Paediatric Nephrology - Glomerular Disease Flashcards

1
Q

What percentage of CO do the kidneys recieve /min?

A

Receives 25% of CO/min

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2
Q

What is the GFR of:

  • neonate
  • age 2
A
  • Neonate 20-30ml/min/1.73m2
  • Age 2 years equals adult at 90-120ml/min/1.73m2
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3
Q

What are 5 functions of the kidneys?

A
  • Waste handling
  • Water handing
  • Salt balance
  • Acid base control
  • Endocrine
    • Red cells/blood pressure/bone health
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4
Q

What is the presentation of glomerular disease usually?

A
  • Haematuria
    • Usually darker in upper tract and lighter in lower tract
  • Proteinuria
    • Seen to be frothy
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5
Q

Glomerulopathy - presentation

A
  • Blood and protein in vary amounts for different diseases
    • Proteinuria signifies glomerular injury
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6
Q

What are the differences between nephrotic and nephritic syndromes for:

  • haematuria or proteinuria
  • IV overload or depletion
A
  • Nephritis syndrome
    • Haematuria
    • IV overload
  • Nephrotic syndrome
    • Proteinuria
    • IV depletion
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7
Q

What does the presentation of difference kidney diseases depend on?

A

Which component of kidneys is affected

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8
Q

Glomerulopathy - aetiology

A
  • 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
  • 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)
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9
Q

What are the 2 classes of glomerulopathy?

A
  • Nephrotic syndrome
    • More proteinuria
  • Nephritic syndrome
    • More haematuria
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10
Q

What can proteinuria be investigated by?

A
  • 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
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11
Q

What is the normal and nephrotic range for:

  • protein creatinine ratio
  • 24 hour urine collection
A
  • 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
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12
Q

Nephrotic syndrome - definition

A
  • Nephrotic range proteinuria
  • Hypoalbuminemia
  • Oedema (increasing 3rd space fluid volume)
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13
Q

Nephrotic syndrome - aetiology

A
  • Due to balance between oncotic (osmotic) vs hydrostatic pressure
    • “Protein is magnet to water”,
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14
Q

Nephrotic syndrome - presentation

A
  • 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
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15
Q

Nephrotic syndrome - investigations

A
  • Urinalysis
    • High protein and blood
    • Raised PCR ratio
  • Bloods
    • Albumin low
    • Normal creatinine
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16
Q

What are the most common nephrotic syndromes in paediatrics?

A
  • Different kinds of nephrotic syndrome by age:
    • Minimal change disease most common in children
17
Q

Nephrotic syndrome - treatment

A
  • 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
18
Q

Nephrotic syndrome - prognosis

A
  • Steroid sensitivity predict diagnosis and prognosis
19
Q

What are typical features of minimal change disease?

A
  • Age 1-10
  • Normal BP
  • No frank haematuria
  • Normal renal function
20
Q

What are atypical features of minimal change disease?

A
  • Suggestions of autoimmune disease
  • Abnormal renal function
  • Steroid resistance
21
Q

What are difference kinds of steroid resistance nephrotic syndromes?

A
  • Acquired
    • Focal segmental glomeruloscerosis (FSGS)
      • Podocyte loss and progressive inflammation and sclerosis
  • Congenital
    • Infant presentation
    • NPHS1 – nephrin
    • NPHS2 – podocin
    • Podocyte loss
22
Q

What can haematuria be investigated by?

A
  • Macroscopic/frank
  • Microscopic
    • Dipstix – trace on 3 or more occasions
23
Q

Haematuria - aetiology

A
24
Q

What are examples of nephritic syndromes?

A
  • Acute post infectious glomerulonpehirits
  • IgA nephropathy
  • IgA related vasculitis (Henoch Schonlein Purpura)
25
Q

What are difference classifications of nephritic syndromes and what components do they affect?

A
  • 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
26
Q

Nephritic syndrome - presentation

A
  • Haematuria
  • Proteinuria
  • Reduced GMR
    • Oliguria
    • Fluid overload – raised JVP, oedema
  • Hypertension
  • Worsening renal failure
27
Q

Nephritic syndrome - investigations

A
  • Renal USS
  • Bloods
  • Maybe biopsy
28
Q

Acute-post infectious glomerulonephritis - aetiology

A
  • Usually group A strep
  • Occurs through autoimmune process – antigen mimicry
29
Q

Acute-post infectious glomerulonephritis - epidemiology

A
  • Commonly in younger children
30
Q

Acute-post infectious glomerulonephritis - management

A
  • Self-limiting
  • Antibiotics
  • Support renal functions
    • Electrolytes/acid base
  • Diuretics for overload/hypertension
31
Q

IgA nephropathy - aetiology

A
  • Usually 1-2 days after URTI
  • Autoimmune process
32
Q

IgA nephropathy - epidemiology

A
  • Most common glomerulonephritis
33
Q

IgA nephropathy - clinical features

A
  • Recurrent macroscopic haematuria
  • +/- chronic microscopic haematuria
  • Varying degree of proteinuria
34
Q

IgA nephropathy - investigations

A
  • Diagnosis confirmed by biopsy
35
Q

IgA nephropathy - treatment

A
  • Mild disease
    • Proteinuria with ACEI
  • Moderate to severe disease
    • Immunosuppression (KDIGO)
36
Q

IgA related vasculitis - aetiology

A
  • 1 to 3 days after viral trigger
    • Usually streptococcus or drugs
37
Q

IgA related vasculitis - epidemiology

A
  • 5 to 15 years
    • Most common childhood vasculitis
38
Q

IgA related vasculitis - clinical diagnosis criteria

A
  • Mandatory papable purpura
  • One of 4
    • Abdominal pain
    • Renal involvement
    • Arthritis or arthralgia
    • Biopsy
      • IgA deposition
39
Q

IgA related vasculitis - management

A
  • 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