Paediatric Nephrology - Part 1 Flashcards
Describe glomerulopathy
Blood and protein in varying amounts dictates the clinical presentation and suggests diagnosis
Proteinuria signifies glomerular injury
What are the renal causes of haematuria and proteinuria?
Range from nephritic syndrome (increasing haematuria and intravascular overload) and nephrotic syndrome (increasing proteinuria and intravascular depletion)
Can be overlap
What component can be affected in acquired glomerulopathy?
Epithelial cell - minimal change disease
Basement membrane - post-infectious glomerulonephritis (PIGN)
Endothelial cell - PIGN and haemolytic uremic syndrome
Mesangial cell - HSP/ IgA nephropathy
Describe congenital glomerulopathy
Rare and layer involved
Podocyte cytoskeletal integrity - congenital nephrotic syndrome
Basement membrane proteins - Alport syndrome and thin basement membrane disease
Endothelial/ microvascular integrity - MPGN
How is proteinuria investiagted?
Dipstix - measure concentration >3+ is abnormal
Protein creatinine ratio - early morning is best
24hr urine collection - gold standard
What is nephrotic syndrome?
Nephrotic range proteinuria, hypoalbuminemia and oedema (increasing 3rd space fluid volume)
What is a typical presentation in nephrotic syndrome?
Looks well and pale
Inflated weight, periorbital oedema, pitting oedema legs, ascites and small pleural effusions
BP is low and frothy urine
How is nephrotic syndrome diagnosed?
Oedema, proteinuria on urine dipstix/ protein creatinine ration/ urine Na is low, and bloods showing low albumin + normal creatinine
Describe minimal change disease - nephrotic
Age 1-10, normal BP, no frank haematuria and normal renal function
Consider renal biopsy if suggestion of autoimmune disease, abnormal renal function and steroid resistance
Steroid response confirms diagnosis
What is the treatment of nephrotic syndrome?
Prednisolone for 8 weeks
Can have SE - personality changes, increased susceptibility to infection, sleep disturbance, GI distress and BP rise
How is infection risk managed in glucocorticoid treatment?
Varicella status
Pneumococcal vaccination
Antibiotic prophylaxis
What are the causes of steroid resistant nephrotic syndrome?
Acquired - focal segmental glomerulosclerosis (FSGS)
Congenital - NPHS1 (nephrin), NPHS2 (podocin) and podocyte loss
How is haematuria investigated?
Macroscopic/ frank - investigate
Microscopic - dipstix adequate
Investigate and look for haemoglobinuria as associated proteinuria - glomerular disease
What are some causes of haematuria?
Urethritis, trauma, UTI, stones, malignancies, cysts, tumours (Wilm’s), renal (glomerulonephritis) and systemic (clotting disorders)
What are some investigations for nephritic syndrome?
Increased waste accumulation - creatinine
Electrolytes normal, FBC with mild anaemia and albumin is slightly low
Exclude UTI and PCR
What is nephritic syndrome?
Haematuria and proteinuria
Oliguria, fluid overload (raised JVP and oedema), hypertension and worsening renal function
Intrarenal cause of AKI
What components are affected in acquired glomerulopathy - nephritic?
Epithelial cells - MCD, FSGS and lupus
Basement membrane - membranous glomerulopathy and MPGN
Endothelial cells - PIGN and HUS
Mesangial cell - HSP/ IgA nephropathy
Describe post infectious glomerulonephritis
Age - usually age 3-5
Cause - group A strep, beta haemolytic and can be throat (shows 7-10 days after) or skin (shows 2-4 weeks after)
How does group A strep cause PIGN?
Nephrogenic antigens on strep which bind to specific sites in glomerulus and deposits in kidney (Antibodies to Ag forming complexes) which activated alternative compliment pathway - AKI
How is acute PIGN diagnosed?
Bacterial culture, positive ASOT, low C3 normalises and differential diagnosis (lupus and IgA nephropathy)
What is the treatment of acute PIGN?
Antibiotic and support renal function with electrolytes + acid balance management
Overload and hypertension - diuretics
Not recurrent
Describe IgA nephropathy
1-2 days after URTIs
Usually older children and adults
Clinically recurrent macroscopic haematuria and chronic microscopic haematuria
Varying degree of proteinuria
Clinical diagnosis and confirmation biopsy
What is the treatment for IgA nephropathy?
Mild- ACEi for proteinuria
Moderate to severe - immunosuppression
Outcome is variable
Describe Henoch schonlein purpura/ IgA related vasculitis
Age of onset is 5-15 years
Clinical diagnosis is mandatory palpable purpura and one of 4 - abdominal pain, renal involvement, arthritis/ arthralgia and biopsy (IgA deposition)
Describe IgA vasculitis
Small vessel and most common childhood vasculitis
IgA nephropathy cross over
IgA vasculitis with nephritis (HSP)
1-3 day post trigger - viral URTI, strep and drugs
4-6 weeks of symptoms
Mesangial cell injury
What is the treatment for IgA vasculitis?
Symptomatic - joints and gut
Glucocorticoid therapy - not helpful for mild but can help with G involvement
Immunosuppression
Long term - hypertensive and proteinuria screening
What conditions are more nephritic presentation?
Post infectious GN, HSP and IgA nephropathy
What conditions present more nephrotic?
FSGS and minimal change disease
