Nephrotic syndrome-oedematous and oliguric
22nd June 2012
Rachel Lennon
Consultant Paediatric Nephrologist
Royal Manchester Children’s Hospital
Nephrotic syndrome: Most common glomerular disorder in children
Endothelial cells
GBM
Podocyte
1 million glomeruli in
each human kidney
Glomerular filtration barrier
180 litres of water and small
solutes- almost no proteins
Glomerular endothelial cells
GBM
Slit
diaphragm
Podocytes
A clinical syndrome: Triad
Albumin
<25g/l
Oedema
Massive proteinuria
Aetiology
• 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
Minimal change nephrotic
syndrome (MCNS):
Commonest in children
Focal segmental
glomerulosclerosis
Mesangioproliferative GN
Membranous
nephropathy
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
Estimate dry weight
ABC
HR/BP/CRT
normal
Oedema
• Predinsolone 60mg/m2/day
• Fluid restriction to 70%
• Low salt diet
• Diuretics: Furosemide and spironalactone
Close monitoring
Daily weight
Fluid balance
Case 2
• 7 year old boy with SSNS
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–
–
–
–
•
•
•
•
•
•
•
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
Urine Na
Haematocrit
Hypovolaemia
Estimate dry weight
Fluid bolus: 1020ml/kg 4.5%
HAS
Reassess
Oedema
• Predinsolone 60mg/m2/day
• Fluid restriction to 70%
• Low salt diet
• Diuretics: Furosemide and spironalactone
Close monitoring
Daily weight
Fluid balance
Case 3
• 5 year old girl with FRNS
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–
–
–
–
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
Estimate dry weight
Urine Na
Haematocrit
HR/BP/CRT
normal
Oedema
Symptomatic
oedema
• Predinsolone 60mg/m2/day
• Fluid restriction to 70%
• Low salt diet
• Diuretics: Furosemide and spironalactone
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
Close monitoring
Daily weight
Fluid balance
3.5g/kg
4hrs
2.5g/kg
3hrs no
diuretic
1g/kg over
1 hour
ISKDCmortality
in MCNS
Δ Nephrotic
syndrome
ABC
Urine Na
Haematocrit
HR/BP/CRT
normal
Hypovolaemia
Oedema
Symptomatic
oedema
• Predinsolone 60mg/m2/day
• Fluid restriction to 70%
• Low salt diet
• Diuretics: Furosemide and spironalactone
Estimate dry weight
Fluid bolus: 1020ml/kg 4.5%
HAS
Reassess
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
Close monitoring
Daily weight
Fluid balance
Questions?