Paediatric Nephrology Flashcards

1
Q

What is the difference between nephrotic and nephritic syndrome?

A

Nephritic syndrome is associated with increasing haematuria and intravascular overload.
Nephrotic syndrome is associated with increasing proteinuria and intravascular depletion.

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2
Q

Which is more common; acquired or congenital glomerulopathies?

A

Acquired

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3
Q

What are some examples of acquired glomerulopathies?

A

Minimal change disease
Post infectious glomerulonephritis
Haemolytic uraemic syndrome
IgA nephropathy

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4
Q

What are some examples of congenital glomerulopathies?

A

Congenital nephrotic syndrome
Alport syndrome
Thin basement membrane disease

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5
Q

How can proteinuria be measured?

A

Dipstix- measures concentration, 3 or more positive usually abnormal. Can give false positives and negatives
Protein creatinine ratio- early morning urine best, normal Pr:Cr ratio is <20mg/mmol, nephrotic range is >250
24hr urine collection- gold standard, normally <60mg/m²/24hrs, nephrotic range in children is >1g/m²/24hrs

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6
Q

When does nephrotic syndrome occur?

A

Nephrotic syndrome occurs when nephrotic range proteinuria progresses to hypoalbuminaemia, which progresses to oedema

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7
Q

What are the typical features of nephrotic syndrome in children?

A

Aged 1-10 (2-5 most common)
Normal blood pressure
No frank haematuria
Normal renal function

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8
Q

How is nephrotic syndrome treated?

A

8 weeks of prednisolone

Second line = immunosuppression

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9
Q

What are the side effects of glucocorticoids in children?

A

Behaviour and mood changes
Sleep disturbance
Increased infection risk
Hypertension

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10
Q

What is the spectrum of steroid sensitivity in paediatric nephrotic syndrome?

A
Non relapsing
Infrequently relapsing
Frequently relapsing
Steroid dependent
Steroid resistant
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11
Q

What are the causes of acquired steroid resistant nephrotic syndrome?

A

Acquired- focal segmental glomerulosclerosis

Congenital

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12
Q

What is nephritic syndrome?

A

Nephritic syndrome is a clinical diagnosis describing glomerulonephritis.

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13
Q

What are the symptoms of nephritic syndrome?

A
Haematuria
Proteinuria 
Reduced GFR
Oliguria
Fluid overload (raised JVP, oedema)
Hypertension
Worsening renal failure
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14
Q

What can nephritic syndrome cause?

A

Acute kidney injury

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15
Q

What is the most common cause of acute post-infectious glomerulonephritis?

A

Group A strep, usually affecting the throat or skin

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16
Q

How is acute post-infectious glomerulonephritis diagnosed?

A

Bacterial culture
Positive ASOT (antistreptolysin O titre)
Low C3- normalises

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17
Q

How is acute post-infectious glomerulonephritis treated?

A

Antibiotics
Support renal function with electrolytes etc
Diuretics if overload/hypertension present

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18
Q

Who is IgA related vasculitis most common in?

A

Ages 5-15

Usually associated with non-streptococcal post infectious glomerulonephritis

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19
Q

How is IgA related vasculitis diagnosed?

A

Diagnosis can be made if there are palpable purpura with at least one of the following:
•Abdominal pain
•Renal involvement
•Arthritis or arthralgia
•IgA deposition on biopsy (rarely required for diagnosis, usually just used for staging severity of vasculitis)

20
Q

Which vessels does IgA related vasculitis affect?

A

The small vessels

21
Q

What is the timeline of IgA vasculitis?

A

IgA vasculitis usually occurs 1-3 days after the trigger, which is most commonly a viral URTI in children but can also be streptococcus or drugs. Symptoms usually last 4-6 weeks and ~1/3 will relapse

22
Q

How is IgA vasculitis treated?

A

Symptomatic treatment for joints, gut etc
Glucocorticoid therapy (not useful in mild disease, may help with GI involvement)
Immunosuppression (in moderate-severe disease only)
Long term hypertension and proteinuria screening

23
Q

When does IgA nephropathy occur and who does it occur in?

A

Usually occurs 1-2 days after an URTI

Most common in older children and adults

24
Q

What are the clinical features of IgA nephropathy?

A

Recurrent macroscopic haematuria
Potential chronic microscopic haematuria
Varying degree of proteinuria

25
How is IgA nephropathy diagnosed?
Diagnosis of IgA nephropathy is usually clinical and should have a negative autoimmune workup. A biopsy can be taken to confirm the diagnosis
26
How is IgA nephropathy treated?
Mild disease is treated with ACE inhibitors | Moderate to severe disease is treated with immunosuppression
27
How is acute renal failure characterised?
Anuria/oliguria <0.5ml/kg/hr Hypertension with fluid overload Rapid rise in plasma creatinine
28
How is acute kidney injury defined in paediatrics?
Serum creatinine >1.5x age specific reference creatinine | Urine output <0.5ml/kg for >8 hours
29
How is an AKI warning score assigned?
AKI 1- creatinine 1.5-2 x reference creatinine AKI 2- creatinine 2-3 x reference creatinine AKI 3- creatinine >3 x reference creatinine
30
How can AKIs be prevented?
Monitor- paediatric early warning score, urine output, weight Maintain- good hydration Minimise- drugs
31
What are the pre-renal causes of AKI?
Volume depletion Oedematous states Hypotension Renal hypoperfusion
32
What are the renal causes of AKI?
Glomerular disease Tubular injury Interstitial nephritis
33
What are the post-renal causes of AKI?
Obstructive uropathies
34
When does typical haemolytic syndrome occur in children?
Typical haemolytic uraemic syndrome occurs post-diarrhoea, with other causes including pneumococcal infection and drugs. The period of risk post-diarrhoea of HUS in children is 14 days after the onset of diarrhoea
35
What is the triad associated with haemolytic uraemic syndrome?
Microangiopathic haemolytic anaemia Thrombocytopenia Acute kidney injury/acute renal failure
36
How is haemolytic uraemic syndrome managed?
Monitor kidney function Maintain- IV saline and fluid Minimise- no Abx/NSAIDs
37
What are the possible long term outcomes of acute kidney injury?
Blood pressure Requires proteinuria monitoring Can progress to CKD
38
What is the most common cause of chronic kidney disease in children?
Congenital abnormalities of the kidney and urinary tract (CAKUT)
39
What are the most common examples of congenital abnormalities of the kidney and urinary tract (CAKUT)?
Vesico-ureteric reflux | Renal dysplasia
40
What syndromes need to be considered when CAKUT is present?
Turner syndrome Trisomy 21 syndrome Branchio-oto-renal syndrome Prune belly syndrome
41
When does chronic kidney disease usually become symptomatic?
At a GFR <60
42
What are the possible paediatric presentations of CKD?
Weight and appetite loss Polyuria Lethargy UTIs
43
Why should UTIs be investigated in children?
UTIs are not normal in children and can be caused by vesico-ureteric reflux. Investigations should be done to screen for children at risk of progressive scarring and diagnose renal dysplasia and other abnormalities
44
What investigations can be done to establish a cause of paediatric UTI?
Ultrasound- structure DMSA (isotope scan- check for scarring/function) Micturating cysto-urethrogram
45
How are paediatric UTIs treated?
Three days of antibiotics for lower tract infections 7-10 days for upper tract infections Plus fluids and management of voiding dysfunction
46
What factors affect the prognosis of CKD?
``` Hypertension Proteinuria Bone health Recurrent UTIs Late referral Acidosis High intake of protein, salt and phosphate ```
47
How is hypertension defined in children?
Blood pressure is affected by height, age and sex and so hypertension in children is taken as anything >95th centile