6.1 Nephrotic Syndrome

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Paediatric Clinical GuidelinesRenal 6.1

November 2002

THE INVESTIGATION AND MANAGEMENT OF NEWLY

PRESENTING CHILDHOOD NEPHROTIC SYNDROME

Revised November 2002

Patient Group Children with Nephrotic Syndrome

Author Dr JHC Evans, Consultant Paediatric Nephrologist, Nottingham

City Hospital NHST, Ext 47428

Background

Nephrotic syndrome is characterised by heavy proteinuria (protein or

albumin:creatinine ratio > 200mg/mmol), hypoalbuminaemia (serum albumin


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3. Investigations

In all new patients

Urine for Dipstick urinalysis

Urine culture

Urine microscopy for casts if gross haematuria

Urine protein/albumin:creatinine ratio (early morning specimen) -

correlates with overnight protein loss and replaces the need for 24hr urine

collection. Do not delay starting treatment in order to obtain.

Blood for

Urea, electrolytes, creatinine and albumin

Full blood count

"Varicella Zoster immunity status"

(Plan tests, single venepuncture is ideal in children who may be difficult to

bleed because of oedema. Femoral stabs should never be performed as

thrombosis is a described complication)

If mixed nephrotic/nephritic picture with macroscopic haematuria and hypertension,

cases should be discussed with a Consultant Nephrologist where other investigations

may be appropriate (C3, C4 complement, C3d, ANF, ASOT, ANCA and

immunoglobulins).

4. Management

Nephrotic syndrome treatment aims to induce remission with steroids (most patients

respond within 5-14 days), and therefore promote diuresis. All other therapies are

symptomatic and aimed at preventing complications. Children who do not respond to

prednisolone within 28 days will require a renal biopsy.

4.1. Cortico-steroidsThere is considerable debate as to the optimal regimen of prednisolone. Prolonged

courses (3-6 months) reduce the likelihood of relapse in the subsequent 2 years, but

this may be at the expense of greater steroid induced adverse effects 3,4. Two regimens

are therefore used. A standard regimen and an intensified regimen. The intensified

regimen is used in children considered at high risk of relapse taking into account

factors such as young age, ethnicity (south asian) severe illness on initial presentation

and delay in achieving initial remission. The choice between the two regimens is an

elective decision that should be made after discussion with the consultant paediatric

nephrologist.

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Standard Regimen

Initial therapy - Prednisolone 60mg/m2/day in a single morning dose (maximum dose

80mg) for 28 days even if proteinuria remits. Methyl prednisolone 60mg/m2/day can

be used intra-venously in the vomiting child. After 28 days, the dose of steroid is

reduced to 40mg/m2 alternate days for the next 28 days and then stopped

Week Prednisolone dosage

1-4 (first 28 days) 60 mg/m2/day (maximum dose 80 mg)

5-8 (next 28 days) 40 mg/m2/alternate day (maximum 40mg)

Intensified Regimen

The first 8 weeks are as in the standard regimen, followed by a further 8 weeks of

tapering dosage.

Week Prednisolone dosage

1-4 (first 28 days) 60 mg/m2/day (maximum dose 80 mg)

5-8 (next 28 days) 40 mg/m2/alternate day (maximum 40mg)

9-12 25 mg/m2/alternate day (maximum 25mg)

13-17 10mg/m2/alt day (maximum 10mg)

A "steroid warning card" should be provided for the patient to carry.

4.2. Oedema and ascites

A balanced no added salt diet is recommended.

Suggested fluid intake 5yrs = 1 litre

Diuretics

(use only if severe and worsening oedema/ascites, in the absence of

hypovolaemia,)

Spirinolactone 2mg/kg/day in two divided doses

Frusemide 1-2mg/kg/day in two divided doses

Albumin infusions

Albumin infusion is only indicated in symptomatic hypovolaemia or severe

diuretic resistant oedema. It should be administered with great caution withfrequent monitoring of vital signs until at least two hours after the infusion is

completed.

Doses;

Severe circulatory failure = 4.5% albumin 20ml/kg over 30-60 mins repeated if

necessary until volume status restored.

Mild hypovolaemia + oedema = 20% albumin 5ml/kg (1g/kg) over 2-4 hrs with IV

frusemide 1mg/kg halfway through the infusion provided signs of hypovolaemia

have resolved.

Severe diuretic resistant oedema = 20% albumin 5ml/kg (1g/kg) over 2-4 hrs with

IV frusemide 1mg/kg half way through infusion

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4.3. Risk of thrombosis

Thrombosis either arterial or venous is rare (5%5) in nephrotic syndrome. The

consequences however can be devastating.

To decrease the risk of thrombosis; Avoid hypovolaemia

Prevent sepsis

Encourage mobilisation and avoid bedrest

Low dose Aspirin*

*Nb: There is no definite evidence (even in adults) that prophylactic anti-platelet or

anticoagulation treatment is of benefit. However, it seems reasonable that patients with

a family history of thrombophilia or raised platelet count (>800) should receive low

dose Aspirin as prophylaxis three times per week (if 30kg 75mg).

4.4. Hypertension

Check volume status, if satisfactory

Anti-hypertensives

Atenolol 0.5-1mg/kg/day in single daily dose

or Nifedipine SR 0.25-2mg/kg/day in two divided doses

4.5. Infection prophylaxis

4.5.1. Antibiotics

Oral Penicillin V (125mg BD if < 5yrs or 250mg BD


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"Immunosuppression" in the context of immunisation is defined as any

child who is receiving or has received in the last 3 months; (a)

Prednisolone 2mg/kg/day for > 1 week, (b) Prednisolone 1mg/kg/day or

equivalent for 1 month (ie 40mg/ m2 alternate days) (c) lower doses of

prednisolone combined with cytotoxic drugs, (d) long term lower dose

immunosuppression.

In some instances where the risk of relapse is high, immunisation may be

deferred. This requires discussion with the consultant.

4.5.3. Varicella Zoster

Chicken pox whilst immunosuppressed can be a very serious illness.

"Varicella zoster immunity status" should be known (and documented) in

each nephrotic patient.

ZIG or Aciclovir may be required please refer to renal protocol 6.3

"Chickenpox and immunosuppression therapy in renal children."

4.6. Dietary advice

A dietician should see the child and family. A balanced no added salt diet is

recommended

4.7. Psychosocial support

Making adequate information available for the family is essential. Childhood

Nephrotic Syndrome booklets and a video are available from the Renal unit at City

hospital. Carers should be instructed on how to dipstick the child's early morning

urine and record this in a daily diary.

Referral to the paediatric community renal nurse and social worker is routine.

A family support group exists; "Childhood Nephrotic Support" (contact Mrs

Sandra Warhurst, 7 Bonnymead, Cotgrave. NG12 3QH. Tel. 0115 9892975).

5. Relapse

Relapse within the first year is common (86%3) and can occur years after the initial

presentation. Relapse can follow immunisation or viral infection. A relapse is defined as

proteinuria of ++ or more for three consecutive days. Prednisolone treatment usually delayed

for at least 5 days unless the child is becoming oedematous.

Duration Prednisolone dosage

Daily until urinary protein negative

or trace for 3 days

60 mg/m2/day (maximum dose 80 mg)

Next 28 days 40 mg/m2/alternate day (maximum 40mg)

Subsequent therapy Individualised (may / may not taper steroids)

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Frequent relapsers are defined as having 2 or more relapses in 6 months and should receive

second line treatments (not covered by this guideline).

Contact NumbersConsultant Paediatric Nephrologists

Dr Jonathan Evans 47428 or bleep via City Hospital switchboardDr Alan Watson 46169 or bleep via City Hospital switchboard

Paediatric Renal Nurse Liz Moore 46488Paediatric Nephrology SpR 46458 )(Lambley Ward) or bleep via City

Hospital switchboard

Audit Points

As patient numbers per year are very low, a retrospective audit of case notes could be

undertaken as there are only minor changes in the new version of the guidelines

compared with the3 previous version.

1. Do patients receive Pneumovac?

2. Are patients managed according to the guidelines

References

1 Kherr KK, Sweet M, Makker SP,: Nephrotic syndrome in children. Curr Prob Pediatr;

18: 197-251.

2 The International Study of Kidney disease in Children: The primary nephrotic

syndrome in children. Identification of patients with minimal change nephrotic

syndrome from initial response to prednisolone. J Pediatr. 1981; 98: 561-564.

3 Evans J :National Audit of Childhood Nephrotic Syndrome. The Royal College of

Paediatrics and Child Health/The British Association for Paediatric Nephrology. 1999

4 Hodson EM, Knight JF, Willis NS, Craig JC. Corticosteroid Therapy for nephroticsyndrome in children (Cochrane Review). The Cochrane Library, Issue 3, 2002.

Oxford Update

5 Mehls O, Andrassy K, Koderisch J, Herzog U, Ritz E,: Hemostasis and

thromboemblism in children with nephrotic syndrome: Differences from adults. J

Pediatr 1987:110:862-867

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PAEDIATRIC CLINICAL GUIDELINESPAEDIATRIC CLINICAL GUIDELINES

ISSUE: VERSION: FINAL

Title: THE INVESTIGATION AND MANAGEMENT OF NEWLY PRESENTING

CHILDHOOD NEPHROTIC SYNDROME

Author: Dr JHC Evans,Job Title: Consultant Paediatric Nephrologist,

Nottingham City Hospital NHST, Ext 47428

First Issued: Date Revised: November 2002Review Date: October 2004

Document Derivation: Consultation Process: PaediatricNephrologists

i.e. References: Cross town Paediatric Clinical GuidelinesGroupIncluded in document Pharmacist

Ratified By: Paediatric Clinical Guidelines CommitteeChaired By:Consultant with Responsibility: Dr Stephanie Smith

Distribution: All wards QMC and CHN

Training issues: Included in Induction Programme

Audit: included in document

This guideline has been registered with Nottingham City Hospital NHS Trust and QMCClinical Guidelines Committee. However, clinical guidelines are guidelines only. Theinterpretation and application of clinical guidelines will remain the responsibility of theindividual clinician. If in doubt contact a senior colleague or expert. Caution is advised whenusing guidelines after the review date.

MANUAL AMENDMENTS RECORD

(please complete when making any hand-written changes/ amendments to guideline and not processedthrough guideline committee)

Date Author Description

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6.1 Nephrotic Syndrome