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Nephrotic Syndrome

Dr.K.A.W.Karunasekera

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Characteristic features

Gross proteinuria (albuminuria)

Hypoalbuminaemia

Oedema

Hyperlipidaemia

Primary 90%

Secondary 10%

Congenital rare

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Primary (idiopathic)

nephrotic syndrome

Cause remains unknown, seen more

commonly in atopic families.85% Minimal change NS

10% focal segmental GS

5% Diffuse mesangial proliferation

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Idiopathic NS contd.

95% of MCNS respond to steroids.

50%-60% mesangial proliferation and20% FSGS respond to steroids.

Idiopathic NS steroid sensitive NS

steroid resistant NS

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Pathophysiology

Basic change is the increase glomerular capillarywall permeability.

Oedema Intra-vascular volume

Renin-angiotensin ADH

aldosterone system

Increased Na+ reabsorption Increased waterreabsorption

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Pathophysiology contd.

Gross proteinuria >= +2

In NS proteinuria >2gram/24hrs.

Oedema appears when albumin

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Clinical Features

Male:female = 2:1

Onset at 2-6 years.

Often a history of URTI.Periorbital oedema, oedema in lowerextremities, generalized oedema, decreased

urine output.Lethargy, irritability, decreased appetite,diarrhoea, abdominal pain, hypertension.

O/E include height, weight, BP

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Investigations

Urine FR : proteinuria >= +2, haematuria,casts.

Urine culture & ABSTProtein : creatinine ratio > 200mg/mmol

Serum proteins : albumin

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Management

Monitoring

Initial height & weight

Daily

weight, urine protein, UOPBP

At homeurine protein

Bed rest : no indication

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Management contd.

Diet : A balanced diet with no added saltonly during oedema.

Antibiotics :Risk of infectionsPneumococci & gram-ve

peritonitis, septicaemia andcellulitis.

Obvious foci must be treated.

Oral penicillin b.d until oedema subsides.

Pneumococcal vaccine.

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Rapid protein loss hypovolaemia

Hypovolaemic crisis:

Abdominal pain, decreased BP, coldextremities, decreased capillary

perfusion, wide central:peripheral

temperature gap.Treatment :Rapid infusion of plasma,

salt poor albumin

Management of

hypovolaemia

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Management contd.

Diuretics : While awaiting prednisolone toact.

Antihypertensives :

hypervolaemia

HT may be or

vasoconstrictive effect to

hypovolaemia

Treatment : Hydralazine, frusemide (if UOP isOK) propranalol,

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Steroid therapy

prednisolone therapy : 1956

Inflammatory / immunosuppressive or both?

Dose for 1st attack :

60mg/m2/d 2-3 divided doses for 4-6 weeks,

then 40mg/m2

/d EOD as a single morningdose for 28 days. (must know the side

effects)

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Management

80% of all types & 92% of MCNS respond toprednisolone.

80% of SSNS will do so with in 14 days.Steroid resistance :

No response to 60mg/ms/d for 4-6 weeks.renal biopsy

Relapse : Protienuria >= +2 for 3days

About 75% of MCNS relapse.

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Management contd.

Frequent relapse : Up to 50% of relapsingchildren

>=2 relapses with in 6/12 of initial responseor

>=4 relapses with in any 12 months period.

Steroid dependence : 2 relapses duringtreatment or with in 14 days after treatment

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Treatment of a relapse

Look for infections and treat if any.

Can wait for 5-7 days to commence

therapy provided the child is free ofoedema.

Prednisolone 60 mg/ms/d until 3 days

free of protein, then 40 mg/ms/d EOD asa single morning dose for 28 days.

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Treatment of frequent

relapses & steroid dependence

According to the individual child.

Prednisolonesingle dose alternate :

0.5-1 mg/kg/d EOD for 6-12 months. Levamizole2.5 mg/kg/d EOD can be given to reduce the

required dose of prednisolone.

Cyclophosphamide or Chlorambucil- Increase the duration of remission

- Decrease frequency of relapses

Renal biopsy is not indicated.

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Cyclophosphamide

therapy

For those who require :

prednisolone up to 1mg/kg/d with its side

effects or Prednisolone >1mg/kg/dDose 2-3 mg/kg/d single dose for 8-12 wks.

Side effects : bone marrow suppression,

increase incidence of infections, alopecia,haemorrhagic cystitis, azoospermia,malignancy

3rd line drugs : Cyclosporin

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Immunization

Should receive normal immunization unless

the patient on daily prednisolone > one week.

If on prednisolone > 1 week, no live vaccinesuntil 3 months after discontinuation.

Live vaccines can be given when on Every

Other Day treatment

No live vaccine, while on cyclophosphamide.

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Indications for renal

biopsy

Before treatment :

Onset

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ComplicationsIncrease risk of infection

Increase risk of thrombosis

Steroid side effectsAbdominal pain :

Hypovolaemia

PeritonitisCellulitis

Renal vein thrombosis

Non s ecific

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Prognosis

SSNS resolves towards the end of 2nd

decade.

SRNS includes some of MCNS, SFGS,

MPGN and prognosis depends ofhistology.

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Congenital NS(Finnish type)

Rare, autosomal recessive

C.F. within 1st 6 months with persistent

oedema and infectionsAdditional featuresprematurity, large

placenta, respiratory distress.

Treatment: Dialysis & renal transplantotherwise death within 2 years.

AN diagnosis : Increase alpha feto protein

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Causes Secondary NS

Other GN : membranous, MPGN

SLE, Henoch Schonlein Purpura

Chronic infections - P.malaria

Tumours - Hodgkin lymphoma

Drugs

Penicillamine, gold, Hg