Paediatric Nephrotic Syndrome Flashcards
In summary, what causes proteinuria/haematuria in children?
glomerular disease - nephrotic syndrome
nephritic syndrome
In summary, what causes acute kidney injury in children?
haemolytic uraemic syndrome
In summary, what causes chronic kidney disease in children?
developmental anomalies leading to reflux nephropathy
What is a normal GFR in neonates?
20-30ml/min/1.73m2
What is a normal GFR in children aged 2?
same as adult: 80-120ml/min/1.73m2
What are the 3 layers of the glomerular filtration barrier?
- fenestrated endothelial cell
- glomerular basement membrane
- podocytes
Is it more common to acquire or have a congenital glomerulopathy?
more common to have an acquired glomerulopathy
When considering a glomerulopathy, what dictates the clinical presentation and most likely diagnosis?
blood and protein in varying amounts
Take home message. What does proteinuria signify?
glomerular injury
Why can glomerulopathies have a progressive nature?
hypertension and the proteinuria can worsen condition
Nephrotic syndrome and nephritic syndrome are 2 overlapping ways that may be used to narrow down diagnoses. What makes conditions have a nephritic syndrome presentation compared to classical nephrotic syndrome presentation?
- nephritic syndrome is characterised by increasing haematuria and intravascular overload
- nephrotic syndrome is characterised by increasing proteinuria and intravascular depletion
What 3 signs define nephrotic syndrome?
- nephrotic range proteinuria (>250mg/mmol)
- hypoalbuminaemia (normal is 36-44g/l)
- oedema
Why does oedema occur in nephrotic syndrome disease processes?
damage in glomeruli results in protein leaking out which allows water to follow it
Where can the oedema occur in nephrotic syndrome?
periorbital, pitting oedema of legs, ascites
What are 3 ways you can test for proteinuria?
- dipstix (+++ usually indicates abnormal)
- protein creatinine ratio (use early morning urine and are looking for nephrotic range proteinuria)
- 24hr urine collection
How would you diagnose a patient with nephrotic syndrome?
- oedema
- proteinuria on dipstix
- protein creatinine ratio
- urine sodium- 10
- bloods (low albumin and normal creatinine)
What is the most common cause of nephrotic syndrome in childhood?
minimal change disease
What is minimal change disease?
- have nephrotic syndrome but only on electron microscopy can the effacement of the podocyte for processes be seen
- this allows the proteinuria
- 85% will have minimal change disease
How would you diagnose/ treat minimal change disease?
- recognise young child with nephrotic syndrome is likely to have minimal change disease
- treat with steroids (prednisolone for 8 weeks) and if responsive then has MCD
- only if the steroids do not work should you consider renal biopsy
What is another common (but much less common than MCD) cause of nephrotic syndrome?
focal segmental glomerulosclerosis (FSGS)
What is focal segmental glomerulosclerosis?
- when some (focal) glomeruli in the kidney become affected by scarring change (sclerosis) in only a segment (segmental) of the glomerulus
- the glomeruli that are affected by the scarring allow proteins to pass through their filtration barrier, ultimately resulting in nephrotic syndrome
How would FSGS be treated?
- can be steroid resistant!
- so tend to use second lin treatment which would be immunosuppression e.g. cyclosporin, tacrolimus
What tends to be the outcome of nephrotic syndrome disease?
- MCD tends to result in remission but can recur
- FSGS can result in remission ht sometimes can progress and result in chronic kidney disease
What are 3 causes of acquired nephrotic syndrome?
- HSP/ IgA
- lupus
- post infectious
