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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.
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.
Therefore children with typical features are started on steroids without recourse
to renal biopsy. Those with atypical features should therefore undergo renal
biopsy before receiving steroid treatment.
Nephrotic Syndrome
Typical Features Atypical Features
Age 1-10 years <1yr, >10years
Normotensive Hypertensive
Normal Adrenal Function Elevated Creatinine
+/- microscopic haematuria Macroscopic Haematuria
3. Initial Investigation
The following investigations should be performed in all children:
• Blood: FBC, U+E’s; Creatinine; LFT’s; 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.
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.
6.1 Prednisolone
When the diagnosis of nephrotic syndrome has been made, prednisolone
treatment can be started in children with typical features. Children with atypical
features should be referred to paediatric nephrology for consideration of renal
biopsy.
6.2 Albumin
As discussed above the clinical indications for albumin are
• Clinical hypovolaemia
• Symptomatic oedema
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.
8.1 Prednisolone
Prednisolone treatment should be restarted once a relapse has been diagnosed.
• 2mg/kg daily (maximum 80 mg) until the urine is negative or trace for 3 days
• 40 mg/m2 (maximum 60 mg) on alternate days for 4 weeks then stop or taper
the dose over 4-8 weeks
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.
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.