Nephrotic Syndrome (NS)

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Nephrotic Syndrome (NS)
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Definition NS is an
accumulation of symptoms and signs and is
characterized by proteinuria, hypoproteinemia,
edema, and hyperlipidemia.

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The vast majority patients (90 of cases) with
NS of childhood are primary.
In children under age 5 years the
disease usually takes the form of idiopathic
(primary) NS of childhood (nil disease, lipoid
Nephrosis).
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Conditions Of Attack Second only to
acute nephri-tis. Incidence age At all
ages, but most commonly between 25 years of age.
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Type 1.Clinical type
Simple NS Nephritic NS 2.Response to steroid
therapy (P331)
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The initial response to cortico- steroids is
a guide to prognosis. (1) Total effect
(2)  Partial effect (3)  Non-effect
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3. Pathologic type (P328) Minimal change
disease, MCD 80 of patients.
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Pathogenesis The primary
disorder is an increase in glomerular permea-
bility to plasma proteins. ?Foot processes of
the visceral epithelium of the GBM.
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1.The construction of theglomerular
basement memb-rane has changed. 2.The loss
of the negative charges
on the GBM.
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?The underlying pathoge-nesis is
unknown, but evidence strongly supports the
impor- tance of immune mechanisms(P328).
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Pathophysiology







1.Proteinuria Fundamental and highly important
change of pathophysiology.
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2.Hypoproteinemia (mainly albumin)
3.Edema Nephrotic edema (pitting edema)
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Hypoproteinemia plasma
oncotic pressure is diminished,
result in a shift of fluid from the vascular to
the interstitial compartment and plasma
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volume??the activation of

the reninangiotensinaldo-sterone
system? tubularsodium chloride reabsorp-tion?.

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4. Hyperlipidemia (Hyper-cholesterolemia)
Ch?, TG?, LDL-ch?,VLDL-ch?.
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Clinical Manifestations There is a
male preponderance of 21. 1.Main
manifestations Edema (varying degrees) is the
common symptom.
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Periorbital swelling and perhaps
oliguria are noticed??increasing
edema??anasarca evident.
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2.General symptoms

Pallid, anorexia, fatigue,abdominal
pain, diarrhea.
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Laboratory Exam 1.Urinary protein
24 24hr total urinary protein gt
0.1g/kg. ( The most are selective
proteinuria. )
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May occur granular and red cell casts.
2.Total serum protein?,lt30g/L . Albumin
levels are low (lt20g/L).
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3.Serum cholesterol and triglycerides
Cholesterol gt5.7mmol/L (220mg/dl). 4.
ESR?gt100mm/hr.
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5.Serum proteins electro-phoresis
Albumin?, a2-G?,?-G?, A/G inversion.
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6.Serum complemen Vary with clinical type. 
7.Renal function
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Complications 1.Infections
Infections is a major compli-cation in children
with NS. Itfrequently trigger relapses.
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Site Respiratory tract, skin, urinary tract
and acute pri-mary peritonitis.
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Causes Immunity lower , severe
edema?malcirculation, protein malnutrition, and
use hormone and immunosuppre-ssive agents.
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2.Electrolyte disturbances (1) Hyponatremia
(2) Hypokalemia (3) Hypocalcemia
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3.Thromboembolic phenomena (
Hypercoagulability ) Renal vein thrombosis
4.Hypovolemic shook 5.Acute renal failure
(prerenal)
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Diagnosis 1.Diagnostic standard
(P330) ?Four characteristics. ?Excluding other
renal disease (second nephrosis).
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2.Clinical type Simple NS or Nephritic NS.
Treatment
1.General measures 1.1 Rest
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1.2 Diet Hypertension and edema Low
salt diet (lt2gNa/ day) or salt-free diet.
Severe edema Restricting fluid intake.
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Increase proteins properly 2g/(kgday)
While undergoing the corti-costeroid
treatment Give VitD 5001000iu/day (or
Rocaltrol)and calcium.
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1.3 Prevent infection 1.4 Diuretics Not
requires diuretics usually.HCT 25mg/(kg
day)Antisterone 35mg/(kg day)Triamterene
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Attention Volume depletion, disorder of
electrolyte and embolism.
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Apparent edema Give low molecular
dextran 1015ml/(kgtime)Dopamine
23ug/(kgmin) and/or Regitine 10mg Lasix
12mg/kg.
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2.Corticosteroid therapy Short-course
therapy Prednisone 2mg/(kgday) or
60mg/m2/day (Max.60mg/day) in 3 or 4 divided
doses for 4wk?maintenance treatment
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Prednisone 1.5mg/kg, single dose for
every-other day4wk.
?Total course of therapy 8 wk.
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  Middle-course long-course therapy ?
Induction of remission Prednisone 1.52mg/(kg
day)(Max.60mg/day) for 4wk untilthe urinary
protein falls totrace or negative levels ?
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?After maintenance treatment Prednisone
2mg/kg , single dose for every-other-day4wk
tapered gradually (2.55mg/2wk)
discontinued.
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?Total course of treatment ?Middle 6mo
?Long 912mo Estimate of curative effect
(P331).
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3. Treatment of relapse and recurrence 3.1
Extend the course of corti- costeroid 3.2
Immunosuppressive agents(Cytotoxic agents)
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? CTX (Cytoxan) 2mg/(kgday) for 812wk .
Total amount 250mg/kg
Side effects nausea, vomiting,WBC?,
trichomadesis, hemo-rrhagic cystitis and the
damage of sexual glands.
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? CB (Chlorambucil) 0.2mg/kg for 8wk .
Total amount 10mg/kg ? VCR Levamisole
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4.Impulsive therapy (1) Methylprednisolone
(MP) 1530mg/kg(lt1g/day10GS 100 250ml,
iv drip (within 12hr) , 3 times/one course. If
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necessary, give another 12 courses after
12wkprednisone 2mg/kg, qodtapered gradually.
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(2) CTX 0.50.75mg/m2 NS/GS iv drip
(1hr), give liquid 2,000ml/(m2.d) . Every
one mo for 68 times.
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(3) CsA 57mg/kg, in 3 divided doses for
36mo. ?expense and nephrotoxicity.
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(4) Anticoagulants Heparin Persantin
5mg/(kgday)for6mo.
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5.Alleviar proteinuria Angiotensin
converting en-zyme inhibitions (ACEI)
Captopril, Enalapril and
Benazepril.
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Prognosis Most cases of
minimal change disease eventually remit
permanently.