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Guideline For The Management of Nephrotic Syndrome: Renal Unit Royal Hospital For Sick Children Yorkhill Division
Guideline For The Management of Nephrotic Syndrome: Renal Unit Royal Hospital For Sick Children Yorkhill Division
Syndrome
Renal Unit
Royal Hospital for Sick Children
Yorkhill Division
Please note: the following guideline has not been assessed according to
the AGREE (Appraisal of Guidelines for Research and Evaluation) criteria.
This will take place at the next guideline review.
Nephrotic Syndrome
Author: Renal Clinicians Group
Date of Review: October 2007
Version: 1.1
Authorised by: Dr J Beattie
Q Pulse Ref: YOR-REN-019
Page 1 of 8
Issue Date: November 2005
Contents
1. Introduction
2. Definition of Nephrotic Syndrome
3. Initial Investigation
4. Referral to Paediatric Nephrology
5. Complications
5.1 Hypovolaemia
5.2 Infection
5.3 Thrombosis
6.
Treatment of Initial Presentation of Nephrotic
Syndrome
6.1 Prednisolone
6.2 Albumin
6.3 Penicillin Prophylaxis
6.4 Salt/Fluid Restriction
6.5 Vaccination
7. Relapse of Nephrotic Syndrome
8. Treatment of Relapse of Nephrotic Syndrome
8.1 Prednisolone
8.2 Albumin
8.3 Salt Restriction
8.4 Penicillin
8.5 Vaccination
9. Treatment of Frequent Relapses
9.1 Low Dose Alternate Day Prednisolone
9.2 Levamisole
9.3 Cyclophosphamide
9.4 Cyclosporin
9.5 Mycophenylate Mofitil (MMF)
10. Follow Up
11. Future Guideline Review
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1. Introduction
This guideline represents our current practice within the Renal Unit and are intended
for use by clinicians. These guidelines are based on previous recommendations
reviewed in the light of recent literature and will be update regularly. They cover
many aspects of the management of typical nephrotic syndrome, but they are
not exhaustive, and many not be relevant for children with atypical nephrotic
syndrome. There is always a paediatric nephrology consultant on call for the unit
who will be happy to discuss difficult or unusual cases.
Nephrotic Syndrome
Author: Renal Clinicians Group
Date of Review: October 2007
Version: 1.1
Authorised by: Dr J Beattie
Q Pulse Ref: YOR-REN-019
Page 2 of 8
Issue Date: November 2005
Classification
3. Initial Investigation
The following investigations should be performed in all children:
Blood: FBC, U+Es; Creatinine; LFTs; ASOT; C3/C4; Varicella titres
Urine: Urine culture andUrinary protein/creatinine ratio
BP
Urinalysis including glucose
A urinary sodium concentration can be helpful in those at risk of
hypovolaemia.
Varicella status should be known in all children commencing steroids.
Hepatitis B status may be appropriate in children at high risk.
Age < 1 yr
Age > 10-12 yrs
Persistent hypertension
Macroscopic haematuria
Low C3/C4
Failure to respond to steroids within 4 weeks
Nephrotic Syndrome
Author: Renal Clinicians Group
Date of Review: October 2007
Version: 1.1
Authorised by: Dr J Beattie
Q Pulse Ref: YOR-REN-019
Page 3 of 8
Issue Date: November 2005
5. Complications
The main complications of nephrotic syndrome are hypovolaemia, infection and
thrombosis.
5.1 Hypovolaemia
The initial examination of children with nephrotic syndrome needs to include
an assessment of their intravascular volume. Whilst these children may be
very oedematous, they may also be intravascularly depleted. Signs of
intravascular depletion are cool peripheries (capillary refill time > 2 secs), a
core-peripheral temperature gap of > 2oC, and tachycardia. Hypotension is a
late sign of hypovolaemia, but paradoxical hypertension may be present. A
urinary sodium of < 10 mmol/l is a useful investigation to confirm
hypovolaemia.
5.2 Infection
Loss of complement components and possibly immunoglobulins results in an
increased risk of infection. Consider antibiotic prophylaxis whilst patients have
significant proteinuria.
5.3 Thrombosis
Loss of proteins such as anti-thrombin III contributes to a pro-coagulant
state. This might be exacerbaterd by hypovolaemia.
Version: 1.1
Authorised by: Dr J Beattie
Q Pulse Ref: YOR-REN-019
Page 4 of 8
Issue Date: November 2005
prednisolone causes gastric irritation, start ranitidine 2mg/kg bid for the duration
of steroid treatment.
6.2 Albumin
As discussed above the clinical indications for albumin are
Clinical hypovolaemia
Symptomatic oedema
A low serum albumin alone is not an indication for intravenous albumin.
If there is evidence of hypovolaemia, give 1 g/kg 20% albumin (5ml/kg) over 4 6 hours. Give 2mg/kg of iv frusemide mid-infusion. If clinically shocked give
10ml/kg 4.5% albumin. Children should be closely monitored during albumin
infusions, and where possible they should be administered during working hours.
125 mg bid
250 mg bid
6.5 Vaccination
Pneumococcal vaccination is recommended for children with NS. Consider giving
at the time of diagnosis. Varicella vaccination is only available on a named
patient basis.
Response To Treatment
Most children with nephrotic syndrome will respond to steroid treatment
within 2-4 weeks. A remission is defined as 3 or more days of trace or
negative on dipstick testing. Treatment is continued for a total of 12
weeks as outlined above. If proteinuria persists beyond the first 4 weeks
of steroid treatment, then children should be referred for renal biopsy.
Nephrotic Syndrome
Author: Renal Clinicians Group
Date of Review: October 2007
Version: 1.1
Authorised by: Dr J Beattie
Q Pulse Ref: YOR-REN-019
Page 5 of 8
Issue Date: November 2005
8.2 Albumin
The indications for albumin infusion are as for the initial presentation. It is less
likely to be needed during a relapse.
8.4 Penicillin
Whilst there is proteinuria (>++) penicillin can be given.
8.5 Vaccination
Consider giving varicella vaccine between relapses in children who are varicella
seronegative.
Referral to/Discussion with Paediatric Nephrology
Frequent relapsers
Steroid dependency
Steroid toxicity
Version: 1.1
Authorised by: Dr J Beattie
Q Pulse Ref: YOR-REN-019
Page 6 of 8
Issue Date: November 2005
If children have frequent relapses, strategies should be adopted to try to reduce the amount
of steroid required.
9.2 Levamisole
Levamisole may be beneficial for children who have occasional relapses. It is less useful for
children who are steroid dependent. The dose is 2.5 mg/kg/ on alt days for 6 months to a
year in the first instance. Reversible neutropenia is a rare but recognised side-effect. A FBC
should be checked monthly for the first 3 months. This drug is not licensed in the UK, and
has to be imported.
9.3 Cyclophosphamide
For children with frequent relapses or those who are steroid dependent consider a course of
Cyclophosphamide 3 mg/kg/day for 8 weeks or equivalent. It is best to avoid cutting the
tablets. FBC should be monitored for the first few weeks of treatment.
9.4 Cyclosporin
Cyclosporin at a dose of 2.5 mg/kg bid usually for 1 year may be useful as a steroid sparing
agent. Levels should be checked after 1-2 weeks; aim for a 12 hour trough of 70 120
nmol/l (85-145 ug/l). For children under 5 yrs of age, tid dosing may be necessary.
Monitor BP and renal function.
Nephrotic Syndrome
Author: Renal Clinicians Group
Date of Review: October 2007
Version: 1.1
Authorised by: Dr J Beattie
Q Pulse Ref: YOR-REN-019
Page 7 of 8
Issue Date: November 2005
10. Follow Up
GENERAL CONSIDERATIONS DURING FOLLOW UP
For children on long-term steroids:1)
Monitor BP
2)
Monitor growth (including bone age and pubertal stage where
appropriate)
3)
Monitor weight dietetic review where appropriate
4)
Glycosuria / HbA1c
5)
Bone mineral density / calcium supplements
6)
Ophthmology review
7)
VACCINATION
Pneumococcal: recommended for all children with NS.
Varicella: consider in varicella negative children with frequent relapses.
Aim to administer vaccine when prednisolone dose is low.
Should any aspect of this guideline change before the planned review (i.e.
new technology or procedural change) then this guideline should be
updated accordingly.
Future review of this guideline should make use of the AGREE document to
ensure that up-to-date evidence and best clinical practice has been used to
inform this guideline.
For further information regarding guideline
development, please contact the Chairperson of the Multi-Professional
Clinical Practice Committee.
Nephrotic Syndrome
Author: Renal Clinicians Group
Date of Review: October 2007
Version: 1.1
Authorised by: Dr J Beattie
Q Pulse Ref: YOR-REN-019
Page 8 of 8
Issue Date: November 2005