Nephrotic syndrome is characterized by heavy proteinuria, hypoalbuminemia, edema, and hyperlipidemia. It is caused by primary glomerular diseases that damage the glomerular filter, with minimal change disease being the most common cause. Symptoms include edema of the eyes and legs that becomes generalized. Diagnosis involves urine tests showing proteinuria and serum tests showing low albumin and normal kidney function. Treatment of initial episodes involves a high protein diet, diuretics, salt restriction, and corticosteroid therapy. Frequent relapses are treated with steroid-sparing agents like levamisole or immunosuppressants. Complications include edema, hyperlipidemia, increased susceptibility to infections
2. IT IS THE CLINICAL MANIFESTATION OF
GLOMERULAR DISEASES ASSOCIATED WITH
HEAVY PROTEINURIA-
>3.5gm/24 hrs URINE.
URINE PROTEIN & CREATININE RATIO >2.
HYPOALBUMINEMIA.
EDEMA.
HYPERLIPIDEMIA>200mg/dl.
7. STEROID SENSITIVE NEPHROTIC
SYNDROME
Renal biopsies- doesn't show significant
abnormalities
Electron microscopy- nonspecific obliteration of
epithelial foot process
Immuneflourescence-doesnot demonstrate
deposition of immune rectents except
occasional mesangial IgM
8. CLINICAL FEATURES
Oedema is first noticed around eyes and
subsequently on legs
-it is soft and pits easily on pressure
-gradually oedema becomes generalised with
ascites ,hydrothorax , hydrocele
-Urine output may fall
-Blood pressure is usually normal
9. DIAGNOSIS
• Urine Analysis-
• +3 or +4 proteinuria
• Red cells and casts absent.
• Serum Cholesterol – elevated.
• Serum Albumin – less than 2gm/dl.
• Blood Urea and Serum Creatinine – normal.
• Complete blood count- normal.
10. Management-Initial episode
High protient diet
-salt moderation
-Treatment of infections
-If significant oedema-diuretis .Aldosterone
antagonist (fursemide,spirnolactone)
-Corticosteroid therapy with prednisolone or
Prednisone(2mg/kg per dayfor 6 weeks )
11. Subsequent course
Relapse
-Infrequent Relapses:3 or less relapses per year
-Frequent Relapses:4 or more repalses per year
-Steroid therapy
-Steroid dependant:relapses following dose
reduction or discontinution
- Steroid resistant:Partial or no response no
initial treatment
12. Alternate Day Prednisolone
-Steroid sparing agents
-Levamisole(2-2.5mg/kg)
-Cyclophosphamide(2-2.5 mg/kg/day)
-Mycophenolate Mofetil(20-25mg/kg/day)
-Cyclosporin(4-5mg/kg/day)
-Tacrolimus(0.1-0.2mg/kg/day)
-Rituximab(375mg IV once a week)
FREQUENT RELAPSES
13. Management of relapses
-Parent education
-Symptomatic therapy for infections in case of
low grade protenuria
-persistant protenuria(3-4+)-
-Prednisolone
( 2 mg/kg/day untill protein is negitive for 3
days )(1.5mg/kg on alternate days for 4 weeks)
14. CLINICAL CONSEQUENCES OF NEPHROTIC SYNDROME
EDEMA.
Nephrotic Range Proteinuria
Fall in Plasma Protein Level
Decrease in Intravascular Oncotic Presssure.
Leakage of Plasma water into the Interstitium
Reduced Intra Vascular Volume
Increased secretion of Vasopressin and Atrial natriuretic factor and
Aldosterone.
Increased sodium and H2O retention by tubules.
15. HYPERLIPIDEMIA - alteration of lipid profile
Cholesterol level
Triglycerides
LDL
HDL
VLDL
INCREASED SUSEPTIBILITY TO INFECTIONS
Urinary losses of Ig
Defects in complement cascade from urinary loss of
complement factors (C3,C5)
S.Pneumoniae, varicella, and gram negative organisms .
Bacterial peritonitis , bacteremia , septicemia.