Paediatric Nephrology - Glomerular Disease Flashcards
What percentage of CO do the kidneys recieve /min?
Receives 25% of CO/min
What is the GFR of:
- neonate
- age 2
- Neonate 20-30ml/min/1.73m2
- Age 2 years equals adult at 90-120ml/min/1.73m2
What are 5 functions of the kidneys?
- Waste handling
- Water handing
- Salt balance
- Acid base control
- Endocrine
- Red cells/blood pressure/bone health
What is the presentation of glomerular disease usually?
- Haematuria
- Usually darker in upper tract and lighter in lower tract
- Proteinuria
- Seen to be frothy
Glomerulopathy - presentation
- Blood and protein in vary amounts for different diseases
- Proteinuria signifies glomerular injury
What are the differences between nephrotic and nephritic syndromes for:
- haematuria or proteinuria
- IV overload or depletion
- Nephritis syndrome
- Haematuria
- IV overload
- Nephrotic syndrome
- Proteinuria
- IV depletion
What does the presentation of difference kidney diseases depend on?
Which component of kidneys is affected
Glomerulopathy - aetiology
- Acquired
- Commoner than congenital
- What component is affected changes presentation
- Epithelial cell (podocyte)
- Minimal change disease
- Basement membrane
- Post infectiours glomeruloneohritis (PIGN)
- Endothelial cell
- PIGN, haemolytic uraemia syndrome (HUS)
- Mesangial cell
- HSP/IgA neuropathy
- Epithelial cell (podocyte)
- Congenital
- Rare
- Can effect any layer
- Podocyte cytoskeletal integrity
- Congenital nephrotic syndrome
- Basement membrane proteins
- Alport syndrome
- Thin basement membrane disease
- Endothelial/microvascular integrity
- Complement regulatory proteins (MPGN)
- Podocyte cytoskeletal integrity
What are the 2 classes of glomerulopathy?
- Nephrotic syndrome
- More proteinuria
- Nephritic syndrome
- More haematuria
What can proteinuria be investigated by?
- Dipstix
- Measures concentration
- Protein creatinine ratio
- Normal <220mg/mmol
- Nephrotic range >250ml/mmol
- 24 hour urine collection (gold standard)
- Normal <60mg/m2/24 hours
- Nephrotic range >1g/m2/24 hours for children, >3.5g/24 hours for adults
What is the normal and nephrotic range for:
- protein creatinine ratio
- 24 hour urine collection
- Protein creatinine ratio
- Normal <220mg/mmol
- Nephrotic range >250ml/mmol
- 24 hour urine collection (gold standard)
- Normal <60mg/m2/24 hours
- Nephrotic range >1g/m2/24 hours for children, >3.5g/24 hours for adults
Nephrotic syndrome - definition
- Nephrotic range proteinuria
- Hypoalbuminemia
- Oedema (increasing 3rd space fluid volume)
Nephrotic syndrome - aetiology
- Due to balance between oncotic (osmotic) vs hydrostatic pressure
- “Protein is magnet to water”,
Nephrotic syndrome - presentation
- Swollen face (worse in mornings)
- Swollen legs
- Family history positive
- Signs
- Inflated weight
- Pale
- Periorbital oedema, pitting oedema legs, ascites, small pleural effusions
- Frothy urine
- Hypotensive or hypertensive
Nephrotic syndrome - investigations
- Urinalysis
- High protein and blood
- Raised PCR ratio
- Bloods
- Albumin low
- Normal creatinine
What are the most common nephrotic syndromes in paediatrics?
- Different kinds of nephrotic syndrome by age:
- Minimal change disease most common in children
Nephrotic syndrome - treatment
- If typical features
- First line - Prednisolone 8 weeks (is a high dose glucocorticoid)
- Side effects – susceptibility to infection, hypertension, personality changes, growth
- Second line - immunosuppresion
- Antibiotic prophylaxis
- First line - Prednisolone 8 weeks (is a high dose glucocorticoid)
Nephrotic syndrome - prognosis
- Steroid sensitivity predict diagnosis and prognosis
What are typical features of minimal change disease?
- Age 1-10
- Normal BP
- No frank haematuria
- Normal renal function
What are atypical features of minimal change disease?
- Suggestions of autoimmune disease
- Abnormal renal function
- Steroid resistance
What are difference kinds of steroid resistance nephrotic syndromes?
- Acquired
- Focal segmental glomeruloscerosis (FSGS)
- Podocyte loss and progressive inflammation and sclerosis
- Focal segmental glomeruloscerosis (FSGS)
- Congenital
- Infant presentation
- NPHS1 – nephrin
- NPHS2 – podocin
- Podocyte loss
What can haematuria be investigated by?
- Macroscopic/frank
- Microscopic
- Dipstix – trace on 3 or more occasions
Haematuria - aetiology
What are examples of nephritic syndromes?
- Acute post infectious glomerulonpehirits
- IgA nephropathy
- IgA related vasculitis (Henoch Schonlein Purpura)
What are difference classifications of nephritic syndromes and what components do they affect?
- Epithelial cell (podocyte)
- MCD, FSGS, lupus
- Basement membrane
- Membranous glomerulopathy, MPGN
- Endothelial cell
- Post infectious glomerulonephritis (PIGN), haemolytic uraemic syndrome, membranoproliferative glomerulonephritis (MPGN), lupus, ANCA vasculitis
- Mesangial cell
- HSP/IgA nephropathy, lupus
Nephritic syndrome - presentation
- Haematuria
- Proteinuria
- Reduced GMR
- Oliguria
- Fluid overload – raised JVP, oedema
- Hypertension
- Worsening renal failure
Nephritic syndrome - investigations
- Renal USS
- Bloods
- Maybe biopsy
Acute-post infectious glomerulonephritis - aetiology
- Usually group A strep
- Occurs through autoimmune process – antigen mimicry
Acute-post infectious glomerulonephritis - epidemiology
- Commonly in younger children
Acute-post infectious glomerulonephritis - management
- Self-limiting
- Antibiotics
- Support renal functions
- Electrolytes/acid base
- Diuretics for overload/hypertension
IgA nephropathy - aetiology
- Usually 1-2 days after URTI
- Autoimmune process
IgA nephropathy - epidemiology
- Most common glomerulonephritis
IgA nephropathy - clinical features
- Recurrent macroscopic haematuria
- +/- chronic microscopic haematuria
- Varying degree of proteinuria
IgA nephropathy - investigations
- Diagnosis confirmed by biopsy
IgA nephropathy - treatment
- Mild disease
- Proteinuria with ACEI
- Moderate to severe disease
- Immunosuppression (KDIGO)
IgA related vasculitis - aetiology
- 1 to 3 days after viral trigger
- Usually streptococcus or drugs
IgA related vasculitis - epidemiology
- 5 to 15 years
- Most common childhood vasculitis
IgA related vasculitis - clinical diagnosis criteria
- Mandatory papable purpura
- One of 4
- Abdominal pain
- Renal involvement
- Arthritis or arthralgia
- Biopsy
- IgA deposition
IgA related vasculitis - management
- Usually self-limiting condition
- Symptomatic treatment
- Analgesia for joints, anti-acids for gut
- Immunosuppression in moderate to severe disease
- Long term – hypertension and proteinuria screening
