Renal disease in a child: Nephrotic syndrome Flashcards

1
Q

Define nephrotic syndrome.

A

Characerised by hypoalbuminaemia, proteinureia and oedema.

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2
Q

Explain the aetiology of nephrotic syndrome.

A

All causes of glomerulonephritis (GN) can cause nephrotic syndrome.

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3
Q

What is primary nephrotic syndrome?

A

Described by histology; Minimal change disease (MCD), focal segmental glomeruloscelrosis (FSGS) and membranous nephropathy (MN)

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4
Q

What are causes of secondary nephrotic syndrome?

A

SLE, post-infectious (group A B-haemolytic streptococcus, syphilis, malaria, TB, varicella, hepatitis B, HIV, EBV), collagen vascular disease, HSP, hereditary nephritis (Alport syndrome), sickle cell disease

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5
Q

What is the pathophysiology of nephrotic syndrome?

A

Proteinuria: Structural damage to the glomerular membrane and reduction of its negatively charged componenets prevent the action of repelling negatively charged proteins, which are therefore excreted in excess.

Hypoalbuminaemia: Secondary to proteinuria and increase breakdown of albumin in the kidney.

Oedema: Hypoalbuminuria leads to decreased intravascular colloid osmotic pressure.

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6
Q

Summarise the epidemiology of nephrotic syndrome.

A

Developed countries: 2-7/100,000/year (UK). MCD is the cause of nephrotic syndrome in 90% of children; most common in boys < 5 years; Peak age: 2-4 years

Developing countries: Infectious cause of GN: malaria (40%), HBV infection (6%) and group A B-haemolytic streptococcal infection (rare)

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7
Q

What are signs and symptoms of nephrotic syndrome?

A

General: Anorexia, lethargy, oliguria, hypertension GI: Diarrhoea, poor feeding, abdominal pain.

Oedema: Swelling of face, ascites, oedema of legs/scrotum Symptoms of complications: Infectious, renal vein thrombosis, loin pain, haematuria.

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8
Q

What are investigations for nephrotic syndrome?

A

Bloods: U&E, decrease albumin, increase ESR/CRP, lipid profile (secondary hyperlipidaemia).

Post-infectious nephropathy: Plasmodium falciparum (thick and thin blood films), ASOT, HBV/EBV/HIV serology, HIV, PCR.

Urine dipstick: 3/4 + protein, microscopic haematuria

MSU: MC&S

24-hour urine collection: Creatinine clearance and 24-hour protein excretion

Renal USS: Other renal diseases may cause proteinuria, e.g. Polycystic kidney disease

Renal biopsy: Reserved for older children with haematuria, increase BP, renal impairment, steroid-resistant patients

Doppler USS, renal angiogram, CT, MRI: If renal thrombosis is suspected

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9
Q

How is nephrotic syndrome treated symptomatically?

A

Limit oedema with low-sodium diet and diuretics

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10
Q

What should be monitored when treating nephrotic syndrome?

A

BP, U&E, Ca2+, weight, fluid balance

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11
Q

How should the initial presentation of nephrotic syndrome be treated?

A

Longer duration (6 months) of initial prednisolone treatment is associated with fewer relapses and lower total prednisolone dose over the first 2 years

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12
Q

How should a relapse of nephrotic syndrome be treated?

A

Prednisolone daily unit in remission, then a slow gradual reduction of dosage

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13
Q

How should steroid-resistant patients be treated?

A

Alternate-day prednisolone with long-term cyclosporine or cyclophosphamide. Steroid-sensitive patients (85-90% cases) respond after 4 weeks, steroid-resistant (10-15% cases) have no remission after 4 weeks

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14
Q

How should hypertension in nephrotic syndrome patients be treated?

A

ACE inhibitors are drug of choice

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15
Q

How should complications in nephrotic syndrome be treated?

A

Penicillin prophylaxis to prevent pneumococcal peritonitis and septicaemia, mobilisation/TED stockings to prevent thrombosis.

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16
Q

What are complications associated with nephrotic syndrome?

A

Renal failure: Secondary to hypovolaemia, diuretics or renal vein thrombosis.

Increase infection susceptibility: Peritonitits, pneumococcal infections due to urinary loss of immunoglobulin.

Hypercoagulability: Renal vein thrombosis and DVT secondary to hypovolaemia, urinary loss of antithrombin III, protein C and S, increase synthesis of fibrinogen in the liver, immobility secondary to leg oedema and steroid therapy.

Hyperlipidaemia: Increase synthesis of triglyceride and cholesterol with albumin in liver.

17
Q

What is the prognosis of nephrotic syndrome?

A

Before the introduction of steroids, 60% of children with the relapsing form of nephrotic syndrome died.

Steroids have revolutionised the prognosis of this condition but prognosis is affected by complications and S/E of treatment.