Nephrotic syndrome

Nephrotic syndrome

1. NEPHROTIC SYNDROME
Soumya Ranjan Parida
Basic B.Sc. Nursing 4
th
year
Sum Nursing College

2. Nephrotic syndrome
Nephrotic syndrome is 15 times more common in childre
than adults.
Incidence – 2-3 / 1lac children /yr
It is characterized by –
- Heavy proteinuria ( > 3.5gm/day or
40mg/m²/hr ),
- Hypoalbuminemia (< 2.5gm/dl ),
- Edema ,
- Hyperlipidemia.
Hematuria , hypertension and impaired renal function
are occasionally associated.

3. Nephrotic Syndrome
Etiology –
•90% Idiopathic nephrotic syndrome
- Minimal change disease ( 85%),
- Mesangial proliferation (5%) ,
- Focal segmental glorulosclerosis (10%)
•10% Secondary nephrotic syndrome
- Membranuous nephropathy
- Membranoproliferative GN
Pathophysiology –
•T cell dysfunction
•Altration of cytokines
•Loss of negative charged glycoproteins

4. Pathophysiology
Heavy proteinuria
Hypoalbuminemia
Reduced plasma oncotic pressure
Extravasation of intravascular fluid
hypovolemia
Aldosterone
Antidiuretic hormone
Distal Na & water reabsorption
Edema

5. Hyperlipidemia –
•Increased hepatic protein synthesis
•Decreased lipoprotein lipase activity
•LDL and VLDL are increased
•HDL may increase, normal or decrease
•Cholesterol and triglyceride are increased
Pathophysiology

6. Idiopathic Nephrotic Syndrome
Pathology –
•Minimal change disease ( 85% )
Minimal increase in mesangium and matrix
IFMC – normal
EMC – Effacement of epithelial cell foot processes
Steriod sensitive – 90%
•Mesangial proliferative ( 5% )
LMC - Diffuse increase in mesangium and matrix.
IFMC – Mesangial IgM and IgA staining.
EMC - Mesangium cell and matrix increased.
Effacement of epithelial cell foot processes
Steriod sensitive – 50%
•Focal segmental glomerulosclerosis ( 10% )
LMC – mesangial proliferation and segmental scarring
IFMC – IgM and C3 staing in the area of scarring

7. Clinical features –
•Sex – M:F 2 : 1
•Age – 2 – 6 yrs
Common –
•Periorbital edema
•Generalized edema
•Oliguria
•Irritability
•Abdominal pain
•Diarrhoea
Uncommon –
•Hypertension
•Gross hematuria

Idiopathic Nephrotic Syndrome

8. Diagnosis –
Urine examination –
•Proteinuria
•Hematuria
•24 hour urinary protein
•Spot urine - protein mg% / creatinine mg %
Blood investigations –
•Sreum creatinine
•Total protein
•Serum albumin
•Serum cholesterol and triglyceride level
•C3-C4 levels – N
•IgG- low, IgM - increased
Idiopathic Nephrotic Syndrome

9. •Glomerular Proteinuria –
Selective – LMW & albumin
Nonselective – HMW IgG
Selectivity = IgG / Transferrin ratio
1gm – 30 gm / day
Hematuria , hypertension ,renal insufficiency
•Tubular proteinuria –
<1gm / day
Low molecular proteinuria
Little or no albumin
•Transient proteinuria
•Orthostatic proteinuria.
R/o infections –
Urine r/m , Urine c/s, Hb ,CBC, ESR , MT test
Chest x- ray , USG abdomen.
Idiopathic Nephrotic Syndrome

10. Treatment
1
st
episode mild to moderate edema –
•Out patient management
•Sodium intake restricted initially
•Oral diuretics judiciously.
Severe edema –
•Hospitalized
•Fluid restrction
•IV diuretics
•25% human albumin – 0.5gm/kg/12hour
Renal biopsy –
•Hematuria, hypertension
•Renal insufficiency
•Hypocomplementemia
•Age beyond 1-8 yrs
•RBC casts

11. Prednisone - 60 mg/m²/day divided into 2-3 doses for at least 4wks
After 4-6 wks - 40 mg/m²/day EOD single morning dose after meals
After 3 months – gradually tapered and stopped
•Steroid responsive –
Urine protein negative, trace or 1+ for 3 consecutive days
•Steroid resistant –
Urinary protein 2+ or more after 8 wks of therapy.
•Steroid dependent –
Relapse while on EOD therapy or within 28 days of stopping
of steroids.
•Relapse –
Reappearance of proteinuria 3+ or 4+ and edema.
•Infrequent relapse –
3 or less in a year.
•Frequent relapse –
4 or more in a year
Treatment

12. Frequent relapses or steroid dependent –
•Prednisolone alternate day –
- 0.3 – 0.7 mg/kg:
- Duration – 9-12 months
•Levamisole –
- 2-2.5mg/kg and prednisolone 1.5 mg/kg on alternate days.
- Duration: 1 – 2yrs.
- Prednisolone dose tapered and discontinued
•Cyclophosphamide –
- 2 mg/kg daily and prednisolone 1.5 mg/kg alternate days.
- Duration : 12 wks
•Cyclosporine –
- 5 mg/kg daily and prednisolone 1-1.5 mg/kg alternate days.
- Duration : 1-3 yrs.
- Prednisolone dose tapered
Treatment

13. Complications
•Edema
•Infection –
- Urinary loss of Ig, properdin factor B
- Defective cell mediated immunity
- Malnutrition , edema, ascitis
- Spontaneous bacerial peritonitis ( Strept. Peumoniae, E.coli )
- Vaccination – pneumococcal, varicella, Infuenza.
•Thrombotic complications –
- Increased prothrombotic factors ( Fibrinogen, thrombocytosis,
hemoconcentration, relative immobilization.
- Decreased fibrinolytic factors ( urinary loss of AT3, protein C, S.
•Acute renal failure
•Steroid toxicity

14. Secondary Nephrotic Syndrome
•Glomerular diseases –
Membranous GN, MPGN, Lupus & HSP nephritis.
•Infection –
Malaria, Schistosomiasis, HBV, HCV, Filariasis, Leprosy, HIV.
•Malignancy –
Lung and GIT carcinoma, Hodgkins lymphoma
•Druga –
- Membranous GN – Penicillamine, captropil ,gold, NSAIDS,
mercury .
- Minimal change disease – Probencid, ethosuximide,
methimazole, lithium.
- MPGN – Procainamide, chlorpropamide, phenytoin,
trimethadion, paramethadione

15. •< 3 months
•Finnish type, AR
•Mutation in NPHS1 gene, ch 19, Nephrin
•Pathology –
Dilatation of proximal tubules, mesangial hypercellularity,
glomerular sclerosis
•Clinical features –
Massive proteinuria, large placenta, marked edema,
prematurity, RDS, seperation of cranial sutures
•Treatment -
ACE inhibitors, indomethacin, unilateral nephrectomy
•Denys-Drash syndrome –
Diffuse mesangial sclerosis, Male pseudohermaphroditism,,
Wimls tumor. Mutation in WT1 chromosome
Congenital Nephrotic Syndrome

16. THANKS