Nephrotic Syndrome

 

Renal disease with a triad of:

• Hypoalbuminemia (S albumin <25 g/L)
• Significant proteinuria (3+ or more on dipstick / Urine Alb:Creat ratio >200 mg/mmol)
• Oedema

Common in younger children (preschool or school age), boys & asian origin

Seek specialist advise from paediatric nephrology

 

Causes:

– Minimal change disease (commonest cause)

– Other renal:

    Focal segmental glomerular sclerosis (FSGS)

    Membranoproliferative glomerulonephritis

– Congenital nephrotic syndrome (very rare)

– Secondary to multisystem disorders (SLE, HSP, diabetes mellitus, HepB or C etc)

 

History

– Oedema: peri-orbital, pedal, scrotal or rarely ascites

– Reduced urine output

– Precipitating cause often absent, but maybe due to gastroenteritis or other illness

– Be alert to clinical features of complications

    Lethargy and abdominal pain if peritonitis or severe intravascular dehydration

    Breathless if severe ascites or pleural effusion

    Severe abdo pain with hematuria or severe headache if thrombosis

 

Examination

– Record weight, HR, RR, BP & Temp

– Assess for dehydration / shock (third space losses)

  Increased HR, thread pulse, prolonged capillary refill, cold peripheries, low BP, confusion

– Extent of pitting oedema- often peri-orbital, but also pedal, scrotal or rarely ascites

– Hypertension may be present if nephritis

– Tachypnoea / Recessions/ dull percussion if pleural effusion

– Abdominal distension and shifting dullness if ascites

– Abdominal tenderness & guarding may indicate peritonitis

 

Investigations

– Confirm significant proteinuria

     3+ or more on dipstick

     Spot urine Albumin:Creat ratio >200 mg/mmol (normal is <20)

     24 hour urine protein excretion > 3g/day

– Urine sodium (<10 mmol) suggests intravascular hypovolaemia

 

Bloods

U&E – raised creatinine or urea if prerenal hypovolemia or renal disease

LFT – low serum Albumin <25g/L

Lipid profile – elevated cholesterol

FBC – raised haematocrit if hypovolaemia

Coagulation

Ig G, A and M – may slow low IgG

Complement C3 and C4 – normal in minimal change disease

Zoster immune status: as a baseline

Hepatitis B and C serology – fo aetiology

Consider ASO titre, Antinuclear and Anti-ds DNA antibodies

 

Management

Supportive- admit to monitor daily weight, input/ output chart, BP

Avoid excess salt intake

Fluid management can be tricky

    Restrict daily fluid intake to 800ml – 1000ml (seek guidance)

Intravascular hypovolemia with low urine output

     Confirm if Urine Sodium < 10mmol/L

     Give 20% Albumin (to draw fluids in to blood vessels), then give Furosemide (seek guidance)

High dose oral Prednisolone 60 mg/m2 for 4wks, then 40mg/m2/d alternate day for 4wks, then taper (seek guidance)

Gut protection with Omeprazole

Penicillin V for prophylaxis  (as low IgG) until oedema persists

 

Complications

– Peritonitis (as low immunity)- suspect if abdominal pain; steroids may mask signs

     Ascitic tap for culture & broad spectrum Antibiotics

– Thrombosis (due to hypercoagulable state) – of renal veins or cavernous sinus in brain

    early imaging if suspecting & specialist management

 

Prognosis

Most have minimal change disease & are steroid responders

Relapse considered if proteinuria >3+ on 3 consecutive mornings, restart high dose steroids (seek guidance)

Non-responders to steroids (at 4 weeks) need further specialist management