Transcript Nephrotic syndrome, NS

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 nephritis.
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 corticosteroids 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 permeability to plasma proteins.
▲Foot processes of the visceral
epithelium of the GBM.
1.The construction of the
glomerular basement membrane has changed.
2.The loss of the negative
charges on the GBM.
◆The underlying pathogenesis is unknown, but evidence
strongly supports the importance 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 reabsorption↑.
4. Hyperlipidemia (Hypercholesterolemia)
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
+
+
2 ～4
1.Urinary protein:
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 electrophoresis :
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 complication in children with NS. It
frequently trigger relapses.
Site: Respiratory tract, skin,
urinary tract and acute primary peritonitis.
Causes: Immunity lower ,
severe edema→malcirculation,
protein malnutrition, and use
hormone and immunosuppressive 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 corticosteroid 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
2
60mg/m /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 corticosteroid
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, hemorrhagic 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
2
0.5~0.75mg/m + 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 enzyme inhibitions (ACEI) :
Captopril, Enalapril and
Benazepril.
Prognosis
Most cases of minimal
change disease eventually
remit permanently.