If 'simple, idiopathic' i.e. not atypical nephrotic syndrome give HIGH DOSE ORAL STEROIDS. 60mg/kg prednisolone for 4 WKS.
Reduce to 40mg/kg for another 4 weeks, on ALTERNATE days. Recent evidence to suggest that if the tapering (alternate day) period is LONGER, reduces proportion of children that develop steroid dependent or recurrent course of nephrotic syndrome.
Further investigations: if complicated or atypical nephrotic syndrome (not responsive to corticosteroid therapy etc) >> RENAL BIOPSY
Majority is steroid sensitive. Some are steroid-resistant (indication for further investigation)
RULE OF THIRDS - for initial steroid sensitive MCD nephrotic syndrome
1/3 resolve directly
1/3 have infrequent relapses
1/3 relapse frequently and become steroid dependent
On presentation commonly: periorbital oedema (worse on waking, first sign)
Scrotal, vulval, ankle, leg odema
SOB - due to pleural effusions and abdo distension
Watch out for abdominal pain - fluid accumulation or peritonitis
Dyspnoea - pleural effusion or bad ascites. Somtimes pneumonia or pulmonary embolus
Minimal change disease. Because on renal light microscopy histology = normal. On electron microscopy = podocyte fusion (abnormal). LOSS of anionic charge across GBM and endothelial membrane but no structural damage/change.
●Age younger than six years of age
●Absence of hypertension
●Absence of hematuria by Addis count
●Normal complement levels
●Normal renal function
*DDx for nephrotic syndrome:
orthostatic proteinuria (benign, found at the end of the day when child has been upright, normal in the morning)
*transient- not abnormal for children to get it during febrile illness and after exercise.
Glomerular abnormalities: if pathological - usually falls into this category. Minimal change disease, glomerulonephritis, genetic (familial nephritidies)
Small kidneys - dysplastic kidneys
Increased glomerular filtration pressure
PRIMARY - nephrotic syndrome in absence of systemic disease (includes idiopathic and inflammatory causes).
Idiopathic - mostly MCD and account for >90% of cases in children under 10yo.
Inflammatory (nephritic disorders --> nephrotic syndrome) like IgA nephropathy, membranoproliferative glomerulonephritis etc
are due to
Loss of immune proteins >> infection
PRO-coagulable state (thromboembolism) presumably due to loss of antithrombin in the urine. Increased synthesis of clotting factors, increased blood viscosity from increased haematocrit. Watch out for thrombosis in brain, limbs and spleen.
Iatrogenic complications - arising from treatment
Hypovolaemia - when becoming oedematous intravascular compartment may become volume-depleted.
Complaining of abdominal pail, feeling faint
Look for low urinary sodium (<20mmol/L)
high packed cell volume of rbcs
If oedema gets really bad - may be painful, cause respiratory distress. Treat with IV albumin - risk of thrombosis and shock
Increased risk of infection with encapsulated bacteria.
Loss of opsonising factors in the urine.
especially pneumococcal infection. SHould have 23 valent polysaccharide pneumococcal vaccine if not already vaccinated
Ascites + pleural effusions promote bacterial growth
Worry about INTRAABDOMINAL infection, sepsis, meningitis, cellulitis, UTIsWATCH for VARICELLA. Especially if they are receiving high dose steroids. Check immune status before they start.
Thromboembolism - significant morbidity associated with TEs in children.
Pulmonary embolism - can be silent in children. Should have high index of suspicion and should be considered if any cardio or resp symptoms. Confirmed with angiography
Renal vein thrombosis - higher risk in infants. Otherwise rare in older children and adults. Suspect if sudden onset haematuria, flank pain
Defining features: proteinuria, hypoalbuminaemia, oedema, hyperlipidaemia
1) nephrotic range proteinuria = >50mg/kg
2) <30g/L albumin in plasma
Required to diagnose