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 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 is the normal and nephrotic range for: - protein creatinine ratio - 24 hour urine collection

Protein creatinine ratio Normal \ 250ml/mmol 24 hour urine collection (gold standard) Normal \ 1g/m2/24 hours for children, \>3.5g/24 hours for adults

Paediatric Nephrology - Glomerular Disease Flashcards by Emilia Sykes

What percentage of CO do the kidneys recieve /min?

Receives 25% of CO/min

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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

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What are 5 functions of the kidneys?

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

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What is the presentation of glomerular disease usually?

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

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Glomerulopathy - presentation

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

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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

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What does the presentation of difference kidney diseases depend on?

Which component of kidneys is affected

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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
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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What are the 2 classes of glomerulopathy?

Nephrotic syndrome
- More proteinuria
Nephritic syndrome
- More haematuria

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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

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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

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Nephrotic syndrome - definition

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

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Nephrotic syndrome - aetiology

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

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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

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Nephrotic syndrome - investigations

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

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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

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
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