

* Chronic kidney disease is defined as either kidney damage or GFR < 60mL/min/1.73m2 for ≥ 3months. Kidney damage is defined as pathologic abnormalities or markers of damage, including abnormalities in blood or urine tests or image studies.

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 i

n 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.

NEPHROTIC SYNDROMENEPHROTIC 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 u

rinary protein loss. It is also due to the catabolis

m of filtered albumin by the proximal tubule as w

ell as to redistribution of albumin within the body.

This in part accounts for the inexact relationship

between urinary protein loss, the level of the ser

um 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.

Hyperlipidemia

Most nephrotic patients have elevated levels of total and

low-density lipoprotein (LDL) cholesterol with low or nor

mal high-density lipoprotein (HDL) cholesterol . Lipoprot

ein (a) [Lp(a)] levels are elevated as well and return to n

ormal with remission of the nephrotic syndrome. Nephrot

ic patients often have a hypercoagulable state and are pr

edisposed to deep vein thrombophlebitis, pulmonary em

boli, 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 SYNDROMEPRIMARY NEPHROTIC SYNDROME

Minimal Change Disease

Focal Segmental Glomerulosclerosis Membranous Nephropathy Membranoproliferative Glomerulonep

hritis (MPGN)

Figure 5a. Pathology of glomerular disease. Light microscopy.

(a) Normal glomerulus; minimal change disease.

Table 4

PRIMARY NEPHROTIC SYNDROMEPRIMARY NEPHROTIC SYNDROME

Minimal Change Disease Focal Segmental Glomerulosclerosis Membranous Nephropathy Membranoproliferative Glomerulonep

hritis(MPGN)

Figure 5b. Segmental sclerosis; focal segmental glomerulosclerosis.

Figure 6. Light microscopic appearances in focal segmental glomerulosclerosis. Segmental scars with capsular adhesions in otherwise normal glomeruli.

Table 5

PRIMARY NEPHROTIC SYNDROMEPRIMARY NEPHROTIC SYNDROME

Minimal Change Disease Focal Segmental Glomerulosclerosis Membranous Nephropathy Membranoproliferative Glomeruloneph

ritis(MPGN)

Figure 7a. Early MN: a glomerulus from a patient with severe nephrotic syndrome and early MN, exhibiting normal architecture and peripheral capillary basement membranes of normal thickness (Silver–methenamine ×400).

Figure 7b morphologically advanced MN

Figure 7c. Morphologically more advanced MN (same patient as in (b))

Table 6

PRIMARY NEPHROTIC SYNDROMEPRIMARY NEPHROTIC SYNDROME

Minimal Change Disease Focal Segmental Glomerulosclerosis Membranous Nephropathy Membranoproliferative Glomeruloneph

ritis(MPGN)

Figure 8. Pathology of membranoproliferative glomerulonephritis type I.

(a) Light microscopy shows a hypercellular glomerulus with accentuated lobular architecture and a small cellular crescent (methenamine silver).

Table 7

Diagnosis and Differential diagnosis

Initial evaluation of the nephrotic patient in

cludes laboratory tests to define whether t

he patient has primary, idiopathic nephroti

c syndrome or a secondary cause related t

o a systemic disease.

Common screening tests include the fasting blood sugar

and glycosylated hemoglobin tests for diabetes, and anti

nuclear antibody test for rheumatoid disease, and the se

rum complement, which screen for many immune compl

ex-mediated disease (Table 3), In selected patients, cry

oglobulins, hepatitis B and C serology, anti-neutrophil cyt

oplasmic 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 glomerula

r involvement.

It leads to a multitude of other consequences , s

uch as predisposition to infection and hypercoag

ulability. In general, the diseases associated with

NS cause chronic kidney dysfunction, but rarely t

hey can cause ARF. ARE may be seen with mini

mal change disease, and bilateral renal vein thr

ombosis.

ComplicationsInfectionCoagulation disordersProtein malnutrition and dyslipidemiaAcute renal failure

Treatment 治疗

1. General treatment

2. Symptomatic treatment (e.

g.diuresis to relieve edem

a, treating dyslipidemias,

anticoagulate treatment, e

tc.)

3. Immunosupressive treatm

ent

一、一般治疗二、利尿消肿三、免疫抑制治疗四、调脂药物五、抗凝治疗

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