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N ephrotic syndrome- - oedematous and oliguric

N ephrotic syndrome- - oedematous and oliguric. 22 nd June 2012 Rachel Lennon Consultant Paediatric Nephrologist Royal Manchester Children’s Hospital . Nephrotic syndrome: Most common glomerular disorder in children. Endothelial cells. GBM. Podocyte.

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N ephrotic syndrome- - oedematous and oliguric

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  1. Nephrotic syndrome- -oedematous and oliguric 22nd June 2012 Rachel Lennon Consultant Paediatric Nephrologist Royal Manchester Children’s Hospital

  2. Nephrotic syndrome: Most common glomerular disorder in children

  3. Endothelial cells GBM Podocyte 1 million glomeruli in each human kidney

  4. Glomerular filtration barrier Glomerular endothelial cells 180 litres of water and small solutes- almost no proteins GBM Slit diaphragm Podocytes

  5. A clinical syndrome: Triad Oedema Albumin <25g/l Massive proteinuria

  6. Congenital Congenital infections Genetic mutations Eg. Nephrin, podocin Acquired No clearly identified mechanism Association with viral infections Circulating factors Recurrence of FSGS post renal transplant Materno-fetaltransmission Aetiology

  7. Minimal change nephrotic syndrome (MCNS): Commonest in children Focal segmental glomerulosclerosis Mesangioproliferative GN Membranous nephropathy

  8. Minimal change nephrotic syndrome Electron microscopy

  9. Induction and maintenance therapy • Glucocorticoids: ISKDC regime • 90% with MCNS initially respond • 33% no further relapse • 33% infrequent relapse • 33% frequent relapse • Prophylactic penicillin • 2nd line therapy • Cytotoxics • Cyclophosphamide • Ciclosporin

  10. Complications • Thrombosis • Haemoconcentration • Increased fibrinogen, factor VII, X, VIII • Decreased anti-thrombin III and plasminogen • Infections • Immunological losses • Pneumococcal infections • Primary peritonitis

  11. Acute management of nephrotic syndrome

  12. Case 1 • 3 year old boy • Facial swelling for 2 weeks • Treated with antihistamines • Urinalysis 3+ protein 1+blood • HR/BP/CRT normal • Periorbital and lower limb oedema • Albumin 15, Urea 4.5 Creat 30, Urine Na 30 • Treatment? • Prednisolone 60 mg/m2/day (Prednos trial?) • Penicillin V • Daily monitoring until remission

  13. Δ Nephrotic syndrome Estimate dry weight ABC HR/BP/CRT normal Oedema • Predinsolone 60mg/m2/day • Fluid restriction to 70% • Low salt diet Close monitoring Daily weight Fluid balance • Diuretics: Furosemide and spironalactone

  14. Case 2 • 7 year old boy with SSNS • Unwell with D&V for 3 days, • Urine 3+ protein • Lower limb oedema • HR 130, BP 100/78, CRT 5 seconds • Albumin 12, Urea 9.5, Creat 42, Urine Na 10 • Treatment? • IV fluid bolus (10ml/kg 4.5%HAS) • Reassess • Urine output • Prednisolone 60 mg/m2/day • Penicillin V • Daily monitoring until remission

  15. Δ Nephrotic syndrome Estimate dry weight ABC Urine Na Haematocrit Fluid bolus: 10-20ml/kg 4.5% HAS Reassess HR/BP/CRT normal Hypovolaemia Oedema • Predinsolone 60mg/m2/day • Fluid restriction to 70% • Low salt diet Close monitoring Daily weight Fluid balance • Diuretics: Furosemide and spironalactone

  16. Case 3 • 5 year old girl with FRNS • Ciclosporin • Oedematous for 2-3 weeks • Symptomatic oedema • HR 120, BP 105/80, CRT <2s • Albumin 8, Ur 7.5, Creat 52, Urine Na 15 • Treatment? • Cautious use of 20% albumin (2.5-5ml/kg dry weight) over 4 hours with IV furosemide at 2 hours. • Risk of life threatening pulmonary oedema • Daily 20% albumin • Prednisolone 60 mg/m2/day • Penicillin V • Daily monitoring until remission

  17. Δ Nephrotic syndrome Estimate dry weight ABC Urine Na Haematocrit HR/BP/CRT normal Symptomatic oedema Oedema 0.5-1g/kg (0.25-5ml/kg) 20% salt poor albumin Over 4 hours Furosemide (1mg/kg) at 2 hours In consultation with Paediatric Nephrologist • Predinsolone 60mg/m2/day • Fluid restriction to 70% • Low salt diet Close monitoring Daily weight Fluid balance • Diuretics: Furosemide and spironalactone

  18. 3.5g/kg 4hrs 2.5g/kg 3hrs no diuretic 1g/kg over 1 hour ISKDC- mortality in MCNS

  19. Δ Nephrotic syndrome Estimate dry weight Fluid bolus: 10-20ml/kg 4.5% HAS Reassess ABC Urine Na Haematocrit HR/BP/CRT normal Hypovolaemia Symptomatic oedema Oedema 0.5-1g/kg (0.25-5ml/kg) 20% salt poor albumin Over 4 hours Furosemide (1mg/kg) at 2 hours In consultation with Paediatric Nephrologist • Predinsolone 60mg/m2/day • Fluid restriction to 70% • Low salt diet Close monitoring Daily weight Fluid balance • Diuretics: Furosemide and spironalactone

  20. Questions?

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