Nephrotic syndrome- -oedematous and oliguric 22 nd June 2012 Rachel Lennon Consultant Paediatric Nephrologist Royal Manchester Childrens Hospital.

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?