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Idiopathic Nephrotic Syndrome. Guideline Developments.

Idiopathic Nephrotic Syndrome. Guideline Developments. David Hughes Consultant Paediatric Nephrologist RHSC, Glasgow Honorary Consultant Paediatric Nephrologist RHSC, Edinburgh. Idiopathic (Primary) Nephrotic Syndrome (INS). Definition Epidemiology The spectrum of Idiopathic NS

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Idiopathic Nephrotic Syndrome. Guideline Developments.

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  1. Idiopathic Nephrotic Syndrome.Guideline Developments. David Hughes Consultant Paediatric Nephrologist RHSC, Glasgow Honorary Consultant Paediatric Nephrologist RHSC, Edinburgh

  2. Idiopathic (Primary) Nephrotic Syndrome (INS) • Definition • Epidemiology • The spectrum of Idiopathic NS • Diagnosis and investigation • Management and Treatment

  3. Definition Characteristic diagnostic features • Nephrotic range proteinuria Suspect for urine ‘stix’ testing > ++. Quantify as urine protein:creatinine (P:CR) >200mg/mmol. • Hypoalbuminaemia serum albumin <25 g/l • Generalised oedema Initial presenting clinical features Onset of oedema may be insidious. Features include • Peri-orbital swelling • Ankle and lower limb swelling – pitting oedema • Abdominal swelling and scrotal/vulval oedema Less commonly • frank haematuria • frothy urine

  4. Paediatric Network Document Report of a working party of Royal College of Paediatrics and Child Health British Association for Paediatric Nephrology NHS Kidney Care Epidemiological considerations Quality standards setting

  5. Epidemiology of INS in childhood • Incidence • 2-7 per 100 000 child population • 19-66 new cases across Scotland • 188 new cases in 14 months in UK (‘95-’96) • (Evans, personal communication) • 6 x more common in Asians • Much rarer in Africans • Prevalence • 12-16 per 100 000 child population • 114-152 cases across Scotland

  6. Epidemiology of INS in childhood • Young age • 1-6 years old – median age 4 years • More common in boys • 2:1 m:f • 1:1 m:f (adolescents) • <5% family history • Minimal Change Disease commonest cause in childhood v. 25 % in adults

  7. Idiopathic NS Spectrum: from MCD to FSGS 80-90 % MCD Non Infrequently Frequently Steroid Steroid Relapsing Relapsing Relapsing Dependent Resistant ~90 % Steroid Sensitive - PROGNOSTIC Multiple aetiologies

  8. Progress of INS in childhood • ~80% minimal change disease • >90% steroid responsive • >70% relapsing course • 80% long term remission during childhood • mortality rates • Published series1-7.2% • Sepsis • Vascular thrombosis • Currently much lower

  9. Quality Standards for INS

  10. Initial clinical assessment to include: • Height, weight, surface area • Volume status • Perfusion • Capillary refill • CPΔT • Heart rate and BP • JVP or hepatomegaly • Urinary sodium

  11. Initial investigation Investigations to be performed in all children • Blood: FBC, U+E’s; Creatinine; LFT’s; Varicella titres • Urine: Urine culture and Urinary protein:creatinine ratio (PCR) • Urinalysis including glucose • Urinary sodium concentration in those at risk of hypovolaemia Investigations to be performed in selected children • ASOT – strep throat infection; anti-DNaseB – strep skin infection • C3/C4 – post-strep GN; MPGN; SLE • Hepatitis B status in children at high risk: FH of HBV; history of travel in endemic areas.

  12. Clinical assessment for complications and early management • Hypovolaemia • with or without shock • Hypertension • ‘urgent’, persistent and paradoxical hypertension • Infection • role of antibiotic prophylaxis • Renal Vein Thrombosis

  13. Hypovolaemia and albumin infusion • With shock • Volume resuscitation • 4.5% albumin 5 – 20ml/kg • Without shock • 20% albumin with furosemide • Nursing guideline • 20% Albumin Infusion Careplan

  14. Atypical featuresEarly discussion with nephrologist • Typical Features • Age 1-10 years • Normotensive • Normal Renal Function • Microscopic haematuria (in up to 25%) • Atypical Features • <1yr, >10years • Hypertensive • Elevated Creatinine • Macroscopic Haematuria • Systemic, extra-renal disease symptoms • Positive family history of nephrotic syndrome Nephrotic Syndrome Flowchart

  15. Medication management • Steroid trial • Antibiotic prophylaxis • Gastro-protection • Albumin and diuretics • Fluid restriction • Symptomatic oedema – skin compromise • Use with care • BNFc hyperlinks for drug therapies

  16. Nursing management • Monitoring progress • Nursing Careplan - Nephrotic Syndrome • Daily weight • Fluid balance • Daily EMU ‘stix’/PC:R • BP • Parent teaching • disease information • urine testing and recording • Albustix for monitoring

  17. Dietetic management • Dietician • salt intake and fluid restriction • limit calorie intake on steroids • normal protein intake • ‘healthy diet’ for all the family • draft dietetic guideline

  18. Pharmacist drug dosing and administration appropriate formulation steroid weaning regimen antibiotic prophylaxis gastro-protection Renal Medication Information Booklet Vaccination Pneumococcal Varicella status non-immune ZIG (passive immunity) for exposure prophylaxis IV aciclovir for active infection Active immunisation when off immunosuppressives Annual flu vaccination Further Pharmacy management

  19. Childhood Nephrotic Syndrome – Biopsy Indications • Systemic disease • Extremes of age • Under 1 year • Older child • Low complement • Gross haematuria • Persistent hypertension • Impaired renal function • Steroid unresponsive at 4-8 weeks

  20. Quality Standards for INS

  21. Discharge management planNephrotic Syndrome - Discharge Planning Checklist

  22. 10.2 Patient and Family Information Provision and LinksWeb links for patient information on Nephrotic Syndrome Edinburgh Renal Unit’s website EdREN Info The 3 links below are all from the NKF website – the 3rd link is about a renal biopsy: A guide to the treatment of Childhood Nephrotic syndrome Nephrotic syndrome in Children Rebecca has a Renal Biopsy This is a North American document: From Kids Kidney Research (adapted from a GOS info sheet): The BKPA website PDF link: A BBC website one: Wikipedia link:

  23. SSNS – drug therapies • Corticosteroid therapy • Cochrane review • Non-corticosteroid therapy • Cochrane review • Newer arrivals

  24. Steroids in INS • The reduction in risk for relapse is associated with both an increase in duration and an increase in dose. • During daily therapy, prednisone is as effective when administered as a single daily dose compared with divided doses. • Alternate-day therapy is more effective than intermittent therapy (three consecutive days of seven days) in maintaining remission.

  25. RHSCG guideline review • 60 mg/m2/day for 4 weeks (maximum 80 mg) • 40 mg/m2/on alternate days for 4 weeks (maximum 60mg) • Reduce dose by 5-10mg/m2 each week for another 4 or 8 weeks then stop

  26. How do you taper steroids? • Steroid Dosing - First Presentation • Excel spreadsheet link from guideline specifying reduction regimens according to BSA ‘bandings’ at presentation • Steroid Dosing - Relapsing • Excel spreadsheet link from guideline specifying reduction regimens according to BSA ‘bandings’ on relapse • Taper options over 4 or 8 weeks

  27. What is the follow up routine?

  28. What is the best approach to relapses? • Information on relapse definition • Confirm relapse if in doubt • Treat to remission and taper steroid • Penicillin prophylaxis until remission • Gastro-protection on high dose steroid

  29. When should I consider asking about second line therapy? • Frequent relapsers • How often? • Twice in first 6 months • Four times in any 12 months • Steroid dependence • How dependent? • > 0.5mg/kg alternate day • Or steroid toxic side effects

  30. What approaches will be considered? • Low dose alternate day steroid • Increase to daily over 6 days for viral URTI • A choice of second line agents • Discuss pros and cons of each • Need for monitoring • Trial of treatment • Duration of treatment if successful • Work through the list if not successful

  31. Non-corticosteroid second line therapies • Levamisole • Alkylating agents • Cyclophosphamide (Chlorambucil) • Calcineurin inhibitors • Ciclosporin (Tacrolimus) • Mycophenolate mofetil • Rituximab

  32. Cochrane review conclusions • Effective second line therapy • Alkylating agents • Levamisole • Cyclosporin • No data to support one agent over another • Ineffective therapies • Azathioprine • Mizoribine

  33. Mycophenolate Mofetil (MMF) • MMF for SDNS: phase II Bayesian trial • 23 children with SDNS • MMF in combination with low-dose alternate-day prednisone • effective to maintain long-term remission and reduces steroid dose • Baudouin et al. Pediatr Nephrol Online First™, 27 September 2011 • Well tolerated • Steroid sparing • Ciclosporin sparing • Improved GFR • Reduction in relapse rate • Increase in relapse rate on MMF cessation

  34. Monoclonal antibody therapy • Rituximab • chimeric human-murine monoclonal antibody • binds specifically to the CD20 antigen on pre-B and mature B lymphocytes, mediating B-cell lysis • not directed against plasma cells. • FRSSNS & SDNS • Response rate >75% • Relapse on B cell repopulation

  35. Rituximab • Give in remission • >75% good initial response • Side effects – acute reactions • ~75% relapse with B-cell repopulation • Responsive to repeat Rituximab treatment • Re-treat at first relapse • Further treatment on B cell re-population • Possible MMF role in maintaining remission on B cell repopulation • Allows withdrawal of CNI, MMF and steroids

  36. Summary • The acute management of nephrotic syndrome can be optimally managed in a general paediatric setting with multi-disciplinary team support • Awareness of atypical and complicating features allows early discussion with specialist centres • Various second line therapies are available with no single optimal treatment • Following specialist discussion most treatments can be administered locally with shared/joint clinic review • Novel therapies requires detailed counselling, careful administration and close follow-up

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