Nephrotic syndrome- -oedematous and oliguric 22 nd June 2012 Rachel Lennon Consultant Paediatric Nephrologist Royal Manchester Children’s Hospital
Mar 30, 2015
Nephrotic syndrome- -oedematous and oliguric
22nd June 2012Rachel Lennon
Consultant Paediatric NephrologistRoyal Manchester Children’s Hospital
Nephrotic syndrome: Most common glomerular disorder in children
Endothelial cells
Podocyte
GBM
1 million glomeruli in each human kidney
Glomerular filtration barrier
Podocytes
Glomerular endothelial cells
GBM
180 litres of water and small solutes- almost no
proteins
Slit diaphragm
A clinical syndrome: Triad
Massive proteinuria
Oedema
Albumin <25g/l
• 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-fetal transmission
Aetiology
Minimal change nephrotic syndrome (MCNS): Commonest in children
Membranous nephropathy
Focal segmental glomerulosclerosis
Mesangioproliferative GN
Minimal change nephrotic syndrome
Electron microscopy
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
Complications
• Thrombosis– Haemoconcentration– Increased fibrinogen, factor VII, X, VIII– Decreased anti-thrombin III and plasminogen
• Infections– Immunological losses
• Pneumococcal infections• Primary peritonitis
Acute management of nephrotic syndrome
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
Δ Nephrotic syndrome
ABC
HR/BP/CRT normal
• Predinsolone 60mg/m2/day• Fluid restriction to 70%• Low salt diet
Oedema
Estimate dry weight
• Diuretics: Furosemide and spironalactoneClose monitoring
Daily weightFluid balance
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
Δ Nephrotic syndrome
ABC
HR/BP/CRT normal
Hypovolaemia
Fluid bolus: 10-20ml/kg 4.5%
HASReassess
• Predinsolone 60mg/m2/day• Fluid restriction to 70%• Low salt diet
Oedema
Estimate dry weight
• Diuretics: Furosemide and spironalactoneClose monitoring
Daily weightFluid balance
Urine NaHaematocrit
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
Δ Nephrotic syndrome
ABC
HR/BP/CRT normal
• Predinsolone 60mg/m2/day• Fluid restriction to 70%• Low salt diet
OedemaSymptomatic
oedema 0.5-1g/kg (0.25-5ml/kg) 20% salt poor albumin
Over 4 hoursFurosemide (1mg/kg) at 2 hours
In consultation with Paediatric Nephrologist
Estimate dry weight
• Diuretics: Furosemide and spironalactoneClose monitoring
Daily weightFluid balance
Urine NaHaematocrit
3.5g/kg 4hrs
2.5g/kg 3hrs no diuretic
1g/kg over 1 hour
ISKDC- mortality in MCNS
Δ Nephrotic syndrome
ABC
HR/BP/CRT normal
Hypovolaemia
Fluid bolus: 10-20ml/kg 4.5%
HASReassess
• Predinsolone 60mg/m2/day• Fluid restriction to 70%• Low salt diet
OedemaSymptomatic
oedema 0.5-1g/kg (0.25-5ml/kg) 20% salt poor albumin
Over 4 hoursFurosemide (1mg/kg) at 2 hours
In consultation with Paediatric Nephrologist
Estimate dry weight
• Diuretics: Furosemide and spironalactoneClose monitoring
Daily weightFluid balance
Urine NaHaematocrit
Questions?