Nephrotic Syndrome by Dr. Nadeem Khawer

NEPHROTIC SYNDROME
NADEEM KHAWAR
Definition
•EDEMA

•PROTEINUREA

•HYPOALBUMINEMIA

•HYPERLIPIDEMIA
EDEMA
Mechanism ?

Complex
Decrease Oncotic Pressure
Increase Renin
Increase ADH
Proteinurea
Ø 24 hour protein excretion = 150 mg
Ø Non Pathologic
§ Posture
§ Febrile
§ Exercise
Ø Pathologic

o Tubular 200mg / 24hrs to 1G / 24hrs

o Glomerular > 1 G / 24 hrs
§ Selective Albumin
§ Non selective Albumin + Ig G


CLASSIFICATION
Nephrotic syndrome
1) Idiopathic -90%
a. M.C.N.S—85%
b. Mes.P.G.N—5%
c. F.S.G.N—15%
2) SECONDORY -10%
a. Mem.
b. M.P.G.N

IDIOPATHIC
1 MINIMAL CHANGE NEPHROTIC SYNDROME
Ø MAJORITY IDIOPATHIC (90%)
Ø KNOWN CAUSES (10%)
a. DRUGS (NSAID.RIFAMPICIN.INTERFERON)
b. HODGKIN’S
c. HIV
d. IDDM
e. Ig A NEPHROPATHY
SECONDARY
Ø INFECTIONS
1. Hepatitis B & A, Malaria
Ø AUTOIMMUNE
1. SLE, HSP, IDDM
Ø DRUGS
Ø
MINIMAL CHANGE NEPHROTIC SYNDROME
§ Children 75 % ( 2 to 6 years old )
§ Male : Female 2 : 1
§ Steroid Sensitive 90 %
§ Hematuria 10 to 20 %
§ Hypertension 10 %
§ CRF nil
History
1. Recent onset generalized edema
2. Exclude CCF , Malabsorption , PCM .
3. Enquire about Urine output , Hematuria.
4. Fever
5. Other Atopic disorders.
6. Drugs used esp. Steroid.
7. Number of relapses if any , Hospitalizations

EXAMINATION
1. Well or Ill looking.
2. Height / Weight.
3. Edema: periorbital , ascites ,scrotal , ankle.
4. Pulse / Blood Pressure.
5. Abdomen.
6. Chest


INVESTIGATIONS
1. 24 hours urine protein / spot urine / A/G ratio
2. Serum Albumin / Cholesterol
3. FBC / CRP / Cultures
4. Electrolytes / Creatinine / Urea
5. Chest X Ray / MT
6. C3
Nephrotic Syndrome Category
•Initial Presentation
•Frequent Relapses
•Steroid Dependent
•Steroid Resistance

Bad Prognostic Factors
•Age- (2 to 10 yrs)
•Hypertension
•Hematuria
•Azotemia
•Non Selective Proteinurea
•Decrease C 3


MANAGEMENT
ü Hospitalize
o Education
o Investigation
o Response to therapy
ü Induce Remission
ü Supportive Care
ü Treat Complications
ü Follow Up
REMISSION
STEROID
o Prednisolone ( Delta Cortril 5 mg )
§ 2 mg / kg /24 hours (60mg/m sq) for 4 weeks then alternate day regime for 4 months
§ Response time up to 8 weeks

CYCLOPHOSPHAMIDE

CYCLOSPORIN
SUPPORTIVE
Ø DIET : salt restriction / protein ?

Ø DIURETICS : caution

Ø HYPOVOLEMIA: Salt free albumin
§ (urine sodium < 10 mmol/l)
Ø Scrotal support


COMPLICATIONS
•INFECTION

•HYPERTENTION

•THROMBOSIS
· FOLLOW UP
STEROID RESPONDER 90 %
1 / 3 NO RELAPSE
1 / 3 INFREQUENT RELAPSE ( < 3 PER YEAR )
1 / 3 FREQUENT RELAPSE OR STEROID DEPENDENT( > 3 PER YEAR )

STEROID RESISTANT 10 %
STEROID RESISTANT / STEROID DEPENDENT NS

•RENAL BIOPSY

•IMMUNOSUPPRESIVE
THANK YOU
NEXT LECTURE
HEMATURIA / AGN