1. Nephrotic Syndrome..…(NS)
Prepared by:NAZURAH

2. Nephrotic syndrome
 Proteinuria
Proteinuria >40mg/m2/hour or an early
morning ur pr creatinine index
>200mg/mmol
Edema
Hypoalbuminemia
 Hypercholesterolemia

3. Classification:
A-Primary Idiopathic NS (INS):
majority
Accounting for 90% of NS in
child. mainly discussed.
Unknown cause
B-Secondary NS:
Include post streptococcal
glomerulonephritis and SLE

4. 1.The construction of the
glomerular basement membrane has changed.
2.The loss of the negative
charges on the GBM.

5. Pathophysiology:
The Main Trigger Of primary Nephrotic Syndrome
and Fundamental and highly important change of
pathophysiology :Proteinuria

6. Pathogenesis of Proteinuria: Increase glomerular permeability for proteins due to loss of
negative charged glycoprotein
 Degree of protineuria: Mild less than 0.5g/m2/day
 Moderate 0.5 – 2g/m2/day
 Sever more than 2g/m2/day
 Type of proteinuria: A-Selective proteinuria: where proteins of low molecular
weight .such as albumin, are excreted more readily than
protein of HMW
 B-Non selective :
 LMW+HMW are lost in urine

7. How many pathological types causes
nephrotic syndrome?

10. Investigations: 1-Urine analysis:-
A-Proteinuria : 3-4 + SELECTIVE.
b-24 urine collection for protein
>40mg/m2/hr
for children
c- volume: oliguria (during stage of edema formation)
d-Microscopically:microscopic hematuria 20%, large number of hyaline cast

11. Investigations: 2-Blood:
 A-serum protein: decrease >5.5gm/dL , Albumin levels are
low ( ＜ 2.5gm/dL).
 B-Serum cholesterol and triglycerides:
Cholesterol ＞ 5.7mmol/L (220mg/dl).
 C-- ESR↑ ＞ 100mm/hr during activity phase
.
 3.Serum complemen: Vary with clinical type.
 4.Renal function

12. Kidney Biopsy:-
 Considered in:
 1-Secondary N.S
 2-Steroid resistant N.S
 3- Gross Hematuria
 4-Hypertension
 5- Renal Impairment

13. Complications of NS:1-Infections:Infections is a major complication in children with
NS. It frequently trigger relapses.
Nephrotic pt are liable to infection because :
A-loss of immunoglobins in urine.
B-the edema fluid act as a culture medium.
C-use immunosuppressive agents.
D- malnutrition
The common infection : URI, peritonitis, cellulitis and UTI
may be seen.
Organisms: encapsulated (Pneumococci, H.influenzae),
Gram negative (e.g E.coli

14. Complication…..
 2-Hypercoagulability (Thrombosis).
 Hypercoagulability of the blood leading to venous or arterial
thrombosis:
 Hypercoagulability in Nephrotic syndrome caused by:


1-Higher concentration of I,II, V,VII,VIII,X and fibrinogen
2- Lower level of anticoagulant substance: antithrombin III

3-decrease fibrinolysis.

4-Higher blood viscosity


5- Increased platelet aggregation
6- Overaggressive diuresis

15. 
3-ARF: pre-renal and renal

4- cardiovascular disease :-Hyperlipidemia, may be a risk
factor for cardiovascular disease.

5-Hypovolemic shock

6-Others: growth retardation, malnutrition,
adrenal cortical insufficiency


16. Management of NS:
General (non-specific )
Corticosteroid therapy

17. General therapy:Normal diet with adequate calories
No added salt to the diet whn child has
edema
Avoiding infection: very important.
Penicillin V is recommended at diagnosis
and during relapses
 Severe edema: Restricting fluid intake
Human albumin (20-25%)- symptomatic
grossly edematous together with IV
frusemide(diurresis)

18. GENERAL ADVICE
Home urine albumin monitoring (1st urine specimen)
Consult doctors if 1)albuminuria >= 2+ for
consecutives day or out 7 days.
2)edematous
Immunisation
on corticosteroid treatment and within 6 weeks
(killed vacines)
after 6 weeks cessation (live vaccine)
pneumococcal vaccine

19. Corticosteroid—prednisone therapy:-
REMISSION : Urine dipstick trace or nil for 3 consecutives days within 28
days.
RELAPSE: Urine albumin excretion > 40mg /m2/hour or urine dipstick
>= 2+ for 3 consecutives days
FREQUENT RELAPSES : >= 2 Relapses within 6 month of initial diagnosis
or >= 4 relapses within 12 month periods
STEROID DEPENDENT NEPHROTIC SYNDROME : >= 2 Consecutives
relapses occuring during steroid taper or within 14days of cessation of
steroid

21. Side Effects With Long Term Use of
Steroids “Steroid toxicity
-Stunted growth
Cataracts
- Pseudotumor cerebri
 hyperglycemia
 myopathy
 peptic ulcer
 poor healing of wound.
-Psycosis
 Hirsutism
-Osteoporosis
 Thromboembolism
- Cushingoid features
-Adrenal gland suppression

22. Alternative agent: When can be used:
 Steroid-dependent patients, frequent relapsers, and steroid-
resistant patients.

Cyclophosphamide Pulse steroids

Cyclosporin A

Tacrolimus

Microphenolate

23. Treatment
Cytotoxic drugs with corticosteroid:
(for steroid dependent or steroid resistant)
Cyclophosphamide (CTX): p.o. or intravenously
Side effects: liver injury, inhibition of bone marrow, etc.
Cyclosporine
(for those failed responsing to combination of steroid and cytotoxic
drugs)
Dose: 5mg/kg/d, bid, p.o.
Side effects: renal and liver toxic injury, expensive, etc.

24. Treatment
Mycophenolate mofetil, MMF
(for steroid dependent or steroid resistant)
Dose:1.5-2g/d, bid, p.o. for 3-6 months, maintaining 0.5 year

25. THE END….
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