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
Q

How is IgA nephropathy diagnosed?

A

Diagnosis of IgA nephropathy is usually clinical and should have a negative autoimmune workup. A biopsy can be taken to confirm the diagnosis

26
Q

How is IgA nephropathy treated?

A

Mild disease is treated with ACE inhibitors

Moderate to severe disease is treated with immunosuppression

27
Q

How is acute renal failure characterised?

A

Anuria/oliguria <0.5ml/kg/hr
Hypertension with fluid overload
Rapid rise in plasma creatinine

28
Q

How is acute kidney injury defined in paediatrics?

A

Serum creatinine >1.5x age specific reference creatinine

Urine output <0.5ml/kg for >8 hours

29
Q

How is an AKI warning score assigned?

A

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
Q

How can AKIs be prevented?

A

Monitor- paediatric early warning score, urine output, weight
Maintain- good hydration
Minimise- drugs

31
Q

What are the pre-renal causes of AKI?

A

Volume depletion
Oedematous states
Hypotension
Renal hypoperfusion

32
Q

What are the renal causes of AKI?

A

Glomerular disease
Tubular injury
Interstitial nephritis

33
Q

What are the post-renal causes of AKI?

A

Obstructive uropathies

34
Q

When does typical haemolytic syndrome occur in children?

A

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
Q

What is the triad associated with haemolytic uraemic syndrome?

A

Microangiopathic haemolytic anaemia
Thrombocytopenia
Acute kidney injury/acute renal failure

36
Q

How is haemolytic uraemic syndrome managed?

A

Monitor kidney function
Maintain- IV saline and fluid
Minimise- no Abx/NSAIDs

37
Q

What are the possible long term outcomes of acute kidney injury?

A

Blood pressure
Requires proteinuria monitoring
Can progress to CKD

38
Q

What is the most common cause of chronic kidney disease in children?

A

Congenital abnormalities of the kidney and urinary tract (CAKUT)

39
Q

What are the most common examples of congenital abnormalities of the kidney and urinary tract (CAKUT)?

A

Vesico-ureteric reflux

Renal dysplasia

40
Q

What syndromes need to be considered when CAKUT is present?

A

Turner syndrome
Trisomy 21 syndrome
Branchio-oto-renal syndrome
Prune belly syndrome

41
Q

When does chronic kidney disease usually become symptomatic?

A

At a GFR <60

42
Q

What are the possible paediatric presentations of CKD?

A

Weight and appetite loss
Polyuria
Lethargy
UTIs

43
Q

Why should UTIs be investigated in children?

A

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
Q

What investigations can be done to establish a cause of paediatric UTI?

A

Ultrasound- structure
DMSA (isotope scan- check for scarring/function)
Micturating cysto-urethrogram

45
Q

How are paediatric UTIs treated?

A

Three days of antibiotics for lower tract infections
7-10 days for upper tract infections
Plus fluids and management of voiding dysfunction

46
Q

What factors affect the prognosis of CKD?

A
Hypertension
Proteinuria
Bone health
Recurrent UTIs
Late referral
Acidosis
High intake of protein, salt and phosphate
47
Q

How is hypertension defined in children?

A

Blood pressure is affected by height, age and sex and so hypertension in children is taken as anything >95th centile