Paediatric Nephrology

Question 1

causes of haematuria and proteinuria nephrotic syndrome

Answer 1

o Acute glomerulonephritis
o Haemolytic uraemic syndrome
o Henoch schonlein purpura

Question 2

paeds kidney problems

Answer 2

•	Proteinuria/haematuria
o	Glomerular disease
	Nephrotic syndrome
	Nephritic syndrome
•	Acute kidney injury
o	Haemolytic uraemic syndrome
•	Chronic kidney disease
o	Developmental anomalies (CAKUT)
	Reflux nephropathy

Question 3

GFR in children

Answer 3

o Neonate 20-30 ml/min/1.73m2

o Age 2yrs = adult 80-120

Question 4

acquired glomerulopathy

Answer 4

Acquired glomerulopathy is a common condition. It is necessary to determine which component is affected.

•	Epithelial cell (podocyte)
o	Mcd, fsgs, lupus
•	Basement membrane
o	Membranous glomerulopathy, mpgn, pign
•	Endothelial cell
o	Infection associated glomerulonephritis (pign), haemolytic uraemic syndrome, membranoproliferative glomerulonephritis (mpgn), lupus
•	Mesangial cell
o	Hsp, iga nephropathy, lupus

Question 5

congenital glomerulopathy

Answer 5

This is much rarer. Determine which layer is involved.

•	Podocyte cytoskeletal integrity
o	Proteins - podocin (ar), nephrin (ar)
•	Basement membrane proteins
o	Alport syndrome (xl)
o	Thin basement membrane disease (ad) 
o	Collagen 4 mutation, xl~85, ar / ad~15%
o	Col4a5, col4a3 or4
•	Endothelial/microvascular integrity  
o	Complement regulatory proteins (mpgn)
o	Complement regulatory proteins complement factors h and i (ad)   and complement c3 (gain-of-function) 

Question 6

glomerulopathy

Answer 6

Blood and protein in varying amounts dictates the clinical presentation and most likely diagnosis. Injury to one part of the gfb affects the other components. Take home message is that proteinuria signifies glomerular injury. It is progressive in nature contributed by hypertension and proteinuria.

Question 7

define nephrotic syndrome

Answer 7

Nephrotic range proteinuria –> hypoalbuminaemia –> oedema

Normal albumin 36-44g/l, oedema if less then 25-30g/l, usually need pcr>600mg/mmol creatinine for oedema

Question 8

what causes oedema

Answer 8

protein loss

Question 9

typical paeds presentation of nephrotic syndrome

Answer 9

•	2.5 years
•	Hx
o	Gastroenteritis 10 days prior
o	3-4 days
	Swollen face (worse in mornings), one eye closed in the morning, legs
o	No allergies
o	Family hx – great grandmother nephrectomy 
•	Examination
o	Looked well, pale
o	Weight 14.9kg
o	Periorbital oedema, pitting oedema legs, ascites, no effusions
o	Bp 98/56 (can be raised)
o	Frothy urine

Question 10

treatment of nephrotic syndrome

Answer 10

If typical features treat with prednisolone for 8 weeks. Side effects from high dose glucocorticoids. Varicella status, pneumococcal vaccination, behaviour. With a longer first course there are fewer relapses. Glucocorticoid toxicity from treatment, gi se – acid blockade, glucose intolerance, hypertension, risk of adrenal crisis, growth.

Question 11

steroid resistant nephrotic syndrome: idiopathic

Answer 11

o Focal segmental glomerulosclerosis (fsgs)
o Podocyte loss
o Progressive inflammation and sclerosis

Question 12

steroid resistant nephrotic syndrome: congenital

Answer 12

o Infant presentations
o Nphs1 – nephrin
o Nphs 2 – podocin
o Podocyte loss