Nephrology Flashcards
Paediatric most common cause for proteinuria/ haematuria
Glomerular disease
- Nephrotic syndrome
- nephritic syndrome
Paediatric most common cause for acute kidney injury
Haemolytic uraemic syndrome
Paediatric most common cause for chronic kidney disease
Developmental anomalies (CAKUT) -reflux nephropathy
5 kidney functions
Waste handling (urea and creatinine) Water handling Salt balance (Na, K, Ca, P) Acid base control (bicarbonate) Endocrine (red cells/ blood pressure/ bone health)
What makes up the glomerular filtration barrier
Endothelial cells:
- Fenestrated
- Vulnerable to immune mediated injury
GBM:
- 2 proteins - Type IV collagen (COL4) and laminin
- Synthesis from podocytes and endothelial cells
- Mesangial cells playing a role in turnover
Podocyte:
- Proteins
- Podocin, nephrin
Mesangial cells:
- Glomerular structural support
- Embedded in GBM
- Regulates blood flow of the glomerular capillaries
What makes up the EM glomerular filtration barrier
Fenestrated endothelial cell,
Glomerular basement membrane,
Podocyte with “slit diaphragms”
Clinical indication of glomerulopathy
Blood and protein
What does proteinuria normally signify?
Glomerular injury
Clinical symptoms of nephritic syndrome (PHARAOH)
Proteinuria Haematuria Azotaemia Red blood cell casts Oliguria Hypertension
Intravascular overload
Clinical symptoms of nephrotic syndrome
Proteinuria
Hypoalbuminaemia
Oedema
Hyperlipidaemia
Intravascular depletion
In acquired glomerulopathy what components are affected
Epithelial cells (podocyte)
- Minimal Change Disease
- FSGS
- Lupus
Basement membrane
- Post infectious glomerulonephritis
- Membranous glomeruopathy
- MPGN (Membranoproliferative glomerulonephritis)
Endothelial cell
- PIGN
- HUS
- MPGN
- Lupus
- ANCA vasculitis
Mesangial cell
- Henoch-Schonlein purpura
- IgA nephropathy
- Lupus
Congenital glomerulopathy layers involved
Podocyte cytoskeletal integrity
(congeital nephrotic syndrome)
-Proteins- Podocin, nephrin
Basement membrane proteins
- Alport syndrome (XL)
- Thin basement membrane disease
Endothelial/ microvascular integrity
-Complement regulatory protein
Testing for proteinuria
Dipstix (>2 usually abnormal)
Protein creatinine ratio (nephrotic range >250mg/mmol)
24hr urine collection (nephrotic range >1g/m2/24hrs)
Nephrotic syndrome treatment
Prednisolone 8 weeks
Second line immunosuppression
Who does nephrotic syndrome clinically present?
pale, periorbital oedema, pitting oedema legs, ascites, small pleural effusions, hypotension, frothy urine
Common cause of nephrotic presentation in children?
Minimal change disease,
Focal segmental glomerulosclerosis
Minimal change disease typical features
age 2-5
normal BP
resolving microscopic haematuria
normal renal function
Atypical features of MCD
What would you consider doing if someone presented with these?
Suggestions of autoimmune disease,
abnormal renal function,
steroid resistance
-Consider renal biopsy
Main side effects noticied by parents in children with glucocorticoid treatment
behaviour change,
mood change,
sleep disturbance
Glucocorticoid treatment, what must the dr think about before
hypertension,
pneumococcal vaccination,
antibiotic prophylaxis
what is on the spectrum for idiopathic nephrotic syndrome in childhood
non relapsing, infrequently relapsing, frequently relapsing, steroid dependent, Steroid resistant (Focal segmental glomerulosclerosis)
Pathology behind steroid resistant nephrotic syndrome
Interaction between lymphocytes (t and B cells) and podocytes
Acquired cause of steroid resistant nephrotic syndrome
Focal Segmental Glomeruloscerosis
causes podocyte loss and progressive inflammation and sclerosis
Congenital causes of steroid resistant nephrotic syndrome
NPHS1-nephrin loss
NPHS2- podocin loss
infant presentation
What does persistent haematuria and proteinuria suggest
glomerular disease
Causes of haematuria
systemic:
-clotting disorders
Renal:
- Glomerulonephritis
- Tumour (Wilm’s nephroblastoma)
- Cysts
Malignancies:
-Sarcomas
Stones
UTI
Trauma
Urethritis