Nephrotic syndrome NS

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					  Nephrotic
Syndrome (NS)
        Definition
  NS is an accumulation of
symptoms and signs and is
characterized by proteinuria,
hypoproteinemia, edema, and
hyperlipidemia.
   In children under age 5 years
the disease usually takes the
form of idiopathic (primary) NS
of childhood (nil disease, lipoid
Nephrosis).
   Conditions Of Attack
    Second only to acute nephri-
tis.
   Incidence age: At all ages,
but most commonly between
2~5 years of age.
          Type
1.Clinical type
  Simple NS ; Nephritic NS
2.Response to steroid therapy
  (P331)
  The initial response to cortico-
steroids is a guide to prognosis.
  (1) Total effect
  (2) Partial effect
  (3) Non-effect
3. Pathologic type (P328)
   Minimal change disease,
MCD: 80% of patients.
        Pathogenesis
   The primary disorder is an
increase in glomerular permea-
bility to plasma proteins.
 ▲Foot processes of the visceral
epithelium of the GBM.
  1.The construction of the
glomerular basement memb-
rane has changed.
  2.The loss of the negative
charges on the GBM.
  ◆The underlying pathoge-
nesis is unknown, but evidence
strongly supports the impor-
tance of immune mechanisms
(P328).
      Pathophysiology
1.Proteinuria: Fundamental
and highly important change of
pathophysiology.
 2.Hypoproteinemia (mainly
albumin)
 3.Edema: Nephrotic edema
(pitting edema)
  Hypoproteinemia plasma
oncotic pressure is diminished,
result in a shift of fluid from
the vascular to the interstitial
compartment and plasma
volume↓→the activation of
the renin–angiotensin–aldo-
sterone system→ tubular
sodium chloride reabsorp-
tion↑.
  4. Hyperlipidemia (Hyper-
cholesterolemia)
  Ch↑, TG↑, LDL-ch↑,
VLDL-ch↑.
 Clinical Manifestations
  There is a male preponderance
of 2:1.
  1.Main manifestations: Edema
(varying degrees) is the common
symptom.
  Periorbital swelling and
perhaps oliguria are noticed
→→increasing edema→→
anasarca evident.
  2.General symptoms:
Pallid, anorexia, fatigue,
abdominal pain, diarrhea.
    Laboratory Exam
 1.Urinary protein: 2+~4+

   24hr total urinary protein
> 0.1g/kg.
   ( The most are selective
proteinuria. )
  May occur granular and red
cell casts.
  2.Total serum protein↓,
<30g/L .
  Albumin levels are low (<
20g/L).
   3.Serum cholesterol and
triglycerides:
 Cholesterol >5.7mmol/L
(220mg/dl).
   4. ESR↑>100mm/hr.
  5.Serum proteins electro-
phoresis :
  Albumin↓, α2-G↑,γ-G↓,
  A/G inversion.
 6.Serum complemen: Vary
with clinical type.
 7.Renal function:
      Complications
  1.Infections
  Infections is a major compli-
cation in children with NS. It
frequently trigger relapses.
 Site: Respiratory tract, skin,
urinary tract and acute pri-
mary peritonitis.
   Causes: Immunity lower ,
severe edema→malcirculation,
protein malnutrition, and use
hormone and immunosuppre-
ssive agents.
2.Electrolyte disturbances
(1) Hyponatremia
(2) Hypokalemia
(3) Hypocalcemia
3.Thromboembolic phenomena
 ( Hypercoagulability )
 Renal vein thrombosis
4.Hypovolemic shook
5.Acute renal failure (prerenal)
        Diagnosis
 1.Diagnostic standard (P330):
 ●Four characteristics.
 ●Excluding other renal disease
(second nephrosis).
2.Clinical type
 Simple NS or Nephritic NS.
       Treatment
1.General measures
1.1 Rest
  1.2 Diet
   Hypertension and edema:
Low salt diet (<2gNa/ day) or
salt-free diet.
   Severe edema: Restricting
fluid intake.
  Increase proteins properly:
2g/(kg·day)
  While undergoing the corti-
costeroid treatment: Give VitD
500~1000iu/day (or Rocaltrol)
and calcium.
1.3 Prevent infection
1.4 Diuretics
 Not requires diuretics usually.
*HCT 2~5mg/(kg · day)
*Antisterone 3~5mg/(kg · day)
*Triamterene
 Attention: Volume depletion,
disorder of electrolyte and
embolism.
  Apparent edema:
  Give low molecular dextran
10~15ml/(kg·time);[+Dopamine
2~3ug/(kg·min) and/or Regitine
10mg +Lasix 1~2mg/kg].
 2.Corticosteroid therapy
  Short-course therapy:
   Prednisone 2mg/(kg·day) or
60mg/m   2/day (Max.60mg/day)

in 3 or 4 divided doses for 4wk
→maintenance treatment:
 Prednisone 1.5mg/kg, single
dose for every-other day×4wk.
 ▲Total course of therapy: 8
wk.
  Middle-course & long-course
therapy:
 ① Induction of remission:
 Prednisone 1.5~2mg/(kg · day)
(Max.60mg/day) for 4wk until
the urinary protein falls to
trace or negative levels     ②
②After maintenance treatment:
 Prednisone 2mg/kg , single
dose for every-other-day×4wk
     tapered gradually (2.5~5
mg/2wk)        discontinued.
 ▲Total course of treatment :
 ★Middle: 6mo
 ★Long: 9~12mo
  Estimate of curative effect
(P331).
  3. Treatment of relapse and
recurrence
  3.1 Extend the course of corti-
 costeroid
  3.2 Immunosuppressive agents
(Cytotoxic agents):
 ① CTX (Cytoxan)
  2mg/(kg·day) for 8~12wk .
  Total amount: 250mg/kg
  Side effects: nausea, vomiting,
WBC↓, trichomadesis, hemo-
rrhagic cystitis and the damage
of sexual glands.
② CB (Chlorambucil)
 0.2mg/kg for 8wk .
 Total amount : 10mg/kg
③ VCR & Levamisole
 4.Impulsive therapy
 (1) Methylprednisolone (MP)
   15~30mg/kg(<1g/day+10%
GS 100~ 250ml, iv drip (within
1~2hr) , 3 times/one course. If
necessary, give another 1~2
courses after 1~2wk
prednisone 2mg/kg, qod
tapered gradually.
  (2) CTX
   0.5~0.75mg/m   2 + NS/GS iv

drip (1hr), give liquid 2,000ml
/(m2.d) .
   Every one mo for 6~8 times.
 (3) CsA
  5~7mg/kg, in 3 divided doses
for 3~6mo.
 ★expense and nephrotoxicity.
(4) Anticoagulants
 Heparin
 Persantin 5mg/(kg·day)for
6mo.
  5.Alleviar proteinuria
  Angiotensin converting en-
zyme inhibitions (ACEI) :
  Captopril, Enalapril and
Benazepril.
        Prognosis
  Most cases of minimal
change disease eventually
remit permanently.

				
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