Nephrotic syndrome - MCD Flashcards
GFR values - neonates
adult
- 20-30ml/min/1.73m²
- 90-120
5 key functions of the kidney
Waste handling - urea, creatinine
Water handling
Salt balance - sodium and calcium phosphate
Acid base control - bicarbonate
Endocrine
red cells / blood pressure / bone health
what proteins are in the GBM?
Type IV collagen (COL4) and laminin
- MESENGIAL CELLS WITHIN
Podocytes - proteins within wall?
Podocin, nephrin
Mesangial cells - what do they regulate?
glomerular capillaries
how do patients present
haematuria and proteinuria
- frothy urine
what does proteinuria signify?
glomerular injury
Nephritic syndrome tends to have?
Increasing haematuria
Intravascular overload
Nephrotic syndrome tends to have?
Increasing proteinuria
Intravascular depletion
what component is affected in Acquired Glomerulopathy
- what disease does this cause?
Epithelial cell (podocyte) -Minimal Change Disease
Basement Membrane condition
Post Infectious Glomerulonephritis (PIGN)
Endothelial cell condition
PIGN, Haemolytic Uraemic Syndrome (HUS)
Mesangial cell condition
HSP / IgA nephropathy
Congenital nephrotic syndrome proteins?
Podocin (AR), nephrin (AR)
Congenital basement membrane proteins - syndromes? (2)
Alport syndrome (XL) Thin basement membrane disease (AD)
Endothelial/microvascular integrity - what proteins?
Complement regulatory proteins (MPGN)
what is a abnormal dipstick reading?
> 3
proteinuria assessment in children?
Protein Creatinine Ratio (practical)
Early morning urine (best)
Creatine range normal ?
20mg/mmol
protein creatine ratio - nephrotic range?
250mg/mmol
gold standard test for proteinuria? and what are the normal values - adults and children
24hr urine collection (gold standard)
normal <60mg/m²/24hrs
Nephrotic range>1g/m²/24hrs
Adults >3.5g/24hrs
what is nephrotic syndrome
- Nephrotic range proteinuria
- Hypoalbuminaemia, low blood albumin
- Oedema - increasing 3rd space fluid volume
Typical presentation of nephrotic syndrome or MCD?
age 2 and a half
Gastroenteritis 10days prior
3 -4 days
Swollen face (worse in mornings), one eye closed in mornings, legs
presentation of nephrotic syndrome in kids?
Looked well, pale Inflated weight 14.9kg Periorbital oedema, pitting oedema legs, ascites, small pleaural effusions BP 98/56 (can be raised) Frothy urine
how do you know when it is nephrotic syndrome?
Oedema
Proteinuria >3
Protein Creatinine Ratio – 1200mg/mmol creatinine - high
Urine Na – 10mmol/l - low
bloods - albumin low
microscopic heamaturia sign?
minimal change disease
Typical features of nephrotic syndrome - MCD? (4)
Age 1 - 10
Normal blood pressure
No Frank haematuria
Normal renal function
Atypical features of MCD - nephrotic syndrome
Suggestions of autoimmune disease
Abnormal renal function
Steroid resistance
renal biopsy
Drug treatment for MCD?
Prednisolone 8 weeks
Prednisolone side effects? (5)
personality changes greater infection rate hypertension GI distress - increased acid growth
infection risk of glucocorticoids (Prednisolone)
Varicella status
Pneumococcal vaccination
Antibiotic prophylaxis
if steroids don’t work for MCD - what condition is this more indicitive of?
Focal Segmental Glomerulosclerosis - podocyte loss
pathogenesis of nephrotic syndrome?
Interaction between lymphocytes (T and B cells) and podocytes
congenital FSGS - Mutations in what genes?
NPHS1 – nephrin
NPHS 2 – podocin
Podocyte loss
