Download Nephrotic syndsome

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
肾脏疾病常见综合征

Nephrotic syndrome
(一)肾病综合征

Nephritic syndrome
(二)肾炎综合征

Asymptomatic urinary
abnormalities
(三)无症状性尿检异常
Acute renal failure or Rapidly
progressive renal failure
竭综合征


Chronic kidney disease(Table 1)
(四)急性及急进性肾衰
(五)慢性肾脏病(表1)
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
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.
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)
Pathology patterns and
clinical presentations of
idiopathic nephrotic syndome
Renal biopsy

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).
PRIMARY NEPHROTIC SYNDROME

Minimal Change Disease

Focal Segmental Glomerulosclerosis

Membranous Nephropathy

Membranoproliferative
Glomerulonephritis (MPGN)
Figure 5a.
Pathology of glomerular disease. Light microscopy.
(a) Normal glomerulus; minimal change disease.
Table 4
PRIMARY NEPHROTIC SYNDROME

Minimal Change Disease

Focal Segmental Glomerulosclerosis

Membranous Nephropathy

Membranoproliferative
Glomerulonephritis(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 SYNDROME

Minimal Change Disease

Focal Segmental Glomerulosclerosis

Membranous Nephropathy

Membranoproliferative
Glomerulonephritis(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 SYNDROME

Minimal Change Disease

Focal Segmental Glomerulosclerosis

Membranous Nephropathy

Membranoproliferative
Glomerulonephritis(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
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
治疗
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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