Paediatric Nephrology - Glomerular Disease

Question 1

What percentage of CO do the kidneys recieve /min?

Answer 1

Receives 25% of CO/min

Question 2

What is the GFR of:

• neonate
• age 2

Answer 2

• Neonate 20-30ml/min/1.73m²
• Age 2 years equals adult at 90-120ml/min/1.73m²

Question 3

What are 5 functions of the kidneys?

Answer 3

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

Question 4

What is the presentation of glomerular disease usually?

Answer 4

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

Question 5

Glomerulopathy - presentation

Answer 5

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

Question 6

What are the differences between nephrotic and nephritic syndromes for:

• haematuria or proteinuria
• IV overload or depletion

Answer 6

• Nephritis syndrome
  
  • Haematuria
  • IV overload
• Nephrotic syndrome
  
  • Proteinuria
  • IV depletion

Question 7

What does the presentation of difference kidney diseases depend on?

Answer 7

Which component of kidneys is affected

Question 8

Glomerulopathy - aetiology

Answer 8

• 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)

Question 9

What are the 2 classes of glomerulopathy?

Answer 9

• Nephrotic syndrome
  
  • More proteinuria
• Nephritic syndrome
  
  • More haematuria

Question 10

What can proteinuria be investigated by?

Answer 10

• 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

Question 11

What is the normal and nephrotic range for:

• protein creatinine ratio
• 24 hour urine collection

Answer 11

• 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

Question 12

Nephrotic syndrome - definition

Answer 12

• Nephrotic range proteinuria
• Hypoalbuminemia
• Oedema (increasing 3^rd space fluid volume)

Question 13

Nephrotic syndrome - aetiology

Answer 13

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

Question 14

Nephrotic syndrome - presentation

Answer 14

• 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

Question 15

Nephrotic syndrome - investigations

Answer 15

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

Question 16

What are the most common nephrotic syndromes in paediatrics?

Answer 16

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

Question 17

Nephrotic syndrome - treatment

Answer 17

• 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

Question 18

Nephrotic syndrome - prognosis

Answer 18

• Steroid sensitivity predict diagnosis and prognosis

Question 19

What are typical features of minimal change disease?

Answer 19

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

Question 20

What are atypical features of minimal change disease?

Answer 20

• Suggestions of autoimmune disease
• Abnormal renal function
• Steroid resistance

Question 21

What are difference kinds of steroid resistance nephrotic syndromes?

Answer 21

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

Question 22

What can haematuria be investigated by?

Answer 22

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

Question 23

Haematuria - aetiology

Answer 23

Question 24

What are examples of nephritic syndromes?

Answer 24

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

Question 25

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

Answer 25

* 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**

Question 26

Nephritic syndrome - presentation

Answer 26

* Haematuria * Proteinuria * Reduced GMR * Oliguria * Fluid overload – raised JVP, oedema * Hypertension * Worsening renal failure

Question 27

Nephritic syndrome - investigations

Answer 27

* Renal USS * Bloods * Maybe biopsy

Question 28

Acute-post infectious glomerulonephritis - aetiology

Answer 28

* Usually group A strep * Occurs through autoimmune process – antigen mimicry

Question 29

Acute-post infectious glomerulonephritis - epidemiology

Answer 29

* Commonly in younger children

Question 30

Acute-post infectious glomerulonephritis - management

Answer 30

* Self-limiting * Antibiotics * Support renal functions * Electrolytes/acid base * Diuretics for overload/hypertension

Question 31

IgA nephropathy - aetiology

Answer 31

* Usually 1-2 days after URTI * Autoimmune process

Question 32

IgA nephropathy - epidemiology

Answer 32

* Most common glomerulonephritis

Question 33

IgA nephropathy - clinical features

Answer 33

* Recurrent macroscopic haematuria * +/- chronic microscopic haematuria * Varying degree of proteinuria

Question 34

IgA nephropathy - investigations

Answer 34

* Diagnosis confirmed by biopsy

Question 35

IgA nephropathy - treatment

Answer 35

* Mild disease * Proteinuria with ACEI * Moderate to severe disease * Immunosuppression (KDIGO)

Question 36

IgA related vasculitis - aetiology

Answer 36

* 1 to 3 days after viral trigger * Usually streptococcus or drugs

Question 37

IgA related vasculitis - epidemiology

Answer 37

* 5 to 15 years * Most common childhood vasculitis

Question 38

IgA related vasculitis - clinical diagnosis criteria

Answer 38

* Mandatory papable purpura * One of 4 * Abdominal pain * Renal involvement * Arthritis or arthralgia * Biopsy * IgA deposition

Question 39

IgA related vasculitis - management

Answer 39

* 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