This document provides guidelines for managing children with nephrotic syndrome at the Royal Victoria Infirmary in Newcastle upon Tyne. It outlines the typical presentation of nephrotic syndrome, investigations performed, and treatment protocols for initial presentation, obtaining remission, and treating relapses. It also defines frequent relapsers and provides guidance on their treatment, including the use of alternate day prednisolone and discussing second-line steroid sparing agents with the Children's Kidney Unit for cases where high maintenance steroid doses are needed.
This document provides guidelines for managing children with nephrotic syndrome at the Royal Victoria Infirmary in Newcastle upon Tyne. It outlines the typical presentation of nephrotic syndrome, investigations performed, and treatment protocols for initial presentation, obtaining remission, and treating relapses. It also defines frequent relapsers and provides guidance on their treatment, including the use of alternate day prednisolone and discussing second-line steroid sparing agents with the Children's Kidney Unit for cases where high maintenance steroid doses are needed.
This document provides guidelines for managing children with nephrotic syndrome at the Royal Victoria Infirmary in Newcastle upon Tyne. It outlines the typical presentation of nephrotic syndrome, investigations performed, and treatment protocols for initial presentation, obtaining remission, and treating relapses. It also defines frequent relapsers and provides guidance on their treatment, including the use of alternate day prednisolone and discussing second-line steroid sparing agents with the Children's Kidney Unit for cases where high maintenance steroid doses are needed.
This document provides guidelines for managing children with nephrotic syndrome at the Royal Victoria Infirmary in Newcastle upon Tyne. It outlines the typical presentation of nephrotic syndrome, investigations performed, and treatment protocols for initial presentation, obtaining remission, and treating relapses. It also defines frequent relapsers and provides guidance on their treatment, including the use of alternate day prednisolone and discussing second-line steroid sparing agents with the Children's Kidney Unit for cases where high maintenance steroid doses are needed.
Copyright:
Attribution Non-Commercial (BY-NC)
Available Formats
Download as PDF, TXT or read online from Scribd
Download as pdf or txt
You are on page 1/ 7
Royal Victoria Infirmary
Queen Victoria Road
Newcastle upon Tyne NE1 4LP
Tel: 0191 233 6161
Fax: 0191 261 5881
Department of Paediatric Nephrology
NEPHROTIC SYNDROME GUIDELINES January 2008
These guidelines illustrate current practice on our unit and may be helpful to paediatricians managing children with nephrotic syndrome in other units. As with all guidelines they do not cover everything and we welcome contact from other paediatricians to discuss any case but particularly if atypical or difficult to manage. We have a strong commitment to shared care with regional consultants and believe this is made easier by having written guidelines to ensure the advice we give is consistent. These guidelines contain some changes from our previous practice and will continue to be updated from time to time in the light of new evidence.
The consultants work a rotational system. Ask the Trust switchboard for the paediatric nephrologist on call.
Denise Simpson and Jenny Booth are keen to be involved in patient care and management to provide home and school support to all patients.
Dr Malcolm Coulthard Denise Simpson 0191 2829599 Dr Heather Lambert Jenny Booth 0191 2829844 Dr Nadeem Moghal Dr Milos Ognjanovic
Date: January 2008 Page 1 of 7 Review Date: May 2011
INTRODUCTION
These guidelines are intended only for use with cases presenting as ‘typical’ nephrotic syndrome and therefore considered likely to be to be steroid responsive.
‘TYPICAL’ PRESENTATION FEATURES INCLUDE:
• Age range 1- 10 years
• Onset less than 4 weeks • No macroscopic haematuria (some steroid sensitive nephrotics have dipstick positive microscopic haematuria) • Normal blood pressure • Normal plasma creatinine (often low normal)
INVESTIGATIONS At initial presentation During in-patient stay Height a Weight a • bd Blood pressure a • bd or more often Blood tests • urea and electrolytes • Blood tests do not • calcium normally need to be • albumin repeated • full blood count • complement - C3 and C4 • Varicella titres Urine tests • dipstick for protein • daily • urinary sodium concentration • daily
No other investigations are necessary.
TREATMENT OF INITIAL PRESENTATION
1. PREDNISOLONE
Give prednisolone 60mg/m2 po daily for 6 weeks (maximum daily dose = 80mg) as a single morning dose. After this daily regimen, give prednisolone 40mg/m2 po on alternate days (maximum dose = 60mg on alternate days) for a further 6 weeks, then stop. The 6/6 week regimen does not affect definitions of non-responding, relapse frequency etc.
See Appendix 1, on page 6, for the calculation of body surface area from weight alone.
To minimise side effects give steroids in the morning.
Date: January 2008 Page 2 of 7 Review Date: May 2011
2. PENICILLIN
This should be given whilst there is proteinuria ++ or more, as prophylaxis against
pneumococcal infection.
Give: under 5 years of age 125mg bd po
5 years and above 250mg bd po
Stop when urine protein free for 3 days
3. RANITIDINE
Ranitidine should be given for the full 12 weeks of steroid treatment in order to reduce gastric symptoms.
Give 2mg/kg per dose bd po. (maximum dose = 150mg bd). It is available in both tablet and syrup form.
4. LOW SALT DIET
This is employed to help prevent excess thirst and fluid retention. Stop when the urine is protein free for 3 consecutive days. (Fluid restriction is only considered if a child continues to gain weight despite a low salt diet and is clinically euvolaemic.)
5. ALBUMIN INFUSION
The type of albumin required is as follows:
• If the child is shocked use 10ml/kg of 4.5% albumin solution. This may need to be repeated if clinically indicated. • In the non-shocked child infuse 5ml/kg (1g/kg) of 20% albumin solution over 4 hours. Give frusemide 2mg/kg IV after 2 hours.
Monitor oxygen saturation and blood pressure during and for 2 hours post infusion. An accurate fluid balance should also be kept.
Indications for albumin infusion
• Clinical hypovolaemia (see over)
• Urine sodium less than 15mmol/l • Oedema causing discomfort, difficulty mobilising etc. • Skin breakdown secondary to oedema
Albumin should not be administered solely on an arbitrary level of plasma albumin. If albumin infusions need to be given more than once a day, or for more than 3 consecutive days, consider discussion with the Children’s Kidney Unit at the Royal Victoria Infirmary.
Date: January 2008 Page 3 of 7 Review Date: May 2011
Hypovolaemia
Hypovolaemia is a common finding in children with the nephrotic syndrome.
Clinical assessment can be very difficult. The following are recognised as markers, but it is important to remember a child may still be hypovolaemic in the absence of all of these.
• Capillary refill time > 2 seconds
• Toe-core temperature gap >2°C • Hypotension (<5th centile), and paradoxically hypertension (>95th centile) can both be features of hypovolaemia • Persistent tachycardia
REMISSION
Remission is defined as no proteinuria for 3 consecutive days on dipstick testing. Both the low salt diet and penicillin can be stopped at this time.
Children who are not in remission after 28 days of treatment with prednisolone 60mg/m2, at either initial presentation or after a relapse, should be referred for a diagnostic renal biopsy.
RELAPSE
Defined as proteinuria, ++ for 7 consecutive days or +++ for 3 consecutive days.
The parents are advised that once a child is in remission they should telephone their GP or local hospital. Treatment is usually commenced immediately at home. Parents are also told to contact their local hospital immediately if the child becomes unwell (especially with abdominal pain) or if they become oedematous.
TREATMENT OF A RELAPSE
1. PREDNISOLONE
NB. Duration of treatment is different from treatment of an initial presentation.
Give prednisolone 60mg/m2 po daily (maximum daily dose = 80mg) as a single morning dose until urine is protein-free for 3 days. After this daily regimen, give prednisolone 40mg/m2 po on alternate days (maximum dose = 60mg on alternate days) for a further 4 weeks, then stop.
Date: January 2008 Page 4 of 7 Review Date: May 2011
PENICILLIN
This should be given whilst there is proteinuria ++ or more.
Give: under 5 years of age 125mg bd po
5 years and above 250mg bd po
Stop when the urine is protein free for 3 days. For convenience, parents are supplied with either enough tablets to start a course without needing to obtain a prescription, or with a bottle of penicillin powder to which they add water.
2. RANITIDINE
Ranitidine is not routinely used but if a child complains of dyspeptic symptoms give 2mg/kg per dose bd po. (maximum dose = 150mg twice daily).
3. LOW SALT DIET
This is also used during relapses. Stop when the urine is protein free for 3 consecutive days.
FREQUENT RELAPSERS
DEFINITION
• 2 or more relapses within 6 months of the initial response or…
• 4 or more relapses within any 12 month period.
TREATMENT OF FREQUENT RELAPSERS
We would advise that children with frequently relapsing nephrotic syndrome are discussed with the Children’s Kidney Unit.
1) ALTERNATE DAY PREDNISOLONE
The dose required for this maintenance regimen varies enormously. The aim is to keep the child on the lowest dose of maintenance steroids that keeps them relapse free. Initially use 0.1 mg/kg prednisolone po on alternate days (minimum dose = 5mg on alternate days). If further relapses occur despite steroid treatment at this level, the dose can be increased to a maximum of 0.5mg/kg prednisolone on alternate days.
If the maintenance dose needed exceeds 0.5mg/kg on alternate days, severe steroid side effects are likely. We therefore recommend that the introduction of 2nd line/steroid sparing agent. We would advise a further discussion with the Children’s Kidney Unit at this stage.
Date: January 2008 Page 5 of 7 Review Date: May 2011
VIRAL INFECTIONS AND BREAKTHROUGH PROTEINURIA Prescribing daily prednisolone (regular dose) instead of alternate day prednisolone for 7 days during viral infections can significantly reduce the risk of relapse in steroid dependent nephrotic syndrome. For example: a child on maintenance prednisolone 5mg alternate days would increase to 5mg daily for 7 – 10 days or until breakthrough protein settled. Relapse criteria remain the same and if fulfilled relapse treatment as protocol should be followed. If breakthrough protein does not settle within 3 weeks on daily prednisolone. RVI should be contacted to discuss management.
VACCINES Pnuemococcal vaccine is now included in the new infant immunisation program (Sept 06) It is also recommended by Department of Health (Aug 06) to be given to all patients with chronic renal disease including nephrotic syndrome. Under 5yrs If not involved in infant immunisation program, one dose of 7 valent conjugate vaccine followed by second dose of 23 valent polysaccharide pnuemococcal vaccine after 2nd birthday but not within 2months of first dose. 5yrs and over If not involved in infant immunisation program, one dose of 23 valent polysaccharide pnuemococcal vaccine if not previously
Influenza vaccine is also recommended by Department of Health (Aug 06) to be given to all patients with chronic renal disease including nephrotic syndrome. 6 – 35months 0.25 – 0.5ml injection, repeated after 4 – 6 week if receiving influenza injection for first time. 3–12years 0.5ml injection, repeated after 4 – 6 week if receiving influenza injection for first time. 13yrs and over Single 0.5ml injection.
Chickenpox contact RVI Oncology protocol for:
Herpes Zoster or Severe Herpes simplex in immunocompromised
3mths -12yrs 1500mg/m2/24hrs >12yrs 30mg/kg/24hrs Dosage frequency 3 doses in 24 hours To be given until no new spots and then change to 5 day course of oral acyclovir
FOLLOW UP
Local clinic reviews are usually required 3 to 6 monthly, depending upon the case. Children receiving maintenance treatment should be under shared care with the Children’s Kidney Unit, and be jointly discussed or reviewed at least yearly
Date: January 2008 Page 6 of 7 Review Date: May 2011
SUMMARY OF SUGGESTED REASONS FOR REFERRAL
• Atypical presentation • Age at presentation less than 1 or more than 10 years • Low C3 and/or C4 • Frequent albumin infusions required • Unusual clinical course e.g. develops hypertension or macroscopic haematuria • Failure to respond to steroids by being in complete remission within 28 days • Maintenance prednisolone of 0.5 mg/kg or more on alternate days • Children with steroid side effects regardless of dose
Thank you for reading the guidelines.
⎯⎯⎯⎯⎯⎯⎯⎯⎯⎯⎯⎯⎯⎯⎯⎯⎯⎯⎯⎯⎯⎯⎯
APPENDIX 1 BODY SURFACE AREA (BSA) ESTIMATION FROM WEIGHT ALONE.
In nephrotic syndrome there is often extra weight due to oedema. If so, use a recent reliable weight (e.g., a clinic weight), or use the weight estimated from the ideal weight-for-height. To do this, plot the height on the 50th centile line and note the age - this is the height-age. Then use the weight chart to find the 50th centile weight for that height-age. Use that weight.
Date: January 2008 Page 7 of 7 Review Date: May 2011
