Nephritic & Nephrotic Syndrome Flashcards
What are the 3 layers of the Glomerular Filtration Barrier?
Endothelial Cell layer - fenestrated
GBM - Type 4 collagen & laminin with embedded mesangial cells (smooth muscle)
Podocytes (Podocin & nephrin proteins)
Define Nephritic vs Nephrotic Syndrome?
Nephritic = High haematuria & intravascular overload
Nephrotic = High proteinuria & intravascular depletion
What would Nephrotic syndrome look like?
OEdema in face, legs, ascites and pleural effusions
~Raised BP
Frothy Urine
~Pale but mostly well
What causes Nephrotic Syndrome?
85% are Minimal Change Disease
15% are termed “Steroid REsistant nephrotic syndrome” which includes:
- Congenital NPHS1 or 2
- Acquired FSGS (Focal segmental glomerular sclerosis)
How do we confirm minimal change disease?
U&Es + Creatinine = Renal function should be normal
BP = Generally Normal
Urine Dipstix = high Protein & maybe haematuria
Trial Prednisalone
Why don’t we biopsy in most nephrotic syndromes?
Most it’s almost always minimal change disease so we just trial treatment
Nephrotic Syndrome is treated with 8wks of prednisolone. what are the major SEs?
Commonest: GI due to high acid Behaviour (irritable/moody/don't sleep) HTN Weight Gain
Uncommon:
Glucose intolerance
Adrenal crisis
Growth problems with recurrent courses
Describe the outcome of MCD?
95% go into remission in 2-4wks
80% will relapse
How do you treat Steroid Resistant Nephrotic Syndromes e.g. FSGS?
Second line immunosuppression
Try to list 8 major causes of childhood haematuria?
UTI
Trauma
Stones
Glomerulonephritis due to:
- Post-infective GN
- IgA nephropathy / HSP
- Membranoproliferative GN
- SLE
- ANCA +ve vasculitis e.g. GPA
What’s the classic presentation of Nephritic Syndrome?
- Haematuria (frank or microscopic)
- Proteinuria
Reduced GFR:
–> Oligura
–> Fluid overload (JVP/oedema)
–> HTN
–> High creatinine
Often comes post-infection, particularly throat
What’s the most common nephritic syndrome in kids vs adults?
Kids - Post-infective Glomerulonephritsi
Adults – IgA Nephropathy
What causes Post-infective Glomerulonephritis?
Group A strep
Throat infections 7-10days later
skin 2-4wks later
Nephrogenic strep antigens bind to sites in glomeruli & form immune complexes which are deposited there
This triggers Alternative Complement activation & Adaptive immune response leading to the nephritis
How can you diagnose Post-infective Glomerulonephritis?
A bacterial culture & throat swab for Strep A
+ve ASO Titre
Low C3 (used up)
Normal Renal US
ANA (SLE) + ANCA (GPA) to rule out autoimmune
So child with nephritic syndrome presents a week after having a throat infection.
Tests show Strep A culture, +ve ASO titre, low C3
Renal US, ANA & ANCA were all -ve
How would you treat the kid?
Diagnosis of Post-infective GN
Abx
Diuretic for fluid overload
Support renal function (e.g. restrict fluid, restrict Na, ACEI etc)
It will be self-limiting
IgA nephropathy is a commoner cause of nephritic syndrome in older kids/adults. What causes it?
Often comes 1-2days post or during an URTI (So much quicker than post-infective GN)
How do you diagnose and treat IgA nephropathy?
Clinical diagnosis with biopsy to confirm
ACEI
If severe give Immunosuppression
What is IgA Vasculitis / HSP?
Henoch-Sholein Purpura
It’s very similar to IgA nephropathy but comes with more systemic problems. You need Palpable purpura + 1 of
- Renal involvement
- Abdo pain
- Arthritis
- Renal biopsy showing IgA deposits
Again it comes 1-3days post viral URTI
How do you treat HSP/IgA vasculitits?
Symptomatic treatments for joint/gut symptoms e.g. analgesia & NSAIDs
CCS may help gut
Immunosuppression if severe
ACEI for kidneys
Long term monitor for HTN & Proteinuria
Which Glomerulonephritidis affect which layer of the Glomerular Filtration BArrier?
Podocytes = MCD & SLE
GMB = Membranous & Post-infective (PIGN)
Endothelial cells = PIGN, HUS, Membranoproliferative glomerulonephritis or SLE
mesangial = HSP, IgA nephropathy or SLE
