Nephrotic syndrome is characterized by proteinuria, edema, hypoalbuminemia, and hypercholesterolemia. The majority of cases are primary or idiopathic nephrotic syndrome of unknown cause. Investigations show proteinuria, low serum albumin, and high cholesterol. Kidney biopsy may be done in certain cases. Complications include infections, hypercoagulability, acute renal failure, and cardiovascular disease. General management focuses on diet, preventing infections, and managing edema. Corticosteroids are the main treatment but frequent relapses may require alternative immunosuppressants like cyclophosphamide, cyclosporine, or mycophenolate mofetil.

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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