childhood nephrotic syndrome - practice guidelines · • nephrotic syndrome – proteinuria •...

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Childhood nephrotic syndrome – practice guidelines

Dr Shuman Haq Consultant Paediatric Nephrologist Southampton Children’s Hospital


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Definitions

•  Nephrotic syndrome – Proteinuria

•  Urine protein : creatinine >200 mg/mmol •  3+ protein on urine dipstick

– Hypoalbuminaemia •  Plasma albumin < 25 g/l


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Definitions

•  Remission – Dipstick negative or trace – 3 consecutive days

•  Relapse – Dipstick 3+ or more – 3 consecutive days


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Definitions

•  Frequent relapser – 2 or more relapses within 6 months of initial

response – OR 4 or more relapses within any 12 month

period •  Steroid dependence

– 2 consecutive relapses during prednisolone treatment or within 14 days of stopping prednisolone


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Definitions

•  Steroid resistance – Failure to go into remission after 4 weeks of

prednisolone at 60 mg/m2 once daily –  (KDIGO - 8 weeks) – Secondary resistance


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History

•  Atypical features (e.g. rash, arthropathy) •  Fluid intake •  Urine output •  Diarrhoea •  Abdominal pain •  Macroscopic haematuria •  Fever


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Examination •  Peripheral perfusion •  Blood pressure •  Oedema (including genital), ascites,

pleural effusions •  Evidence of infection (including peritonitis) •  Evidence of thrombosis – swollen, painful,

red leg, neurology •  Atypical features (rash, arthropathy)


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Remember complications

•  Hypovolaemia •  Infection •  Thrombosis


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Initial investigations

•  Urine protein creatinine ratio •  U & E’s •  Plasma albumin •  FBC •  Varicella antibodies (even if history positive) •  ASOT, anti-DNAse B •  C3/C4 •  Other investigations if indicated (e.g. ANA)


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Management

•  Admit – Confirm diagnosis – Counsel parents – Commence therapy


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Management

•  Do not fluid restrict or give diuretics •  Normal protein intake •  If suspect hypovolaemia – urine sodium •  If hypovolaemic - may need 4.5% or 20%

albumin •  Restrict salt intake •  Daily weight, and urine dipstick •  Strict input / output chart •  Prophylactic penicillin


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Management – counsel parents

•  Information leaflet - www.infokid.org.uk •  30% - 1or no relapses •  40% - infrequent relapses •  30% - frequent relapses or steroid dependence •  Relapse occur less often as enter teenage years •  90% achieve permanent remission during

adolescence •  Very unlikely to get chronic kidney disease


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Management – counsel parents

•  Discuss complications of steroid therapy •  Provide with steroid card •  Teach how to dip urine •  Provide or suggest diary for dipstick

results •  Ensure they understand definitions of

relapse and remission and when to ring


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Intravenous albumin

•  4.5% v 20% •  Hypovolaemia •  Oedema

– Splinting of diaphragm – Marked discomfort – Marked scrotal or labial swelling


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20% albumin

•  5 ml/kg over 4 hours – Sometimes give less or more slowly

•  IV Frusemide 1 mg/kg half way through infusion

•  Monitor as per blood transfusion •  Pulmonary oedema can occur several

hours after infusion completed •  Do not give at night


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Prednisolone - first presentation •  60 mg/m2 (max 60 mg) once daily for 4

weeks •  40 mg/m2 (max 40 mg) alternate days for 4

weeks •  30 mg/m2 alternate days for 2 weeks •  20 mg/m2 alternate days for 2 weeks •  10 mg/m2 alternate days for 2 weeks •  Stop (total of 14 weeks)


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Prednisolone – relapses •  First relapse

–  60 mg/m2 (maximum of 60 mg) until remission –  then 40 mg/m2 (maximum of 40 mg) alternate days for

4 weeks and then stop (unless steroid dependent) •  Subsequent relapses

–  Individualised weaning schedule dependent on previous history

–  Most will require longer wean (up to 12 weeks)


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Steroid sparing agents •  Low dose alternate day steroids

–  10 mg/m2 or 0.5 mg/kg on alternate days

•  Levamisole 2.5 mg/kg on alternate days •  Cyclophosphamide 2 mg/kg od for 12 weeks •  Tacrolimus 0.05 mg/kg bd •  Mycophenolate mofetil 600 mg/m2 bd (max dose

of 1 g) •  Rituximab 750 mg/m2 (max dose 1 g), 2 doses a

fortnight apart


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Referral to paediatric nephrologist

•  Age <12 months or >10 years •  Macroscopic haematuria •  Persistent hypertension •  Systemic disease •  Low serum C3 or C4 •  Steroid resistance •  Steroid dependence •  Frequent relapser


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Sharing is caring

•  Guideline will be put on PIER website after ratification by the other Wessex centres

•  www.pier.uhs.nhs.uk •  Now lets see if you have been listening…


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Definition of a relapse: urine dipstick 1+ protein on 3 consecutive days

A.  True B.  False


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Definition of remission: urine dipstick negative or trace for protein on 3 consecutive days



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Definition of steroid resistance: failure to respond to 2 weeks prednislone at 60 mg/m2//d



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Definition of a paediatric nephrologist: a happy go lucky, carefree type of Dr - not in any way anally retentive



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Thrombosis is a complication of untreated NS



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Increased intravascular fluid volume is a complication of untreated NS



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Infection is a complication of untreated NS



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Scrotal oedema is a complication of untreated NS



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Call your friendly paediatric nephrologist if: NS pt age is < 12 months or > 10 years



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Call your friendly paediatric nephrologist if: microscopic haematuria present



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Call your friendly paediatric nephrologist if: C3 or C4 low



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When to call your friendly nephrologist: if you feel like a friendly chat with Arvind


childhood nephrotic syndrome - practice guidelines · • nephrotic syndrome – proteinuria •...

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