Guideline for the Management of Nephrotic 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

1 Low Dose Alternate Day Prednisolone 9. and many not be relevant for children with “atypical” nephrotic syndrome.5 Mycophenylate Mofitil (MMF) 10.1 Authorised by: Dr J Beattie Q Pulse Ref: YOR-REN-019 Page 2 of 8 Issue Date: November 2005 Nephrotic Syndrome Author: Renal Clinicians Group Date of Review: October 2007 .2 Levamisole 9. Definition of Nephrotic Syndrome • • • Nephrotic range proteinuria (> 1g/m2/day) Hypoalbuminaemia (<25 g/l) Oedema Version: 1. Complications 5.2 Albumin 8.3 Penicillin Prophylaxis 6. There is always a paediatric nephrology consultant on call for the unit who will be happy to discuss difficult or unusual cases. They cover many aspects of the management of “typical” nephrotic syndrome.4 Salt/Fluid Restriction 6. Introduction 2.1 Prednisolone 8.4 Penicillin 8. Definition of Nephrotic Syndrome 3. Follow Up 11. Initial Investigation 4.3 Salt Restriction 8. Treatment of Frequent Relapses 9.4 Cyclosporin 9.3 Cyclophosphamide 9. Introduction This guideline represents our current practice within the Renal Unit and are intended for use by clinicians. but they are not exhaustive.1 Prednisolone 6.5 Vaccination 7.Contents 1.1 Hypovolaemia 5.3 Thrombosis 6.2 Albumin 6.2 Infection 5. Future Guideline Review Page(s) 2 2-3 3 3 4 4 4 4 4 4-5 5 5 5 5 6 6 6 6 6 6 6 6-7 7 7 7 7 7 8 8 1.5 Vaccination 9. 2. Treatment of Initial Presentation of Nephrotic Syndrome 6. Referral to Paediatric Nephrology 5. These guidelines are based on previous recommendations reviewed in the light of recent literature and will be update regularly. Relapse of Nephrotic Syndrome 8. Treatment of Relapse of Nephrotic Syndrome 8.

• Hepatitis B status may be appropriate in children at high risk. were it performed. Referral to Paediatric Nephrology • • • • • • 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. Children who present with the typical features of nephrotic syndrome (see below) are generally responsive to steroid treatment and a renal biopsy. Those with atypical features should therefore undergo renal biopsy before receiving steroid treatment. Those with atypical features are more likely to be unresponsive to steroid treatment. Initial Investigation The following investigations should be performed in all children: • Blood: FBC.microscopic haematuria Macroscopic Haematuria 3. and a biopsy more likely to show FSGS or one of the other forms of nephrotic syndrome. SLE. U+E’s. Therefore children with typical features are started on steroids without recourse to renal biopsy. C3/C4. • Varicella status should be known in all children commencing steroids. would be likely to show minimal change nephrotic syndrome. Nephrotic Syndrome Typical Features Atypical Features Age 1-10 years <1yr. LFT’s. Creatinine. 4. >10years Normotensive Hypertensive Normal Adrenal Function Elevated Creatinine +/. MPGN) Congenital nephrotic syndrome This document relates only to the management of idiopathic nephrotic syndrome.1 Authorised by: Dr J Beattie Q Pulse Ref: YOR-REN-019 Page 3 of 8 Issue Date: November 2005 .Classification • • • Idiopathic (primary) nephrotic syndrome o Minimal change (80-90%) o Focal segmental glomerulosclerosis (FSGS) (10-20%) Secondary nephrotic syndrome (HSP. 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. ASOT.

5. There is increasing evidence that longer initial courses of prednisolone are associated with a lower incidence of relapse. The dose of prednisolone is based on surface area. Signs of intravascular depletion are cool peripheries (capillary refill time > 2 secs). but paradoxical hypertension may be present. a core-peripheral temperature gap of > 2oC. Consider antibiotic prophylaxis whilst patients have significant proteinuria. Varicella status should be documented clearly in the casenotes and on HISS.1 Authorised by: Dr J Beattie Q Pulse Ref: YOR-REN-019 Page 4 of 8 Issue Date: November 2005 . Complications The main complications of nephrotic syndrome are hypovolaemia. 6. and tachycardia. they may also be intravascularly depleted.1 Prednisolone When the diagnosis of nephrotic syndrome has been made. If Nephrotic Syndrome Author: Renal Clinicians Group Date of Review: October 2007 Version: 1. and therefore a 12-week initial course is recommended.2 Infection Loss of complement components and possibly immunoglobulins results in an increased risk of infection. This might be exacerbaterd by hypovolaemia. prednisolone treatment can be started in children with typical features. Hypotension is a late sign of hypovolaemia. 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.3 Thrombosis Loss of proteins such as anti-thrombin III contributes to a pro-coagulant state. and they should be aware of the side effects and risks of steroid treatment. infection and thrombosis. A urinary sodium of < 10 mmol/l is a useful investigation to confirm hypovolaemia. Patients should be issued with a steroid warning card.5. Treatment of Initial Presentation of Nephrotic Syndrome 6. Children with atypical features should be referred to paediatric nephrology for consideration of renal biopsy. or as divided doses during the day. • 60 mg/m2/day for 4 weeks (maximim 80 mg) • 40 mg/m2/on alternate days for 4 weeks (maximum 60mg) • Reduce dose by 5-10mg/m2 each week for another 4 weeks then stop Prednisolone can be given as a single dose in the morning with food. 5.

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. Children should be closely monitored during albumin infusions. If there is evidence of hypovolaemia. and some centres do not use prophylaxis. Treatment is continued for a total of 12 weeks as outlined above. A remission is defined as 3 or more days of trace or negative on dipstick testing. and where possible they should be administered during working hours. Grossly oedematous children are at risk of cellulitis and may benefit from antibiotic prophylaxis. Fluid restriction may also be helpful. Nephrotic Syndrome Author: Renal Clinicians Group Date of Review: October 2007 Version: 1. There is no evidence that antibiotic prophylaxis is of benefit.4 Salt/Fluid Restriction A low salt diet is used to try to prevent further fluid retention and oedema. then children should be referred for renal biopsy. Penicillin V can be given while there is proteinuria and discontinued when the child goes into remission.5 Vaccination Pneumococcal vaccination is recommended for children with NS. Give 2mg/kg of iv frusemide mid-infusion. 6. If proteinuria persists beyond the first 4 weeks of steroid treatment. give 1 g/kg 20% albumin (5ml/kg) over 4 6 hours. 6. children are at increased risk of infection.prednisolone causes gastric irritation.3 Penicillin Prophylaxis Whilst nephrotic. Varicella vaccination is only available on a named patient basis. 6. Consider giving at the time of diagnosis.5% albumin. Dose: Under 5 yrs 5yrs or above 125 mg bid 250 mg bid 6. start ranitidine 2mg/kg bid for the duration of steroid treatment. particularly with encapsulated organisms such as pneumococcus.1 Authorised by: Dr J Beattie Q Pulse Ref: YOR-REN-019 Page 5 of 8 Issue Date: November 2005 . These restrictions are lifted once the child goes into remission. If clinically shocked give 10ml/kg 4. Response To Treatment Most children with nephrotic syndrome will respond to steroid treatment within 2-4 weeks.

It is less likely to be needed during a relapse.7.2 Albumin The indications for albumin infusion are as for the initial presentation. 8. 8. 8. and the families instructed to get in contact should a relapse of proteinuria occur.1 Authorised by: Dr J Beattie Q Pulse Ref: YOR-REN-019 Page 6 of 8 Issue Date: November 2005 .5 Vaccination Consider giving varicella vaccine between relapses in children who are varicella seronegative. Diagnosis and Treatment of Frequent Relapses Frequent relapsers are diagnosed if there is: • 2 or more relapses within the first 6 months of presentation • 4 or more relapses within any 12 month period This becomes steroid dependency if the relapses are occurring during steroid tapering. Relapsing Nephrotic Syndrome Up to 60 . 8. Treatment of Relapse Nephrotic Syndrome 8. or if there is ++ for more than 1 week. Varicella status should be repeated 6 monthly in those who are nonimmune.4 Penicillin Whilst there is proteinuria (>++) penicillin can be given. These are diagnosed if there is +++ or ++++ proteinuria for 3 or more days. • 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. Nephrotic Syndrome Author: Renal Clinicians Group Date of Review: October 2007 Version: 1.70 % of children with nephrotic syndrome may have one or more relapse. Urine should be checked initially twice weekly.3 Salt Restriction Whilst there is proteinuria (>++) a no added salt diet is advised.1 Prednisolone Prednisolone treatment should be restarted once a relapse has been diagnosed. then weekly after the first episode. Referral to/Discussion with Paediatric Nephrology • Frequent relapsers • Steroid dependency • Steroid toxicity 9.

9. It is less useful for children who are steroid dependent.1 Authorised by: Dr J Beattie Q Pulse Ref: YOR-REN-019 Page 7 of 8 Issue Date: November 2005 . FBC should be monitored for the first few weeks of treatment.1 Low Dose Alternate Day Prednisolone Low dose alternate day steroid treatment (< 10-15 mg/alt days) may prevent relapses. Levels should be checked after 1-2 weeks.4 Cyclosporin Cyclosporin at a dose of 2. Doses of 600mg /m2/bid have been used. This drug is not licensed in the UK. Reversible neutropenia is a rare but recognised side-effect.5 Mycophenylate Mofitil (MMF) There is some experience of using MMF in children with difficult to treat NS. Monitor BP and renal function. 9. It may be useful for those children showing signs of cyclosporin toxicity.2 Levamisole Levamisole may be beneficial for children who have occasional relapses. The use of MMF is associated with gastro-intestinal intolerance. and result in less steroid being given overall. FBC should be monitored for leucopenia. For children under 5 yrs of age. strategies should be adopted to try to reduce the amount of steroid required.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. and has to be imported. 9. 9. aim for a 12 hour trough of 70 – 120 nmol/l (85-145 ug/l). A FBC should be checked monthly for the first 3 months. tid dosing may be necessary.5 mg/kg bid usually for 1 year may be useful as a steroid sparing agent. The dose is 2. This is an unlicensed indication for MMF. mainly diarrhoea. Nephrotic Syndrome Author: Renal Clinicians Group Date of Review: October 2007 Version: 1.5 mg/kg/ on alt days for 6 months to a year in the first instance. It is best to avoid cutting the tablets.If children have frequent relapses. 9.

For further information regarding guideline development. please contact the Chairperson of the Multi-Professional Clinical Practice Committee. Aim to administer vaccine when prednisolone dose is low. new technology or procedural change) then this guideline should be updated accordingly. 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. 11. Nephrotic Syndrome Author: Renal Clinicians Group Date of Review: October 2007 Version: 1. Varicella: consider in varicella negative children with frequent relapses.10.e. Future Guideline Development • • Should any aspect of this guideline change before the planned review (i. 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.1 Authorised by: Dr J Beattie Q Pulse Ref: YOR-REN-019 Page 8 of 8 Issue Date: November 2005 .

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