Childhood nephrotic syndrome practice guidelines

Transcription

1 Childhood nephrotic syndrome practice guidelines Dr Shuman Haq Consultant Paediatric Nephrologist Southampton Children s Hospital

2 Definitions Nephrotic syndrome Proteinuria Urine protein : creatinine >200 mg/mmol 3+ protein on urine dipstick Hypoalbuminaemia Plasma albumin < 25 g/l

3 Definitions Remission Dipstick negative or trace 3 consecutive days Relapse Dipstick 3+ or more 3 consecutive days

4 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

5 Definitions Steroid resistance Failure to go into remission after 4 weeks of prednisolone at 60 mg/m 2 once daily (KDIGO - 8 weeks) Secondary resistance

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

7 Peripheral perfusion Blood pressure Examination Oedema (including genital), ascites, pleural effusions Evidence of infection (including peritonitis) Evidence of thrombosis swollen, painful, red leg, neurology Atypical features (rash, arthropathy)

8 Remember complications Hypovolaemia Infection Thrombosis

9 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)

10 Management Admit Confirm diagnosis Counsel parents Commence therapy

11 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

12 Management counsel parents Information leaflet % - 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

13 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

14 Intravenous albumin 4.5% v 20% Hypovolaemia Oedema Splinting of diaphragm Marked discomfort Marked scrotal or labial swelling

15 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

16 Prednisolone - first presentation 60 mg/m 2 (max 60 mg) once daily for 4 weeks 40 mg/m 2 (max 40 mg) alternate days for 4 weeks 30 mg/m 2 alternate days for 2 weeks 20 mg/m 2 alternate days for 2 weeks 10 mg/m 2 alternate days for 2 weeks Stop (total of 14 weeks)

17 Prednisolone relapses First relapse 60 mg/m 2 (maximum of 60 mg) until remission then 40 mg/m 2 (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)

18 Steroid sparing agents Low dose alternate day steroids 10 mg/m 2 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/m 2 bd (max dose of 1 g) Rituximab 750 mg/m 2 (max dose 1 g), 2 doses a fortnight apart

19 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

20 Sharing is caring Guideline will be put on website after ratification by the other Wessex centres Now lets see if you have been listening

21 Definition of a relapse: urine dipstick 1+ protein on 3 consecutive days A. True B. False

22 Definition of remission: urine dipstick negative or trace for protein on 3 consecutive days A. True B. False

23 Definition of steroid resistance: failure to respond to 2 weeks prednislone at 60 mg/m 2/ /d A. True B. False

24 Definition of a paediatric nephrologist: a happy go lucky, carefree type of Dr - not in any way anally retentive A. True B. False

25 Thrombosis is a complication of untreated NS A. True B. False

26 Increased intravascular fluid volume is a complication of untreated NS A. True B. False

27 Infection is a complication of untreated NS A. True B. False

28 Scrotal oedema is a complication of untreated NS A. True B. False

29 Call your friendly paediatric nephrologist if: NS pt age is < 12 months or > 10 years A. True B. False

30 Call your friendly paediatric nephrologist if: microscopic haematuria present A. True B. False

31 Call your friendly paediatric nephrologist if: C3 or C4 low A. True B. False

32 When to call your friendly nephrologist: if you feel like a friendly chat with Arvind A. True B. False